Cogan’s syndrome: clinical evolution of deafness and vertigo in three patients

  • A. Baumann
  • A. Helbling
  • S. Oertle
  • R. Häusler
  • D. Vibert
Otology

Abstract

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.

Keywords

Cogan’s syndrome Hearing loss Vertigo Keratitis Autoimmune disease Therapy 

Copyright information

© Springer-Verlag 2004

Authors and Affiliations

  • A. Baumann
    • 1
  • A. Helbling
    • 2
  • S. Oertle
    • 2
  • R. Häusler
    • 1
  • D. Vibert
    • 1
  1. 1.Department of ENT and Head and Neck SurgeryUniversity Hospital InselspitalBerneSwitzerland
  2. 2.Clinic of Rheumatology and Policlinic of Clinical Immunology and AllergologyUniversity Hospital InselspitalBerneSwitzerland

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