Immune-mediated inner ear disease: 10-year experience

https://doi.org/10.1016/j.semarthrit.2004.07.001Get rights and content

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 Meniere’s (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.

Section snippets

Methods

A chart review of all known patients diagnosed between 1990 and 1999 with AIED by the Otolaryngology Division of the University of Texas Southwestern Medical School was conducted. Additionally a list of all patients undergoing testing for antibodies to inner ear antigens was requested from the laboratory. Charts of patients positive for autoantibodies to inner ear antigens were reviewed and patients with a clinical diagnosis of immune-mediated inner ear disease were included in this review. The

Results

The average age at initial presentation was 50 years (range 22 to 80). Twenty-two patients were men and 20 were women. Thirty-three of 42 (79%) had evidence of bilateral disease during their evaluation. Otologic symptoms evaluated included presence of fluctuating hearing loss in at least 1 ear (26/42 or 62%), aural fullness (25/42 or 60%), and tinnitus in 1 or both ears (35/42 or 83%). The number of patients with vestibular complaints was 33/42 (79%). True vertigo spells occurred in 26

Management

A total of 33 patients received corticosteroids at some point during their treatment (Fig 1). Twenty-three of 33 (70%) showed a response to treatment, although 9 of those 23 were brief trials (less than 2 weeks duration) or subjective improvement only. The remaining 14 patients had documented pre- and posttreatment audiometric data following corticosteroid treatment. Of the 14 responders, hearing stabilized in 6 and improved in 7. One patient had an improvement in vestibular symptoms with no

Discussion

Patients with AIED are frequently managed by the rheumatologist after referral from the otolaryngologist. This review describes our experience of the clinical and laboratory manifestations of these patients as well as their poor long-term outcome. These patients were characterized by a sensorineural hearing loss which is usually bilateral and occurs rapidly, over weeks to months. The hearing loss often fluctuated and stabilized at a certain level, or progressed without fluctuation. Vestibular

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*

Otolaryngology Fellow

Chairman Emeritus, Dept. of Otoloaryngology

Clincial Professor of Internal Medicine

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