National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Cogan's syndrome


Other Names:
Cogan syndrome
Cogan syndrome is a rare autoimmune disease that affects the eyes and inner ears. Symptoms of the syndrome include irritation and pain in the eyes, decreased vision, hearing loss, and vertigo. Other symptoms may include joint or muscle pain or inflammation of the blood vessels.[1] 

The exact cause of Cogan syndrome is not well-understood. It is thought that the syndrome is caused by an autoimmune response that causes the immune system to attack the tissues of the eyes and ears. Cogan syndrome is not known to run in families.[2] Diagnosis of Cogan syndrome is based on observing symptoms associated with the syndrome and ruling out other possible causes of the symptoms. Treatment options generally include corticosteroids and immunosuppressive agents.[2] 
Last updated: 12/5/2017
Cogan syndrome primarily affects the eyes and the inner ears. Symptoms of Cogan syndrome generally begin in adolescence to mid-adulthood. The first symptoms typically either affect only the inner ears or only the eyes but often progress to affect both the eyes and the ears. Symptoms affecting the eyes include redness, irritation and pain, excessive tear production, sensitivity to light (photophobia), and decreased vision. When the eyes are examined by an ophthalmologist, swelling of specific tissues of the eye (interstitial keratitis) may be identified.[1][3] Symptoms affecting the ears may include sensorineural hearing loss, ringing in the ears (tinnitus) and dizziness (vertigo).[1] 

Cogan syndrome can also affect the blood vessels. This can cause symptoms in other parts of the body including pain or cramping in the muscles (myalgia), fever, headache, diarrhea, and stomach pain. In some cases, people with Cogan syndrome may have a heart murmur or other heart problems.[1][2] 
Last updated: 12/5/2017
Cogan syndrome is an autoimmune disease, which means that it occurs when the immune system begins to attack the tissues of the body. This is called an autoimmune response. Specifically, the immune systems of people with Cogan syndrome begin to attack the tissues of the eyes and the inner ears. In some cases, the autoimmune response may also be directed against blood vessels.[1] The exact reason that people with Cogan syndrome have an autoimmune response against the tissues of the eyes and the inner ears is not well-understood.[2] For some people, it may be that symptoms of Cogan syndrome begin after an infection.[3][4]
Last updated: 12/5/2017
Changes (mutations) in a specific gene are not known to cause Cogan syndrome. This means that Cogan syndrome is not thought to be passed directly from parent to child, and the syndrome is not thought to run in families.[2] In general, family members of an individual with an autoimmune disease are thought to be at an increased risk to develop an autoimmune disease themselves. Although there are not reports of Cogan syndrome running in families, it is possible that family members of people with Cogan syndrome are at an increased risk to develop other autoimmune diseases. 
Last updated: 12/5/2017
Cogan syndrome is diagnosed when a doctor observes signs and symptoms consistent with the syndrome. If Cogan syndrome is suspected, other diseases that may have similar signs and symptoms must be excluded. These diseases include syphilis, Lyme disease, Epstein-Barr virus, and Meniere disease.[1][2] Laboratory tests to exclude other diseases may include blood tests, urinalysis, and studies to analyze liver function.[2] In some cases, blood tests for a specific antibody related to Cogan syndrome may be completed.[2][3]

If a diagnosis of Cogan syndrome is suspected, it is suggested that the affected individual see an ophthalmologist and otolaryngologist to determine if there are other symptoms consistent with Cogan syndrome.[2] 
Last updated: 12/5/2017
Most people with Cogan syndrome respond well to corticosteroids or immunosuppressive agents. However, in some cases the medications may not be effective, or the syndrome may have progressed too quickly for the medications to relieve symptoms. In these cases, the symptoms of Cogan syndrome may lead to permanent vision or hearing loss.[1] Permanent hearing loss is more common than permanent vision loss, and hearing loss may not be improved with the use of medications.[3] The medications used to treat Cogan syndrome may have side-effects, so the risks and benefits of each medication must be considered.[5]

The long-term outlook for people with Cogan syndrome also depends on the involvement of the blood vessels. If the blood vessels are affected by the autoimmune response associated with Cogan syndrome and the treatment is not effective, symptoms such as stomach pain or headaches may persist. Life-threatening complications of Cogan syndrome are rare, but may include an aneurysm of the aorta.[2][3]
Last updated: 12/5/2017

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Cogan's syndrome. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Patient Registry

  • The Autoimmune Registry supports research for Cogan's syndrome by collecting information about patients with this and other autoimmune diseases. You can join the registry to share your information with researchers and receive updates about participating in new research studies. Learn more about registries.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Organizations Providing General Support

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

  1. Roat MI. Cogan Syndrome. Merck Manuals. December 2016; http://www.merckmanuals.com/professional/eye-disorders/corneal-disorders/cogan-syndrome.
  2. Cimaz R and Lega JC. Cogan syndrome. Orphanet. October 2014; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=1467.
  3. Hain TC. Cogan’s Syndrome. American Hearing Research Foundation. October 2012; http://american-hearing.org/disorders/cogans-syndrome/.
  4. Greco A, Gallo A, Fusconi M, Magliulo G, Turchetta R, Marinelli C, Macri GF, De Virgilio A, and de Vincentiis M. Cogan’s syndrome: An autoimmune inner ear disease. Autoimmunity Reviews. January 2013; 12(3):396-400. https://www.ncbi.nlm.nih.gov/pubmed/22846458.
  5. McCallum R and St. Clair EW. Cogan’s syndrome. The Vasculitis Foundation. March 2013; http://www.vasculitisfoundation.org/mcm_article/cogans-syndrome/.