Cogan's Syndrome

What Is It?

Cogan's syndrome is a rare, rheumatic disease characterized by inflammation of the ears and eyes. Cogan's syndrome can lead to vision difficulty, hearing loss and dizziness. The condition may also be associated with blood-vessel inflammation (called vasculitis) in other areas of the body. It most commonly occurs in a person's 20s or 30s. The cause is not known. However, one theory is that it is an autoimmune disorder in which the body's immune system mistakenly attacks tissue in the eye and ear.

Symptoms

The most common symptoms of Cogan's syndrome include:

• Red, painful, light-sensitive eyes and/or blurred vision
• Hearing loss, which may become profound and permanent
• Vertigo (a sensation of the room spinning; may be called dizziness)
• Poor balance
• Nausea, vomiting
• Fever, fatigue, weight loss

More rarely, Cogan's syndrome can cause enlarged lymph nodes, rash, chest pain, arm pain and shortness of breath.

Diagnosis

The diagnosis of Cogan's syndrome is established by finding the typical combination of problems associated with this disease in the eye and inner ear. Until both the eye and the inner ear are affected, the diagnosis may be uncertain. Either area may be involved first, or the eye and ear problems may develop at the same time.

Your doctor will want to rule out an infection (especially syphilis, tuberculosis, viral infection and chlamydia) or another rheumatic disease (including rheumatoid arthritis, sarcoidosis and Wegener's granulomatosis) as the cause of your symptoms. In addition, a detailed eye examination by an ophthalmologist is important to look for a particular type of corneal inflammation (called interstitial keratitis) or other eye abnormalities. Careful examinations of the nervous system (for example, reflexes, strength and sensation) and the ears, including hearing tests and specialized tests of balance and inner-ear function, are also important.

Expected Duration

Cogan's syndrome is a chronic disease. The disease activity may wax and wane or respond well to treatment, but usually the disease is prolonged or even lifelong.

Prevention

There is no way to prevent Cogan's syndrome.

Treatment

Anti-inflammatory medications, including steroids and nonsteroidal anti-inflammatory drugs (NSAIDs), that are applied to the eye may be effective for mild eye disease. If these medications do not work well enough, oral antibiotics, such as tetracycline (sold under many brand names), may be recommended in case an infection is causing the eye inflammation.