This rare autoimmune syndrome is characterized by bilateral interstitial keratitis with profound sensorineural hearing loss. There can be systemic vasculitic manifestations, and there are both typical and atypical presentations described . There can be an association with both ANCA antibodies and rheumatoid factor suggestive of a potential overlap in terms of pathogenesis and potential contribution to vasculitis. There can be associated larger artery aneurysm formation which can include the carotid artery . This is reflective of the larger vessel involvement seen in both Cogan’s syndrome and Behcet’s disease . Of note, there is considerable overlap in the manifestations of these two disorders with both diagnosed on the basis of clinical manifestations with lack of confirmatory diagnostic testing. Despite its recognition as a cause of CNS vasculitis, it is felt that Cogan’s syndrome is not only quite rare but uncommonly results in neurological manifestations .