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Cogan's syndrome is a chronic inflammatory disorder of unknown cause affecting mostly young adults. Two main observation of the disease are bilateral interstitial keratitis and vestibuloauditory dysfunction. Association between Cogan's syndrome and systemic vasculitis as well as aortitis are exist. The diagnosis of the disease is based upon the presence of inflammatory eye disease and vestibuloauditory dysfunction . In this article, the classic Cogan's syndrome has been reported in a 47-year-old woman. Two months prior to admission, the patient had been suffering from headache, vertigo, nausea, vomiting, right leg claudication, musculoskeletal pains, bilateral hearing loss and blindness. Ophthalmologic examination revealed that visual acuity was 0.1 bilaterally and on slit lamp examination, there was a conjunctival hyperemia, bilateral cataract and interstitial keratitis. Pure tone audiogram (PTA) and auditory brain stem response (ABR) showed bilateral sensorineural hearing loss. The patient was initially treated with pulse intravenous methylprednisolone and was followed by oral prednisolone and cyclophosphamide, which in follow-up showed partial improvement.