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.

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.

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.

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.

There is no way to prevent Cogan’s syndrome.

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.

For more severe disease, oral corticosteroids may be necessary. When large amounts of steroids are required or the disease is severe and is not responding to steroid therapy, other immunosuppressive therapy often is recommended. These immunosuppressive medicines include methotrexate (Rheumatrex), cyclophosphamide (Cytoxan, Neosar), cyclosporine (Neoral, Sandimmune), or azathioprine (Imuran). In some cases, combinations of these medicines are prescribed. Vascular surgery is needed occasionally when vasculitis damages blood vessels.

Diuretic medications, which increase urination and removal of fluid from the body, may reduce balance difficulties when excess fluid is present in the inner ear. Antihistamines or benzodiazepines, such as diazepam (Valium) or clonazepam (Klonopin) and bed rest may reduce the sensation of imbalance.

When hearing is impaired and does not improve with medical treatment, cochlear implants may be helpful. Cochlear implants are electronic devices that translate sounds into electrical signals that are transmitted to the brain, bypassing the malfunctioning part of the ear.

If the front of the eye is damaged severely by inflammation, a corneal transplant can improve vision. A corneal transplant is surgery that replaces the scarred cornea with one from an organ donor.

If you experience any significant visual or auditory problems or difficulties with balance, contact your health-care provider for evaluation, especially if these symptoms persist.

Many people with Cogan’s syndrome have permanent visual or hearing deficits. The condition is fatal in less than 10 percent of patients. However, most patients are able to manage their symptoms and limit complications of the disease with therapy.

Diseases and Conditions Center

All ArmMed Media material is provided for information only and is neither advice nor a substitute for proper medical care. Consult a qualified healthcare professional who understands your particular history for individual concerns.