ABSTRACT
Cogan’s syndrome (CS) is a disease of young adults consisting of episodes of acute interstitial keratitis with vestibuloauditory dysfunction. Episodes of vestibuloauditory dysfunction in CS are frequently indistinguishable clinically from episodes of Meniere’s disease. The eye manifestations of CS are typically interstitial keratitis with iritis and conjunctivitis. Atypical cases of CS are seen with ocular inflammatory disease other than keratitis, most often mild anterior scleritis or episcleritis. In CS, the comea, conjunctiva and cochlear are infiltrated with mononuclear inflammatory cells during active disease. On cyclophosphamide and prednisone, the patient developed myocardial ischemia; at catherization an occlusion of the distal right coronary artery was found and successful angioplasty performed. CS is a rare tissue-specific inflammatory disease affecting the eyes, ears, and large vessels. While severe hearing loss, if treated early on, can be reversed with systemic steroids, the rare complications of vision-threatening eye disease and large vessel vasculitis have been more difficult to control with either prednisone or cyclophosphamide therapy.
