Cogan's syndrome consists of vestibuloauditory dysfunction, ocular lesion and nonreactive serologic test for syphilis. Many researchers believe that Cogan's syndrome is an immune-mediated systemic inflammatory condition. A 41 years old female with bilateral total hearing loss, tinnitus, dizziness and ocular lesion is presented. She has been treated with steroid and had improvement in ocular and vestibular symptoms but no change in bilateral total hearing loss.