Autoimmune inner ear disease

Other Names: AIED

Autoimmune inner ear disease (AIED) has been defined as a condition of bilateral sensorineural hearing loss (SNHL), caused by an ‘uncontrolled’ immune system response. The inner ear can be the direct target of the immune response, but it can be additionally damaged by a deposition of circulating immune complexes or by systemic immune-mediated diseases.

Epidemiology

The estimated yearly incidence of AIED is <5 cases per 100,000 and its estimated prevalence is about 15/100,000 (in the United States, the expected annual AIED prevalence is 45,000 patients); it is reported that AIED is more prevalent in women, in their third and the sixth decades of life.

Cause

AIED is generally caused by either antibodies or immune cells that cause damage to the inner ear. There are several theories that propose a cause of AIED:

Bystander damage – Physical damage to the inner ear may lead to cytokine release that signals for an immune response. This may be a component of the "attack/remission cycle" of Meniere's disease. Cross-reactions – Accidental damage of the inner ear by antibodies or T-cells that recognize an inner ear antigen that is similar to a bacterial or viral antigen Genetic factors – Predisposition to developing an autoimmune disorder based on genes inherited Intolerance – The immune system may not be aware of all the antigens present in the inner ear until physical damage releases some of these antigens. As a result, the immune system treats these unfamiliar antigens as foreign and mounts an immune response. Currently, the cross-reactions theory appears to be the favored mechanism of AIED pathogenesis.

Signs and symptoms

Signs and symptoms of AIED are:

• Progressive hearing loss in both ears
• Typically will begin in one ear and gradually affect the other
• Hearing loss may begin suddenly
• Tinnitus (ringing or buzzing in ears)
• Decrease in word recognition capability
• Loss of balance (vestibular symptoms)
• Degree of balance loss can change throughout the course of the disease

Diagnosis

Since AIED symptoms are fairly common to many hearing loss disorders, it may be difficult to diagnose AIED without performing multiple medical tests. Some examples of these tests include:

• Hearing Tests for Progressive Hearing and Balance loss
• Audiometry (measure of hearing acuity and sound intensity)
• Rotatory Chair Test (determines if inner ear is responsible for balance loss)
• Electrocochleography (ECOG) (recording of electrical potential in inner ear due to sound)
• Blood Tests for General Autoimmune Diseases
• Erythrocyte sedimentation rate (test for inflammation)
• Rheumatoid Factor (indicator of autoimmune disorders)

There are also blood tests specific to inner ear disorders:

• Anti-cochlear antibody test (testing for antibodies against cochlear cells)
• Lymphocyte Transformation Assay (testing whether an individual has developed a T-cell response against a certain drug)

Though it has also been proposed that the use of anti heat shock protein 70 antibodies may be useful in the detection and diagnosis of AIED.

Treatment

AIED treatment is a rapidly changing field. Several medical therapies have been proposed in the treatment of AIED, with corticosteroid therapy being the most effective. However, corticosteroid therapy (specifically with prednisone and dexamethasone) has demonstrated limited effectiveness in some patients, suggesting the need for novel treatment methods.

The goal of most AIED treatments is to administer corticosteroids over a certain period of time, re-evaluate hearing at each appointment, and eventually taper off corticosteroid administration. Ideally, patients can be tapered off with hearing fully recovered, though this is the least likely outcome.

Often, tumor necrosis factor-α (TNF-α) inhibitors must be administered alongside corticosteroids to achieve a favorable outcome and eventual end to corticosteroid treatment. Recent papers have indicated that the TNF-α inhibitor, infliximab, has the potential to allow for sustained patient improvement and alleviation of symptoms.

Cytotoxic agents such as cyclophosphamide and methotrexate have been used in AIED treatment in the past; however, findings have suggested limited symptom alleviation from these drugs.

NIH genetic and rare disease info

• NIH info on Autoimmune inner ear disease

Autoimmune inner ear disease is a rare disease.

Autoimmune inner ear disease Resources
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