What are the ocular and auricular manifestations of Cogans syndrome?
Nonsyphilitic interstitial keratitis (IK) is the classic ocular manifestation and typically presents as the acute onset of unilateral or bilateral redness, pain, photophobia, and increased lacrimation. Other forms of ocular inflammation including uveitis, scleritis, choroiditis, and retinal artery occlusion can occur with or without concomitant keratitis.
Vestibuloauditory dysfunction is usually acute in onset and presents in a Ménière-like manner, with episodes of tinnitus, vertigo, and SNHL. Hearing loss is fluctuating but progressive, leading to deafness in >50% of cases. Unilateral involvement is typical, but bilateral disease can occur. Vestibular dysfunction may be prominent, manifesting as vertigo, ataxia, and nausea.
Eye and ear involvement usually occur within 1 to 6 months of each other but can be separated by years.
Atypical Cogan’s syndrome is defined as non-IK inflammatory ocular manifestations, typical or atypical ocular manifestations with audiovestibular symptoms different from Ménière-like episodes, or >2 years between the onset of eye and ear manifestations.
