Posterior Scleritis with Optic Nerve Edema Presenting in a Case of Atypical Cogan’s Syndrome

Cogan's syndrome (CS) is a rare, systemic autoimmune vasculitis characterized by ocular and cochlear manifestations (Yaginuma et al.). Features of typical CS are non-syphilitic interstitial keratitis (IK) within 2 years of Ménière-like sensorineural hearing loss, characterized by vertigo, instability, nausea, vomiting, and tinnitus (Espinoza et al.). Atypical CS is defined as having other forms of ocular inflammation (with or without IK), such as episcleritis, scleritis, optic neuritis, non-Ménière-like sensorineural hearing loss, or greater than 2 years between the ocular and audiovestibular manifestations. Ocular involvement has a good prognosis with treatment; however, 38% of atypical CS patients become deaf in both ears. However, prompt diagnosis and treatment with oral Prednisone of at least 1 mg/kg/day can prevent permanent hearing loss (Grasland et al.). This disease can also affect multiple organ systems, and although it remains idiopathic, its proposed mechanism is believed to be autoimmune in nature (Espinoza et al.). We report the clinical course of a young female presenting with ocular and auditory symptoms without systemic involvement, ultimately leading to a diagnosis of atypical Cogan's syndrome.