Bilateral progressive hearing loss and vestibular dysfunction with inner ear antibodies.
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.
Yukawa
Kumiko
K
Department of Otolaryngology, Tokyo Medical University, 6-7-1 Nishishinjuku Shinjuku-ku, Tokyo, Japan. spicemomo@hotmail.co.jp
Hagiwara
Akira
A
Ogawa
Yasuo
Y
Nishiyama
Nobuhiro
N
Shimizu
Shigetaka
S
Kawaguchi
Sachie
S
Nakamura
Mari
M
Ito
Hiroyuki
H
Tomiyama
Shunichi
S
Suzuki
Mamoru
M
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
D000893:Anti-Inflammatory Agents; D001323:Autoantibodies; D005839:Gentamicins; D007166:Immunosuppressive Agents; D001623:Betamethasone; D011239:Prednisolone