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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. This eye condition causes pain, lacrimation (tearing of the eye) and photophobia (eye pain with exposure to light). Shortly following these ocular (eye) symptoms, patients develop bilateral audiovestibular (ear) 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. Other symptoms that may occur include headache, fever, arthralgia (joint pain), and systemic vasculitis (inflammation of the blood vessels). The symptoms typically deteriorate progressively within days. 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

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]

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]

This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).

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