Cogan’s Syndrome

Cogan’s syndrome (CS) is a rare autoimmune disorder characterized by interstitial keratitis, vestiboloauditory symptoms, and a wide spectrum of ocular inflammation. Typical (classical) and atypical variants of CS are usually defined to have different clinical combinations. Typical CS is classically presented by ocular involvement that is primarily interstitial keratitis with cochlear vestibular symptoms, such as sudden onset of nausea, vomiting, tinnitus, vertigo along with progressive hearing loss. Atypical CS is presented by scleritis, iritis, and frequently with systemic immune-mediated inflammation, such as systemic vasculitis. Treatment includes corticosteroids a first-line treatment, other immune-suppressive agents such as anti-TNF drugs. In severe and unresponsive cases, biological drugs are considered to be efficient and with a favorable outcome.