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.

To analyze data from 42 patients with documented immune mediated inner ear disease to further define this syndrome.

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.

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.

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.