Cogan's Syndrome

Timothy C. Hain, MD
Last update: 06/20/2008

• What is Cogan’s syndrome?
• What causes Cogan’s syndrome?
• How is Cogan’s syndrome treated?
• References
  
  
  What is Cogan’s syndrome?
  
  Cogan's syndrome is defined as nonsyphilitic interstitial keratitis (an inflammation of the eye) and bilateral audiovestibular deficits (hearing problems and dizziness). It is more common in Caucasians than in other races. 
  
  Onset of the disease is generally a brief episode of inflammatory eye disease, most commonly interstitial keratitis.  Other inflammatory eye conditions that may occur include uveitis, episcleritis or scleritis.  These  eye conditions cause pain, lacrimation (tearing of the eye) and photophobia (eye pain with exposure to light).
  
  Shortly following these ocular symptoms, patients develop bilateral audiovestibular symptoms, including hearing loss, vertigo (dizziness) and tinnitus (ringing in the ears).  Approximately half of patients ultimately develop complete hearing loss, but only a minority experience permanent visual loss.
  
  Systemic symptoms that may occur include headache, fever, arthralgia (joint pain), and systemic vasculitis (inflammation of the blood vessels).  Vasculitis may affect any blood vessels, including the aorta.  Aortitis placed patients at an increased risk of aortic aneurysm, a potentially fatal complication. 
  
  The symptoms typically deteriorate progressively within days. While the ESR, WBC, and C-reactive protein test may be abnormal and there may be thrombocytosis or anemia, none of these findings are reliable indicators of the disease. A slit-lamp examination is essential. Recent work has suggested that high-resolution MRI and antibodies to inner ear antigens may be helpful. Very little has been published in recent years on Cogan's syndrome.
  
  What causes Cogan’s syndrome?
  
  It is currently thought that Cogan’s syndrome is an autoimmune disease.  The inflammation in the eye and ear are due to the patient’s own immune system producing antibodies that attack the inner ear and eye tissue.  Autoantibodies can be demonstrated in the blood of some patients, and these antibodies have been shown to attack inner ear tissue in laboratory studies.  Infection with the bacteria Chlamydia pneumoniae has been demonstrated in some patients prior to the development of Cogan’s syndrome, leading some researchers to hypothesis that the autoimmune disease may be initiated by the infection.  Chlamydia pneumoniae is a common cause of mild pneumonia, and the vast majority of patients who are infected with the bacteria do not develop Cogan’s syndrome.
  
  How is Cogan’s syndrome treated?
  
  Immediate treatment for Cogan’s syndrome is with steroids, which reduce the inflammatory response.  TNF-alpha is powerful anti-inflammatory agent that has been used in a small number of patients who do not respond to steroid treatment.  Once hearing loss has occurred, anti-inflammatory medication may not improve hearing.  Cochlear implantation may be used in some patients, and studies have shown it to produce significant improvement in hearing loss in some patients.
  A patient with Cogan's syndrome volunteered to put her story on line, and it can be found by clicking here.
  See also: Autoimmune Inner Ear Disease
  
  References:
  
  
• Andler, W., M. Hulse, et al. (1977). "[Cogans's syndrome in childhood (author's transl)]." Monatsschr Kinderheilkd 125(3): 161-4.
  
  
• Cundiff J, Kansal S et al.  Cogan’s syndrome: a cause of progressive hearing deafness.  Am J Otolaryn.  27(1):68-70, 2006 Jan-Feb.
  
  
• Fricker M, Baumann A et al.  A novel therapeutic option in Cogan diseases? TNF-alpha blockers.  Rheum Int.  27(5):493-5, 2007 Mar.
  
  
• Gluth MB, Baratz KH et al.  Cogan syndrome: a retrospective review of 60 patients throughout a half century.  Mayo Clin Proc.  81(4):483-8, 2006 Apr.
  
  
• Goncalves RM, Curi AL et al.  Posterior scleritis in Cogan’s syndrome.  Ocular Immun and Inflam.  12(2):149-52, 2004 Jun.
  
  
• Grasland A, Puchot J et al.  Typical and atypical Cogan’s syndrome: 32 cases and review of the literature.  Rheum. 43(8):1007-15, 2004 Aug.
  
  
• Helmchen C, Jager L, Buttner U, Reiser M, Brandt T. Cogan's syndrome: High resolution MRI indicators of activity. JVR 8,2,155-167, 1998.
  
  
• Lepur D, Vranjican Z et al.  Chlamydia pneumoniae infection as a trigger for Cogan’s syndrome.  J Inf.  52(3):223-6, 2006 Mar.
  
  
• Low AH, Su JW et al.  Cogan’s syndrome with recurrent carotid and aortic aneurysms: a potentially fatal disorder mimicking Marfan syndrome.  Scan J Rheum. 36(1):71-3, 2007 Jan-Feb.
  
  
• Lunadi C, Bason C et al.  Autoantibodies to inner ear and endothelial antigens in Cogan’s syndrome. Lancet.  360(9337):915-21, 2002 Sep 21.
  
  
• Pasanisi E, Vincenti V et al.  Cochlear implantation and Cogan syndrome.  Oto and Neurol.  24(4):601-4, 2003 Jul.