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Research Article| Volume 17, ISSUE 3, P581-594, September 1997

Autoimmune Inner Ear Disease—A Real Entity?

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      The term, "inner ear disease" denotes a clinical entity comprising sensorineural hearing loss (nerve deafness), dizziness (Meniere's disease), or both conditions. Inner ear disease may be associated with immune responses to inner ear proteins or tissue or improvement after immunosuppressive treatment. Systemic immunologic disease may also be present. Although such findings suggest autoimmunity, specific organ-related antigens provoking autoimmune reactions to inner ear components have yet to be identified. Inner ear disease with evidence of immunologic involvement is therefore frequently referred to as "immune-mediated inner ear disease" (IMIED), rather than true autoimmune disease. IMIED likely represents a heterogeneous group of diseases with a common endpoint, and diagnosis is made primarily by clinical profile in association with laboratory testing to rule out neoplasia or infection.
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