Cogan's syndrome: ophthalmic, audiovestibular, and systemic manifestations and therapy.

Cogan's syndrome is an idiopathic inflammatory disease which may present as interstitial keratitis, inflammation of other ocular structures, Meniere's-like attacks, or systemic vasculitis. Although the ocular manifestations respond to topical steroids and are rarely serious, permanent deafness may result if systemic steroid therapy is not promptly instituted for audiovestibular dysfunction, while major morbidity and even death may occur if systemic sequelae such as vasculitis and aortic insufficiency are not recognized. For these reasons, ophthalmologists must suspect Cogan's syndrome in any patient presenting with ocular inflammation who develops hearing loss, vertigo, ataxia, tinnitus, systemic vasculitis, or aortic insufficiency.