Cogan's syndrome: Ophthalmic, audiovestibular, and systemic manifestations and therapy

Cogan's syndrome (CS) is a rare multisystem disease characterized by acute nonsyphilitic interstitial keratitis and audiovestibular dysfunction including neurosensory hearing loss, tinnitus, and vertigo. This disease was first recognized as a discrete clinical entity by David Cogan in 1945, when he reported a series of 4 patients with characteristic findings, although prior isolated reports can be found in the literature [1]. The systemic associations of CS were recognized early historically by both Cogan [2-4] and others [5-8]. The most important systemic manifestations include vasculitis and aortic insufficiency. The accurate diagnosis of CS assumes an importance disproportionate to its incidence, as early systemic steroid therapy may prevent permanent deafness, whereas systemic workup and management can prevent life-threatening complications.