Cogan's Syndrome

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 [81]. 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 [82]. This is reflective of the larger vessel involvement seen in both Cogan’s syndrome and Behcet’s disease [83]. 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 [84].

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