Category Archive ENT

Autoimmune Inner-Ear Disease – Causes, Symptoms, Treatment

Autoimmune inner-ear disease refers to a progressive, bilateral, although often asynchronous, sensorineural hearing loss, and vestibular function presumed autoimmune condition in which there is sudden or rapidly progressive fluctuating sensorineural hearing loss in the absence of any other neurologic or systemic immunologic abnormalities.

The autoimmune inner ear disease is a clinical syndrome with uncertain pathogenesis that is often associated with rapidly progressive hearing loss that, especially at the early stages of the disease, maybe at monoaural localization, although more often it is at binaural localization. It usually occurs as sudden deafness, or a rapidly progressive sensorineural hearing loss.

These include, but are not limited to, Vogt‐Koyanagi‐Harada syndrome, Cogan’s syndrome, Susac’s syndrome, systemic lupus erythematosus, rheumatoid arthritis, granulomatosis with polyangiitis (ie, Wegener’s granulomatosis), Behçet’s disease, systemic sclerosis, inflammatory bowel disease (eg, Crohn’s, ulcerative colitis), relapsing polychondritis, and temporal arteritis.

Causes of Autoimmune Inner-Ear Disease

The causes of AIED can include:

  • The body’s uncontrolled immune system attacks the inner ear protein, forming immune complexes and antibodies and causing progressive hearing loss in both ears.
  • Cochlin is a protein located in the inner ear that is attacked by the immune system.
  • The Endolymphatic sac, a structure of the inner ear, can become dilated as the immune response of the inner ear.

Symptoms of Autoimmune Inner-Ear Disease

Common symptoms of AIED can include:

  • Progressive SNHL in both ears that occurs over weeks to months that is not always the same in both ears
  • Fluctuating hearing
  • Dizziness or imbalance (approximately 50 percent of AIED cases)
  • Ringing in the ears, or tinnitus
  • Ear fullness (approximately 25 to 50 percent of AIED cases)
  • Conductive hearing loss may be present due to Eustachian tube obstruction from inflamed middle ear lining and/or if AIED is because of systemic autoimmune diseases
  • Symptoms of systemic autoimmune diseases, such as fatigue, achy muscles, swelling and redness, low-grade fever, and more
  • Dizziness or problems with your balance
  • Fullness in your ear
  • Tinnitus (ringing, roaring, or hissing in your ear)
  • Vertigo (a sense that you’re spinning)

Diagnosis of Autoimmune Inner-Ear Disease

Because of the difficulty in the differential diagnosis of AIED, many have proposed the use of lab tests to assist in the medical diagnosis. McCabe originally recommended an “immune screen” to include

  • Erythrocyte sedimentation rate, which is a general indicator of inflammation;
  • Rheumatoid factor, which is a marker for rheumatoid arthritis and other autoimmune diseases;
  • Anti-nuclear antibody titer, to check for lupus and other autoimmune diseases;
  • quantitative immunoglobulin determination; and
  • A leukocyte migration inhibition test.

Later, Campbell and Klemens (2000) listed other medical tests they commonly use to detect AIED:

  • CBC (complete blood count) to check for leukemia or other hemolytic disorders;
  • FTA/ABS blood screen for syphilis;
  • MRI, with contrast, of the brain and cerebellopontine angle to check for MS, vascular lesions, and space-occupying lesions;
  • Lymphocyte blast transformation to check for inner ear antigen, which may underlie AIED (the efficacy of this test is controversial);
  • Rheumatoid factor and antinuclear antibody, as mentioned above;
  • Lipid panel to check for dyslipidemias; and
  • Steroid trial.

The diagnosis is based on history, findings on physical examination, blood tests, the results of the hearing and vestibular tests, MRI scans, and response to immunosuppressive medications. The usual clinical picture is a subacute bilateral progressive sensorineural hearing loss.

Steroid responsiveness is the most useful method of making the diagnosis, and ordinarily, the diagnosis is made by observing a bilateral progressive sensorineural hearing loss that responds to steroids. This is a rather difficult criterion to meet, as it requires three features — bilateral, progression, and steroid responsiveness. There might be few people where steroid responsiveness has been established, as this generally requires contact with a subspecialty physician. The observation of progression requires a time series of audiograms, quite different from the “point of service” type audiograms used in most settings.

Other tests may be proposed based on the clinical situation.

As auditory neuropathy can present with a progressive bilateral sensorineural hearing loss, ABR testing should be done in persons with enough hearing for the test to be practical. Otoacoustic emission tests should be done in those in whom ABR testing cannot be done. MRI scans of the brain is useful to diagnose Susac’s syndrome (see above), as well as to exclude possible confounding disorders, such as acoustic neuroma.

While specific tests for autoimmunity to the inner ear would be desirable, at this writing (2020) we know of none that are both commercially available and proven to be useful. (Garcia Berrocal et al, 2002).

Having an autoimmune disease, per se, combined with hearing loss is not a good way of establishing that hearing loss is autoimmune, as both autoimmune disease and hearing loss are very common. In other words, coincidence is not the same as cause. Here is a partial list of blood tests.

  • Immunofluorescence of supporting cells of guinea pig organ of Corti (cochlea) has also been shown to correlate with disease and steroid responsiveness. According to Gray and others, immunofluorescence is more sensitive and specific (86%, 41%) than is Western Blot (59%, 29%) (Gray and others, ARO abstracts, 1999, #246).
  • Western Blot is helpful in some hands (Garcia et al, 2003). Yeom et al (2003) recently reported that immunofluorescence is more sensitive and specific than anti-HSP 70. The specificity of both tests to us seems unacceptably low.
  • The lymphocyte transformation assay, like the anti-cochlear antibody test, is present of uncertain value.
  • Antiendothelial antibodies may be associated with some cases of AIED (Cadoni et al, 2003). At this writing, there is no commercially available test.
  • Several studies have reported an association between autoimmune thyroid disease and ear disease (Brenner et al, 2004; Medugno et al, 2000), which is the rationale for testing for anti-microsomal or thyroid peroxidase antibodies.
  • It has recently been reported that antibodies to sulfoglucuronosyl glycolipids are common in autoimmune inner ear disease. (Yamawaki M, 1998). It remains to be seen if this finding will be confirmed and whether a commercial assay will be developed.
  • At this writing (2011), it is clear that anti-cochlear antibody (also called anti-HSP70) blood tests are not useful. Antibodies to HSP-70 can also be found in Lyme disease, ulcerative colitis, cancers, and in about 5% of healthy individuals. In Meniere’s disease, Hornibrook et al (2011) found no significant difference between 80 subjects with definite Meniere’s disease and controls. Yeom et al (2003) suggested that all anti-HSP tests are directed against the wrong substrate. Whether this is true or not, because of the poor specificity of anti-HSP 70 testings, diagnosis is generally based on evidence from broader tests of autoimmunity, or a positive response to steroids.

A small study suggested that FDG PET scans may be useful in AIED. (Mazlumzadeh et al, 2003). More investigation of this modality is needed before its role in diagnosis can be defined.   The cost is clearly prohibitive.

As there are no specific tests for AIED, a common approach is to look for other evidence for autoimmune involvement.

  • ANA, ANCA, Sed Rate, and CRP
  • Thyroid (anti-microsomal and thyroglobulin antibodies)
  • Rheumatoid Factor
  • Complement C1Q
  • Smooth Muscle Antibody
  • anti-gliadin and anti-endomysial antibodies (for Celiac disease)
  • HLA testing, especially for HLA-B27

As commented above, positives on these blood tests do not prove that hearing loss is caused by autoimmune disease — some of them simply document inflammation. Response of hearing to steroids is far more specific. It is best to get these tests done prior to starting immunosuppressive drugs.

  • FTA (for Syphilis) as of 2020, syphilis is on the upswing again.
  • HBA1C (for diabetes, which is often autoimmune-mediated also)
  • HIV (HIV is associated with auditory neuropathy as well as syphilis)
  • Lyme titer (we are dubious that this is helpful as Lyme has very little to do with hearing loss)

Recently it has been suggested that blood levels of TNF, tumor necrosis factor, are both diagnostic and predictive of treatment response (Svarkic, 2012). As TNF is a nonspecific inflammatory cytokine, we are dubious as we think that TNF should logically be elevated in many conditions.

MRI testing is mainly done to exclude other entities, but occasionally enhancement is seen of the cochlea.

Treatment of Autoimmune Inner-Ear Disease

  • Autoimmune inner ear disease (AIED) is a reversible form of sensorineural hearing loss when immunosuppressive treatment is given.
  • AIED is described as progressive, bilateral although asymmetric sensorineural hearing loss that responds to treatment with corticosteroids.
  • Primary AIED exists in the absence of systemic disease and is probably an inner ear-specific autoimmune disease involving T-cell targeting of inner ear-specific antigens such as cochin.
  • Secondary AIED may be the consequence of systemic immune abnormalities that involve the inner ear.

    Systemic treatment

    • Corticosteroids administered prior to the development of AIED in mice lead to retained cochlear function, although no changes in cochlear histopathology could be noted.
    • High-dose corticosteroids for 2–4 weeks are recommended for patients with suspected AIED.
    • Treatment with low-dose methotrexate was not found to be efficacious in AIED.
      Animal models with keyhole limpet hemocyanin-induced labyrinthitis treated with etanercept, a TNF-α inhibitor, found decreased cochlear inflammation with reduction of hearing loss.
    • Clinical studies on the treatment of AIED with etanercept are mixed but promising, and a randomized controlled trial has yet to be conducted.
    • Treatment with cyclophosphamide remains an option, but toxic risks often deter clinicians and patients from its use.
    • Other treatment options include combination cytotoxic agents and plasmapheresis for potential use in patients with AIED.

      Intratympanic treatment

      • In animal models, it was found that medications can be delivered to the inner ear through the round window and intratympanic administration.
      • Intratympanic steroids for the treatment of AIED have produced mixed results.
      • Intratympanic injection of infliximab, a humanized TNF-α monoclonal antibody, may provide a steroid-sparing treatment option.

        The treatment most widely used for AIED is corticosteroids therapy. The initial dosage regimen is 60 mg or 1 mg/kg per day of prednisone or 6-methylprednisolone for a month. Shorter courses or lower doses have proved to be ineffective and increase the risk of relapse[rx]. In rapidly progressive forms 1 mg/kg per day is maintained for 4 wk until the audiogram is stable and the dose is then tapered over 8 wk to 10-20 mg per day, which is maintained for another 6 wk. In cases of sudden hearing loss, 1 mg/kg per day of 6-methylprednisolone is administered for four weeks. In severe hearing loss (over 70 dB) three pulses of 500 mg are administered, and then the above-mentioned dosage regimen is applied. When patients receive high doses of corticosteroids, active tuberculosis must be ruled out, and glycemia, potassium and blood pressure must be monitored. Tapering must be gradual, slower if glucocorticoids have been given at higher doses or for a longer time.

        methotrexate was offered to these patients at a dose of 15 mg/week. If the patient refused methotrexate treatment or if methotrexate therapy failed (as evidence of no response or hearing loss relapse), then they were offered two intratympanic injections of methylprednisolone 0.3–0.5 ml at 40 mg/ml given 1 week apart.

        Plasmapheresis

        Plasmapheresis is typically reserved in severe cases of autoimmune disorder that progress rapidly with vasculitis, leukopenia, thrombocytopenia or organ involvement despite immunosuppression.[rx] It has been reported to help stabilize hearing when it has been used.[rx, rx]

        Other immunosuppressants

        Some patients do not respond to corticoids or require high doses to control the disease, and other immunosuppressants such as methotrexate or cyclophosphamide have been tried. The empirical basis for using these drugs is the observation that in certain cases their effect enhances that of the corticosteroids, thus obtaining remission of one or more symptoms that are not achieved with corticosteroids alone, or allowing reduction of the required dose of corticosteroids to maintain the patient symptom-free.

        Methotrexate: A meta-analysis showed that there was no benefit with methotrexate compared with corticosteroids alone[rx]. However, vertigo or instability can improve with long treatments.

        The most frequently employed regimen is 7.5 mg weekly administered in one single dose. Once the response is achieved, the drug is given orally (15 mg weekly) for 12 mo. Methotrexate is associated with blood toxicity (leukopenia, thrombocytopenia), liver toxicity (elevated liver enzymes, periportal fibrosis, cirrhosis), and gastrointestinal toxicity (nausea, vomiting, mucositis). Folic acid supplements reduce the adverse effects, preserve their efficacy, and are, therefore, recommended.

        Cyclophosphamide: This drug was used by McCabe[rx], who advocated its use as the treatment of choice, in his original series of cases. However, because of its adverse effect profile (gonadal, bladder, and bone marrow toxicity), it is not frequently used and is limited to those patients who do not respond to corticosteroids or do not maintain their response after dose tapering. The oral dose is 1-2 mg/kg per day for 4-6 wk. Intravenously, the starting dose is 0.75 g/m2 or 0.5 g/m2 if the glomerular filtration rate is lower than a third of the normal value, and this is repeated every 1-3 mo. The white cell count should not be lower than 2000/mm3 and neutrophils should remain over 1000/mm3. When both cyclophosphamide and high doses of corticosteroids are employed trimethoprim/sulphamethoxazole or dapsone is administered to prevent Pneumocystis carinii pneumonia.

        NOVELTIES IN AIED THERAPY

        The overall response rate to corticosteroids is 60%, but the response rate varies considerably. In most responders, the dose can be lowered or corticosteroids can be withdrawn without relapse, but some patients can present a corticosteroid-dependant hearing loss. Hearing loss may become refractory to corticosteroids, and other immunosuppressants should be considered in these cases. Finally, treatment can result in unacceptable adverse reactions (gastritis, peptic ulcer, fluid retention, glucose intolerance, avascular necrosis of the femoral head, psychiatric problems, sleep disorders, cataracts, osteoporosis, cushingoid habitus) and this has prompted the search for new drugs or different modes of administration such as the intratympanic route.

        Intratympanic therapy

        The use of intratympanic corticosteroids is an attractive therapeutic approach because it is minimally invasive and, since the drug is applied directly to the affected ear, side effects are minimized. However, there is no consensus regarding the doses and length of treatment. Moreover, it is not easy to control the dose that actually enters the inner ear (part of it is absorbed in the middle ear and part is eliminated through the Eustachian tube); as a result, its efficacy has so far not been fully determined[rx].

        Biological therapy agents

        Biological therapy agents are fusion proteins (made from a fusion gene, which is created by joining parts of two or more genes) or monoclonal antibodies designed to block specific components of the inflammatory cascade. Tumor necrosis factor α inhibitors and lymphocyte CD20 receptor antagonists have recently been tested on AIED patients

        Among the biological therapy agents, the most frequently used are tumor necrosis factor alpha-blockers. Tumor necrosis factor (TNF) is a proinflammatory cytokine produced by multiple cells, especially macrophages, that stimulates the maturation and migration of dendritic cells, activates neutrophils and NK cells, and increases vascular permeability. It was isolated by Carswell et al[rx] in 1975 when they were seeking to identify the factors responsible for Meth A sarcoma necrosis. It is expressed early in the inflammatory response in different inner ear structures. Of the different TNF-α blockers that have been developed, etanercept, infliximab, and adalimumab have been tested on AIED patients. X-ray or Mantoux screening is recommended before initiating treatment with TNF-α blockers because TNF-α is a key component in the body’s defense against M. tuberculosis and other granulomatous diseases.

        Etanercept: The results obtained so far are promising but not conclusive, as very few studies have been performed[30]. Anecdotally, it has been used together with methotrexate with good results, allowing corticosteroid therapy to be withdrawn[rx]. The usual dose is 25 mg administered by subcutaneous injection twice a week or 50 mg once a week for an indefinite period of time. Side effects that have been a concern are infections including tuberculosis and sepsis, tumors such as lymphomas, anemia, and pancytopenia, demyelinating diseases, congestive heart failure, and hypersensitivity. However, a meta-analysis that examined the adverse reactions with etanercept and other biologic therapies in 163 randomized controlled studies with 50010 participants and 46 extension studies with 11954 participants reported that the severe adverse reactions rate for the biological products was not different from that of the control therapy (e.g., corticosteroids)[rx].

        Adalimumab – It is administered by subcutaneous injection of 40 mg every two weeks for an indefinite period of time. The dose can be increased to 40 mg weekly if a decrease in the response is observed. It has been employed successfully in one patient with autoimmune sensorineural hearing loss and rheumatoid arthritis[rx].

        Infliximab – The usual regimen is a slow intravenous infusion (2 h) of 3 mg/kg at the start of treatment, and at 2 and 6 wk, followed by maintenance therapy every 8 wk indefinitely. Intratympanic administration of infliximab can help to reduce corticosteroids doses in patients with AIED[rx].

        B lymphocyte CD20 receptor antagonist – Apart from TNF-α blockers other biological therapy agents such as rituximab have been recently tested on patients with AIED. Rituximab is a chimeric monoclonal antibody that binds to the CD20 receptor of B lymphocytes, thereby inducing apoptosis and reducing their number. The few studies that have used rituximab in AIED patients have yielded encouraging results[rx,rx]. However, more studies are needed for reliable conclusions to be reached. The recommended dose is 1000 mg in intravenous injection, followed by second injection perfusion of 1000 mg 2 wk later. The most common side effect associated with rituximab is a reaction to the injection (low blood pressure, nausea, eruption, fever, itching, urticaria, throat irritation, tachycardia, peripheral edema). Infections of the upper airway and urinary tract have also been reported (but not in AIED patients).

        TNFα Antagonists

        TNFα is a pro‐inflammatory cytokine and is an indicator of steroid responsiveness in AIED.[rx] Using an established mouse model of AIED immunized with KLH antigen, etanercept has been found to decrease the number of infiltrating cells in the cochlea in response to TNFα.[rx] Several open‐pilot studies show variable hearing results with etanercept in steroid-responsive patients. In one reported study of 12 patients, 58% had hearing improvement.[rx] In another with 23 patients, 30% had improved hearing and 58% had a stable hearing.[rx] However, a pilot placebo‐controlled study of steroid-responsive AIED patients found no difference in the hearing improvement between etanercept and placebo.[rx]

        Yet another TNF αantagonist, infliximab, delivered by local intratympanic (IT) infusion once weekly for 4 weeks has been found to stabilize hearing and allow 4 of 5 steroid‐dependent patients to wean off steroids, or improve hearing loss in 3 of 4 steroid‐responsive patients who relapsed after steroid cessation.[rx] Another study of 10 steroid‐dependent AIED patients who underwent IT golimumab therapy found that 6 had stable thresholds in the injected ear and 7 patients were able to wean off steroids.[rx]

        TNFα antagonist is not useful in steroid-refractory AIED. In a study of 8 patients who did not respond to steroids, systemic treatment with infliximab was not helpful in hearing improvement.[rx]

        IL‐1β Antagonists

        One of the challenges of AIED is the treatment of steroid non-responders. While steroids are known to suppress IL‐1β through indirect pathways, one study suggests that the IL‐1β pathway is abnormally upregulated in steroid-resistant patients.[rx] They also showed that IL‐1β antagonist anakinra can decrease IL‐1β in otherwise steroid‐nonresponsive monocytes. This is promising for the potential use of anakinra for steroid‐nonresponsive patients. A phase I/II study showed that in an intention to treat analysis, 58% response rate with anakinra injection in steroid‐nonresponsive AIED.[rx] The drug was well tolerated, aside from the risk of injection site reaction rate of 70%.

        B‐Cell Antagonists

        Rituximab is a B‐cell inhibitor targeting CD20. A small open pilot study of 7 patients tolerated rituximab without significant side effects and 5 were able to maintain the post‐steroid hearing improvement.[rx] There is one case report of a Cogan’s syndrome patient who did not respond to prednisone, methotrexate, cyclophosphamide, cyclosporine, and adalimumab (TNFα inhibitor), but did have hearing improvement after rituximab.[rx] In a retrospective study, hearing improved in only 2 of 5 treated with rituximab, but all patients improved tinnitus, aural fullness, and vertigo.[rx]

        Cochlear Implantation

        For those patients whose hearing could not be salvaged, cochlear implantation is an excellent rehabilitative option.[rx, rx, rx] Although neo‐ossification (which required drill out) and intraluminal fibrosis was seen in 50% of implanted ears, all ears were implanted and the outcomes on word and sentence scores were not significantly different between AIED and postlingually deaf control patients.[rx] This option is especially important for those patients unable to tolerate the side effects of immunomodulating drugs and go on to develop bilateral deafness.

