We report a case of ASNHL (autoimmune sensorineural hearing loss) with systemic symptoms. The patient was a 64-year old female who consulted us with complaints of bilateral hearing loss and left tinnitus. Later, she had cranial polyneuropathy (L: V VII VIII, R: VIII XII), diabetes insipidus, headache and fever. Her blood sedimentation rate, CRP and γ-globulin were increased. Antinuclear antibodies and antihypophysis antibodies were found in her serum. Her general condition and hearing level were improved by steroid and cyclophosphamide therapy. Autoimmune vasculitis was suspected, but histopathological studies of granulation tissue around the left stapes and left superficial temporal artery were not specific. Steroid therapy is being continued.