Cogan's syndrome.

Abstract:

:Work originating in the 1940s led to the characterization of a rare, chronic inflammatory disorder with a unique predilection for the cornea and vestibuloauditory apparatus, now called Cogan's syndrome (CS) after the ophthalmologist who first described it. CS occurs primarily in young adults and typically presents with interstitial keratitis (IK) and Ménière's-like episodes developing within several months of each other. The inflammatory process may target other ocular sites, and the disease itself may be accompanied by aortitis or a Takayasu's-like or medium-sized vessel vasculitis. Morbidity in CS results from deafness and complications from cardiovascular disease. Most evidence suggests that the ocular and vestibuloauditory manifestations are not a consequence of vasculitis but rather mediated by other immunologic mechanisms, possibly organ-specific autoimmunity. The cornerstone of treatment in CS is corticosteroids, topically for IK and systemically for inner ear dysfunction. Early corticosteroid therapy appears to be critical for reversing hearing loss. Cochlear implants can partially restore auditory function and have been a salvation for patients who suffer from deafness as a result of permanent cochlear damage.

journal_name

Curr Opin Rheumatol

authors

St Clair EW,McCallum RM

doi

10.1097/00002281-199901000-00008

subject

Has Abstract

pub_date

1999-01-01 00:00:00

pages

47-52

issue

1

eissn

1040-8711

issn

1531-6963

journal_volume

11

pub_type

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