        References

        Epistaxis (Nosebleed) – Causes, Symptoms, Treatment

        Epistaxis (nosebleed) is one of the most common ear, nose, and throat (ENT) emergencies that present to the emergency department or the primary care clinic. There are two types of nosebleeds: anterior (more common), and posterior (less common, but more likely to require medical attention). The source of 90% of anterior nosebleeds is within Kiesselbach’s plexus (also known as Little’s area) on the anterior nasal septum. There are five named vessels whose terminal branches supply the nasal cavity:

        • 1) Anterior ethmoidal artery
        • 2) Posterior ethmoidal artery
        • 3) Sphenopalatine artery
        • 4) Greater palatine artery
        • 5) Superior labial artery

        The watershed area of these five vessels is in the anterior nasal septum, comprising Kiesselbach’s plexus. This lies at the entrance to the nasal cavity and so is subject to extremes of heat and cold, and of high and low moisture, and is easily traumatized. The mucosa over the septum in this area is especially thin, making this the site of the majority of epistaxis. More rarely, vessels in the posterior or superior nasal cavity will bleed, leading to the so-called “posterior” epistaxis. This is more common in patients on anticoagulants, patients who are hypertensive, and patients with underlying blood dyscrasia or vascular abnormalities. Management will depend on the severity of the bleeding and the patient’s concomitant medical problems.

        Pathophysiology

        Nosebleeds are caused by the rupture of a blood vessel within the nasal mucosa. Rupture can be spontaneous, initiated by trauma, use of certain medications, and/or secondary to other comorbidities or malignancies. An increase in the patient’s blood pressure can increase the length of the episode. Anticoagulant medications, as well as clotting disorders, can also increase the bleeding time.

        Most nosebleeds occur in the anterior part of the nose (Kiesselbach’s plexus), and an etiologic vessel can usually be found on careful nasal examination.

        Bleeding from the posterior or superior nasal cavity is often termed a posterior nosebleed.  This is usually presumed due to bleeding from Woodruff’s plexus, which are the posterior and superior terminal branches of the sphenopalatine and posterior ethmoidal arteries. These are often difficult to control and are associated with bleeding from both nostrils or into the nasopharynx, where it is swallowed or coughed up, presenting as hemoptysis.  It can generate a greater flow of blood into the posterior pharynx and have a higher risk for airway compromise or aspiration due to increased difficulty in controlling the bleed.

        Causes of  Epistaxis (Nosebleed)

        There are multiple causes of epistaxis which can be divided into local, systemic, environmental, and medication-induced.

        • Nasal fractures are caused by physical trauma to the face. Common sources of nasal fractures include sports injuries, fighting, falls, and car accidents in the younger age groups, and falls from syncope or impaired balance in the elderly.[rx]

        Local causes:

        • Digital manipulation
        • Deviated septum
        • Trauma
        • Chronic nasal cannula use

        Systemic causes:

        • Alcoholism
        • Hypertension
        • Vascular malformations
        • Coagulopathies (von Willebrand disease, hemophilia)

        Environmental factors:

        • Allergies
        • Environmental dryness ( more common in winter months)

        Medications:

        • NSAIDs (ibuprofen, naproxen, aspirin)
        • Anticoagulants (warfarin)
        • Platelet aggregation inhibitors (clopidogrel)
        • Topical nasal steroid sprays
        • Supplement/alternative medications (vitamin E, ginkgo, ginseng)
        • Illicit drugs (cocaine)

        While epistaxis is a very common spontaneous problem, rarer etiologies such as neoplasms or vascular malformations must always be in the differential diagnosis, particularly if additional symptoms such as unilateral nasal obstruction, pain, or other cranial nerve deficits are noted.

        Symptoms of Nasal Bony Fractures

        Symptoms of a broken nose include bruising, swelling, tenderness, pain, deformity, and/or bleeding of the nose and nasal region of the face.

        • Pain or tenderness, especially when touching your nose
        • Swelling of your nose and surrounding areas
        • Bleeding from your nose
        • Bruising around your nose or eyes
        • Bruising, swelling and tenderness around the nose
        • A deformed, twisted or crooked nose
        • Blockage of one or both nostrils
        • A deviated septum
        • A bruise-like discoloration under the eyes
        • Crooked or misshapen nose
        • Difficulty breathing through your nose
        • Discharge of mucus from your nose
        • Feeling that one or both of your nasal passages are blocked

        The patient may have difficulty breathing, or excessive nosebleeds (if the nasal mucosa are damaged). The patient may also have bruising around one or both eyes.

        Diagnosis of Epistaxis (Nosebleed)

        History and Physical

        The history should include duration, severity, frequency, laterality of the bleed, inciting event, and interventions provided prior to seeking care.  Inquire about anticoagulant, aspirin, NSAID, and topical nasal steroid use. Obtain a relevant family history, particularly relating to coagulopathy and vascular/collagen disease, as well as any history of drug and alcohol use.

        Prepare proper equipment and proper personal protective equipment (PPE) before beginning the physical examination.  Equipment may include a nasal speculum, bayonet forceps, headlamp, suction catheter, packing, silver nitrate swabs, cotton pledgets, and topical vasoconstrictors and anesthetic. Have the patient in a seated position in an exam chair in a room with suction available. Carefully insert the speculum and slowly open the blades to visualize the bleeding site. A headlight is essential to allow for hands-free illumination, and clot may need to be suctioned from the nasal cavity to identify the bleeding source.

        A posterior nosebleed is not easy to visualize and may be suggested by active bleeding into the posterior pharynx without a visualized vessel on nasal examination. Nasal endoscopy greatly increases the success in identifying the bleeding source.

        Evaluation

        Differentiating an anterior or posterior is key in management. Diagnosis of anterior bleeding is can be made by direct visualization using a nasal speculum and light source. A topical spray with anesthetic and epinephrine may be helpful for vasoconstriction to help control bleeding and to aid in the visualization of the source. Usually, the diagnosis of posterior bleeding is made after measures to control anterior bleeding have failed. Clinical features of posterior bleeding can include active bleeding into the posterior pharynx in the absence of an identified anterior source; high-flow posterior bleeds may cause blood to emanate from both nares. Labs may be obtained if necessary, including a complete blood cell count (CBC), type and cross match, and coagulation studies, though should not delay treatment of an active bleed. Imaging such as x-ray or computed tomography have no role in the urgent or emergent management of active epistaxis.

        Treatment of Epistaxis (Nosebleed)

        Start with a primary survey and address the airway, ensure the airway is patent.  Next, assess for hemodynamic compromise. Obtain large-bore intravenous access in patients with severe bleeding and obtain labs. Reverse blood clotting as necessary, if there is a concern with medication use.

        All patients with moderate to severe nose bleeding should have two large-bore intravenous lines and infusion of crystalloid. The monitoring of oxygen and hemodynamic stability is vital.

        Treatment for anterior bleeding can be started with direct pressure for at least 10 minutes. Have the patient apply constant direct pressure by pinching the nose over the cartilaginous tip (instead of over the bony areas) for a few minutes to try to control the bleed. If that is ineffective, vasoconstrictors such as oxymetazoline or thrombogenic foams or gels can be employed. It is important to remove all clot with suction before any attempt at treatment is made. The reasons are twofold: 1) Clot will prevent any medication from reaching the vessel itself and 2) if packing becomes necessary, the clot can be pushed into the nasopharynx and aspirated. If topical treatments are unsuccessful, proceed with nasal examination to identify and cauterize the vessel with silver nitrate. If this too is unsuccessful, anterior nasal packing is necessary. This can be performed with absorbable packing material such as surgicel or fibrillar, or with devices such as anterior epistaxis balloons, or nasal tampons (Rapid Rhino). If silver nitrate is used to cauterize a septal blood vessel, only use it on one side of the septum to prevent septal perforation. Thermal coagulation is painful and should rarely be attempted in an emergent setting.

        Traditional petrolatum gauze can be used if one does not have access to balloons or tampons.

        If none of this is successful, the bleeding may be from the posterior or superior nasal cavity. Symptoms can include active bleeding from both nostrils or active bleeding present in the posterior pharynx. Longer (7.5cm) nasal tampons are available that provide some more posterior pressure and can be employed in this situation. Formal posterior nasal packing should only be performed by experienced personnel as it requires admission and telemetry monitoring, and sometimes intubation. It is associated with higher rates of complications like pressure necrosis, infection, or hypoxia, and may trigger a nasal-cardiac reflex (sudden bradycardia after nasal packing – if this occurs, remove the pack immediately). Foley catheters can be used by experienced personnel to tamponade a posterior bleed. If a posterior pack is placed, a formal petrolatum gauze anterior pack must be placed as well to create a closed, tamponaded space in the nasopharynx.

        If all of these measures are unsuccessful, the patient should be intubated for airway protection and interventional radiology consulted emergently for embolization. If this service is unavailable, operative ligation of the sphenopalatine and ethmoid arteries can be performed in the operating room by an otolaryngologist.

        Complications

        • Septal hematoma
        • Septal abscess
        • Avascular necrosis of nasal septal cartilage leading to saddle deformity
        • Nasal obstruction
        • Blowout fractures: Extraocular muscle entrapment and diplopia
        • Nasolacrimal duct injury: Due to the close relationship of the duct to the nasal bones
        • Fracture of cribriform plate and cerebrospinal fluid (CSF) rhinorrhoea
        • Inability to reduce: Fractures that cannot be reduced by closed techniques are candidates for open reduction.
        • Airway compromise and hemorrhage.
        • Nasofrontal duct and or lacrimal duct disruption as a result of direct damage or due to displaced fracture segments.
        • Facial deformity, as full correction of telecanthus or nasal depression can be difficult to achieve, and some patients will retain a degree of asymmetry. Depending on the surgical approach, patients may experience temporary or permanent paralysis and or anesthesia of the forehead. Scars that cannot be hidden in the hairy scalp or skin folds may be prominent.
        • Infection of the incision site, soft tissues, and meninges are recognized complications from these injuries.
        • Mucocele formation is a complication of sinus or lacrimal drainage disruption and can become infected.
        • Mental health, as patients with facial injuries are at greater risk of developing post-traumatic stress disorder or anxiety-related disorders. Particularly those who were victims of assault.

        References

        Glossopharyngeal Neuralgia – Causes, Symptoms, Treatment

        Glossopharyngeal neuralgia (GN) is a rare pain syndrome that affects the glossopharyngeal nerve (the ninth cranial nerve that lies deep within the neck)  and causes sharp, stabbing pulses of pain in the back of the throat and tongue, the tonsils, and the middle ear.  The excruciating pain of GN can last for a few seconds to a few minutes, and may return multiple times in a day or once every few weeks.  Many individuals with GN relate the attacks of pain to specific trigger factors such as swallowing, drinking cold liquids, sneezing, coughing, talking, clearing the throat, and touching the gums or inside the mouth.  GN can be caused by compression of the glossopharyngeal nerve, but in some cases, no cause is evident.  Like trigeminal neuralgia, it is associated with multiple sclerosis.  GN primarily affects the elderly.

        Glossopharyngeal neuralgia (GN) is a rare and pain syndrome in the sensory distribution of the ninth cranial nerve also known as the glossopharyngeal nerve. As per ICHD-3 (International Classification of Headache Disease- 3) classification, glossopharyngeal neuralgia is a disorder characterized by a brief episodic unilateral pain, with sharp and stabbing in character, with abrupt onset and cessation, in the glossopharyngeal nerve distribution (angle of the jaw, ear, tonsillar fossa and the tongue base). It also involves the pharyngeal and auricular branches of CN X. Pain is commonly triggered by coughing, talking and swallowing. Pain in glossopharyngeal neuralgia follows a relapsing and remitting pattern. It falls under the International Classification of Diseases (ICD) category as ICD-10-CM-G52.1.

        Pathophysiology

        The glossopharyngeal nerve is a mixed sensorimotor nerve that exits the brainstem from the upper medulla. From that point, it leaves the skull through the jugular foramen along with the vagus and accessory nerves. It continues its path between the internal jugular vein and the internal carotid artery as it descends and then continues beneath the styloid process. It then curves to make an arch on the side of the neck as it passes under the hyoglossus muscle to its final distribution of the base of the tongue, the palatine tonsil, and glands of the mouth. A motor efferent supplies the stylopharyngeus muscle which is essential in swallowing. Sensory afferents provide information from the inner surface of the tympanic membrane, the upper pharynx as well as the posterior one-third of the tongue. Another important branch is the one to the carotid body and sinus is known as Hering’s nerve. It communicates with the vagus nerve and carries information from chemoreceptors in the carotid body and baroreceptors in the carotid sinus; this is important clinically as activation of the visceral sensory branch of the glossopharyngeal neuralgia can activate the vagus nerve (tractus solitarius and dorsal motor nucleus) and produce a reflex arrhythmia. This vagal activation may explain the cardiac-related syncopal episodes sometimes associated with glossopharyngeal neuralgia.  Overall the glossopharyngeal nerve is a very small nerve that runs deep in the neck, and it is sometimes resected accidentally during open neck dissections. For this same reason, it is often called ‘the neglected cranial nerve.’ Any infectious, inflammatory, or compressive etiologies across the glossopharyngeal nerve’s path from the end organs to the brainstem may result in hyperexcitability of the nerve and produce pain.

        Types of Glossopharyngeal Neuralgia

        There have been multiple attempts to classify GPN on a different basis. The various ways the disease has been classified are

        Otitic type – Pain in and around the ear

        • This is a commoner form of the two in the anatomical classification. The pain is often described in relation to the ear. The pain can be of any type, ranging from burning, sharpshooting, shock-like, pressure, pinprick, etc.

        Oropharyngeal – Pain is in and around the throat and face region

        • This form has a more varied distribution, and significant overlap may occur with other cranial nerve distribution areas.

        The International Headache Society (IHS) Classification of GPN

        The basis of classification is that pain occurs as episodic or constant basal pain that persists between the episodes of peaks and troughs of pain.

        The types proposed by IHS are

        • Classical GPN- episodic pain
        • Symptomatic GPN- continuous pain, a commoner
        Idiopathic type

        No demonstrable lesion is found in these cases. Most often, these are attributed to nerve ganglion compression by vessel or by compression of the glossopharyngeal nerve as it exits or enters the brainstem. This is supported by the fact that microvascular decompression (MVD) eliminates GPN symptomatology. Most of the cases belong to this type of GPN.

        Secondary type (Symptomatic)

        In this, a demonstrable lesion can be found, which includes trauma, neoplasm, infection, vascular malformation, or elongated styloid process [rx]. The secondary nature of GPN is suspected when there are neurological deficits, like numbness in the distribution of a glossopharyngeal nerve, absence of symptom-free interval in between the attacks, and pain distribution different from the glossopharyngeal nerve area.[]

        Causes of Glossopharyngeal Neuralgia

        Essential or idiopathic glossopharyngeal neuralgia is where etiology is unknown.

        Secondary causes of glossopharyngeal neuralgia include:

        • Vascular compression mainly at the nerve root: the most common cause
        • Demyelinating diseases: e.g., multiple sclerosis
        • Inflammatory and autoimmune diseases: e.g., Sjogren disease
        • Intraoral and peritonsillar infections
        • Intracranial space-occupying lesions especially medullary tumors or tumors originating from CP angle
        • Posterior fossa and cervical malformations
        • Eagle syndrome or stylalgia: If the styloid process is over 25mm or stylohyoid ligament is calcified they can cause compression of the glossopharyngeal nerve
        • Oropharyngeal cancers include carcinoma of the tongue and benign tumors like schwannomas.

        Symptoms of Glossopharyngeal Neuralgia

        Patients describe an attack as burning or jabbing pain, or as an electrical shock that may last a few seconds or minutes. Swallowing, chewing, talking, coughing, yawning or laughing can trigger an attack. Some people describe the feeling of a sharp object lodged in the throat. The pain usually has the following features:

        The pain usually has the following features:

        • Affects one side of the throat
        • Can last several days or weeks, followed by a remission for months or years
        • Occurs more frequently over time and may become disabling

        About 10% of patients also have potentially life-threatening episodes of heart irregularities caused by involvement of the nearby vagus nerve, such as:

        • slow pulse
        • sudden drop in blood pressure
        • fainting (syncope)
        • seizures

        Diagnosis of Glossopharyngeal Neuralgia

        History and Physical

        The ICHD-3 provides the following diagnostic criteria for glossopharyngeal neuralgia:

        A. Recurrent paroxysmal painful attacks in unilateral glossopharyngeal nerve distribution and should fulfill the criterion.

        B. Pain should have all the following characteristics:

        • Duration – Pain lasts from a few seconds to about 2 minutes
        • Intensity – Severe
        • Type of pain – Sharp, stabbing, shooting or electrical shock-like sensations
        • Precipitating factors – The pain is precipitated or exacerbated by coughing, yawning, swallowing or talking

        C. Pain should not be accounted for by any other ICHD-3 diagnosis.

        The physical examination of patients with glossopharyngeal neuralgia is generally benign, and the painful areas do not show any signs of sensory abnormalities for both light touch and pinprick. Sometimes glossopharyngeal neuralgia is associated with dysesthesias and/or hyperalgesia in the affected areas. If there is an absent cough or gag reflex, a detailed investigation into the etiology of the pain is necessary. In some rare cases, glossopharyngeal neuralgia and trigeminal neuralgia can occur concurrently.

        Evaluation

        The diagnosis of glossopharyngeal neuralgia is mainly clinical and should meet all criteria as mentioned in ICHD-3. A detailed history and physical examination of the patient is therefore mandatory. A thorough ENT examination is necessary including a throat exam and neck palpation. All the patients should have basic laboratory evaluations include complete blood count, basic metabolic panel, and erythrocyte sedimentation rate, anti-nuclear antibodies to rule out any underlying infection, inflammation, malignancy or temporal arteritis.

        Persistent symptoms are infrequent and warrant further investigation. Complications including syncope should have a cardiology evaluation with an echocardiogram and Holter monitoring.

        The primary role of imaging is to identify potential causes of nerve compression at the base of the skull.

        • Computed tomography (CT) – CT scans do not show the nerve directly but can identify an elongated and ossified styloid process in the axial images.
        • X-Ray – An elongated and heavily calcified styloid process can be present on the cervical spine plain radiograph on the lateral view.
        • Magnetic Resonance Imaging (MRI) – Neurovascular compression of the glossopharyngeal nerve is most visible on MRI. Thin section T2 weighted images are ideal for seeing pathology. An MRI with contrast should be performed to see any abnormal enhancement of the nerve, vessels, or surrounding structures. The most common vascular source of nerve compression is from the posterior inferior cerebellar artery (PICA). The vertebral artery and anterior inferior cerebellar artery are the second and third most common culprit vessels. The MRI can also show demyelinating brain lesions, tumors in the posterior fossa, or any malformations.
        • Magnetic resonance angiogram (MRA) – MRA should also be done to evaluate for avascular loop compressing on the nerve root entry zone.

        Treatment of Glossopharyngeal Neuralgia

        Medical management –  Glossopharyngeal neuralgia is usually responsive to pharmacotherapy especially with carbamazepine or oxcarbazepine.

        Carbamazepine – Starting dose 200 mg/day in a single dose (extended-release), or two divided doses (immediate-release tablet) or in four divided doses (oral solution). Increase the dose gradually with increments of 200 mg/day as needed. If the dose exceeds 200mg per day, it is advisable to administer extended-release capsules in two divided doses. Maintenance dose – 400 to 800 mg daily in two divided doses (immediate-release tablet) forms) or four divided doses (oral solution); maximum dose: no be more than 1,200 mg/day.

        Characteristics and management of glossopharyngeal neuralgia in the maxillofacial region.

        Diseases Clinical features Pharmacological treatments Surgical /local treatments Limitation
        Glossopharyngeal neuralgia Pain, dull type
        Pain duration, short duration
        Intensity, mild to moderate
        Localization, diffuse
        Characteristics, usually pain in the throat/ mouth floor
        Trigger point, swallowing
        Carbamazepine GN nerve block (i) Chance of trauma to the internal jugular vein and carotid artery
        (ii) Hematoma formation
        Gabapentin
        pregabalin
        Myotherapy
        Percutaneous radiofrequency thermal rhizotomy
        (i) Regular monitoring is not possible
        (ii) Recurrence
        (iii) Hoarseness of voice
        (iv) Vocal cord paralysis, and dysphagia (difficulty in swallowing)
        Injections (i) As it is a painful procedure, the patient feels uncomfortable during injection
        Direct section of the nerve in the cerebellopontine angle (i) High morbidity with neurologic and life threatening condition
        (ii) Thromboembolic complication
        (iii) Meningitis
        (iv) Cerebrospinal fluid leak,
        (v) Cutaneous flap distension
        (vi) Facial nerve dysfunction
        (vii) Ocular dysfunction
        (viii) Tinnitus
        Sedatives
        (on condition)
        Microvascular decompression (i) Low recurrence of pain
        (ii) Chance of nerve damage result
        (iii) Hoarseness
        (iv) Difficulty swallowing (dysphagia)
        (v) Unsteady gait

        The other neuropathic pain medicines recommended by the International Association for the Study of Pain (IASP) are as below:

        • Gabapentin (100 to 5000mg/day in 1 to 4 divided doses),
        • Duloxetine (20 to 90mg/day),
        • Valproic acid (125-2500mg/day in 1 to 2 divided doses),
        • Clonazepam (0.5-8mg/day),
        • Lamotrigine (50 to 500mg/day in 1 to 2 divided doses),
        • Baclofen (10 to 80mg/day in 1 to 4 divided doses),
        • Phenytoin (200 to 600mg/day in 1 to 3 divided doses),
        • Pregabalin (75 to 500mg/day in 1 to 2 divided doses) and
        • Topiramate (50 to 1000mg/day in 1 to 2 divided doses)

        As a general rule, these medications should be started at low doses and titrated up as needed based on their effectiveness, tolerability, and side effects. This pain condition often shows a relapsing-remitting course, and so medication can be tapered down to a low maintenance dose. Combining two or more medications with different mechanisms of action can help achieve better pain relief while avoiding side effects. A short course of opioids can be useful for intractable pain.

