Cogan’s Syndrome: Atypical Ophthalmic Manifestations

Abstract

The purpose of this report is to describe a case of Cogan’s syndrome (CS) with atypical ophthalmic features, such as diffuse anterior scleritis and bilateral peripheral ulcerative keratitis. The article also discusses the clinical aspects, treatment and prognosis of this rare disease. A 66-year-old woman presented with bilateral a symmetric diffuse anterior scleritis and peripheral ulcerative keratitis. Two years later, the patient complained of hearing loss and the audiometry showed sensorineural hearing impairment. The ancillary tests for infectious or autoimmune diseases were unremarkable, leading to the diagnosis of atypical CS. Therefore, the relevance of this article is to emphasize that CS should be considered in patients with recurrent eye inflammation and audiovestibular symptoms. The peripheral ulcerative keratitis is an atypical finding that has not been previously described related to CS. The diagnosis of the syndrome in the early stages is important for appropriate therapy and prevention of hearing impairment and permanent visual damage.