Systemic Disorders With Optic Nerve and Retinal Findings

Cogan's syndrome is a vasculitic-autoimmune process with critical ocular features. In its original classical form it consists of nonluetic interstitial keratitis, with audiovestibular dysfunction, often with pain. More recently, it has been pointed out that there are nonclassical forms as well, particularly in that the use of topical corticosteroids before a definitive diagnosis is made may drastically alter the natural history of the ocular disease. These other eye symptoms may include subepithelial nummular lesions, episcleritis, and uveitis. As seen here, retinal vasculitis has been reported in the absence of anterior segment signs and has been a presenting feature in this entity, so the ophthalmologist must inquire about audiovestibular symptoms in patients with inflammatory ocular disease.