        Adjuvant care: Cold and hot compresses, physical therapy, and psychological counseling are all options in addition to medical therapy. The success rate is variable but can be helpful.

        Interventional pain management techniques

        Glossopharyngeal nerve blocks merit consideration for both diagnostic and therapeutic purposes. This block can be an option in conjunction with pharmacotherapy. A diagnostic block with a local anesthetic should be tried first to confirm the origin of the pain. If diagnostic blocks are successful, chemical neurolysis or thermal radiofrequency ablation can be performed on the nerve. Chemical neurolytic agents such as alcohol, glycerol, or phenol are typical choices. Radiofrequency ablation is typically performed at the jugular foramen to target the inferior petrous ganglion of Andersch. Accurate needle placement is critical as life-threatening bradycardia and hypotension can occur if the vagus nerve gets stimulated during the procedure. There are two common approaches to block the glossopharyngeal nerve: the intraoral and extra-oral approaches. The extra-oral technique is preferred since it is safe and easy to perform. Complications are not uncommon with glossopharyngeal neuralgia blocks. The glossopharyngeal nerve is in the vicinity of the internal jugular vein, and the carotid artery and intravascular injection can easily occur. The concomitant block of the recurrent laryngeal nerve may cause hoarseness of the voice. Always avoid bilateral glossopharyngeal nerve blocks at the same time to avoid complete vocal cord paralysis. Blockade of the vagus nerve may result in tachycardia and hypertension via blockade of parasympathetic fibers.

        Surgical Therapy

        Once the patient becomes refractory or intolerant to medications, surgery is the next treatment option. However, surgical therapy is associated with high morbidity of the patients and is limited to younger patients. These surgical procedures for the lesions may be classified as follows:

        • Extracranial, such as direct surgical neurotomies or percutaneous radiofrequency thermal rhizotomy[]
        • Intracranial, such as a direct section of glossopharyngeal and vagal nerves in the cerebellopontine angle[,]
        • Central procedures, such as percutaneous or open trigeminal tractotomy-nucleosome or nucleus caudalis DREZ operation

        These days, the best-established surgical treatments are MVD of vascular roots[] and a rhizotomy of the glossopharyngeal nerve with upper vagal nerve roots.[] In essential GPN, the primary pathology, being vascular compression of the nerve roots, responds well to MVD. However, in secondary GPN, first, address the underlying pathology: Tumor resection, posterior fossa decompression in Chiari malformation, embolization of an arteriovenous malformation, coagulation of choroid plexus overgrowth, fistulectomy for Eagle’s Syndrome.[] In secondary GPN, when MVD is not possible, the intracranial root section is considered curative and is most widely employed. In the largest case series by Rushton et al.[] and in a smaller series by Taha et al.,[] there were no recurrences after a preganglionic section of the ninth and upper tenth nerve roots. However, sectioning of cranial nerve fibers IX–X, open or percutaneous tractotomy-nucleosome is followed by severe and persistent dysphonia and dysphagia.[,,] This is because all neural destructive or ablative procedures carry the risk of neuritis, deafferentation pain, and neurovascular injury.[]

        With the refinements of microsurgical and anesthesiological techniques (Brainstem evoked potentials), MVD has proven to be an effective and safe available treatment and should be considered the first-line treatment in drug-resistant GPN.[] In a study by Resnic et al.,[] MVD provided complete pain relief in 76% of the cases and substantial improvement in a further 16%. Sampson et al.[] found pain relief of more than 10 years by MVD, hence indicating its efficacy and safety even on long term follow-up. MVD should be considered when a patient experiences typical GPN symptoms and has a PICA loop near the glossopharyngeal nerve[] and especially in patients with isolated symptom of throat pain.[]

        Recently, various case reports have been published, which have shown beneficial effects of pulsed radiofrequency neurolysis (PRN) and gamma knife surgery (GKS). PRN is a non-destructive neuromodulatory method to treat both, idiopathic and secondary GPN.[,] Short pulses of radiofrequency energy, delivered at a constant temperature, produce central and peripheral neuromodulatory effects.[,] In GKS system, an 80 Gy dose is stereotactically directed to the isocenter with MR imaging-based target localization and 4-mm collimation.[,] It might serve as a potential alternative to other percutaneous techniques and surgical options for patients with secondary GPN. Stereotactic radiosurgery (SRS) with GKS system offers a less-invasive option for patients with GPN. Till date, Pollock and Boes have reported the largest series of patients (5 patients), with suspected GPN being treated with SRS directed at the glossopharyngeal and vagus nerves, within the jugular foramen with a failure rate of 40%.[] These new techniques offer a promising direction that might spare patients from pain and potential morbidity of surgery.

        There are several surgical modalities used for the treatment of glossopharyngeal neuralgia based on the etiology of the pain. The compression of the glossopharyngeal nerve by a vascular structure is the most common cause of secondary glossopharyngeal neuralgia. Microvascular decompression (MVD) of the glossopharyngeal nerve is the most widely used surgical modality to correct vascular compression of the nerve. Alternatively, a resection of the glossopharyngeal nerve alone or with branches of the vagus nerve can also be performed.

        Extracranial techniques are percutaneous radiofrequency rhizotomy and direct surgical resection. These techniques are ideal in patients with essential glossopharyngeal neuralgia who failed medical management but unable to tolerate an open intracranial resection. Resection of the ipsilateral styloid process also known as fistulectomy is a therapeutic option for Eagle syndrome. The physician must rule out other central causes of glossopharyngeal neuralgia before pursuing this surgery.

        Intracranial techniques include rhizotomy or an intracranial root resection of the glossopharyngeal nerve and/or vagus nerves from its origin in the brainstem at the cerebellopontine angle. Persistent dysphagia and hoarseness of voice are the most common complications if these surgeries. Stereotactic radiosurgery with gamma knife surgery provides a less-invasive option, but data on safety and efficacy is limited.

        Complications

        Syncope and cardiac dysrhythmias: When the glossopharyngeal nerve gets irritated, it sends feedback via the dorsal motor nucleus of the Xth nerve. These signals also stimulate the nucleus tractus solitarius in the midbrain. Thus, during the acute glossopharyngeal neuralgia attack, abnormal stimulation produces amplified vagal response, which, in turn, results in bradycardia, hypotension, and cardiac dysrhythmias. These autonomic changes cause cerebral hypoperfusion, slow waves on EEG, seizures, and syncope. Convulsive movements, limb clonus, automatic smacking movements of the lips, and upward turning of the eyes are signs of cerebral hypoxia.

        The cardiovascular complications occur during the painful episodes or immediately after the pain symptoms resolve. Management of glossopharyngeal neuralgia pain attacks with drugs and/or surgical treatment can help manage these complications. Some patients only develop cardiovascular manifestations of glossopharyngeal neuralgia without painful paroxysms, also known as non-neurologic glossopharyngeal neuralgia. These patients can receive therapy with glossopharyngeal nerve avulsion or microvascular decompression.

        References

        Ear Irrigation – Indications, Contraindications

        Ear Irrigation/Cerumen, or ear wax, is a naturally occurring substance that is produced at the lateral one-third of the external auditory canal (EAC). Anatomically, this region houses a collection of pilosebaceous glands that includes ceruminous glands, hair follicles, and sebaceous glands. The modified sweat produced by the ceruminous glands has bacteriocidal and fungicidal properties, functioning to lubricate and clean the EAC. As dead skin cells slough off and move out of the ear canal, they combine with the oily secretions of sebaceous glands as well as the modified sweat of the ceruminous glands. The combination of these substances is what makes up cerumen, consisting primarily of dead keratin cells . Cerumen serves as a protective barrier to trap foreign particles. There are a number of pathologies that may present in the EAC including sebaceous cysts, furuncles, and even glandular tumors, but what most commonly plagues patients is the buildup and impaction of cerumen.

        The American Academy of Otolaryngology defines cerumen impaction as “an accumulation of cerumen that is associated with symptoms, prevents the necessary assessment of the ear, or both” . While cerumen is typically expelled from the EAC spontaneously with the aid of jaw movement, this mechanism may fail some patients and lead to impaction. Impaction is more likely to occur when this normal extrusion of cerumen is prevented in some way; whether that be with the use of hearing aids, persistent use of earplugs/earbuds for noise reduction or music, or by the simply attempting to clean the ears with Q-tips or cotton swabs . Common symptoms include a feeling of fullness in the ear, ear pain or otalgia, itchy ear, the sensation of imbalance, cough, and of course decreased hearing . Roughly 5% of healthy adults, 10% of children, 57% of older persons, and 33% of patients with mental retardation suffer from impaction of cerumen .

        Irrigation of the external auditory canal is one of the many options in treating cerumen impaction and a method that is readily available to the likes of general practitioners and emergency rooms. Irrigation may be performed by non-clinicians; resulting in its own advantages/disadvantages and can be attempted alone or with the pre-treatment of a cerumenolytic agent, such as acetic acid, mineral oil, or hydrogen peroxide. It is important to note, however, that a thorough history and physical exam through the use of otoscopy should be obtained to ensure the tympanic membrane (TM) is intact, without perforation or tympanostomy tubes, and to assess for any anatomic abnormalities prior to any irrigation attempts. If multiple attempts to remove impacted cerumen—including a combination of treatments—are ineffective, clinicians should refer the patient to an otolaryngologist.

        Anatomy and Physiology

        The EAC in most adults tends to follow a posterosuperior to an anteroinferior trajectory, laterally to medially. In children less than 3 years of age, the EAC is largely directed posterosuperior. The lateral one-third of the EAC is made up of fibrocartilage whereas the medial two-thirds is the osseous or bony portion of the canal that contains skin that is tightly adherent to the periosteum without any subcutaneous tissue. The TM is the most medial portion of the EAC, separating it from the middle ear. Approximately 6mm lateral to the TM there is a narrowing of the bony canal known as the isthmus. This may play an important role in a foreign body and cerumen removal alike, as material medial to this point proves to be quite difficult to remove.

        Two tracts or canals exist in the external auditory canal which extend to surrounding structures. More laterally, there are the Fissures of Santorini. These fissures are lymphatic channels that traverse between the incomplete cartilaginous coverings of the lateral one-third of the canal and connect this portion of the canal to the parotid gland, the glenoid fossa, and the infratemporal fossa. More medially, there may be an embryologic defect at the inferior tympanic ring known as the Foramen of Huschke that will connect the medial EAC to the parotid gland and glenoid fossa region. Both of these channels may permit extension of infection or malignant tumors to these surrounding structures, thus special consideration of these possibilities should be kept in mind while performing irrigation of cerumen.

        If irrigation was successful in removing the cerumen impaction, one should be able to evaluate the tympanic membrane anatomy. The normal coloring of a tympanic membrane is pearly gray and translucent. There is a cone of light in the anterior, inferior quadrant of the tympanic membrane, and it points towards the nose. One should also be able to observe the umbo and the handle of the malleus. The tympanic membrane is somewhat conical in shape, with a concavity noted at the umbo. A normal tympanic membrane has no perforation. If the provider observes a bulging tympanic membrane, with a distortion of the cone of light, and little to no visibility of the umbo and the handle of the malleus, this may be indicative of an infection or fluid in the middle ear space — a serous or purulent otitis media. The presence of a eustachian tube dysfunction may result in a retraction of the TM or a serous otitis media.

        The provider should be mindful of the temperature of the water while irrigating the EAC, attempting to keep the water temperature close to the patient’s natural body temperature. Water that is too cold or hot may cause a sensation of dizziness due to the proximity of the lateral semicircular canal to the EAC. The vestibulocochlear nerve has two parts: the vestibular nerve and the cochlear nerve. The semicircular canals of the inner ear are innervated by the vestibular nerve, which is responsible for orientation in space, balance, and coordination. The cochlear nerve is responsible for hearing.

        Indications

        Cerumen impaction irritates the may result in the feeling of fullness in the ear, ear pain or otalgia, itchy ear, the sensation of imbalance, cough, and of course, decreased hearing . Another indication of impactions is an inability to visualize the tympanic membrane due to cerumen when inspection of the tympanic membrane is needed.

        Ear irrigation may also be used for caloric stimulation. This method is discussed as a separate topic.

        Contraindications

        There are a few contraindications to performing irrigation of the ear including lack of patient consent. These contraindications are a patient’s inability to sit upright, a patent tympanostomy tube, a patient who is unwilling or unable to sit still, a foreign body present in the ear canal, a perforated tympanic membrane, an opening into the mastoid, and severe swimmer’s ear (otitis externa). Also, a history of middle ear disease, ear surgery, inner ear problems (especially vertigo), or radiation in the area is an additional reason to choose another method for cerumen dis-impaction.

        Equipment

        Face Shield (universal precautions)

        To safely perform ear irrigation, one should use an otoscope. You will need your cerumenolytic of choice. The water you will use for irrigation must be warmed before use. You can either use a thirty milliliter to a 60-mm syringe with a 16 or 18 gauge intravenous (IV) catheter attached (with the needle removed) or a pulsating water device (such as a WaterPik) to irrigate the impacted cerumen out of the ear. You will also need an ear irrigation basin or emesis basin to catch the water and pieces of cerumen as it leaves the ear.

        Due to the availability of syringes and IV catheters when compared to pulsating water devices, the syringe and IV catheter method is more common.

        A cerumen spoon or alligator forceps can be used to remove loose cerumen pieces following the ear irrigation procedure.

        Personnel

        An assistant can help by holding traction on the pinna. This straightens the ear canal, allowing for more efficient and effective cerumen removal.

        Preparation

        Some providers may choose to soften the wax before irrigation. Multiple agents may be used including mineral oil, 1% sodium docusate solutions, and carbamyl peroxide solutions.

        Warm the solutions and the water that will be used during the irrigation to near body temperature to prevent dizziness. Cold or hot solutions put in the ear are likely to have an uncomfortable effect on the patient, and it may make them dizzy or nauseous.

        If using an IV catheter and syringe, ensure the needle is removed from the IV catheter.

        Technique

        • Ask the patient to sit upright. Place your cerumenolytic of choice in the external auditory canal and leave it in the ear for fifteen to thirty minutes before initiating irrigation.
        • Draw up the warm water into the syringe and attach the IV catheter to the end of the syringe. Place the IV catheter into the external ear canal, no further than the cartilage/bone junction. The cartilaginous portion usually makes up the lateral one-third of the external auditory canal.
        • Hold the emesis or ear irrigation basin tightly to the skin below the ear, in an attempt to catch the water during irrigation. This will help keep the patient from getting wet.
        • Direct the IV catheter superiorly and posteriorly in the ear canal so that the water will separate the cerumen from the tympanic membrane. Do not direct the water stream directly at the tympanic membrane, because this can cause perforation. Do not inject too rapidly as this may result in trauma, bleeding, and pain.
        • Following irrigation, you can remove any loose pieces of wax with a cerumen scoop or alligator forceps, being careful not to damage the external auditory canal and the tympanic membrane.
        • To dry the remaining moisture in the external auditory canal, apply several drops of isopropanol. This step is especially contraindicated if the tympanic membrane is ruptured.

        Following prolonged irrigation

        • Topical steroid containing suspension drops, such as ciprofloxacin/dexamethasone drops, may be soothing to the external auditory canal. Some providers will prescribe these for a few days following the ear irrigation procedure.
        • Many providers prescribe antibiotic drops (example: fluoroquinolones) to patients at high risk for severe infections, such as diabetic patients. These drops are usually prescribed for several days following the ear irrigation procedure to prevent the complication of otitis externa.

        If multiple attempts to remove impacted cerumen—including a combination of treatments—are ineffective, clinicians should refer the patient to an otolaryngologist.

        Complications

        Irrigation of the ear can lead to otitis externa, vertigo, perforation of the tympanic membrane, and middle ear damage if the tympanic membrane is perforated. These complications are less common with the syringe and IV catheter technique than when compared to the pulsating water device technique.

        Using a cerumen spoon to remove the remaining wax can cause damage to the skin covering the external auditory canal.

        Symptoms of complications include sudden pain, ringing in the ears, loss of the ability to hear, nausea, and dizziness. If a patient experiences any of these symptoms, the provider should immediately stop and examine the ear canal and tympanic membrane with an otoscope.

        If the tympanic membrane is ruptured, prescribe the patient oral antibiotics to treat otitis media prophylactically. Refer the patient to an otolaryngologist for specialty consult.

        References

        Pediatric Hearing Loss – Causes, Symptoms, Treatment

        Pediatric hearing loss is a broad category that covers a wide range of pathologies. Early detection and prompt management are essential for the development of normal language and psychosocial functioning, as well as to identify potentially reversible causes or other underlying problems.  Hearing is measured in decibels, and the severity of the hearing loss is graded by hearing thresholds. The normal hearing range is 0-20 decibels (dB) which equates to being able to perceive sound quieter than a whisper. Mild hearing loss corresponds to a range of 20-39 dB, moderate 40-69 dB, severe 70-89 dB and profound is greater than 90 dB.

        There are three main types of hearing loss; conductive, sensorineural and mixed. The former typically occurs due to a problem transmitting sounds at the level of the external or middle ear. The major cause of conductive hearing loss in children is otitis media with effusion (glue ear). Sensorineural hearing loss results from a disruption of the auditory pathway at any point from the cochlea of the inner ear through to the brainstem, and despite being relatively uncommon in children as a whole, it is the primary cause of permanent hearing loss in the pediatric population. Mixed hearing loss occurs when there are both conductive and sensorineural components.

        Causes of Pediatric Hearing Loss

        Hearing loss can be broadly characterized as congenital or acquired in the pediatric population.

        Congenital Causes

        Congenital hearing loss can be classified as genetic and non-genetic in etiology. The former category is responsible for greater than half of congenital causes and can be due to either an autosomal dominant, recessive or sex-linked mutation. Genetic causes are often further subdivided into syndromic versus non-syndromic categories based on whether the patient suffers from an underlying genetic syndrome. Approximately 30% of the genetic causes of hearing loss are syndromic. The most common cause of congenital hearing loss is autosomal recessive non-syndromic hearing loss.

        TORCH organisms (toxoplasmosis, rubella, cytomegalovirus (CMV) and herpes) have been identified as key infective causative agents. CMV is the most common cause of congenital non-genetic hearing loss in the developed world. Other congenital causes include trauma, ototoxic medications used in the antenatal period and several perinatal risk factors such as prematurity, low birth weight, and hyperbilirubinemia.

        Acquired Hearing Loss

        Otitis media with effusion is the number one cause of acquired hearing loss in children. It is beyond the scope of this article to cover this in detail, but it classically has a bimodal beak at 2 years and 5 years of age and is characterized by a conductive hearing loss associated with flattened tympanogram. It typically resolves without intervention as the eustachian tube matures or following the insertion of a ventilation tube in the middle ear. Adenoidal hypertrophy can contribute to this clinical picture.Infections also present another major category for acquired hearing loss, with a particularly strong link with bacterial meningitis, mumps, and measles. Other reasons include primary otological pathologies such as cholesteatoma, impacted wax and otosclerosis as well as trauma.

        High-risk factors in neonates:

        • Congenital infections
        • Family history
        • Craniofacial anomalies
        • Hyperbilirubinemia
        • Birth weight 1500 g
        • Low Apgar
        • Bacterial meningitis
        • Need to prolonged intubatio

        Pathophysiology

        Any condition that lowers the transmission of sound from the external space to the cochlea will cause conductive hearing loss. This include cerumen, abnormalities of the helix or auricle, effusions, and fixed ossicular chain. Besides cholesteatoma, other masses include glomus tumors, schwannomas of the facial nerve and hemangiomas.

