title: Atypical Cogan's syndrome: a case report and summary of current treatment options.
abstract: Cogan's syndrome is a rare chronic vasculitis, characterized in its typical form by progressive sensorineural bilateral hearing loss, vestibular symptoms and non-syphilitic interstitial keratitis. Only a few cases have been reported in children, most of whom have been diagnosed with the typical form. Early diagnosis and treatment are crucial to ensure a favorable prognosis. Systemic treatment usually begins with high dosage corticosteroids. In case the initial treatment fails, other immunosuppressive drugs are used (cyclophosphamide, methotrexate, cyclosporine A and azathioprine). Additional treatment possibilities, such as plasmapheresis, TNF-alpha blockers (etanercept and infliximab), rituximab, tocilizumab and mycophenolate mofetil have been described over the past few years.
authors.affiliation: Department of Otorhinolaryngology, University Hospital Ostrava, 17 listopadu 1790, 708 52 Ostrava, Czech Republic.
authors.forename: Debora
authors.initials: D
authors.lastname: Jančatová
authors.affiliation: Department of Otorhinolaryngology, University Hospital Ostrava, 17 listopadu 1790, 708 52 Ostrava, Czech Republic; Faculty of Medicine, University of Ostrava, Syllabova 19, 703 00 Ostrava, Czech Republic. Electronic address: karol.zelenik@fno.cz.
authors.forename: Karol
authors.initials: K
authors.lastname: Zeleník
authors.affiliation: Department of Otorhinolaryngology, University Hospital Ostrava, 17 listopadu 1790, 708 52 Ostrava, Czech Republic; Faculty of Medicine, University of Ostrava, Syllabova 19, 703 00 Ostrava, Czech Republic.
authors.forename: Pavel
authors.initials: P
authors.lastname: Komínek
authors.affiliation: Department of Otorhinolaryngology, University Hospital Ostrava, 17 listopadu 1790, 708 52 Ostrava, Czech Republic; Faculty of Medicine, University of Ostrava, Syllabova 19, 703 00 Ostrava, Czech Republic.
authors.forename: Petr
authors.initials: P
authors.lastname: Matoušek
mesh_terms: D000293:Adolescent; D001379:Azathioprine / Q000627:therapeutic use; D055952:Cogan Syndrome / Q000175:diagnosis* / Q000188:drug therapy*; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006312:Hearing Loss, Bilateral / Q000209:etiology; D006319:Hearing Loss, Sensorineural / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008775:Methylprednisolone / Q000627:therapeutic use; D009325:Nausea / Q000209:etiology; D014717:Vertigo / Q000209:etiology; D014839:Vomiting / Q000209:etiology
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D001379:Azathioprine; D008775:Methylprednisolone
keywords: Cogan's syndrome; Corticosteroids; Immunosuppressive therapy; Interstitial keratitis; Sensorineural hearing loss; Vertigo