title: Immunosuppressive therapy for autoimmune inner ear disease.
abstract: Autoimmune inner ear disease (AIED) is a rare disease that is diagnosed after clinical suspicion and response to corticosteroids. AIED manifests as progressive, bilateral, although often asynchronous, sensorineural hearing loss and can be associated with vestibular symptoms. Since its description as a defined disease entity in 1979, the initial mainstay of treatment remains high-dose corticosteroids. Several animal models have been developed to assist in determining efficacy of immunosuppression in AIED, and several clinical studies have also investigated the role of both steroid and steroid-sparing treatments. Here we discuss the basic science and clinical research surrounding the history of immunosuppressive therapy in AIED.
authors.affiliation: Cleveland Clinic, Lerner Research Institute, Department of Immunology, 9500 Euclid Avenue, Cleveland, OH 44195, USA. tuohyv@ccf.org
authors.forename: Maria C
authors.initials: MC
authors.lastname: Buniel
authors.forename: Katie
authors.initials: K
authors.lastname: Geelan-Hansen
authors.forename: Peter C
authors.initials: PC
authors.lastname: Weber
authors.forename: Vincent K
authors.initials: VK
authors.lastname: Tuohy
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D001327:Autoimmune Diseases / Q000188:drug therapy* / Q000276:immunology / Q000503:physiopathology; D035843:Biomedical Research; D006319:Hearing Loss, Sensorineural; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007759:Labyrinth Diseases / Q000188:drug therapy* / Q000276:immunology / Q000503:physiopathology; D023421:Models, Animal
chemical_list: D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents