Prolonged prodrome, systemic vasculitis, and deafness in Cogan’s syndrome

Cogan’s syndrome is a rare, multisystem disease which occurs predominantly in children and young adults. It was originally described as the combination of interstitial keratitis and audiovestibular disturbance, but other forms of ocular disease, as well as systemic vasculitis, have since been recognised as part of the syndrome. Diagnosis can be diYcult if the various manifestations occur separately, but early recognition is important because prompt treatment may prevent deafness. Two cases are presented here illustrating the features of this disease, and providing histological evidence of systemic vasculitis in both. (Ann Rheum Dis 2001;60:69–71) In 1945 Cogan described a syndrome of interstitial keratitis and audiovestibular dysfunction in young adults. The potential for systemic features including vasculitis to occur as part of this syndrome was highlighted by Cody and Williams in 1960. Although uncommon, it is important to recognise Cogan’s syndrome quickly and attempt to prevent deafness. Corticosteroids are the mainstay of treatment; however, further immunosuppressive treatment may be required for associated vasculitis.