        Sensorineural hearing loss is due to interruption of sound transmission after the cochlea. This may be due to damage to the hair cells or damage to the 8th cranial nerve. Even mild distortions in the hair cells can result in severe hearing loss.

        Categories of hearing loss are as follows:

        • Slight hearing loss: 16-25dB
        • Mild hearing loss:    26-40dB
        • Moderate hearing loss: 41-55dB
        • Severe hearing loss: 71-90dB
        • Profound hearing loss: 90dB
        History and Physical

        Hearing loss can present in different ways depending on the age of the child. Hearing loss in neonates is almost exclusively picked up via newborn screening program assessments. In older children, parents or other professionals such as school teachers, may notice delayed language skills, behavioral problems or listen to the television at raised volumes. In history, it is important to ascertain whether there are any associated otological symptoms such as otorrhoea, otalgia, tinnitus, or vertigo. A thorough history is required including asking about any other neurological symptoms, medical history including drug history and precipitating events such as trauma, recent viral infections or new medications.

        The examination will involve assessing the ear including the appearance of the pinna particularly inspecting for any deformities such as microtia or anotia. Otoscopic examination of the external auditory canal and tympanic membrane is crucial, with special attention on the attic for cholesteatoma. An examination should also include assessment of cranial nerves, a full neurological assessment, and assessment of balance depending on the age of the child.

        With the implementation of Universal Newborn Hearing Screening program, today most patiets are identified within a few months after birth, with intervention started by 6 months.

        Evaluation

        Hearing assessment in children is age and ability dependent and will be addressed per age group here.[9]

        Neonates

        Otoacoustic Emissions

        In the UK, all newborns and those who require less than 48 hours of special care in neonatal intensive care (NICU), are offered evoked otoacoustic emission (OAE) testing within the first 4-5 weeks of birth as part of a Newborn Hearing Screening Programme. Oto-acoustic emissions are outer hair vibrations that are detected in the external auditory canal in response to a click stimulus. This test is easy to perform and does not involve a general anesthetic.

        Automated Auditory Brainstem Response

        This investigation is offered to all newborns who have spent over 48 hours in the neonatal intensive NICU and is also offered to those who do not pass two OAE tests. It involves measuring brainstem electrophysiological responses to click stimuli using electrodes placed on the scalp. This assesses hearing throughout the entire hearing pathway; form the external ear through to the brainstem.

        6-8 months                                               

        Distraction techniques

        An assistant engages the child’s attention, and the tester, whilst placed behind and to the side of the child, makes sounds of different intensities. The child is assessed to see whether they turn to the side of the noise.

        9 – 36 months

        Visual Reinforcement Audiometry

        The child is placed at a table with some toys with two speakers either side that produce sounds. If the child looks towards the speaker playing a sound they are delivered a visual reinforcement (such as a flashing light).

        24-60 months

        Conditioned Play Audiometry

        The child is conditioned to perform a task in response to an auditory stimulus such as placing a ball in a cup. Once the task is learned the sound volume is reduced in order to determine their hearing threshold.

        Over 60 months

        Pure Tone Audiometry

        A 5 years of age most children can undergo pure tone audiometry. Hearing thresholds are determined by presenting sounds of various frequencies and at various intensities until the quietest sound is reliably detected 50% of the time. This test requires a higher level of attention and therefore is rarely done below the age of 5 years.

        Other investigations

        Additional investigations will be tailored to the precise clinical picture. In syndromic children, chromosomal testing is advised. There is also a role for imaging in the form of either computed tomography (CT) or magnetic resonance imaging (MRI)

        Some authors advocate measurement of renal function and testing for connexin-26, which is a marker who sensorineural hearing loss. In some children, imaging studies may prove useful and detect abnormalities of the cochlea or the cochlear nerve. Finally, ECG may be useful in children with Jervell Lange Nelsen syndrome. The ECG will reveal a prolonged QT interval, which can lead to syncopal attacks and death.

        Treatment of Pediatric Hearing Loss

        Treatment for hearing loss depends on the type of hearing loss present, the underlying cause and often there is an element of patient/parent preference.

        Conductive hearing loss due to otitis media is treated with antibiotics. Some children may benefit from a myringotomy tube. Sensorineural loss cannot be treated with medical measures. Mild cases may be treated with amplifcation aids and speech therapy is useful. However, amplifcation of sound can result in ear pain and discomfort.

        Conservative management

        A key element to managing hearing loss in family support and advice. There are a number of behavioral measures that can be used to improve hearing without the need for adjuncts or surgical intervention. The principles of this are rooted in creating a deaf-friendly environment such as limiting background noise, talking face-on, and clear intonation. There are also a range of hearing assist devices that can be used such as television listeners. It is also crucial that the child educational support which could be in the form of special equipment or positioning in the classroom.

        • Hearing Aids – There are a variety of hearing aid types that are used in specific situations. Each type will be briefly covered here.
        • Binaural air conduction hearing aids – rely on at least a partially functioning inner ear and central auditory processing system. They work by converting sound detected by a microphone into digital signals which can then be amplified and re-converted into audible sounds that are transmitted to the ear. They can be classified based on whether these key parts are housed in an earpiece that sits externally (behind-the-ear), inside the canal (in-the-canal) or further inside the canal (in-the-ear).
        • Bone conduction – hearing aids are used typically in conductive hearing loss when there are ear problems that impede the use of regular air-conduction hearing aid such in children with external ear deformities (anotia, microtia) or when there are chronic ear infections. Bone-anchored hearing aids (BAHA) are fitted surgically under general anesthetic over two stages. A titanium implant is fixed into the temporal bone. Through this setup, a sound is conducted directly to the inner ear by way of the bone, bypassing the middle ear. Typically the BAHA is fitting from 4 years of age once the temporal bone has developed, however, soft-band bone-conducting aids can be used from several weeks of age.
          Contralateral routing of sound (CROS) – hearing aids are used when there is a unilateral sensorineural hearing loss. The sound in the problem ear is diverted to the better hearing ear without amplification. In cases where neither ear has normal hearing but one side is significantly better, a variation on this can be used called a BiCROS.
        • Cochlear Implant – Cochlear implants work by converting sound into digital signals that are transmitted directly to the auditory nerve via an electrode array. In the UK, the National Institue of Clinical Excellence (NICE) recommends cochlear implants in children who have severe to profound deafness in one or two ears with minimal benefit from conventional hearing aids after 3 months of use.

        Other options

        Ventilation tubes are indicated in conductive hearing loss secondary to flue ear, or less frequently in the context of recurrent otitis media. They are inserted surgically and typically self-extrude on average a year of insertion. Children found to have cholesteatoma invariably require surgical clearance of disease via a mastoidectomy.

        Differential Diagnosis

        • Acute otitis media
        • Cholesteatoma
        • Congenital stenosis
        • Exostoses
        • Foreign body
        • Hemotympanum
        • Impacted cerumen
        • Keratosis obturans
        • Middle ear tumor
        • Otitis externa

        References

        Presbycusis – Causes, Symptoms, Diagnosis, Treatment

        Presbycusis refers to bilateral age-related hearing loss. In literal terms, presbycusis means “old hearing” or “elder hearing.” It becomes noticeable around age 60 and progresses slowly; however, there is evidence that certain stressors can speed the rate of deterioration. The diagnosis can be confirmed with audiometry. The hallmark of presbycusis is the impaired ability to understand high-frequency components of speech (voiceless consonants, such as p, k, f, s, and ch). There is no cure; however, hearing aids that amplify sounds can be used to mitigate symptoms. Anatomically, presbycusis involves multiple components of the auditory system. It is primarily due to age-related changes in hair cells, the stria vascularis, and afferent spiral ganglion neurons.

        Presbycusis (also spelled presbyacusis, from Greek press “old” + akousis hearing), or age-related hearing loss, is the cumulative effect of aging on hearing. It is a progressive and irreversible bilateral symmetrical age-related sensorineural hearing loss resulting from degeneration of the cochlea or associated structures of the inner ear or auditory nerves. The hearing loss is most marked at higher frequencies. Hearing loss that accumulates with age but is caused by factors other than normal aging (nosocusis and sociocusis) is not presbycusis, although differentiating the individual effects of distinct causes of hearing loss can be difficult.

        During the normal hearing, sound, in the form of air vibration, is captured by the funnel-shaped external ear and is directed to the tympanic membrane. This causes the tympanic membrane to vibrate at a specific frequency and amplitude. This movement is amplified by three small bones in the middle ear: the malleus, incus, and stapes. From there, the signal proceeds as vibrations that are transmitted through the fluid within the inner ear to the cochlea. In the cochlea, receptors known as hair cells transform the information encoded in the vibrations into a neurologic signal which travels to the auditory cortex via the cochlear nerve.

        Pathophysiology

        Examples of microscopic changes seen in this condition are hair cell degeneration of the cochlea and giant stereociliary degeneration.

        There are four pathological phenotypes of presbycusis:

        • Sensory – characterized by degeneration of the organ of Corti, the sensory organ for hearing. Located within the scala media, it contains hair cells with stereocilia, which extend to the tectorial membrane. The organ’s outer hair cells play a significant role in the amplification of sound and is extremely sensitive to external and internal factors. If the outer hair cells are damaged, they do not regenerate. This results in a loss of sensitivity of hearing, as well as an abnormal perceived loudness in the aspect of the tonotopic spectrum that the damaged cells serve.
        • Neural – characterized by degeneration of cells of the spiral ganglion.
        • Strial/metabolic – characterized by atrophy of stria vascularis in all turns of cochlea. Located in the lateral wall of the cochlea, the stria vascularis contains sodium-potassium-ATPase pumps that are responsible for producing the endolymph resting potential. As individuals age, a loss of capillaries leads to the endolymphatic potential becoming harder to maintain, which brings a decrease in cochlear potential.
        • Cochlear conductive – due to stiffening of the basilar membrane thus affecting its movement. This type of pathology has not been verified as contributing to presbycusis.

        In addition, there are two other types:

        • Mixed
        • Indeterminate

        The shape of the audiogram categorizes abrupt high-frequency loss (sensory phenotype) or flat loss (strial phenotype).

        The mainstay of SNHL is trial, with only about 5% of cases being sensory. This type of presbycusis is manifested by a low-frequency hearing loss, with unimpaired speech recognition.

        Classically, audiograms in neural presbycusis show a moderate downward slope into higher frequencies with a gradual worsening over time. A severe loss in speech discrimination is often described, out of proportion to the threshold loss, making amplification difficult due to poor comprehension.

        The audiogram associated with sensory presbycusis is thought to show a sharply sloping high-frequency loss extending beyond the speech frequency range, and clinical evaluation reveals a slow, symmetric, and bilateral progression of hearing loss.

        Causes of Presbycusis

        Presbycusis is multifactorial in origin. In addition to age-related degeneration leading to physiologic and anatomic changes, other contributing factors include genetic factors, hormones, exposure to loud noises or ototoxic agents, history of ear infection, and the presence of certain systemic diseases.

        Age-related Factors

        Presbycusis can be broken down further with regards to which structures and functions are primarily affected. Some argue that there is little clinical utility in subdividing presbycusis as there is no significant change in approach or treatment, and oftentimes mixed pathology is present. Presently, there are thought to be six categories of presbycusis: sensory, neural, trial, mechanical, mixed, and indeterminate.

        • Sensory presbycusis loss of receptor hair cells at the basal aspect of the cochlea resulting in characteristic high-frequency hearing loss.
        • Neural presbycusis loss of cochlear nerve fibers as well as the loss of spiral ganglion neurons.
        • Strial presbycusis degeneration of stria vascular cells. These cells are essential for maintaining the appropriate ion composition of endolymph to generate the endocochlear potential for signal transduction. Sometimes referred to as metabolic presbycusis.
        • Mechanical presbycusis (cochlear conductive) – due to physical changes of the cochlear duct. This is accompanied by a specific audiogram pattern.
        • Mixed presbycusis – characterized by pathologic changes in more than one of the above structures
        • Indeterminate presbycusis – cases in which changes to the above structures are not significant.
        • Genetic Factors – Genetic factors, specifically, differences in mitochondrial DNA expression genes related to oxidative stress, have been found in patients with presbycusis when compared to controls.
        • Ototoxic Factors – There are multiple medications associated with ototoxicity, including salicylates, loop diuretics, aminoglycoside, and certain chemotherapeutic agents. Additionally, some work and environmental-related exposures to chemicals such as toluene, styrene, lead, carbon monoxide, mercury, and other toxins have been shown to cause ototoxicity. Minimizing exposure to these agents can help to prevent age-related hearing loss.
        • Noise Exposure Factors – Some long-term studies have shown that individuals who have sustained noise-induced cochlear damage in their youth go on to develop more severe presbycusisAnatomically, noise exposure may lead to damage and subsequent loss of spiral ganglion neurons.
        • Hormonal Factors – Glucocorticoids, sex hormones, and glutamate signaling are thought to play a role in presbycusis. Prolonged corticosterone levels and loss of nuclear factor kappa B have been associated with increased spiral ganglion neuron loss. The use of progestin and combination hormone replacement therapy in postmenopausal is associated with a more frequent incidence of hearing loss.

        Symptoms of Presbycusis

        Primary symptoms:

        • sounds or speech becoming dull, muffled or attenuated
        • need for increased volume on television, radio, music and other audio sources
        • difficulty using the telephone
        • loss of directionality of sound
        • difficulty understanding speech, especially women and children
        • difficulty in speech discrimination against background noise (cocktail party effect)
        • hyperacusis, heightened sensitivity to certain volumes and frequencies of sound, resulting from “recruitment”
        • tinnitus, ringing, buzzing, hissing or other sounds in the ear when no external sound is present

        Diagnosis of Presbycusis

        Presbycusis is generally insidious in onset, and mild cases are difficult to detect. It is imperative that primary care physicians screen for hearing loss, especially geriatricians and those caring for adults age 60 and beyond. Screening for hearing impairment is part of the ‘Welcome to Medicare Visit.” Often, family members and friends are more aware of hearing loss than patients themselves. A common initial presentation is a difficulty discriminating speech in specific situations, such as a room with significant background noise. Some patients complain of tinnitus, or ringing in the ears, however, this is not specific to presbycusis. Formal questionnaires exist, such as the Hearing Handicap Inventory for the Elderly-Screening (HHIE-S). However, some investigators found this formal screening tool to be less sensitive and more time-consuming than the single question “Do you have a hearing problem now?” Gathering a thorough history regarding the patient’s ability to communicate, and ideally getting input from a close contact can help to identify individuals who should be sent for further audiometry testing. Asking about recreational or occupational exposure to loud noises, the use of ototoxic medications, and family history of age-related hearing loss is also important. Referral to an otolaryngologist should be considered if the patient’s hearing loss is acute, unilateral, or accompanied by neurologic symptoms, such as facial numbness or weakness, loss of balance, or dizziness.

        The general physical exam is usually unremarkable in patients with presbycusis. It is common for older adults to have age-related benign opacification of the tympanic membrane and build-up of cerumen. If a moderate amount of cerumen is present, this should be removed to rule out impaction or obstruction as a potential cause of hearing loss. Tuning forks may be used to discriminate between conductive and sensorineural hearing loss; however, their use is limited by patient cooperation and physician subjectivity. Determining whether the pattern of hearing loss is sensorineural or conductive is an important first step in the diagnosis. This can be done by performing both Weber’s test and the Rinne test using a tuning fork. These tests should not be used as screening or diagnostic tools, but simply for differentiating between conductive and sensorineural hearing loss. Presbycusis is sensorineural in origin; therefore, the Rinne test should reveal that air conduction is heard longer than bone conduction in both ears. Weber’s test should localize toward the ear with better hearing, signifying a contralateral sensorineural loss. Weber’s test may vary and may result in a falsely normal result if hearing loss is symmetric.

        Evaluation

        Routine physical exam maneuvers alone are not sufficient for diagnosing presbycusis. An in-office screening audiometry test administered by trained personnel should be considered by providers such as geriatricians who have a large population of older adults at high risk of presbycusis. There should be a low threshold for referral for definitive auditory testing in patients suspected of presbycusis. Imaging is usually not performed except in cases in which there is a discrepancy between presentation and auditory testing, or there are associated neurological changes.

        There are many variations of audiometry testing and central auditory testing that are beyond the scope of this review. In general, an audiometry exam tests the ability to hear sounds at varying intensity (loudness) and frequencies (tone). Commonly, pure tone testing (also known as an audiogram) is performed in patients with suspected age-related hearing loss. Pure tones are delivered through the use of headphones to one ear at a time. Patients are asked to respond if they hear a sound. The results are presented in the form of an audiogram, a graph with hearing level (in decibels) on the y-axis and frequency (in hertz) on the x-axis. In presbycusis, the highest frequency sounds are typically affected first, followed by lower and lower frequency sounds as the condition progresses. Presbycusis is characterized by bilateral hearing loss above 2000 Hz. On a standard audiogram, presbycusis appears as an overall down-sloping line that represents impaired hearing at higher frequency sounds.

        • Laboratory testing – for diseases commonly associated with hearing loss, such as dyslipidemia, diabetes, and renal dysfunction, may be indicated but are not required for diagnosis.Tinnitus occurring in only one ear should prompt the clinician to initiate further evaluation for other etiologies. In addition, the presence of a pulse-synchronous rushing sound may require additional imaging to exclude vascular disorders.
        • Otoscopy – An examination of the external ear canal and tympanic membrane performed by a medical doctor, otolaryngologist, or audiologist using an otoscope, a visual instrument inserted into the ear. This also allows some inspection of the middle ear through the translucent tympanic membrane.
        • Tympanometry – A test administered by a medical doctor, otolaryngologist or audiologist of the tympanic membrane and middle ear function using a tympanometer, an air-pressure/sound wave instrument inserted into the ear canal. The result is a tympanogram showing ear canal volume, middle ear pressure and eardrum compliance. Normal middle ear function (Type A tympanogram) with a hearing loss may suggest presbycusis. Type B and Type C tympanograms indicate an abnormality inside the ear and therefore may have an additional effect on the hearing.

        Laboratory studies

        This may include a blood or other sera test for inflammatory markers such as those for autoinflammatory diseases.

        • Audiometry – A hearing test administered by a medical doctor, an otolaryngologist (ENT) or audiologist including pure tone audiometry and speech recognition may be used to determine the extent and nature of hearing loss, and distinguish presbycusis from other kinds of hearing loss. Otoacoustic emissions and evoked response testing may be used to test for audio neuropathy. The diagnosis of a sensorineural pattern hearing loss is made through audiometry, which shows a significant hearing loss without the “air-bone gap” that is characteristic of conductive hearing disturbances. In other words, air conduction is equal to bone conduction. Persons with cochlear deficits fail otoacoustic emissions testing, while persons with 8th cranial nerve (vestibulocochlear nerve) deficits fail auditory brainstem response testing.
        • Magnetic resonance imaging (MRI) – As part of differential diagnosis, an MRI scan may be done to check for vascular anomalies, tumors, and structural problems like enlarged mastoids. MRI and other types of scan cannot directly detect or measure age-related hearing loss.

        Treatment of Presbycusis

        There is no cure for presbycusis. Hearing aids are the mainstay of treatment and have been shown to have a significantly positive effect on the quality of life and communication. Hearing aids do have limitations. They do not repair normal hearing, but instead simply amplify sounds. Devices can be very expensive and often are not covered by the patient’s insurance. While smaller hearing aids are potentially more comfortable and discrete, decreased dexterity in geriatric patients may make these devices less convenient. Importantly, the management of hearing aids does not stop once the devices are fitted. Learning to use hearing aids and adjusting to both the physical discomfort and cognitive adjustment takes significant effort and practice. A collaborative, interdisciplinary approach involving the primary care provider and audiologist is recommended for continued auditory rehabilitation. Patients often require encouragement as many find hearing aids uncomfortable, unattractive, and embarrassing. Hearing aids are indicated at certain thresholds of hearing loss. Cochlear implants can be offered to patients with severe bilateral hearing loss that is not improved with hearing aids. Specific criteria exist for patients to be considered candidates, and often include a predetermined level of impairment in word identification.

        As extrinsic factors are thought to have a role in the progression of presbycusis, wearing earplugs or earmuffs to attenuate sounds may be helpful if the patient needs to be exposed to loud noises. A diet low in saturated fat may help slow hearing loss. Maintaining a healthy, active lifestyle is a logical form of risk reduction in light of the fact that hearing loss is associated with stroke, myocardial ischemia, hypertension, hyperlipidemia, and diabetes. Smoking should be discouraged, as cessation has been shown to delay age-related hearing loss.

