Cogan’s Syndrome

Timothy C. Hain, MD

Last update: 10/2012

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.

Learn about a patient with Cogan’s syndrome.

See also: Autoimmune Inner Ear Disease

References:

  • Andler W, Hulse M, Bruch PM, Partsch CJ. 1977. [Cogans's syndrome in childhood (author's transl)]. Monatsschrift fur Kinderheilkunde 125: 161-4
  • Antonios N, Silliman S. 2012. Cogan syndrome: an analysis of reported neurological manifestations. The neurologist 18: 55-63
  • Balaskas K, Majo F, Ikonomidis C, Guex-Crosier Y. 2011. Contribution of Serology in the Diagnosis of Cogan’s Syndrome. Klinische Monatsblatter fur Augenheilkunde 228: 352-3
  • Bovo R, Ciorba A, Trevisi P, Aimoni C, Cappiello L, et al. 2011. Cochlear implant in Cogan syndrome. Acta oto-laryngologica 131: 494-7
  • Cundiff J, Kansal S, Kumar A, Goldstein DA, Tessler HH. 2006. Cogan’s syndrome: a cause of progressive hearing deafness. American journal of otolaryngology 27: 68-70
  • Fricker M, Baumann A, Wermelinger F, Villiger PM, Helbling A. 2007. A novel therapeutic option in Cogan diseases? TNF-alpha blockers. Rheumatology international 27: 493-5
  • Gluth MB, Baratz KH, Matteson EL, Driscoll CL. 2006. Cogan syndrome: a retrospective review of 60 patients throughout a half century. Mayo Clinic proceedings. Mayo Clinic 81: 483-8
  • Goncalves RM, Curi AL, Campos WR, Orefice F, Machado DO. 2004. Posterior scleritis in Cogan’s syndrome. Ocular immunology and inflammation 12: 149-52
  • Grasland A, Pouchot J, Hachulla E, Bletry O, Papo T, Vinceneux P. 2004. Typical and atypical Cogan’s syndrome: 32 cases and review of the literature. Rheumatology (Oxford) 43: 1007-15
  • Greco A, Gallo A, Fusconi M, Magliulo G, Turchetta R, et al. 2012. Cogan’s syndrome: An autoimmune inner ear disease. Autoimmunity reviews
  • Kontorinis G, Giourgas A, Neuburger J, Lesinski-Schiedat A, Lenarz T. 2010. Long-term evaluation of Cochlear implantation in Cogan syndrome. ORL; journal for oto-rhino-laryngology and its related specialties 72: 275-9
  • Lepur D, Vranjican Z, Himbele J, Klinar I, Barsic B. 2006. Chlamydia pneumoniae infection as a trigger for a Cogan’s syndrome. The Journal of infection 52: 223-6
  • Low AH, Su JW, Sin KY, Fong KY, Thumboo J. 2007. Cogan’s syndrome with recurrent carotid and aortic aneurysms: a potentially fatal disorder mimicking Marfan syndrome. Scandinavian journal of rheumatology 36: 71-3
  • Wang JR, Yuen HW, Shipp DB, Stewart S, Lin VY, et al. 2010. Cochlear implantation in patients with autoimmune inner ear disease including cogan syndrome: a comparison with age- and sex-matched controls. The Laryngoscope 120: 2478-83