title: Bilateral progressive hearing loss and vestibular dysfunction with inner ear antibodies.
abstract: Autoimmune inner ear disease (AIED) is a clinical syndrome of uncertain etiology. We present the neuro-otological findings of 2 cases of bilateral hearing loss, dizziness and the antibody profiles of the inner ears. Case 1 had bilateral progressive hearing loss, vestibular dysfunction and abnormal eye movement as the disease progressed. She had inner ear antibodies against 42 and 58kDa protein antigency on Western blot immune assay, and responded to glycocorticosteroid but not to immunosuppressant treatment. Intratympanic steroid injection temporally eliminated her symptoms. However, she developed idiopathic Cushing's syndrome and underwent labyrinthectomy. Case 2 became deaf as a teenager and experienced dizziness 10 years after becoming deaf. He reacted strongly to 68kDa protein and was a good responder to immunosuppressant with steroid. As we still lack a definitive diagnostic test for AIED, careful observation of the clinical course is critical for differential diagnosis regarding the bilateral progressive hearing loss.
authors.affiliation: Department of Otolaryngology, Tokyo Medical University, 6-7-1 Nishishinjuku Shinjuku-ku, Tokyo, Japan. spicemomo@hotmail.co.jp
authors.forename: Kumiko
authors.initials: K
authors.lastname: Yukawa
authors.forename: Akira
authors.initials: A
authors.lastname: Hagiwara
authors.forename: Yasuo
authors.initials: Y
authors.lastname: Ogawa
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Nishiyama
authors.forename: Shigetaka
authors.initials: S
authors.lastname: Shimizu
authors.forename: Sachie
authors.initials: S
authors.lastname: Kawaguchi
authors.forename: Mari
authors.initials: M
authors.lastname: Nakamura
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Ito
authors.forename: Shunichi
authors.initials: S
authors.lastname: Tomiyama
authors.forename: Mamoru
authors.initials: M
authors.lastname: Suzuki
mesh_terms: D000328:Adult; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage / Q000009:adverse effects; D001323:Autoantibodies / Q000097:blood*; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D001623:Betamethasone / Q000008:administration & dosage / Q000009:adverse effects; D003131:Combined Modality Therapy; D003480:Cushing Syndrome / Q000139:chemically induced; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D007758:Ear, Inner / Q000276:immunology* / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D005839:Gentamicins / Q000008:administration & dosage; D006312:Hearing Loss, Bilateral / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000009:adverse effects; D008297:Male; D011239:Prednisolone / Q000008:administration & dosage / Q000009:adverse effects; D015837:Vestibular Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D014724:Vestibular Function Tests; D055815:Young Adult
chemical_list: D000893:Anti-Inflammatory Agents; D001323:Autoantibodies; D005839:Gentamicins; D007166:Immunosuppressive Agents; D001623:Betamethasone; D011239:Prednisolone