Autoimmune Inner Ear Disease, AIED

Autoimmune inner ear disease or "AIED" consists of a syndrome of progressive hearing loss and/or dizziness which is caused by antibodies or immune cells which are attacking the inner ear. It can also be a sudden hearing loss on one side or both ears accompanied by pain.

The classic picture is reduction of hearing accompanied by tinnitus (ringing, hissing, roaring) which occurs over a few months. Variants are bilateral attacks of hearing loss and tinnitus which resemble Meniere's disease, and attacks of dizziness accompanied by abnormal blood tests for self-antibodies. About 50% of patients with AIED have balance symptoms.

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

Blood tests for autoimmune disorders include:

Sed Rate,CRP
ANA
Rheumatoid Factor
Complement C1Q
Thyroid (TSH, anti-microsomal antibodies)
anti-gliadin antibodies (for Celiac disease)

Blood tests for conditions that resemble autoimmune disorders include:

RPR (for Syphilis)

Lyme titer

HBA1C (for diabetes, which is often autoimmune mediated also)

HIV (HIV is associated with auditory neuropathy)

What Are the Symptoms of AIED?

The symptoms of AIED are sudden hearing loss in one ear progressing rapidly to the second ear. The hearing loss can progress over weeks or months. Patients may feel fullness in the ear and experience vertigo. In addition, a ringing, hissing, or roaring sound in the ear may be experienced. Diagnosis of AIED is difficult and is often mistaken for otitis media until the patient develops a loss in the second ear. One diagnostic test that is promising is the Western blot immunoassay.Meniere's disease and AIED seem to be closely intertwined. In fact, immune dysfunction is increasingly being recognized as one of the factors that causes Meniere's disease. Researchers now feel that a "significant percentage" of the people with bilateral Meniere's disease have AIED as the underlying cause. One study indicated that immune system dysfunction was responsible for 16% of people with bilateral Meniere's disease and 6% of all people with any variety of Meniere's disease. In another study, 96% of the patients with Meniere's disease had elevated levels of circulating immune complexes, compared to just 20% of the controls. In yet another study, specific immune system antibodies were detected in 47% of people with Meniere's disease. This all shows that a large percentage of the people with Meniere's disease have an underlying immune system problem.

What Is the Treatment for AIED?

Most patients with AIED respond to the initial treatment of steroids, prednisone, and methotrexate, a chemotherapy agent. Some patients may benefit from the use of hearing aids. If patients are unresponsive to drug therapy and hearing loss persists, a cochlear implant maybe considered.

History

Until recently it was thought that the inner ear could not be attacked by the immune system. Studies have shown that the perisacular tissue surrounding the endolymphatic sac contains the necessary components for an immunological reaction. The inner ear is also capable of producing an autoimmune response to sensitized cells that can enter the cochlea through the circulatory system.

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: Autoimmune sensorineural hearing loss: clinical course and treatment outcome.

Loveman DM, de Comarmond C, Cepero R, Baldwin DM.

Department of Internal Medicine, Texas Tech University Health Sciences Center at Odessa, TX, USA. Donald.Loveman@ttuhsc.edu

BACKGROUND: Autoimmune sensorineural hearing loss (ASHL) originally was defined both by the presence of progressive hearing loss (with or without vertigo) and a positive response to glucocorticoid therapy. Subsequently, antibodies to a 68-kd inner-ear antigen were identified in a high percentage of patients with ASHL. OBJECTIVE: To analyze the disease progression and treatment outcome of ASHL. METHODS: Retrospective chart review of 30 patients with a diagnosis of ASHL referred to Texas Tech Rheumatology Clinic. All subjects were tested for anti-68-kd antibody, had audiometric evidence of hearing loss, and were treated with glucocorticoids. RESULTS: The median duration from onset of symptoms to audiometric testing was 25.5 weeks (mean, 144 weeks; range, 3-1,040 weeks), and to testing for anti-68-kd antibody was 40 weeks (mean, 157.3; range, 4-1,092 weeks). Ninety percent of subjects tested positive for anti-68-kd antibodies. By audiometric testing, 50% of subjects were steroid-responsive; minimal improvement or no change was recorded in 12%, and worsening occurred in 39% after steroid therapy. The 3 subjects who were anti-68-kd antibody-negative were steroid-unresponsive, and 1 progressed to complete deafness. CONCLUSIONS AND RELEVANCE: This series of patients with ASHL suggests a more variable and benign course with a better prognosis than previously reported. Immunosuppressive therapy other than steroids rarely was required, and rapid progression to complete hearing loss was very uncommon in this cohort.

PMID: 15505769 [PubMed - indexed for MEDLINE]

: Immune-mediated inner ear disease: 10-year experience.

Broughton SS, Meyerhoff WE, Cohen SB.

Otolaryngology, University of Texas Southwestern Medical School, Dallas, Texas, USA.

BACKGROUND: Autoimmune inner ear disease (AIED) was first described in 1979 and the disease has become more widely recognized over the last decade. Limited information is available regarding clinical features of the disease, disease course, and response to treatment. OBJECTIVE: To analyze data from 42 patients with documented immune mediated inner ear disease to further define this syndrome. METHODS: A retrospective chart review was conducted on all patients considered to have AIED by the Otolaryngology division physicians and on all patients positive for antibody testing to inner ear antigens from 1990 to 1999. Patients who were antibody positive with a clinical diagnosis of AIED were included in this review. RESULTS: Patients with AIED presented with rapidly progressive, frequently bilateral (79%), often fluctuating sensironeural hearing loss. Mean age at presentation was 50 years (22-80) with no gender predilection. Tinnitus (83%), vestibular complaints (79%), and Menieres (50%) were common concomitant symptoms along with hearing loss. Seven of 42 (17%) of the patients had evidence for other systemic autoimmune disorders. In 4 of the patients the onset of vestibuloauditory complaints preceded the diagnosis of autoimmune disorder. Thirty-three of 42 demonstrated antibodies to inner ear antigens but other autoantibodies were infrequent except in patients with systemic autoimmune disorders. Twenty-three of 33 (70%) of patients treated with corticosteroids improved clinically, often short-term. Sixteen patients received treatment with other immunosuppressive drugs including methotrexate, cyclophosphamide, azathioprine, mycophenolic mofetil, and intravenous immunoglobulin (IVIg) generally with a limited modest response. Using clinical trial criteria for response, only 5/35 (14%) demonstrated improvement over a mean 34.4 months follow-up. CONCLUSIONS: Immune-mediated inner ear disease is not a uniform disease with simple diagnosis or treatment. The course of the disease often results in significant long-term disability due to hearing loss and response to aggressive immunosuppression including corticosteroids is poor.

PMID: 15505770 [PubMed - indexed for MEDLINE]
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