Cogan's syndrome.

Published

Journal Article (Review)

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.

Full Text

Duke Authors

Cited Authors

  • St Clair, EW; McCallum, RM

Published Date

  • January 1999

Published In

Volume / Issue

  • 11 / 1

Start / End Page

  • 47 - 52

PubMed ID

  • 9894630

Pubmed Central ID

  • 9894630

Electronic International Standard Serial Number (EISSN)

  • 1531-6963

International Standard Serial Number (ISSN)

  • 1040-8711

Digital Object Identifier (DOI)

  • 10.1097/00002281-199901000-00008

Language

  • eng