RESEARCH - Cogan syndrome (a syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms)

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Mayo Clin Proc. 2006 Apr;81(4):483-8.

Cogan syndrome: a retrospective review of 60 patients throughout a half

century.

Department of Otorhinolaryngology, Mayo Clinic College of Medicine,

Rochester, MN 55905, USA.

OBJECTIVE: To evaluate the disease manifestations and clinical course of

patients affected by Cogan syndrome (a syndrome of nonsyphilitic

interstitial keratitis and vestibuloauditory symptoms) at a single

institution during roughly a half century. PATIENTS AND METHODS: Medical

records of all patients diagnosed as having Cogan syndrome at the Mayo

Clinic in Rochester, Minn, and who were followed up from 1940 to 2002 were

comprehensively reviewed. Otolaryngologic, ophthalmologic, and systemic

manifestations of disease were analyzed. Analysis included patient

demographics, presenting manifestations, delayed manifestations, laboratory

testing, physical examination features, therapeutic interventions, disease

course, and hearing and vision outcomes. RESULTS: Sixty patients were

identified as having Cogan syndrome, with follow-up from 1940 to 2002. Most

patients presented initially with vestibuloauditory symptoms, most commonly

sudden hearing loss (50%). The most common inflammatory ophthalmologic

condition noted was bilateral interstitial keratitis. Headache (40%), fever

(27%), and arthralgia (35%) were the most frequently encountered systemic

manifestations. Evidence of aortitis was found in 12% of patients. Complete

hearing loss was eventually noted in 52% of affected patients, whereas

permanent loss of any degree of vision was uncommon. Cochlear implantation

outcomes were uniformly good. Death directly or indirectly attributed to the

effects of Cogan syndrome was noted in 4 patients.

CONCLUSIONS: The major disease-related morbidities were due to

vestibuloauditory disease and only infrequently due to systemic

manifestations such as vasculitis, with or without aortitis. Cochlear

implantation has been of major benefit in modern hearing rehabilitation for

this patient population. We advise caution before institution of protracted

courses of high-dose corticosteroids and/or chemotherapy for patients

without pronounced systemic disease or severe eye disease unmanageable by

topical or periocular corticosteroids alone.

PMID: 16610568

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