27. Cogan's Syndrome

  1. Gary S. Hoffman MD, MS3,
  2. Cornelia M. Weyand MD, PhD4,
  3. Carol A. Langford MD, MHS3 and
  4. Jörg J. Goronzy MD, PhD4
  1. Rex M. McCallum MD, FACP, FACR1 and
  2. E. William St. Clair MD2

Published Online: 3 MAY 2012

DOI: 10.1002/9781118355244.ch27

Inflammatory Diseases of Blood Vessels, Second Edition

Inflammatory Diseases of Blood Vessels, Second Edition

How to Cite

McCallum, R. M. and St. Clair, E. W. (2012) Cogan's Syndrome, in Inflammatory Diseases of Blood Vessels, Second Edition (eds G. S. Hoffman, C. M. Weyand, C. A. Langford and J. J. Goronzy), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781118355244.ch27

Editor Information

  1. 3

    Department of Rheumatic and Immunologic Diseases, Center for Vasculitis Care and Research, Cleveland Clinic, Lerner College of Medicine, Cleveland, OH, USA

  2. 4

    Department of Medicine, Stanford University School of Medicine, Stanford, CA, USA

Author Information

  1. 1

    Department of Medicine, Division of Rheumatology, University of Texas Medical Branch, Galveston, TX, USA

  2. 2

    Department of Medicine, Division of Rheumatology and Immunology, Duke University Arthritis Center, Duke University Medical Center, Durham, NC, USA

Publication History

  1. Published Online: 3 MAY 2012
  2. Published Print: 8 JUN 2012

ISBN Information

Print ISBN: 9781444338225

Online ISBN: 9781118355244



  • Cogan's syndrome;
  • autoimmune hearing loss;
  • interstitial keratitis;
  • cochlear hydrops;
  • vasculitis;
  • aortitis;
  • vertigo;
  • oscillopsia


Cogan's syndrome (CS) is characterized by interstitial keratitis, which rarely produces blindness, and recurrent Meniere's-like vestibulo-auditory events with frequent progression to hearing loss and deafness. It is a rare disease with a mean age of onset at 29 years. The mortality rate is unknown. Approximately half of the patients report an antecedent upper respiratory illness. Inflammatory cardiovascular disease develops in 15% of patients. The diagnosis of CS requires the presence of ocular inflammation and inner ear dysfunction not explained by another illness. Because the differential diagnostic considerations in CS cross a broad spectrum of illnesses and toxicities, the proper assessment of CS and its management often requires the collaboration of numerous specialties. The exact etiology of CS remains a mystery, although immune mechanisms likely have a pivotal role in its pathogenesis. Treatment consists typically of corticosteroids and the judicious use of other immunosuppressive therapies.