title: Diagnostic and therapeutic dilemmas in rapidly progressive sensorineural hearing loss and sudden deafness. A reappraisal of immune reactivity in inner ear disorders.
abstract: Sera from 76 patients with a clinical diagnosis of idiopathic rapidly progressive sensorineural hearing loss (SNHL) (n = 15), sudden deafness (n = 31) and with other etiologies of their hearing loss (n = 30) were analysed by western blot assay. Seventy-three percent of the cases with rapidly progressive SNHL had cross-reacting antibodies (27, 45, 50, 68 kD). The overall response to immunoprogressive therapy was effective in only 50% of cases. Sixty-five percent of the patients with sudden deafness also had cross-reacting antibodies (27, 45, 50, 80 kD). In these cases steroid therapy was more effective in re-establishing the hearing than no treatment, regardless of the western blot outcome. Spontaneous recovery occurred in approx. 50% of cases, but only in those with a positive assay. The antigenic epitopes detected with immunoblotting were not cochlea specific; they were also found in protein extracts of other organs (cranial nerves, kidney, brain).
authors.affiliation: Department of Otorhinolaryngology, Utrecht University, The Netherlands.
authors.forename: J E
authors.initials: JE
authors.lastname: Veldman
authors.forename: T
authors.initials: T
authors.lastname: Hanada
authors.forename: F
authors.initials: F
authors.lastname: Meeuwsen
mesh_terms: D001327:Autoimmune Diseases / Q000188:drug therapy / Q000276:immunology*; D015153:Blotting, Western; D003429:Cross Reactions; D003520:Cyclophosphamide / Q000627:therapeutic use; D000939:Epitopes / Q000276:immunology; D006319:Hearing Loss, Sensorineural / Q000188:drug therapy / Q000276:immunology*; D003639:Hearing Loss, Sudden / Q000188:drug therapy / Q000276:immunology*; D006801:Humans; D007165:Immunosuppression Therapy; D011241:Prednisone / Q000627:therapeutic use
chemical_list: D000939:Epitopes; D003520:Cyclophosphamide; D011241:Prednisone