Cogan syndrome

Heather E Moss MD PhD (Dr. Moss of Stanford University has no relevant financial relationships to disclose.)
Raymond P Roos MD, editor. (Dr. Roos of the University of Chicago owns stock in Amgen, Best Doctors, Express Scripts, Ionis, and Merck.)
Originally released May 13, 1996; last updated March 13, 2017; expires March 13, 2020

This article includes discussion of Cogan syndrome, Cogan keratitis syndrome, keratitis-deafness syndrome, nonsyphilitic interstitial keratitis, oculovestibulo-auditory syndrome, and atypical Cogan syndrome. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Cogan syndrome is a rare, likely autoimmune, inflammatory disease that affects the inner ear and ocular cornea. It is characterized by an acute phase of relapsing ocular and aural involvement lasting months to years, followed by a chronic phase of relatively low disease activity. Patients typically recover from the ocular symptoms, but many develop permanent hearing loss. Some also develop systemic necrotizing vasculitis that affects vital organs, such as the aorta and aortic valves, brain, and kidneys, with life-threatening complications. Oral glucocorticoid therapy is the mainstay of treatment.

Key points

 

• Cogan syndrome is characterized by ocular disease and audiovestibular dysfunction.

 

• Typical ocular disease is interstitial keratitis.

 

• Typical audiovestibular symptoms are acute attacks resembling Ménière disease and progressive hearing loss.

 

• A large proportion of patients have systemic manifestations, with a minority having life threatening sequelae of vasculitis.

 

• Immunosuppressive therapy is used to control symptoms.

Historical note and terminology

The syndrome of nonsyphilitic interstitial keratitis in association with audiovestibular dysfunction was first reported in 1934 (Morgan and Baumgartner 1934); shortly thereafter it was classified as a distinct clinical entity by David Cogan (Cogan 1945). Oliner and colleagues were the first group to hypothesize that Cogan syndrome was a manifestation of a generalized arteritis (Oliner et al 1953).

Atypical Cogan syndrome is defined on the basis of atypical audiovestibular or ocular symptoms. Haynes and colleagues defined as atypical those cases with audiovestibular symptoms that were not Ménière-like or that occurred more than 2 years before or after the onset of eye symptoms (Haynes et al 1980). Cody and Williams defined atypical Cogan syndrome as the presence of an inflammatory eye lesion in addition to or instead of interstitial keratitis (Cody and Williams 1960).

The synonyms of Cogan syndrome include Cogan keratitis syndrome, keratitis-deafness syndrome, nonsyphilitic interstitial keratitis, and oculovestibulo-auditory syndrome.

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