Síndrome de Cogan

Cogan syndrome (CS) is typified by nonsyphilitic interstitial keratitis and Meniere-like auditory involvement. It can present atypically with other ocular and audiovestibular symptoms and associated systemic manifestations. Its name derives from the author who first described the disease. CS affects adults of both sexes, with a mean age of 30 years. The prevalence is higher in Caucasians. The pathogenesis of this syndrome is unknown, but is probably the result of an autoimmune mechanism triggered by an infection. The diagnosis is mainly clinical, using the criteria established by Haynes et al. in 1980 for “typical CS” and “atypical CS”. A differential diagnosis should be performed with other systemic diseases that cause similar eye and inner ear manifestations. The course is variable and deafness is a common complication. Prompt treatment and its maintenance are the basis of a favorable outcome.