Cogan's syndrome: a cause of progressive hearing deafness

In 1934 Morgan and Baumgartner first described a nonsyphilitic interstitial keratitis (IK) associated with vestibuloauditory dysfunction (Morgan RF, Baumgartner CF, Menier's disease complicated by recurrent interstitial keratitis. Excellent result following cervial ganglionectomy. West J Surg 1934;42:628). Cogan was the first to describe this syndrome as a clinical entity with the report of 5 additional cases in 1945 (Cogan DA: Syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms. Arch Ophthalmol 1945;33:144-9). Since that time, more than 100 cases of Cogan's syndrome (CS) have been in the literature. Cogan's syndrome is a rare clinical disease, which primarily affects young adults; however, published reports range from 2.5 to 60 years for age of onset (Kundell Sp, HD Ochs: Cogan's syndrome in childhood. J Pediatr 1980;97:96-8). This disease primarily affects whites and is believed not to be hereditary. Typical CS is characterized by IK and vestibuloauditory dysfunction. The IK usually occurs with sudden onset and is characterized by photophobia, lacrimation, and eye pain. The vestibuloauditory dysfunction is usually bilateral, presenting with tinnitus, sensorineural hearing loss, and acute episodes of vertigo. Atypical CS presents with significant inflammatory eye disease (ie, scleritis, episcleritis, retinal artery occlusion, choroiditis, retinal hemorrhages, papilloedema, exophthalmos, or tenonitis) with or without IK (Laryngoscope 1960;70:447-9). In this report, we describe a typical case of CS, in which the hearing loss was unresponsive to corticosteroid therapy.