Immune-mediated inner ear disease: 10-year experience
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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Financial Support—not applicable.
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Otolaryngology Fellow
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Chairman Emeritus, Dept. of Otoloaryngology
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Clincial Professor of Internal Medicine