        There is an abundance of ongoing research regarding the genetic and metabolic components of age-related hearing loss. Due to the potential role of oxidative damage, it was thought that antioxidants might slow the progression of hearing loss. While the administration of alpha-lipoic acid has been shown to prevent age-related hearing loss in rats, an antioxidant-enriched diet in humans did not delay the progression of hearing loss. Other agents, such as coenzyme Q-10 and Ginko biloba, have been studied and lack sufficient evidence for use. Additionally, the use of these supplements is controversial as prolonged administration has been associated with an increase in overall mortality. There are ongoing investigations into potential gene and hormone therapies for hearing loss.

        References

        Examination of The Ear – Indications, Contraindications

        Examination of the ear is a vital skill for a variety of medical professionals, including otolaryngologists, primary care physicians, emergency care physicians, paramedics, and pediatricians. Pathologies that result in positive ear examination findings range from the common and benign, such as viral labyrinthitis, to the rare and potentially life-threatening, such as acute bacterial mastoiditis.

        Anatomy and Physiology

        Knowledge of the anatomy of the external, middle, and inner ear systems is vital to enable clinicians to interpret what may be vague or misleading symptoms. The interaction and anatomical proximity of the ear to the brain, skull-base, and cranial nerves must also be appreciated, as certain pathology may involve these structures. In general, abnormalities of the external and middle ears will produce conductive hearing loss, and abnormalities of the inner ear will produce sensorineural hearing loss.

        • External Ear – The external ear comprises of the auricle (pinna), the external auditory meatus and canal, and the external (lateral) layer of the tympanic membrane. The function of the external ear is the funneling of acoustic waves to the tympanic membrane and, therefore, the middle ear. Embryologically the pinna is formed by the fusions of six mesenchymal proliferation, known as Hillocks of His, derivations of the first and second branchial arches. Incomplete fusion of these Hillocks can lead to the development of preauricular pits or sinuses. The entire external ear can be visualized directly during an ear examination.
        • Middle Ear – This is a complex air-filled cavity, found within the temporal bone of the skull. It contains the three ossicles, the internal (medial) layer of the tympanic membrane, and the Eustachian tube orifice. It is lined with respiratory epithelium, which is continuous with the lining of the Eustachian tube, and therefore of the upper aerodigestive tract. The ossicles are termed malleus, incus, and stapes (lateral to medial). The primary function of the middle ear is to transmit acoustic waves from the tympanic membrane of the external ear to the oval window of the inner ear via the ossicles. During the examination, certain parts of the middle ear can be viewed through the tympanic membrane: the lateral process of the malleus, the incudostapedial junction, and occasionally the promontory. The compliance of the middle ear can be measured with tympanometry, not part of the standard ear examination – this measures impedance to soundwave transmission through the middle ear. A number of pathologies will result in an abnormal tympanogram, such as fluid within the middle ear, or disruption of the ossicular chain.
        • Inner Ear – The inner ear is comprised of the vestibular system and cochlea, both of which have a bony and membranous portion. The function of the inner ear is both the conversion of acoustic vibrations into neural impulses for hearing, as well as the detection and transmission of cranial movement for balance. No parts of the inner ear can be directly visualized during the ear examination. However, signs of inner ear disease, such as sensorineural hearing loss or vestibular dysfunction, can be elicited.

        Indications

        Symptoms that mandate an ear examination include otalgia, otorrhea, vertigo, tinnitus, aural fullness, hearing loss, and facial weakness. Careful history taking should differentiate between true vertigo (sensation of the room spinning, indicating vestibular system dysfunction) and the sensations of faintness (usually due to a pre-syncopal episode), light-headedness (a non-specific symptom) or disequilibrium due to cerebellar or gait disturbances. Patients often use the word ‘dizzy’ to describe all of these sensations.

        Less common prompts for ear examination include patients with postnasal space disease, such as nasopharyngeal carcinoma, to exclude sequela resulting from an occluded Eustachian tube orifice. Patients that have sustained trauma to the head and neck may have reduced consciousness, and an ear examination may be required if there is any concern regarding the lateral basal skull injury. This may be prompted by the presence of CSF otorrhoea or CT scan evidence of temporal bone fracture.

        If any hearing impairment is identified during routine hearing screening (such as during the NHS Newborn Hearing Screening Program in the UK), then a formal ear examination must follow.

        Contraindications

        A lack of patient consent in a patient with the capacity to make such a decision precludes ear examination. Caution must be taken in patients with otalgia as the examination can be uncomfortable.

        Equipment

        An otoscope will allow for the assessment of the pinna, external auditory canal, and tympanic membrane. The light also enables the examiner to perform a close examination of the pre- and postauricular areas. Disposable specula of varying sizes are required for the otoscope. To assess the mobility of the tympanic membrane, pneumatic otoscopy can be performed. This requires an otoscope with a pneumatic bulb, and speculae with rubber rings to create an air-tight seal within the canal.

        A tuning fork is required for the differentiation between conductive and sensorineural hearing loss. The ideal tuning fork of choice is one that has a long period of tonal decay that also causes minimal vibration sense (to avoid the patient sensing vibration and to confuse this with sound). Otolaryngologists traditionally utilize a 512-Hz tuning fork as it provides the greatest balance between these two characteristics.

        Personnel

        An effective ear examination requires only the examiner and patient. If the examination is of a child, then both the parent of the child, and either an experienced nurse or play-specialist can be useful to maximize patient compliance.

        Preparation

        The patient should be sat on a chair, suited to their habitus and comfort. The chair should ideally be in the center of the room, as part of the examination requires the examiner to stand behind the patient. Prior to the examination, one must first ask the patient if they are in any pain. The patient should also be asked whether they have any ear-related symptoms (specifically discharge, pain, hearing loss) – and which they think is their better hearing ear. Convention states that one first examines the better ear.

        Clear and courteous description of what is required of the patient during the examination will reduce the possibility of discomfort or confusion, especially during tests that require patient cooperation such as the tuning fork tests.

        As with all clinical encounters, the examiner should review clinical notes of previous encounters, any relevant referral letters, and review any investigations such as clinical imaging or hearing tests.

        To reduce the risk of cross-contamination of pathogens, the WHO recommended ‘5 moments of hand hygiene’ should be adhered to throughout the examination.

        Technique

        The examiner must develop their system when approaching the ear examination; this instills fluency and structure and ensures nothing is missed. Most examiners first perform a general inspection, before focused inspection of the ears, palpation, and otoscopy. This is then followed by what is known as the “four F’s”: free-field hearing test, the fistula test, (tuning) forks, and the facial nerve. Depending on the clinical scenario, examinations of the other cranial nerves, vestibular system, nose, throat, neck, or cerebellar systems may follow.

        Patients that present with ear pathology usually are well and assessed in the outpatient clinic. However, any suspicion that the patient is unwell should prompt assessment according to the Advanced Life Support ‘ABCDE’ algorithm.

        Inspection and palpation

        The ears are first visualized with the patient in a seated position facing the examiner. The examiner should note any asymmetry of the ears or any prominence, and a note should be made of any resting facial asymmetry. Starting with the ‘better’ ear, and using the otoscope as a light source, the examiner should inspect the preauricular area for any surgical or traumatic scars, masses, evidence of pits or sinuses, or skins changes such or erythema or desquamation. The pinna itself requires a detailed inspection for congenital malformations, scars, erythema, edema, masses, or exudate from the external auditory canal. If there are any piercings, a note should be made of any signs of infection, and the examiner should be vigilant for signs of perichondritis. Finally, the postauricular area should be inspected for scars and erythema. Mastoid erythema, swelling, loss of the post-auricular sulcus, and anteroinferior displacement of the pinna are all signs of mastoiditis. The ‘worse’ ear should then be inspected in turn.

        Following this inspection, the mastoid and tragus should be palpated for tenderness, indicating mastoiditis and otitis externa, respectively. Pre- and post-auricular lymphadenopathy should also be noted.

        Otoscopy

        The pinna should be gently pulled in a posterosuperior direction, having warned the patient. This results in straightening of the external auditory canal and subsequent alignment of the cartilaginous and bony portions of the canal. The otoscope should be gently inserted into the external auditory canal. Any discomfort doing so should be noted. The canal should be assessed for any edema, exudate, wax, foreign bodies, and the presence or absence of a mastoid cavity (from a previous ‘canal-wall-down’ mastoidectomy). The tympanic membrane, if visible, should be assessed for perforation, sclerosis, retraction. The presence or absence of a normal light reflex should be noted. The attic area, immediately superior to the tympanic membrane, should be carefully inspected for signs of cholesteatoma. In cases of middle ear effusion, fluid levels or bubbles may be seen behind the tympanic membrane.

        Pneumatic otoscopy is a simple test to assess the mobility of the tympanic membrane. The combined features of a bulging tympanic membrane and reduced mobility of pneumatic otoscopy are highly suspicious for acute otitis media. The tip of the speculum is inserted into the canal as with routine otoscopy, ensuring an airtight seal is formed. If there is any concern about an air leak, then a speculum with a rubber seal should be utilized, although this is not always required. Paying careful attention to the light reflex, positive pressure is introduced into the ear canal by gently squeezing the pneumatic bulb, and releasing it. In a normal ear, the tympanic membrane should move briskly. If there is fluid in the middle ear, such as in the presence of acute otitis media, then mobility will be restricted.

        Free-field hearing test

        This is a bedside test of hearing. It is not as reliable as formal audiometry and is highly examiner-dependent. However, it has value as a screening tool for hearing impairment. The test can reveal whether any hearing impairment is present and estimate the degree of the impairment.

        Firstly, explain to the patient that they will be required to repeat words or phrases that they hear back to the examiner. To eliminate the possibility of lip-reading, stand behind the patient, and say a test word to be repeated back. The examiner then stands to the side of the test ear (conventionally the better hearing ear) and gently presses on the tragus of the non-test ear, to mask it. The examiner then speaks a number of test words at arm’s length, and then at a half arm’s length. At each distance, the test words are spoken at three volumes: whispered, conversational, and loud.

        The following descriptors of hearing can then be noted if the patient correctly identifies 50% or more words at each level:

        Arm’s length test words:

        • Whispered: normal hearing
        • Conversational: mild-moderate hearing loss
        • Loud: severe hearing loss

        Half-arm’s length test words:

        • Whispered: mild hearing loss
        • Conversational: moderate hearing loss
        • Loud: profound hearing loss

        Fistula test

        A perilymphatic fistula is an abnormal connection between the inner ear and the middle ear, allowing perilymph to leak into the middle ear. This can be secondary to dehiscence at the oval or round windows, or otic capsule. Etiologies include trauma, cholesteatoma, otological surgery, or barotrauma. The pressure of the middle ear is transiently increased during the test. If a perilymphatic fistula is present, this pressure may be directly transmitted to the inner ear.

        Prior to performing the test, the patient is warned that it may make them feel dizzy. The test is, therefore, performed sitting down. The patient’s tragus is firmly palpated, and the examiner assesses the patient’s eyes: nystagmus toward the test ear implies a perilymphatic fistula.

        (Tuning) Fork tests

        Many otological pathologies result in hearing loss, either conductive, sensorineural, or both. Conductive loss is due to a blockage of the vibrations reaching the cochlea, due to ossicular disruption or tympanic membrane perforation, for example. Sensorineural hearing loss is due to damage to the cochlear itself, or due to damage to the vestibulocochlear nerve.

        Weber’s and Rinne’s tuning fork tests allow the differentiation between these two types of hearing loss. Weber’s test is performed first. The examiner strikes the tuning fork and places it in the center of the patient’s forehead, with the examiner’s other hand providing counter-pressure on the back of the patient’s head. The patient is then asked to state whether they can hear it loudest in the left, right, or center.

        Interpretation of Weber’s test:

        1. Normal hearing or symmetrical hearing loss: heard in the center.
        2. Asymmetrical sensorineural hearing loss: heard loudest in the normal ear.
        3. Asymmetrical conductive hearing loss: heard loudest in the affected ear.

        Rinne’s test is then performed by striking the tuning fork and holding it 2 cm away from the external acoustic meatus (air conduction). After 2 to 3 seconds, the base of the fork should then be firmly pressed on the mastoid, using the examiner’s other hand to provide contralateral counter-pressure (bone conduction). The patient is then asked which sound was heard loudest

        Interpretation of Rinne’s test:

        1. Air conduction heard louder than bone conduction [Rinne’s positive]: either normal hearing or sensorineural hearing loss.
        2. Bone conduction heard louder than air conduction [Rinne’s negative]: conductive hearing loss.

        Therefore in left sensorineural hearing loss, Weber’s will lateralize to the right, and Rinne’s will be positive on both sides. In the left conductive hearing loss, Weber’s will lateralize to the left, Rinne’s will be negative on the left and positive on the right.

        Facial nerve function testing

        The facial nerve has a complex intratemporal course, running through the middle ear. Therefore, a number of otological pathologies, including cholesteatoma, surgery, trauma, and middle ear infection, can cause facial nerve palsy. Examining for this is most commonly performed by assessing the branchiomotor component of the nerve, which innervates the muscles of facial expression. The examiner should ask the patient to lift their eyebrows, squeeze their eyes shut and to resist forceful eye-opening, puff out their cheeks, and show their teeth. Any weakness should be graded using the House-Brackmann grading system.

        References

        Hyperacusis – Causes, Symptoms, Treatment

        Hyperacusis is a rare disorder of loudness perception, where sounds that are ordinarily considered innocuous become intolerable. Patients may perceive this sensation as painful, frightening, unpleasant, or excessively loud. Hyperacusis often co-exists with tinnitus and can cause significant distress, with patients regularly reporting impairment in their social, occupational, and recreational activities. Avoiding sound sources and seeking medical attention are common behaviors.

        Although a definitive cure is yet to be identified, research in this field is accelerating, and there has been a 10-fold increase in the number of peer-reviewed studies on the condition in the past four decades.

        Pathophysiology

        Measured in decibels (dB), loudness discomfort level (LDL) describes the point at which a subject perceives a sound as being uncomfortably loud. In patients with hyperacusis, LDL is decreased by 16–18 dB compared to the general population, in whom the average LDL is around 100 dB. Regardless of the degree of hearing loss, LDL in hyperacusis is decreased across the whole frequency spectrum, suggesting an increase in auditory gain as the underlying mechanism. Supported by experimental studies, the central auditory gain model proposes that a reduction in auditory input can lead to a dysfunctional increase in neuronal gain, leading to over-amplification of sound and the symptoms of hyperacusis. This mechanism is also demonstrated through studies that induce temporary auditory deprivation through wearing earplugs. Even if only worn in one ear, there is a bilateral increase in perceived loudness. The plasticity of the central auditory system is, therefore, implicated in using sound therapy as a potential treatment for hyperacusis.

        There are several other proposed mechanisms. The prevalence of hyperacusis in those suffering from Williams syndrome led to the theory that 5-hydroxytryptamine (5-HT) dysfunction increases auditory sensitivity, however, the high rate of otitis media in Williams syndrome may be more contributory. Stapes hypermobility is also cited as a cause of peripheral hyperacusis, and conditions that involve paralysis of the facial nerve (i.e., Bell’s palsy, Ramsay-Hunt syndrome, and Lyme disease) are involved in the etiology of the condition. This cohort responds well to oval window reinforcement, which is reported to improve LDL and subjective symptoms.

        Causes of Hyperacusis

        Although there are many associations, a direct underlying cause for hyperacusis is rarely found. The most common cause of hyperacusis is high noise exposure. The association between hyperacusis and hearing loss is complicated. Occupational and recreational noise exposure and noise-induced hearing loss are commonly cited as major risk factors. Indeed, studies show that professional musicians are more likely to suffer from hyperacusis, especially those playing pop/rock music and exposing themselves to prolonged periods of amplified sound. However, in a multivariate analysis of 850 participants, no significant correlation was reported between hearing threshold loss and hyperacusis. We can reasonably conclude from this that hyperacusis often occurs in conjunction with hearing loss, but it is not essential in the development of the condition.

        Those that suffer from hyperacusis are more likely to be comorbid. Psychiatric conditions, functional diseases (i.e. fibromyalgia, chronic fatigue syndrome), and joint and back disorders are particularly common. Fifty-six percent of patients referred to secondary care for hyperacusis met the criteria for at least one psychiatric illness, with 47% having an anxiety disorder. Concurrently suffering from tinnitus is remarkably common, with rates reported to be as high as 86%. Migraine, post-traumatic stress disorder, Lyme disease, and Williams syndrome are also implicated in the etiology of the disease, with up to 90% of those with Williams syndrome reporting hyperacusis symptoms.

        Diagnosis of Hyperacusis

        The subjectivity of hyperacusis has given rise to several definitions in the literature, the simplest of which separates the presentation into four categories: loudness, annoyance, pain, and fear. Loudness hyperacusis is defined as the perception of moderately-intense sounds as uncomfortably loud. In annoyance hyperacusis, the chief complaint is that of a negative emotional reaction manifesting as irritability and anxiety. Fear hyperacusis is that which results in avoidance behaviors, and pain hyperacusis can present as a stabbing pain felt in the ear. Hyperacusis is almost exclusively a bilateral phenomenon. In patients with unilateral symptoms, one must consider an alternative cause, such as an acoustic shock leading to tonic tensor tympani syndrome.

        Phonophobia and misophonia present similarly (and indeed the terms are often used synonymously), however, an important distinction must be made between these conditions. Hyperacusis is sound sensitivity arising from within the auditory system, and therefore can be triggered by any generic, low-intensity sound. Contrarily, phonophobia, and misophonia are disorders associated with the limbic system, and there is no abnormality in the peripheral or central auditory system. Phonophobia is a psychiatric disease in which there is a fear of a specific sound, while misophonia is a psychological disease in which a specific sound triggers emotional and physical reactions.

        A full neurotological examination is essential in detecting any associated or underlying causes of hyperacusis, some of which are reversible. Otoscopy and pneumatoscopy are important for assessing the mobility of the tympanic membrane, and cranial nerve examination can reveal facial nerve dysfunction. A detailed history will assess for any underlying psychiatric illness and also establish potential risk factors, including noise exposure and acoustic trauma.

        Evaluation

        The diagnosis of hyperacusis usually involves determining LDL using pure-tone audiometry and using questionnaires to determine disease severity. Many questionnaires exist, including the Geräuschüberempfindlichkeit (GÜF) and the Multiple Activity Scale for Hyperacusis (MASH). However, the most widely used is the hyperacusis questionnaire (HQ). When the result of a test is considered alone, there is no agreed consensus on the diagnostic cut-off values for either the HQ or LDL, however, in combination, the results are more sensitive. Ninety-five percent of patients diagnosed with hyperacusis have LDL ≤ 77 dB (average LDL in non-hyperacusic patients is 100 dB) and an HQ score of ≥ 22. Unfortunately, these cut-offs only inform of the presence or absence of hyperacusis, and not on its impact on a patient’s life. Furthermore, it is important to ensure that the evaluation does not cause undue discomfort for the patient and a subsequent breakdown in clinical rapport.

        Based on the history and physical examination, clinicians may decide to undertake further investigations if an alternative underlying cause is suspected, such as serological tests for Lyme disease or high-resolution computed tomography or magnetic resonance imaging of the brain for those cases with facial paralysis.

        Treatment of Hyperacusis

        Treatment for hyperacusis can broadly be categorized into those which target the physical symptoms, and those which aim to reduce the psychological burden of the condition.

        Cognitive-behavioral therapy (CBT) is one of the most effective components of hyperacusis therapy alongside counseling and education. Through providing patients with the techniques required to manage the emotional reaction to sound, CBT has been shown to increase LDL and reduce hyperacusis severity as assessed by the HQ. Directive counseling uses a similar approach of identifying and discussing repressed behaviors, although much of the literature is directed towards its use in tinnitus.

        Tinnitus retraining therapy (TRT) involves educating the patient about their condition alongside gradual sound enrichment, and its use in hyperacusis is becoming increasingly popular. Prolonged low-level noise exposure has been shown to have a reversing effect on the enhanced neural gain, which is thought to be the underlying mechanism of hyperacusis. Significant improvements in LDL have been seen after 6 months of sound generator therapy. Increasing the mean level of the acoustic environment (i.e. greater auditory stimulation) has been reported to have a beneficial symptomatic effect.

        Surgery may be indicated in select cases, including those refractory to the above treatments or in conductive hyperacusis secondary to superior semi-circular canal dehiscence syndrome. Round and oval window reinforcement is a simple, reversible procedure that has been shown to have a high success rate and a sustainable reduction in symptoms.

        Alternative treatments often are given special attention for chronic pain and may include supplements and vitamins, acupuncture, exercise, yoga, meditation, massage, relaxation therapy, and hypnosis.

