Condition 101 About Cogan's Syndrome

What is the definition of Cogan's 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. 

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. 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. 

What are the alternative names for Cogan's Syndrome?

  • Cogan syndrome

What are the causes for Cogan's Syndrome?

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. 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. For some people, it may be that symptoms of Cogan syndrome begin after an infection.

What are the symptoms for Cogan's Syndrome?

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. Symptoms affecting the ears may include sensorineural hearing loss, ringing in the ears (tinnitus) and dizziness (vertigo). 

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. 

What is the outlook (prognosis) for Cogan's Syndrome?

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. Permanent hearing loss is more common than permanent vision loss, and hearing loss may not be improved with the use of medications. The medications used to treat Cogan syndrome may have side-effects, so the risks and benefits of each medication must be considered.

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.

How is Cogan's Syndrome diagnosed?

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. Laboratory tests to exclude other diseases may include blood tests, urinalysis, and studies to analyze liver function. In some cases, blood tests for a specific antibody related to Cogan syndrome may be completed.

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. 

Is Cogan's Syndrome an inherited disorder?

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. 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. 

Top Global Doctors For Cogan's Syndrome

Thierry Zenone
Valence, FR
Paolo C. Mora
Parma, IT
Jelka G. Orsoni
Parma, IT

Latest Advances On Cogan's Syndrome

  • Condition: Cogan's syndrome during pregnancy
  • Journal: BMJ case reports
  • Treatment Used: Intravenous immunoglobulin (IVIG)
  • Number of Patients: 1
  • Published —
The purpose of the study was to report a case of a woman with Cogan's syndrome who was treated with intravenous immunoglobulin.
  • Condition: Ocular Surface Discomfort with Cogan's Epithelial Basement Membrane Dystrophy (EMBD)
  • Journal: Journal of ocular pharmacology and therapeutics : the official journal of the Association for Ocular Pharmacology and Therapeutics
  • Treatment Used: Topical Heparan Sulfate Mimetic Polymer (HSMP)
  • Number of Patients: 22
  • Published —
This study tested the safety and efficacy of using HSMP to treat patients with ocular surface discomfort with EMBD.

Clinical Trials For Cogan's Syndrome

Clinical Trial
  • Status: Recruiting
  • Participants: 20000
  • Start Date: July 2010
Coordination of Rare Diseases at Sanford