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Immune-mediated inner ear disease: 10-year experience
Shelley S Broughton1, William E Meyerhoff, Stanley B Cohen
1Otolaryngology, University of Texas Southwestern Medical School, Dallas, Texas, USA.
Seminars in Arthritis and Rheumatism
|October 27, 2004
Summary
Autoimmune inner ear disease (AIED) often causes rapid, bilateral hearing loss and related symptoms. Treatments like corticosteroids show limited long-term effectiveness, leading to significant disability.
Area of Science:
- Otolaryngology
- Immunology
- Neurology
Background:
- Autoimmune inner ear disease (AIED) is increasingly recognized, but clinical features, disease progression, and treatment responses remain poorly understood.
- First described in 1979, AIED requires further definition to improve patient outcomes.
Purpose of the Study:
- To define the clinical syndrome of immune-mediated inner ear disease.
- Analyze data from 42 patients diagnosed with AIED.
Main Methods:
- Retrospective chart review of patients diagnosed with AIED between 1990 and 1999.
- Inclusion criteria: clinical diagnosis of AIED and positive antibody testing to inner ear antigens.
Main Results:
- AIED typically presents as rapidly progressive, fluctuating, and often bilateral sensorineural hearing loss.
- Common symptoms include tinnitus, vestibular complaints, and Meniere's disease; 17% had other autoimmune disorders.
- Corticosteroid treatment yielded short-term improvement in 70%, but only 14% showed sustained response over a mean follow-up of 34.4 months.
Conclusions:
- Immune-mediated inner ear disease is heterogeneous, lacking simple diagnostic or treatment protocols.
- The disease frequently leads to long-term hearing loss disability.
- Response to immunosuppressive therapies, including corticosteroids, is generally poor.