        References

         

        The Weber Test – Indications, Contraindications

        The Weber test is a useful, quick, and simple screening test for the evaluation of hearing loss. The test can detect unilateral conductive and sensorineural hearing loss. The outer and middle ear mediate conductive hearing. The inner ear mediates sensorineural hearing. The Weber test is often combined with the Rinne test to detect the location and nature of hearing loss.

        Anatomy and Physiology

        To understand the Weber test, one has to understand the basic anatomy of hearing.

        The ear anatomically consists of the sound-conducting system (outer and middle ear) and sound-transducing system (the cochlea).

        • The outer ear: Pinna and external ear canal
        • The middle ear: Tympanic membrane, ossicular chain (malleus, incus, stapes) and middle ear space
        • The inner ear: Cochlea (organ of hearing), vestibular labyrinth (organ of balance)

        The purpose of the outer ear is to direct sounds onto the tympanic membrane. The sound vibrations are then transmitted through the middle ear via the ossicular chain before it reaches the cochlea. The cochlea plays an important role in transducing these vibrations into nerve impulses via the auditory nerve (vestibulocochlear nerve) which is then delivered along the central pathways to the auditory cortex where it is processed and perceived as sound. This pathway is termed air conduction. However, sound can also be transmitted via bone conduction where vibrations are transmitted via the skull and delivered directly to the cochlea which is buried within the temporal bone.

        Hearing loss may occur due to interruption at any point along these pathways.

        The Weber test, along with its paired Rinne test, is commonly used to distinguish the site and likely cause of hearing loss. Conductive hearing loss is due to problems with the sound-conducting system, while sensorineural hearing loss is due to problems with the sound-transducing system, the auditory nerve or its central pathways. Occasionally, one can get a mixed hearing loss, which is a combination of the 2 hearing loss.

        Indications

        In normal hearing, an individual will hear equally on both sides of the ear.  The Weber test is a test of lateralization and is of most value useful in those with an asymmetrical hearing loss.

        Weber Test Principles

        The inner ear is more sensitive to sound via air conduction than bone conduction (in other words, air conduction is better than bone conduction).

        In the presence of a purely unilateral conductive hearing loss, there is a relative improvement in the ability to hear a bone-conducted sound. This can be explained by the following:

        • Masking effect – The sound heard via the affected ear has less environmental noise reaching the cochlea via air conduction (for example, the environmental noise is masked) as compared to the unaffected ear which receives sounds from both bone conduction and air conduction. Therefore, the affected ear is more sensitive to bone-conducted sound.
        • Occlusion effect Most of the sound transmitted via bone conduction travels through to the cochlea. However, some of the low-frequency sounds dissipate out of the canal. A conductive hearing loss (in other words, when an occlusion is present) will, therefore, prevent external dissipation of these frequencies and lead to increased cochlear stimulation and increased loudness in the affected ear.

        In the presence of sensorineural hearing loss, the sound will be perceived louder in the unaffected ear which has the better cochlear.

        Equipment

        An ideal tuning fork of choice for the Weber test would be one that has a long period of tone decay, in other words, the tone maintains/lasts long after the tuning fork has been struck, and cannot be detected by sense of bone vibration, therefore preventing misinterpretation of the vibration as sound.

        • 512-Hz Tuning Fork – In clinical practice, the 512-Hz tuning fork has traditionally been preferred. At this frequency, it provides the best balance of time of tone decay and tactile vibration. Lower-frequency tuning forks like the 256-Hz tuning fork provide greater tactile vibration. In other words, they are better felt than heard. Higher-frequency tuning forks, for example, the 1024-Hz tuning fork, have a shorter tone decay time.
        • 256-Hz Tuning Fork: An Alternative – The 256-Hz tuning fork, along with 128-Hz tuning fork, is commonly used as part of neurological examination due to their greater tactile vibration characteristic. However, evidence suggests that the 256-Hz provides better reliability when compared to the 512-Hz. ,

        Preparation

        • Ideally, the test should be carried out in a quiet room
        • Verbal consent should be gained prior to performing the test
        • Clear instructions should be given to the patient to avoid misinterpretation of the test

        Technique

        Tuning Fork

        The audiometric tuning fork generally consists of the tines (the U-shaped prongs), the stem, and the footplate.

        Striking the Tuning Fork 

        • Hold the tuning fork by the stem between the thumb and first finger.
        • Strike the tines one-third of the way from the free end of the prong onto a firm but the elastic object (e.g., the clinician’s knee or elbow). This will produce a relatively pure tone.
        • Avoid striking the tines onto a hard surface as this may damage the tuning fork and produce multiple overtones.

        Performing Weber Test 

        • Place the vibrating tuning fork on the vertex (other common sites used are the midline of the forehead, bridge of the nose, and chin), equidistant from both ears. These vibrations will be conducted through the skull and reach the cochlea.
        • Ask the patient whether it is heard loudest in either one side or the midline (e.g., “Is the sound louder in your right ear, left ear, or the middle?”)

        Interpretation

        Normal Hearing

        • Weber test does not demonstrate lateralization: In a normal subject, the sound should be heard in the middle and equally on both sides.
        • Rinne test: Normal/positive in both ears (AC greater than BC)

        Unilateral Sensorineural Hearing Loss

        • Weber test lateralizes to the unaffected ear, in other words, it is heard louder in the better ear.
        • Rinne test: Normal/positive on the affected ear (AC greater than BC); normal/positive on the unaffected ear (AC greater than BC)

        Note: an abnormal/negative response on the affected ear (BC greater than AC) can also occur in a severe sensorineural hearing loss, also called a dead ear. This is termed a “false negative.” Rinne “true negative” only occurs if there is a conductive hearing loss element. However, when testing a dead ear, the bone conduction is perceived to be heard louder than air conduction due to cross-over of bone conduction detected by the opposite normal-functioning cochlear, resulting in a Rinne false negative.

        Unilateral Conductive Hearing Loss

        • Weber test lateralizes to the affected ear, in other words, it is heard louder in the poorer ear.
        • Rinne test: Abnormal/negative on the affected ear (BC greater than AC); normal/positive on the unaffected ear (AC greater than BC)

        Symmetrical Conductive Hearing Loss

        • Weber test does not demonstrate lateralization
        • Rinne test: Abnormal/negative on the affected ear (BC greater than AC)

        Complications

        The Rinne test is the complement for the Weber test. They are screening tests and do not replace formal audiometry. It is important to note that further examinations and investigations such as otoscopy, audiometry, tympanometry, and imaging may be required to correctly diagnose the cause of the hearing loss and allow appropriate management.

        Clinical Significance

        Clinical Use

        • In the primary care setting, it is useful to use the Weber test along with Rinne test to help the clinician differentiate between conductive hearing loss with a sensorineural hearing loss. This will guide the clinician to the need for further examination, investigation, and management.
        • In the post-operative setting, the test is commonly used as a quick bedside test for examining a complication of a dead ear (complete sensorineural hearing loss).
        • The Weber and Rinne tuning fork tests can be used to confirm audiometric findings, particularly when the audiogram is not consistent with clinical findings
        • In the assessment of a patient with bilateral conductive hearing loss, the Weber test is a quick and useful test for the otorhinolaryngology (ENT) surgeon to help determine which side of the ear to operate on first. Usually, the ear with the more significant conductive hearing loss is preferred.

        Possible Causes (Non-Exhaustive) of Hearing Loss

        Conductive Hearing Loss

        Outer Ear Causes

        • Impacted wax
        • Infection affecting the outer ear (otitis externa)
        • A foreign body within the external ear canal
        • Squamous cell carcinoma
        • Congenital microtia

        Middle Ear Causes

        • Tympanic membrane trauma
        • Infection affecting the middle ear (acute otitis media)
        • Glue ear (otitis media with effusion)
        • Otosclerosis
        • Cholesteatoma
        • Congenital malformation
        • Temporal bone trauma

        Sensorineural Hearing Loss

        Inner Ear Causes

        • Hereditary hearing loss
        • Presbycusis
        • Labyrinthitis
        • Meniere disease
        • Viral cochleitis
        • Vascular insult
        • Autoimmune conditions
        • Noise exposure
        • Vestibular schwannoma
        • Ototoxic drugs
        • Trauma

        References

        Eustachian Tube Dysfunction (ETD) – Symptoms, Treatment

        Eustachian tube dysfunction (ETD) is the inability of the Eustachian tube to adequately perform these functions. However, the precise function and mechanisms of the Eustachian tube and the underlying causes of dysfunction are complex and not fully understood. From a diagnostic perspective, ETD is also poorly defined.

        Eustachian tube dysfunction may occur when the mucosal lining of the tube is swollen, or does not open or close properly. If the tube is dysfunctional, symptoms such as muffled hearing, pain, tinnitus, reduced hearing, a feeling of fullness in the ear or problems with balance may occur. Long-term ETD has been associated with damage to the middle ear and the eardrum. Complications include otitis media with effusion (glue ear), middle ear atelectasis (retraction of the eardrum), and chronic otitis media., However, the role of the Eustachian tube in the development of other middle ear conditions is not fully understood. Middle ear ventilation is increasingly seen as being associated with other mechanisms, such as those relating to gaseous exchange through the middle ear mucosa., Therefore, it may be that problems with middle ear ventilation (and therefore symptoms and signs previously attributed to ETD) may not all be associated with problems with or dysfunction of the Eustachian tube. Abnormal patency (patulous Eustachian tube) is a separate condition, in which the Eustachian tube remains intermittently open, causing an echoing sound of the person’s own heartbeat, breathing, and speech.

        Causes of Eustachian Tube Dysfunction

        The lining of the Eustachian tube can become swollen and the Eustachian tube can become dysfunctional following the onset of an infectious or inflammatory condition such as an upper respiratory tract infection, allergic rhinitis or rhinosinusitis, leading to difficulties in pressure equalization, discomfort and other symptoms., Nasal septal deviation has also been associated with symptoms of ETD; this is based on some studies which suggested that, in patients who were unable to equalize pressure during scuba training or submarine service, submucous resection of the nasal septum resolved apparent ETD symptoms. Dysfunction of the Eustachian tube may also be related to failure of the muscles associated with Eustachian tube opening. Extrinsic compression of the Eustachian tube potentially due to inflammation or enlargement of the adenoids, tumour or trauma may also result in ETD,, although these conditions and their management are beyond the scope of this review. The incidence of ETD is disproportionately high in patients with cleft palate who may be considered a discrete clinical population. Other potential risk factors include tobacco smoke, reflux and radiation exposure. There appears to be no association with sex, although it has been suggested that ethnicity and geographical factors (such as proximity to the poles) are associated with increased incidence and prevalence.

        There are limited data on ETD prevalence and incidence, which may reflect the lack of consensus regarding how ETD is defined. A UK national study of hearing reported that 0.9% of the 2708 adults assessed (from an initial sampling of 48,313) were considered to have ETD, based on otoscopic examination and audiological assessment. However, this may be an underestimate; a recent study stated that most otolaryngologists encounter a much higher incidence of the condition in their practices.

        ETD happens if the Eustachian tube becomes blocked, if the lining of the tube swells, or if the tube does not open fully to allow air to travel to the middle ear.

        Common colds and other nasal, sinus, ear or throat infections

        • By far the most common cause of ETD is the common cold (upper respiratory tract infection).
        • The blocked nose or thick mucus that develops during a cold or other infection, may block the Eustachian tube.
        • An infection may also cause the lining of the Eustachian tube to become inflamed and swollen.
        • Most people have had a cold when they haven’t been able to hear so well – this is due to ETD.
        • Symptoms may last for a week or so (sometimes longer) after the other symptoms of the infection have gone. This is because the trapped mucus and swelling can take a while to clear even when the germ causing the infection has gone.
        • Sometimes the infection that sets it off is very mild but even so, in some people, ETD can still develop.

        Glue ear

        • Glue ear is a condition where the middle ear fills with glue-like liquid.
        • It is a common condition in children.
        • The Eustachian tube becomes congested and prevents the free flow of air into the middle ear, causing the difference in air pressure mentioned above. The eardrum becomes tight, reducing its ability to vibrate. This results in dulled hearing. The situation is made worse by the glue-like fluid damping down the vibrations of the drum even further.
        • It clears by itself in most cases but some children need an operation to solve the problem.

        Allergies

        Allergies that affect the nose, such as persistent rhinitis and hay fever, can cause extra mucus and inflammation in and around the Eustachian tube and lead to having symptoms for several months.

        Smoking

        • Smoking can stop the tiny hairs that line the Eustachian tube from working.
        • Smoking can also cause tissues at the back of the nose and throat (including the adenoids) to enlarge, blocking the Eustachian tube.
        • If you smoke and are having problems with long-term (chronic) ETD you should try to stop smoking.

        Blockages

        • Anything that causes a blockage to the Eustachian tube can cause muffled hearing – for example, enlarged adenoids in children.
        • Rarely, a tumor behind the eardrum or at the back of the nose (the nasopharynx) can mimic the symptoms of ETD. These types of tumors are very uncommon and usually cause other symptoms in addition to ETD, such as headache, a hoarse voice, and a constantly blocked nose.

        For most people who experience ETD, it settles by itself within a couple of weeks. But in some people, it seems to go on for a long time – many months. It is not known why some people are more prone to this happening than others.

        Some common causes of long-term (chronic) ETD:

        • Chronic sinusitis – up to half of people with chronic ETD.
        • Persistent rhinitis.
        • Smoking-related changes to the nose and throat.

        In around 1 in 5 people who have long-term ETD, no cause is found. There is no evidence that there is a genetic cause and it doesn’t appear to run in families.

        Symptoms of Eustachian Tube Dysfunction

        Symptoms of ETD may include:

        • fullness in the ears
        • feeling like your ears are “plugged”
        • changes to your hearing
        • ringing in the ear, also known as tinnitus
        • clicking or popping sounds
        • ticklish feelings in the ears
        • pain

        Other symptoms that may also develop

        Ear pain

        • Due to the eardrum being tense and stretched.
        • Pain may come and go.
        • Rarely causes constant ear pain. If your ear is hurting all the time, it may be due to a different cause and you should see a doctor.
        • Ringing or buzzing in the ear (tinnitus):
          • This is as well as muffled hearing.
          • ETD doesn’t cause tinnitus on its own.
        • Dizziness:
          • Mild dizziness (vertigo) may occur.

        Diagnosis of Eustachian Tube Dysfunction

        There are no comprehensive guidelines on diagnosis of ETD. Diagnosis is generally based on medical history and clinical examination to identify potential underlying causes. The UK national survey defined ETD as the presence of a normal or abnormal but intact tympanic membrane with a middle ear pressure of < –100  mmH2O and an air–bone gap of ≥ 15 decibels (dB). The criteria were used for a presumptive diagnosis of ETD. The authors noted that it was a relatively non-specific category, which may include patients in the early or late stages of an episode of otitis media with effusion. However, the presence of either of these signs is not usually considered to be either necessary or sufficient for the diagnosis of ETD in clinical practice; while negative middle ear pressure often indicates ETD, patients with ETD may have normal middle ear pressure and those with negative middle ear pressure may be asymptomatic. Moreover, while an intact eardrum was a requirement of the survey criteria, several investigators include patients with perforated eardrums.

        Although not used in the survey, symptoms of dysfunction are usually a necessary condition for diagnosis in clinical practice. Common diagnostic factors include the inability to ‘clear’ or ‘pop’ the ear with changes in barometric pressure, together with other patient-reported symptoms (e.g. aural fullness, pain, muffled hearing). There are a number of tests that are used to inform diagnosis: otoscopy, tympanometry, and nasal endoscopy are initial options in a secondary care setting. Evidence on the predictive value of Eustachian tube function tests is limited, and several tests may be needed for a more reliable and comprehensive assessment of the Eustachian tube function. Currently, there is no commonly used patient-reported outcome measure. A scale for the assessment of ETD [the 7-item Eustachian Tube Dysfunction Questionnaire (ETDQ-7)] was tested for validity; this is a questionnaire addressing a range of symptoms associated with ETD, which is completed by the patient. The data available on reliability were based on a relatively small number of patients (n = 50) and controls (n = 25), but the test discriminated patients and controls and exhibited good test-retest reliability. However, this represents a recent development and it is not yet widely used. Another relevant scale which is also completed by patients, the 22-item Sinonasal Outcome Test (SNOT-22), has been used to assess symptoms of the related condition of rhinosinusitis.

        The lack of clearly defined diagnostic criteria, together with the uncertainty relating to the etiology of ETD, presents a key challenge in undertaking a review of interventions for its treatment. Lack of consensus on the necessary features for diagnosis, including clinical history, requires additional awareness of the risk of error and bias in the selection of studies, as well as increasing the probability of clinical heterogeneity in the included studies.

        Treatment of Eustachian Tube Dysfunction

        Although ETD symptoms are common, they are often mild and generally resolve after a few days. Simple actions such as swallowing, yawning, chewing or forced exhalation against a closed mouth and nose can help to equalise pressure in the middle ear and resolve symptoms. However, symptoms sometimes persist, in which case treatment may be desirable. There are a number of non-surgical and surgical treatment options aimed at improving Eustachian tube function, but there is limited consensus about management.

        Non-surgical

        Non-surgical management strategies include:

        • Active observation, which involves monitoring the symptoms to determine whether or not they naturally resolve.
        • Supportive care, which includes advice about self-management such as to swallow, yawn, or chew to help equalize the pressure in the middle ear.
        • Pressure equalization methods, which is a technique whereby the Eustachian tube is reopened by raising the pressure in the nose. This can be achieved in several ways, including forced exhalation against a closed mouth and nose (Valsalva maneuver). Other methods include blowing up a balloon through each nostril, using an anesthetic mask or the use of mechanical devices., The aim is to introduce air into the middle ear, via the Eustachian tube, equalizing the pressures and allowing better fluid drainage.
        • Nasal douching, in which the nasal cavity is washed with a saline solution to flush out excess mucus and debris from the nose and sinuses.
        • Decongestants, antihistamines, nasal or oral corticosteroids are aimed at reducing nasal congestion and/or inflammation of the lining of the Eustachian tube.
        • Antibiotics, for the treatment of rhinosinusitis.
        • Simethicone, which is currently being investigated in adults to assess whether or not it can help to break up bubbles that may block the opening of the Eustachian tube in the back of the nose during a cold, allowing air to pass between the nose and middle ear. This is not currently a management option used in the UK.

        Surgical

        We understand that, currently, the main surgical treatment in the UK is a pressure-equalizing tube (also known as tympanostomy tube, ventilation tube, or grommet) which is inserted into the eardrum through a small incision. Pressure equalizing tubes typically extrude after 6–9 months. Long-acting tubes are occasionally used, although these may be prone to crusting, infection, obstruction, and permanent tympanic membrane perforation. This may be performed under either general or local anesthesia. Newer surgical methods which are mainly used in the context of research include:

        • Balloon dilatation (dilatation) of the Eustachian tube, a procedure which aims to dilate the Eustachian tube and improve its function. It consists of introducing a balloon catheter into the Eustachian tube through the nose, under transnasal endoscopic vision. The balloon is filled with saline. The pressure is maintained for approximately 2 minutes, following which the balloon is emptied and removed. The procedure has been performed experimentally under local and general anesthesia.
        • Transtibial application of fluids, an emerging approach for the application of fluids to the middle ear via the Eustachian tube. The transtibial application approach involves placing a nasal microendoscope within the Eustachian tube under local anesthesia via its nasopharyngeal opening. Subsequently, fluids are applied through an additional working channel after microendoscopic evaluation.
        • Eustachian tuboplasty, an emerging treatment in which a laser or rotary cutting tool is used to strip away enlarged mucous membranes and cartilage to clear obstruction to the Eustachian tube. Tuboplasty has been used in patients with chronic ETD as an alternative to pressure equalizing tubes which may have extruded on numerous occasions., The intervention has also been used for middle ear atelectasis or serous effusion.

        There is no consensus on indications for treatment, or on the optimal timing of the interventions. Surgical interventions are generally (though not exclusively) used where ETD is resistant to other interventions. A step-up approach is usually adopted, from primary to secondary and tertiary care settings. Treatment choice is based on etiology, severity and persistence of symptoms, as well as the degree of invasiveness of the treatment and surgical preference.

        References

        Meniere’s Disease – Causes, Symptoms, Diagnosis, Treatment

        Meniere’s disease is a disease of the inner ear, characterized by the clinical triad of recurrent vertigo, fluctuating sensorineural hearing loss, and tinnitus.[] The relapsing nature of the disease may significantly affect the patients’ quality of life, especially during periods of acute symptomatology.[,] Vertigo mainly influences the physical dimension, while tinnitus and hearing loss influence the psychosocial dimension of patients’ lives.[]

        Meniere disease is a disorder of the inner ear characterized by hearing loss, tinnitus, and vertigo. In most cases, it is slowly progressive and has a significant impact on the social functioning of the individual affected.

