title: Endolymphatic hydrops as a cause of audio-vestibular manifestations in relapsing polychondritis.
abstract: Relapsing polychondritis (RP) is characterized by inflammation and subsequent degeneration of cartilage. We report a 61-year-old woman who had RP with audio-vestibular manifestations. She was also diagnosed as having a myelofibrosis with myeloid metaplasia (MMM). Bilateral endolymphatic hydrops (EH) was confirmed by dominant -SP/AP of the electrocochleogram (ECochG). When thalidomide and prednisolone were prescribed for the treatment of MMM, symptoms of RP -- including the inner ear dysfunction -- were ameliorated. Isosorbide, one of the osmotic diuretics commonly used for the treatment of Meniere's disease (MD) in Japan, was also effective in keeping her free from inner ear dysfunction. This is the first report to confirm the existence of EH in a patient with RP with audio-vestibular manifestations. We suppose that an immunological imbalance due to MMM, in conjunction with a specific immunogenetic background, may have played a role in the pathogenesis of RP and the formation of EH in this patient.
authors.affiliation: Department of Otolaryngology, Osaka University School of Medicine, Osaka, Japan. jmurata@ent.med.osaka-u.ac.jp
authors.forename: Junko
authors.initials: J
authors.lastname: Murata
authors.forename: Arata
authors.initials: A
authors.lastname: Horii
authors.forename: Manabu
authors.initials: M
authors.lastname: Tamura
authors.forename: Kenji
authors.initials: K
authors.lastname: Mitani
authors.forename: Masao
authors.initials: M
authors.lastname: Mizuki
authors.forename: Takeshi
authors.initials: T
authors.lastname: Kubo
mesh_terms: D000208:Acute Disease; D001300:Audiometry, Evoked Response; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D002097:C-Reactive Protein / Q000378:metabolism; D003937:Diagnosis, Differential; D004234:Diuretics, Osmotic / Q000008:administration & dosage; D004305:Dose-Response Relationship, Drug; D004359:Drug Therapy, Combination; D004427:Ear Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D004431:Ear, External; D018159:Endolymphatic Hydrops / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D006801:Humans; D007075:Immunoglobulin M / Q000097:blood; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D007547:Isosorbide / Q000008:administration & dosage; D008575:Meniere Disease / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D008875:Middle Aged; D011081:Polychondritis, Relapsing / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D011241:Prednisone / Q000008:administration & dosage; D055728:Primary Myelofibrosis / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D013792:Thalidomide / Q000008:administration & dosage; D016896:Treatment Outcome
chemical_list: D004234:Diuretics, Osmotic; D007075:Immunoglobulin M; D007166:Immunosuppressive Agents; D013792:Thalidomide; D002097:C-Reactive Protein; D011241:Prednisone; D007547:Isosorbide