Cogan's syndrome is a rare, idiopathic, chronic inflammatory disorder characterized vestibuloauditory dysfunction and interstitial keratitis. Some evidence suggests an autoimmune mechanism, but the true etiology unknown. The classic ophthalmic feature is interstitial keratitis causing patchy, granular corneal stromal infiltrates that may result in anterior stromal and subepithelial scarring. Patients may have other signs of ocular inflammation including uveitis, conjunctivitis, episcleritis, scleritis or retinal vasculitis. The inner ear disease associated with Cogan's syndrome can manifest as vertigo, ataxia, tinnitus or hearing loss. Anti-inflammatory and/or immunosuppressive therapy are the treatments based on the clinical features.
This young patient presented initially for bilateral hearing loss without visual complaints. Cogan's syndrome was on the differential and he was sent for ophthalmic evalution. He was found to have bilateral, patchy granular corneal opacities in the mid-stroma peripherally with a clear central cornea in each eye. He was asymptomatic from this with 20/20 vision in each eye. There were no other signs of ocular inflammation.
Ophthalmic Atlas Images by EyeRounds.org, The University of Iowa are licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.
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