        The current diagnostic criteria defined by the Barany society by Lopez-Escamez can help differentiate between a probable and a definite Meniere’s disease.

        Patients with a definite Meniere disease according to the Barany Society have

        • Two or more spontaneous episodes of vertigo with each lasting 20 minutes to 12 hours
        • Audiometrically documented low- to medium- frequency sensorineural hearing loss in one ear, defining and locating to the affected ear on in at least one instance prior, during or after one of the episodes of vertigo
        • Fluctuating aural symptoms (fullness, hearing, tinnitus) located in the affected ear
        • Not better accounted for by any other vestibular diagnosis

        Probable Meniere disease can include the following clinical findings

        • Two or more episodes of dizziness or vertigo, each lasting 20 minutes to 24 hours
        • Fluctuating aural symptoms (fullness, hearing, or tinnitus) in the affected ear
        • The condition is better explained by another vestibular diagnosis

        Stages of Meniere’s Disease

        Meniere’s disease commonly affects people in various stages, with symptoms developing over time.

        • Early stage – During this time, a person will experience sudden and often out-of-the-blue episodes of vertigo that last anywhere from 20 minutes to an entire day. An person’s ear may feel blocked or full, and they may have some hearing loss, which typically goes away after the episode fades. It is also common to feel the effects of tinnitus.
        • Middle stage – Symptoms of vertigo tend to become less severe during this stage, while hearing loss and tinnitus will increase in severity. Many people will also experience long-term remission (the disease goes away) that can last several months.
        • Late stage – During the late stages of Meniere’s disease, patients will not suffer from vertigo as often, and some people will be relieved from it forever. However, tinnitus and hearing loss will likely get progressively worse, and people will likely experience unsteady balance regularly. Most people at this stage feel unstable in dark conditions, for example.

        Causes of Meniere’s Disease

        Studies of the temporal bone revealed endolymphatic accumulation in the cochlea and the vestibular organ in patients with Meniere disease. Current research links endolymphatic hydrops to a hearing loss of >40dB. Vertigo may or may not be associated. Therefore endolymphatic hydrops is not entirely specific for Meniere disease and can be found in cases of idiopathic sensorineural hearing loss.

        • The exact etiology of Meniere disease remains unclear – Different theories exist, but genetic and environmental factors play a role. The relation to common comorbidities remains elusive.
        • Migraine – Migraine occurs more often in patients diagnosed with Meniere disease although there might be an overlap between basilar migraine wrongly diagnosed as Meniere disease.
        • Autoimmune Diseases – Several autoimmune diseases are associated with Meniere disease namely rheumatoid arthritis, systemic lupus erythematosus and ankylosing spondylitis.

        Potential causes or triggers of Meniere’s disease include:

        • Head injury
        • Infection to the inner or middle ear
        • Allergies
        • Alcohol use
        • Stress
        • Side effects of certain medications
        • Smoking
        • Stress or anxiety
        • Fatigue
        • Family history of the disease
        • Respiratory infection
        • Recent viral illness
        • Abnormal immune response
        • Migraines

        Symptoms of Meniere’s Disease

        Signs and symptoms of Meniere’s disease include:

        • Recurring episodes of vertigo – You have a spinning sensation that starts and stops spontaneously. Episodes of vertigo occur without warning and usually last 20 minutes to several hours, but not more than 24 hours. Severe vertigo can cause nausea.
        • Hearing loss – Hearing loss in Meniere’s disease may come and go, particularly early on. Eventually, most people have some permanent hearing loss.
        • Ringing in the ear (tinnitus) – Tinnitus is the perception of a ringing, buzzing, roaring, whistling or hissing sound in your ear.
        • Feeling of fullness in the ear – People with Meniere’s disease often feel pressure in an affected ear (aural fullness).

        A person with Ménière’s disease may experience any or all of these symptoms

        • Vertigo and dizziness, often so severe that it is temporarily disabling. There may be a sense that the room is spinning, twisting, or rocking. Balance can be severely affected. The sensation can last from a few minutes to several hours. After vertigo goes away, a sense of imbalance can remain for hours or days.
        • Nausea and vomiting during an episode of vertigo.
        • A feeling of pressure or fullness in the affected ear.
        • Ringing, buzzing, or other noises in the affected ear (tinnitus). This ringing is often low-pitched and may distort normal sounds.
        • Hearing loss that comes and goes but gets progressively worse over time. Low-pitched hearing often is affected earlier in the disease.

        Paying attention to these warning symptoms can allow a person to move to a safe or more comfortable situation before an attack.

        • balance disturbance
        • dizziness, lightheadedness
        • headache, increased ear pressure
        • hearing loss or tinnitus increase
        • sound sensitivity
        • vague feeling of uneasiness

        During an attack of early-stage Ménière’s disease, symptoms include:

        • spontaneous, violent vertigo
        • fluctuating hearing loss
        • ear fullness (aural fullness) and/or tinnitus

        In addition to the above main symptoms, attacks can also include:

        • anxiety, fear
        • diarrhea
        • blurry vision or eye jerking
        • nausea and vomiting
        • cold sweat, palpitations or rapid pulse
        • trembling

        Following the attack, a period of extreme fatigue or exhaustion often occurs, prompting the need for hours of sleep.

        The periods between attacks are symptom free for some people and symptomatic for others. Many symptoms have been reported after and between attacks:

        • anger, anxiety, fear, worry
        • appetite change
        • clumsiness
        • concentration difficulty, distractibility, tendency to grope for words
        • diarrhea
        • fatigue, malaise, sleepiness
        • headache, heavy head sensation
        • lightheadedness (faintness)
        • loss of self-confidence and self-reliance
        • nausea, queasiness, motion sickness
        • neck ache or stiff neck
        • palpitations or rapid pulse, cold sweat
        • sound distortion and sensitivity
        • unsteadiness (sudden falls, staggering or stumbling, difficulty turning or walking in poorly lit areas, tendency to look down or to grope for stable handholds)
        • vision difficulties (problems with blurring, bouncing, depth perception, glare intensification, focusing, watching movement; difficulty looking through lenses such as binoculars or cameras)
        • vomiting

        Diagnosis of Meniere’s Disease

        History and Physical

        In the emergency room or in the general practice the physician will differentiate between the vertigo of central, peripheral, and cardiovascular cause. Red flags for a central origin of vertigo, according to Harcourt et al., are neurological symptoms or signs, acute deafness, new type or onset of headache, or vertical torsional rotatory nystagmus.If Meniere disease is suspected, the patient should be questioned about the character of vertigo, hearing loss, and earlier episodes. A full otologic history is part of the clinical investigation.

        If Meniere disease is suspected, one should perform a full otologic examination, facial nerve testing, and assessment of nystagmus with Frenzel goggles, Rinne, and Weber tests. 

        • Rinne and Weber – Will show sensorineural hearing loss in acute Meniere disease or advanced disease.
        • Frenzel goggles – May show horizontal nystagmus with a fast-beating component away from the affected vestibular organ in the acute setting.
        • Head impulse testing (HIT) – In contrast to other peripheral vestibular disorders, this test has a low sensitivity in Meniere disease.

        Hearing assessment

        • A hearing test (audiometry) assesses how well you detect sounds at different pitches and volumes and how well you distinguish between similar-sounding words. People with Meniere’s disease typically have problems hearing low frequencies or combined high and low frequencies with normal hearing in the midrange frequencies.

        Balance assessment

        • Between episodes of vertigo, the sense of balance returns to normal for most people with Meniere’s disease. But you might have some ongoing balance problems.

        Tests that assess function of the inner ear include:

        • Videonystagmography (VNG) – This test evaluates balance function by assessing eye movement. Balance-related sensors in the inner ear are linked to muscles that control eye movement. This connection enables you to move your head while keeping your eyes focused on a point.
        • Rotary-chair testing – Like a VNG, this measures inner ear function based on eye movement. You sit in a computer-controlled rotating chair, which stimulates your inner ear.
        • Vestibular-evoked myogenic potentials (VEMP) testing – This test shows promise for not only diagnosing but also monitoring Meniere’s disease. It shows characteristic changes in the affected ears of people with Meniere’s disease.
        • Posturography – This computerized test reveals which part of the balance system — vision, inner ear function, or sensations from the skin, muscles, tendons, and joints — you rely on the most and which parts may cause problems. While wearing a safety harness, you stand in bare feet on a platform and keep your balance under various conditions.
        • Video head impulse test (vHIT) – This newer test uses video to measure eye reactions to the abrupt movement. While you focus on a point, your head is turned quickly and unpredictably. If your eyes move off the target when your head is turned, you have an abnormal reflex.
        • Electrocochleography (ECoG) – This test looks at the inner ear in response to sounds. It might help to determine if there is an abnormal buildup of fluid in the inner ear, but isn’t specific for Meniere’s disease.

        Tests that may be used to aid in diagnosis include

        • A hearing test, also called audiometry — This simple test can tell whether you are experiencing hearing problems, how much hearing you have lost, and what type of hearing problems you have. People with Ménière’s disease have a particular type of damage to nerves important for normal hearing, which may make it difficult to tell the difference between similar-sounding words such as “boat” and “moat.”
        • Computed tomography (CT) – or magnetic resonance imaging (MRI), scans that allow physicians to see the brain, middle ear, and other structures inside the head — These scans can check for tumors and other problems that can cause symptoms that are similar to Ménière’s.
        • Electronystagmography or rotational testing — These tests use the nerve connection between the ears and the eyes to examine your body’s balance system. In a darkened room, electrodes are placed near the eyes. Then, the ear canal is stimulated with water, air or changes in position. The electrodes measure how the inner ear responds. In Ménière’s disease, your doctor can spot typical changes caused by the buildup of fluid in the inner ear.

        Evaluation

        • Audiometric evaluation –  is mandatory in all patients with Meniere disease. Fluctuating low frequency unilateral sensorineural hearing loss is characteristic of the disease. The hearing loss can progress to all frequencies. Tinnitus is common and ipsilateral.
        • BERA (brainstem evoked response audiometry) –  is sufficient. There is no need to perform imaging in the acute setting but may be done within a few weeks after onset of symptoms. High-resolution MRI imaging may directly show endolymphatic hydrops in the affected organs. More research is underway to show if this is of clinical use.
        • Vestibular (caloric) function testing – may show a significantly under-functioning affected organ in 42% to 74% and a full loss of function in 6% to 11%.
        • ECochG – has also been widely used in the diagnosis of Meniere’s disease. During the EcoChG, a needle electrode is placed either through the tympanic membrane on the promontory, or on the tympanic membrane, or simply in the ear canal.
        • Electronystagmogram. This evaluates your balance. You will be placed in a darkened room and have your eye movements measured as cool and warm air blows through your ear canal.
        • Vestibular-evoked myogenic potential (VEMP) – This measures your reaction to sudden, loud noises.
        • Video head impulse test (VHIT) – This uses video images to see how well you can focus and how your eyes respond to sudden movement.
        • Auditory brainstem response test (ABR) – With this test, you wear headphones, and a computer measures your brain waves as you respond to different sounds. It’s typically only used for people who can’t have other types of hearing tests (like babies) or who can’t have imaging tests.
        • Additional imaging tests. Your doctor also might recommend an MRI or CT scan to rule out the possibility that something other than Meniere’s is causing your symptoms.

        The components measured are

        • a) cochlear microphonics,
        • b) summating potentials (SP), and
        • c) action potentials (AP). The cochlear microphonics and the summating potentials reflect the cochlear bioelectric activity, while the action potentials reflect the activity of distal afferent fibers of the 8th nerve. In ECoChG, we determine the amplitude of the SP and the AP from a common baseline.

        Treatment of Meniere’s Disease

        Different treatment options for Meniere disease exist with substantial variability between countries. None of the treatment options cure the disease. As many treatments have a significant impact on the functioning of surrounding structures, one should start with non-invasive approaches with the fewest possible side effects and proceed to more invasive steps.

        • Sodium restriction diet – Low-level evidence suggests that restricting sodium intake may help to prevent Meniere attacks.
        • Betahistine –  Substantial disagreement in the medical community about the use of betahistine exists. A Cochrane review found low-level evidence to support the use of betahistine with substantial variability between studies. Medical therapy in many medical centers often starts with betahistine orally.
        • Intratympanic steroid injections – may reduce the number of vertigo attacks in patients with Meniere disease.
        • Intratympanic gentamycin injections – Gentamycin has strong ablative properties towards vestibular cells. Side effects are a sensorineural hearing loss because of a certain amount of toxicity towards cochlear cells.
        • Vestibular nerve section or labyrinthectomy – Nerve section is a therapeutic option in patients who failed the conservative treatment options and labyrinthectomy when surgical options failed. Labyrinthectomy leads to a complete hearing loss on the affected side.
        • Anti-vertigo medications – such as meclizine or betahistine, to relieve or prevent vertigo and dizziness
        • Anti-nausea medications – such as prochlorperazine, to relieve nausea and vomiting
        • Diuretics, such as hydrochlorothiazide (HydroDIURIL) – reduce the amount of fluid that builds in the inner ear.
        • Meclizine, chewable – Dose ranges from 12.5 twice/day to 50 mg three times/day. This medication is over the counter. No prescription is necessary.
        • Lorazepam (Ativan) 0.5 mg – The usual dose is twice/day or both at the same time at the onset. This medication is effective even if it is not swallowed (i.e. you can just suck on it). Tiredness is expected.
        • Promethazine (Phenergan) – orally (12.5) or rectal suppository (25 mg). The usual dose is once every 12 hours as needed for vomiting.
        • Prochlorperazine (Compazine, orally or suppository) – Usual dose is 5-10 mg every 12 hours as needed for vomiting.
        • Ondansetron (orally or sublingual) – The usual dose is 8mg q 12 hrs for vomiting. This medication formerly was very expensive, but now it can be obtained at reasonable prices at places such as Costco. Although Ondansetron isn’t as strong as Phenergan or Compazine and doesn’t always work, it also doesn’t have many side effects either. One can certainly work and drive after taking nearly any dose of ondansetron. The same cannot always be said for meclizine, lorazepam, and clonazepam, Phenergan, or Compazine.
        • Dexamethasone (Decadron) – 4 mg orally for 4-7 days. Or a Medrol dose packs this convenient, rapid, but not very effective treatment is gradually being replaced by steroid injections through the eardrum. It is usually an “add-on at the time of a physician’s visit for persistent symptoms. Being hyper is the most common side effect. Often people feel that they don’t need sleep and do a lot of cleaning.
        • Diet – People with MD are often advised to reduce their salt intake.[rx][rx] Reducing salt intake, however, has not been well studied.[rx] Based on the assumption that MD is similar in nature to a migraine, some advise eliminating “migraine triggers” like caffeine. However, the evidence for this is weak.[rx] There is no high-quality evidence that changing diet by restricting salt, caffeine or alcohol improves symptoms.[rx]
        • Physical therapy – While the use of physical therapy early after the onset of MD is probably not useful due to the fluctuating disease course, physical therapy to help to retrain of the balance system appears to be useful to reduce both subjective and objective deficits in balance over the longer term.[rx][rx]
        • Vestibular rehabilitation therapy – VRT is an exercise program that retrains your brain to use other senses, such as your vision, to help with your balance.
        • Positive pressure therapy (Meniett device) – This approach uses a device to apply pressure to your ear canal through a tube. This improves how fluid moves through your ear. You can do these treatments at home.
        • Counseling – The psychological distress caused by vertigo and hearing loss may worsen the condition in some people.[rx] Counseling may be useful to manage the distress,[rx] as may education and relaxation techniques.[rx]

        MEDICATIONS USED BETWEEN ATTACKS (also see flowchart below)

        Diuretics — those in common use all tend to be a combination of a thiazide (that is potassium decreasing) and a sodium channel blocker (e.g. triamterene or amiloride). The combinations have the advantage that they may not require potassium supplementation.

        • Triamterine/HCTZ (Dyazide or Maxide).
        • Moduretic(amiloride/HCTZ)
        • Acetazolamide

        Diuretics that do not contain sulfa (Ponka, 2006)

        • Amiloride
        • Ethacrynic acid
        • Spironolactone
        • Triamterene

        Note that we have four chemical groups of diuretics here sodium channel blockers (triamterene, amiloride), loop diuretics (ethacrynic acid), carbonic anhydrase inhibitors (acetazolamide), and aldosterone antagonists (spironolactone).

        When there is a sulfa allergy, one may try amiloride by itself or ethacrynic acid. Loop diuretics such as Edecrin should be used in low doses and with caution because they are ototoxic. Note that the diuretics listed are mainly ones that increase serum potassium. If it is true that the positive effect in Meniere’s of diuretics is to increase aldosterone as has been suggested by several Japanese authors, spironolactone, as well as eplerenone, would be bad choices as they are aldosterone antagonists.

        Vestibular Suppressants

        Benzodiazepines – these drugs have fallen out of favor because of their addictive properties.

        • Clonazepam(Klonopin) 0.5 mg twice a day or as needed
        • lorazepam (Ativan) 0.5mg twice a day or as needed
        • diazepam (Valium) 2 mg twice a day or as needed

        Antihistamines

        • meclizine (Antivert, Bonine, Dramamine non-drowsy) 12.5 mg to 25 mg as needed up to 3-4 times/day
        • diphenhydramine (Benadryl)

        Note that antihistamines that do not cross into the brain are not used because they don’t work — i.e. loratadine, cetirizine, fexofenadine.

        Calcium Channel Blockers – these drugs are rarely used as well. They are more commonly used for migraines.

        • Verapamil  120-240 mg. Sustained-release should be used. Watch out for drug interactions.
        • Nimodipine
        • Cinnarizine (not available in the USA)
        • Flunarizine (not available in the USA)

        Steroids (commonly for severe bouts) – commonly used, the evidence is not strong for efficacy

        • Dexamethasone
        • Prednisone
        • Methylprednisolone (usually in a self-tapering “dose pack”).

        Immune suppressants (rarely used, see AIED)

        • Methotrexate (very rarely)
        • Steroids (see above)
        • Enbrel (injectable drug), Humira (injectable)

        Agents that are controversial

        • Serc (betahistine) – commonly used, maybe placebo, but often worth trying. The usual dose is 16 mg twice/day but more can be used too.
        • Antifungals such as Mycostatin (Nystatin) – Evidence is weak, and no rationale. (Leong et al, 2014)
        • Histamine injections – (irrational treatment as histamine is broken down rapidly in the body).
        • Homeopathic treatments – such as VertigoHeel. As is the case with all homeopathic treatments, VertigoHeel is a placebo.
        • Antiviral therapy – (such as acyclovir, no evidence for effectiveness)
        • Intratympanic dexamethasone or other steroids – (becoming more common, reasonable evidence for temporary effectiveness, no rationale for a long term effect)

        What can be done to reduce the frequency and severity of Meniere’s disease attacks (i.e. prevention)?

        The purpose of treatment between attacks is to prevent or reduce the number of episodes and to decrease the chances of further hearing loss and damage to the vestibular system. Permanent tinnitus (ringing in the ears), constant imbalance, or a progressive hearing loss may be the consequence of long-term Meniere’s disease. Hearing aids may be necessary.

        Standard medical treatments

        • The hydrops diet regimen – will probably be recommended. This is an important part of treatment for virtually all patients with Meniere’s disease. STRICT adherence to this dietary regimen will result in stabilization in most patients.
        • Between attacks, diuretic medication – may be prescribed to help regulate the fluid pressure in the inner ear, thereby reducing the severity and frequency of the Meniere’s episodes. Dyazide (a combination of triamterene and hydrochlorothiazide) is the most common medication for this purpose, and others are listed above.
        • Vestibular suppressants such as Antivert (meclizine) – or Clonazepam, and anti-emetics (e.g. Phenergan or ondansetron) are used on an as-needed basis.

        Not so standard treatments

        • Verapamil (typical dose: 120 SR) – sometimes reduces the frequency of attacks. Nimodipine and Flunarizine have also been used. These medications are all calcium channel blockers. The evidence is not as good that these medications work. Because Menieres and Migraine are very often combined, this medication’s main role may be to treat the migraine associated vertigo that can be confused with or accompany Meniere’s disease. This medication is especially logical if the dizzy attacks are associated with headaches. There have been recent reports that other migraine medications are useful in patients who have failed diuretic treatment.
        • Some physicians prescribe Histamine injections – Most physicians in the USA consider this treatment to be ineffective.
        • Prednisone or other steroids (e.g. Decadron) – are occasionally helpful in short bursts. We would most often use these when considering a destructive treatment.
        • Medications – that do not have much of a track record that can be tried under the supervision of your doctor. There also some unusual medications which are either considered “alternative” or which are available only outside the US which might be worth considering.

