Ew. Chynn et Fa. Jakobiec, COGANS-SYNDROME - OPHTHALMIC, AUDIOVESTIBULAR, AND SYSTEMIC MANIFESTATIONS AND THERAPY, International ophthalmology clinics, 36(1), 1996, pp. 61-72
Cogan's syndrome (CS) is a rare multisystem disease characterized by a
cute nonsyphilitic interstitial keratitis and audiovestibular dysfunct
ion including neurosensory hearing loss, tinnitus, and vertigo. This d
isease was first recognized as a discrete clinical entity by David Cog
an in 1945, when he reported a series of 4 patients with characteristi
c findings, although prior isolated reports can be found in the litera
ture [1]. The systemic associations of CS were recognized early histor
ically by both Cogan [2-4] and others [5-8]. The most important system
ic manifestations include vasculitis and aortic insufficiency. The acc
urate diagnosis of CS assumes an importance disproportionate to its in
cidence, as early systemic steroid therapy may prevent permanent deafn
ess, whereas systemic workup and management can prevent life-threateni
ng complications.