Cogan's syndrome: clinical evolution of deafness and vertigo in three patients.
The aim of this study was to evaluate the clinical symptoms, the otoneurological examinations, the treatment and the clinical course of three patients suffering from Cogan's syndrome, a rare disease based on the clinical association of a non-syphilitic interstitial keratitis with a cochleo-vestibular deficit. This case series involved three patients with follow up. The clinical course of the three patients (aged 30, 48 and 49 years) with Cogan's syndrome during a follow-up period of 2 to 6 years is reported. All patients underwent complete otoneurological, ophthalmologic and rheumatologic examinations and were treated with immunosuppressive therapy such as glucocorticoids and cyclophosphamide in two and glucocorticoids and methotrexate in one patient. Using immunosuppressive therapy, ophthalmologic symptoms disappeared rapidly in two patients. Hearing improved only in one and stabilized in a second patient. One patient died after 6 years of treatment because of complications of generalized vasculitis. Early diagnosis and rapid initiation of a combined immunosuppressive therapy such as corticosteroids and cyclophosphamide seem to be important in controlling the disease and avoiding persistent deafness. Whether systemic complications and a fatal outcome also can be prevented is still questionable.
Department of ENT and Head and Neck Surgery, University Hospital Inselspital, 3010, Berne, Switzerland.
A
A
Baumann
A
A
Helbling
S
S
Oertle
R
R
Häusler
D
D
Vibert
D000328:Adult; D001301:Audiometry, Pure-Tone; D003051:Cochlea / Q000503:physiopathology; D003316:Corneal Diseases / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology*; D003520:Cyclophosphamide / Q000627:therapeutic use; D018450:Disease Progression; D004359:Drug Therapy, Combination; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology; D005260:Female; D005500:Follow-Up Studies; D006319:Hearing Loss, Sensorineural / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007634:Keratitis / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology*; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D013577:Syndrome; D014717:Vertigo / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology*; D014722:Vestibule, Labyrinth / Q000503:physiopathology
D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D008727:Methotrexate