        What the author recommends in his practice in Chicago for medical prevention of Meniere’s. These drugs are administered to most of his patients, generally in the following sequence:

        • Low sodium (2000 mg) diet (1-month trial)
        • A salt-wasting diuretic such as dyazide (1-month trial)
        • Betahistine (2-week trial, often combined with verapamil)
        • Verapamil 120 SR (one-month trial dyazide is stopped) – Verapamil is essentially a migraine prevention medication.
        • These are combined with symptomatic drugs such as meclizine, benzodiazepines, and antiemetics, to be taken during attacks.

        Surgery

        If vertigo attacks associated with Meniere’s disease are severe and debilitating and other treatments don’t help, surgery might be an option. Procedures include

        • Endolymphatic sac procedure – The endolymphatic sac plays a role in regulating inner ear fluid levels. During the procedure, the endolymphatic sac is decompressed, which can alleviate excess fluid levels. In some cases, this procedure is coupled with the placement of a shunt, a tube that drains excess fluid from your inner ear.
        • Labyrinthectomy – With this procedure, the surgeon removes the balance portion of the inner ear, thereby removing both balance and hearing function from the affected ear. This procedure is performed only if you already have near-total or total hearing loss in your affected ear.
        • Vestibular nerve section – This procedure involves cutting the nerve that connects balance and movement sensors in your inner ear to the brain (vestibular nerve). This procedure usually corrects problems with vertigo while attempting to preserve hearing in the affected ear. It requires general anesthesia and an overnight hospital stay.
         References

        What Is Tinnitus? – Causes, Symptoms, Treatment

        What Is Tinnitus?/Tinnitus is defined as a sound a person hears that is generated by the body, rather than by outside source. Most tinnitus is subjective. This means the examiner cannot hear it, and there are no tools to measure or hear that sound. Objective tinnitus can arise from an aneurysm. This can be objectified and heard by the examiner. Other objective tinnitus investigation includes temporomandibular joint disease (TMJD) and tensor tympani muscle spasm.

        Tinnitus is defined as a phantom auditory perception-it is a perception of sound without corresponding acoustic or mechanical correlates in the cochlea. Tinnitus is the perception of sound that does not originate from a source external to the individual’s body. When discussing tinnitus, it is first crucial to categorize it between either subjective or objective, as well as between pulsatile and non-pulsatile tinnitus. In subjective tinnitus, which is more common, only the patient can perceive the sound. On the other hand, in objective tinnitus, both the individual and potentially the examiner can hear the sound. For example, subjective tinnitus is classically caused by a sensorineural hearing loss in patients experiencing presbycusis. The examiner is not able to perceive the tinnitus; however, the patient can.

        Pathophysiology

        When there is a danger or threat, humans normally react with typical fight or flight response. This is the reason why the onset of tinnitus can be so distressing. A broken finger does not necessarily trigger this response, but tinnitus does. Cognitive therapy is done to stop the unwanted reaction.

        However, stress is not a cause of tinnitus. Because humans cannot objectify tinnitus, the pathophysiology is not understood. Lesions that put pressure on the eighth cranial nerve may cause tinnitus. An increase in fluid pressure in the inner ear causes tinnitus. Symptoms associated with increased inner ear pressure include hearing loss, vertigo, tinnitus, and feeling of pressure in the ear. MRI shows that many areas of the brain are involved in tinnitus including the cognitive and emotional areas, as well as the auditory. Sound first enters the brain via the amygdala center. Therefore, learning that tinnitus is not a danger is therapeutic.

        Types of Tinnitus

        Based on the outcomes of the doctor’s examination, he or she will determine which type of tinnitus you have. Doctors distinguish between the following types of tinnitus:

        • Subjective and objective tinnitus – Subjective tinnitus can only be heard or perceived by the person who has it. Possible causes include problems with the auditory (hearing) system or the nerves that belong to it. In objective tinnitus, which is very rare, the doctor can hear the sounds too or detect the nerve signals causing the sounds. This is the case with tinnitus that is caused by blood-vessel-related problems, for instance. Here the doctor can hear a pulsing noise in the carotid artery in the neck with the help of a stethoscope.
        • Primary and secondary tinnitus – If no clear cause can be found, it is referred to as primary tinnitus or idiopathic tinnitus. If there is an identifiable cause, it is known as secondary tinnitus. Possible causes include a perforated eardrum or a vascular (blood vessel) disease.
        • Acute and chronic tinnitus – If the sounds last longer than three months, it is considered to be chronic tinnitus.
        • Various levels of severity – Tinnitus can be mild and hardly affect your everyday life, or only occur from time to time but then be distressing when it does. Sounds that are constantly and clearly heard are more serious: They can have a big impact on your daily life and work, for instance, because it is hard to sleep and concentrate properly.

        Causes of Tinnitus

        There are many causes of tinnitus

        • The most common cause of subjective tinnitus is noise trauma – For example, an employee who works in a noisy industry loses hearing at the 4000 Hz tone. Now the employee hears a sound which is similar to the 4000 tones.
        • Metabolic diseases – Heart, hypertension, diabetes are associated with an onset of tinnitus. Various drugs are ototoxic to some individuals or at sufficient doses. For example, high doses of aspirin cause tinnitus, and the issue resolves when aspirin is stopped.
        • Ear diseases – cause tinnitus including Meniere disease or lesions affecting the eighth cranial nerve.
        • Blocked ear (auditory) – canal due to a build-up of ear wax
        • Chronic middle ear infection
        • A ruptured (perforated) eardrum
        • Otosclerosis – a bone disease in the middle ear and inner ear that can lead to hearing loss
        • Ménière’s disease – a disease of the inner ear, causing symptoms such as tinnitus, vertigo and hearing loss
        • Problems affecting the muscles or joint of the jaw

        Twenty percent of persons visiting tinnitus clinics have normal hearing. Some have somatosensory tinnitus. Here, stimulation from cervical or TMJD has activated the dorsal cochlear nucleus and sends impulses to the auditory center. Evidence for this is that stimulation similar to whiplash or TMJD has been shown to cause anatomical changes in the dorsal cochlear nucleus.

        Salicylates

        • The symptoms of tinnitus include ringing, buzzing, roaring, hissing, or whistling in the ears. The noise may be intermittent or continuous. Most of the time, only the person who has tinnitus can hear it.
          Aspirin, of course, is the most commonly used drug known for its effects on hearing and tinnitus. After just 48 hours on a dosage of about 4.8 g/day, there is 10–15 dB of hearing loss and this can grow with continued use to as much as 40–50 dB. Typically, the hearing loss is essentially flat across frequency, but in some reports, the high frequencies are more affected than the low.

        Quinine

        • Quinine and other antimalarial drugs (e.g., quinidine, chloroquine, and hydroxychloroquine) have long been known for their ability to produce temporary hearing loss and tinnitus. With the decline of malaria in the United States, however, these drugs have become infrequent sources of tinnitus in this country, and as a consequence little has been written about this form of tinnitus.

        Tobacco

        • Fowler (1942) asserted that smoking is a common cause of tinnitus and that at least a month’s cessation is necessary to eliminate it as a causative factor. Whether or not Fowler was correct in this belief has yet to be satisfactorily established. Tyler indicates that new information on tobacco is forthcoming.

        Caffeine

        • This agent is frequently mentioned for its ability to produce or exacerbate tinnitus  but no systematic studies of it were found.

        Alcohol

        • Alcohol has the curious characteristic of being cited as both a cause and a treatment for tinnitus (see ”Alcohol” in this chapter). Unfortunately, anecdotes are the primary source of this information at this time

        Cocaine

        • Tinnitus is sometimes mentioned as a concomitant to cocaine use, and its vasoconstrictive actions make this claim believable. However, no information was found on the dose levels needed, the time course of onset and decline, etc.

        Marijuana

        • It has been asserted that marijuana can markedly increase preexisting tinnitus (CIBA Foundation, 1981:168), but no quantitative information appears to exist.

        Oral Contraceptives

        • Some oral contraceptives can produce a hearing loss and associated tinnitus (Brown et al., 1981). The effects are thought to be due to vascular changes. Detailed information was not found.

        Heavy Metals

        • Tinnitus is a common side-effect of heavy-metal treatment for cancer. For at least, the evidence indicates that the symptom is reversed upon withdrawal of the drug.

        Symptoms of Tinnitus

        The word “tinnitus” comes from the Latin word for “ringing.” But the sounds that people with tinnitus hear also include whistling, buzzing, humming, hissing, clicking, or knocking. They may be heard in one or both ears. Some people say it feels like the sound is coming from inside their heads, whereas others say it sounds like it is coming from outside. Tinnitus may be constant or it may come and go. It is sometimes very quiet and then really loud again.

        Diagnosis of Tinnitus

        History and Physical

        A physical exam should focus on the ear and the nervous system. The ear canal should be inspected for discharge, foreign body, and cerumen. The tympanic membrane should be inspected for signs of infection and tumor (red or bluish mass). A bedside hearing test should be done. Cranial nerves, particularly vestibular function, are tested along with peripheral strength, sensation, and reflexes. A stethoscope should be used to listen for vascular noise over the course of the carotid arteries and jugular veins and over and adjacent to the ear.

        Evaluation

        • X-rays – are not usually done for tinnitus unless there is an unexplained difference in hearing and balance in the ears.
        • An audiogram – is a hearing test measuring hearing levels to determine hearing loss. The patient is asked to match which of the tones matches their tinnitus. The audiologist introduces that sound as to volume, and the patient estimates how loud they hear their tinnitus. Hearing via the bone of the ear is tested and compared with the hearing via the earphone called an air-bone test. If the patient hears better with the bone test, this suggests a condition called otosclerosis which is treatable. Patients with otosclerosis, in whom the stapes fail to move well, can have surgery that corrects the otosclerosis and restores air conduction. In some patients, the tinnitus is relieved. In others, tinnitus remains or becomes worse.
        • The audiologist – measures how long tinnitus is relieved by masking tone. The longer the tinnitus is inhibited, the better the prognosis.
        • Dopplers – should be ordered for suspected arterial etiologies such as carotid stenosis before ordering more advanced radiological studies. In general, both CT and MRI are complementary imaging modalities to identify vascular etiologies of pulsatile tinnitus. Suspected arterial etiologies not well defined by duplex should get a CTA. CTA may also identify aneurysms, which may manifest as pulsatile tinnitus. MRV better evaluates venous etiology. CT scan – It is appropriate to get a CT of the temporal bones if there is clinical suspicion for temporal bone pathology. Patients with focal nerve deficits are candidates for brain imaging, either CT or MRI, to evaluate for more serious causes of pulsatile tinnitus. Other conditions may require further evaluation by specialists such as IIH, which can be diagnosed by normal brain CT/MRI and increased opening pressure on lumbar puncture. Performing an optic exam on these patients may reveal papilledema.

        Treatment of Tinnitus

        Non-Pharmacological

        The American Academy of Otolaryngology has issued clinical practice guidelines for tinnitus. These include:

        • Stress Reduction  This includes using biofeedback, measured breathing, etc. Although stress itself is not a cause of tinnitus, as in any condition, stress and anxiety can make the condition worse.
        • Cognitive Therapy – The more the patient understands what tinnitus is and is not the less negative effect. Once the patient fully cognizes – understands that tinnitus is similar to itching, the symptoms are reduced.
        • Sleep improvement  Tinnitus can affect normal sleep and therapy should be directed to better sleep hygiene.
        • Masking – When the body hears the same sound from the cell phone or sound device, this reduces the symptoms.  There are various forms of masking. Essentially these masking sounds take the attention away from the internal tinnitus sound and replace it with relaxing sounds.
        1. Introduction of the same sound
        2. Introduction of an altered sound
        3. Music with the tinnitus sound removed
        4. White noise or pleasant sounds

        Medication

        • Magnesium, alpha-lipoic acid, N-acetyl cysteine – and others have been tested for protection of hearing from noise. When these are effective, it is difficult to differentiate from the placebo effect or from the impact of having a program where the patient feels they are in charge of bringing the brain into the healing process. Recently beneficial results have been reported using deep brain stimulation. In theory, this alters unwanted neural circuits.
        • Alprazolam  – Medication such as alprazolam can reduce symptoms, but can have adverse effects including habituation.
        • Anti-depressants – may be indicated for patients who do not respond to protocol therapy.
        • Carbamazepine – (Tegretol is a registered trademark) This drug is an oral anticonvulsant and mild antidepressant that is best known in this country for its effective use on trigeminal and glossopharyngeal neuralgia; in Europe, it has been used extensively against epilepsy.
        • Lidocaine – (also known as lignocaine; Xylocaine is a registered trademark) Several recent reports indicate a high rate of improvement in tinnitus following the administration of lidocaine. This amide is commonly used as a local anesthetic in surgery of the middle ear and upper respiratory tract, but it is also a potent short-term anticonvulsant that has vasodilation as one of its effects.
        • Niacin – A member of the vitamin B complex, niacin has three common forms—niacinamide, nicotinic acid, and nicotinamide. The amino acid tryptophan can also be converted into niacin by the body. Niacin is a peripheral vasodilator and thus has been used in the treatment of peripheral vascular disorders and migraine headaches. It also has a long history in the treatment of some forms of Meniere’s Disease.
        • Vitamin A – Graham provides a summary of the studies concerned with vitamin A therapy. The early reports of both reductions in tinnitus and improvements in hearing sensitivity following massive intramuscular injection of vitamin A were not confirmed in later studies, and the issue seems to have been dropped.
        • Tocainide Hydrochloride – (Toward is a registered trademark) Unlike lidocaine itself, which must be administered intravenously, this analog of lidocaine can be taken orally because of a difference in its metabolism by the liver. Additional attractions are that it has a physiological half-life of about 11 hours as compared to about 1.5 hours for lidocaine, and it has fewer side effects. Tocainide is currently being used experimentally as an oral antiarrhythmic agent in cardiac patients.
        • Phenytoin Sodium – (also known as diphenylhydantoin; Dilantin is a registered trademark) This drug is an oral anticonvulsant similar in action to carbamazepine. In the paper discussed above, a brief mention is also made of Dilantin. It was apparently used on four patients who developed an allergic reaction to carbamazepine, but the only indication as to how these patients fared in the comment that Dilantin is always less effective than carbamazepine in treating tinnitus. Shea and Harell reported that none of 15 patients treated with this drug had any relief from tinnitus, even though all had some relief from the lidocaine injection.
        • Primidone – (Mysoline is a registered trademark) This anticonvulsant has been tried against trigeminal neuralgia with mixed results. Emmett and Shea briefly mention a study of its effectiveness against tinnitus. Patients initially received 250 mg twice daily; the dosage was increased monthly in increments of 250 mg/day up to a maximum of 2 g/day or until the tinnitus was relieved. Details are few, but apparently 27 percent (11/41) of the patients reported 80–100 percent relief, and an additional 59 percent (24/41) reported 20–80 percent relief from their tinnitus. Exactly when in the regimen these judgments were made is not revealed, yet this appears to be an important issue given the high incidence of side effects reported.
        • Sodium Fluoride – This compound has come to be recognized for its ability to reverse the process of demineralization in the cochlear capsule that leads to the condition of otospongiosis. Since this condition is often accompanied by a sensorineural-type hearing loss and by tinnitus and vertigo, it is of theoretical as well as practical interest here that these latter symptoms are often diminished or abolished as the otospongiosis is reversed. The explanation offered is that the otospongiotic focus gives off cytotoxic enzymes that then enter the perilymph, causing deterioration of cochlear elements critical to the normal transduction process, and, as a side effect, producing concomitant tinnitus. The administration of sodium fluoride cannot reverse the hearing loss (although according to Shambaugh it does arrest it), but it can eliminate the tinnitus and vertigo.
        • Sodium Valproate – (Depakene, Epilim, and Ergenyl are registered trademarks) Goodey (1981) briefly mentions this drug, indicating that it reduces tinnitus in about the same proportion of subjects as does carbamazepine, but that, at the doses used to date, the amount of relief experienced is less than with carbamazepine. Apparently side effects are minor.
        • Sodium Amylobarbitone – Noting a relationship between drugs that are effective on trigeminal neuralgia and on tinnitus (e.g., carbamazepine), Donaldson (1978) selected this fast-acting barbiturate, known for its effectiveness on trigeminal neuralgia, for a study on tinnitus. Forty patients with tinnitus of varying severity were randomly assigned to the experimental or control groups. Prior to treatment, all patients were assessed audio metrically, were asked to match the pitch and loudness of their tinnitus, and were asked to rate their tinnitus on a four-point scale (from “only noticeable in quiet environments” to “interferes with sleep, and patient engages in some activity to distract attention from it”). The experimental group was then put on a regimen of 50 mg in the morning, 50 mg in the early afternoon, and 80 mg at night; tinnitus was reassessed after 6 and 12 weeks. The drug was withdrawn after 12 weeks, and a final assessment was made at 18 weeks.
        • Arlidin a vasodilator, and chlortrimeton an antihistamine – have been used singly and in combination with some success against some forms of tinnitus.
        • Nortriptyline – Goodey indicates that the tricyclics, especially nortriptyline, may be worth investigating as tinnitus-reducing agents.
        • Diazepam (Valium) – is surely one of the most widely prescribed drugs for tinnitus and its psychological concomitants, but Goodey says that there is no evidence that it has any value in this regard, and it can make depressed patients worse.
        • Mexiletine – McCormick, and Thomas utilized a double-blind cross-over design in a study of mexiletine—a pharmacological relative of lidocaine that has an oral form. The patients’ numerical estimates of the severity of their tinnitus were unaffected both by this drug and by the placebo. It has been asserted that barbiturates do not cause tinnitus and it has been suggested that the aminoglycosides may produce only temporary tinnitus.
        • Heparin – is reported to have produced temporary relief from tinnitus in a number of heart patients
        • Trowbridge injected a 5 percent solution of ethylmorphine hydrochloride – an analgesic and vasodilator—directly through the tympanic membranes of patients suffering from tinnitus that he judged to be caused by structures of the middle ear (the tympanic plexus). He injected repeatedly at 4-day intervals and claimed reduction or elimination of tinnitus for the majority of patients so treated. He also claimed improved audiometric measures. No recent application of these procedures was found.
          A hearing aid – is always of benefit when tinnitus is associated with hearing loss. Some aids come with built-in soothing or masking sounds. Success is variable.

        Treatments that haven’t been proven to work

        Treatments for tinnitus that have not yet been proven to help include the following:

        • Acupuncture
        • Antidepressants – for example, tricyclic antidepressants and selective serotonin reuptake inhibitors (SSRIs). SSRIs can cause side effects such as a dry mouth, feeling faint, and a decreased libido.
        • Electromagnetic stimulation – This involves the use of electromagnets to try to influence the nerve signals that are responsible for the tinnitus. One example is known as repetitive transcranial magnetic stimulation (rTMS). This procedure involves placing a special coil on the scalp, where it generates a magnetic field. But rTMS has not yet been shown to work in good-quality studies.
        • Relaxation techniques – like progressive muscle relaxation, autogenic training, or yoga.
        • Epilepsy drugs – such as medication gabapentin. The possible side effects include sleepiness, dizziness, and – in the long term – weight gain.
        • Ginkgo biloba – Several studies involving a total of more than 1,000 participants didn’t provide any proof that ginkgo products effectively relieve tinnitus symptoms. But they can cause side effects such as gastrointestinal (stomach and bowel) problems or allergic reactions. Ginkgo can also interact with other medications. For instance, it can increase the effect of anticoagulant (blood-thinning) medication, which can cause bleeding.
        • Hyperbaric oxygen therapy – This involves sitting in a special high-pressure chamber and breathing in pure oxygen. The aim is to increase the transport of oxygen to the ears and brain. Hyperbaric oxygen therapy is most commonly used in people who have hearing loss as well as tinnitus.
        • Hypnosis – This involves getting people into a deep state of relaxation where they are at a different level of consciousness. The therapist then uses hypnotic suggestion to try to change how they perceive the tinnitus sounds.
        • Dietary supplements – Dietary supplements such as certain vitamin or zinc supplements haven’t been proven to relieve tinnitus symptoms.
        • Sound therapy – In this treatment, special noise generators produce a sound (usually a shushing sound). Some noise generators, known as “noise maskers,” distract patients from the tinnitus sound by drowning them out. Others integrate the tinnitus sounds into other sounds in order to make them less noticeable. They are worn like hearing aids. You can also play recordings of the sounds of ocean waves or install a tabletop fountain to produce a sound background that can mask the tinnitus.
        • Filtered music – Certain smartphone apps alter the frequency of music you play on your phone on the basis of your individual frequency of tinnitus. Listening to music using the app for one to two hours per day is claimed to reduce the volume of tinnitus sounds.

        References

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