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Clinical diagnosis of immune inner‐ear disease
Author(s) -
Hughes Gordon B.,
Nalepa Nancy J.,
Kinney Sam E.,
Barna Barbara P.,
Calabrese Leonard H.
Publication year - 1988
Publication title -
the laryngoscope
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.181
H-Index - 148
eISSN - 1531-4995
pISSN - 0023-852X
DOI - 10.1288/00005537-198803000-00001
Subject(s) - medicine , sensorineural hearing loss , disease , rheumatoid arthritis , hearing loss , autoimmune disease , immune system , dermatology , pediatrics , surgery , immunology , audiology
Abstract The purpose of this study was to develop a high‐risk clinical profile of patients with autoimmune (immune‐mediated) inner‐ear disease. The records of 52 patients diagnosed over the past 5 years were reviewed. Age, sex, bilateral versus unilateral involvement, otologic symptoms, concomitant systemic immune disease, and presenting clinical diagnoses were recorded. The presenting diagnoses were Cogan's syndrome, Meniere's syndrome, Dandy's syndrome without hearing loss, or progressive sensorineural hearing loss without dizziness. Because Cogan's and Dandy's syndromes were relatively uncommon, the typical high‐risk clinical profile was a middle‐aged patient (often female) with bilateral, asymmetric, progressive sensorineural hearing loss, with or without dizziness, and occasional systemic immune disease such as rheumatoid arthritis. When a more common clinical diagnosis cannot be reached in suspicious patients, immune laboratory tests should be obtained and a trial of immunotherapy offered. Positive test results and beneficial response to therapy support a presumptive diagnosis of immune inner‐ear disease.

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