title: Autoimmune inner ear disease: diagnostic and therapeutic approaches in a multidisciplinary setting.
abstract: Autoimmune Inner Ear Disease (AIED) is a clinical syndrome of uncertain pathogenesis. It is associated with bilateral rapidly progressive hearing loss. The hearing loss may be associated with vestibular symptoms. Autoimmunity has been proposed as the pathogenesis of this sort of hearing loss, although the mechanism of the disease is poorly understood. It is well accepted that the endolymphatic sac is an immunocompetent organ and circulating autoantibodies against inner ear antigens have been reported, as have viral antigens in the endolymph, although the sensitivity, specificity, and roles of those antibodies in a disease process are poorly defined. We will describe the clinical aspects of the disease, the histopathology, the immunologic indicators, the types of presentation, both from the audiologic and vestibular point of view, clinical trials for treatment and the follow-up. One of our conclusions is that many of these patients respond favorably to the treatment Methotrexate.
authors.affiliation: Department of Internal Medicine, Division of Rheumatology Mayo Clinic and Mayo Graduate School of Medicine Rochester, Minnesota 55905, USA.
authors.forename: Eric L
authors.initials: EL
authors.lastname: Matteson
authors.forename: David A
authors.initials: DA
authors.lastname: Fabry
authors.forename: Scott E
authors.initials: SE
authors.lastname: Strome
authors.forename: Colin L W
authors.initials: CL
authors.lastname: Driscoll
authors.forename: Charles W
authors.initials: CW
authors.lastname: Beatty
authors.forename: Thomas J
authors.initials: TJ
authors.lastname: McDonald
mesh_terms: D001323:Autoantibodies / Q000378:metabolism; D001324:Autoantigens / Q000378:metabolism; D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D016022:Case-Control Studies; D004427:Ear Diseases / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D004710:Endolymph / Q000276:immunology; D004712:Endolymphatic Sac / Q000276:immunology; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D014055:Tomography, Emission-Computed; D014717:Vertigo / Q000209:etiology
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D007166:Immunosuppressive Agents; D008727:Methotrexate