Cogan's syndrome

What is Cogan's syndrome?

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 that can cause major organ damage or, in a small number of cases, even death. 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 of Cogan's syndrome

The most common symptoms of Cogan's syndrome include:

• red, painful, light-sensitive eyes 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.

Diagnosing Cogan's syndrome

A doctor diagnoses Cogan's syndrome after 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 affected 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 granulomatosis with polyangiitis) as the cause of your symptoms. Your doctor will refer you to an ophthalmologist for an eye examination to look for eye problems, including a condition called interstitial keratitis, which is inflammation of the cornea, the transparent covering of the eye. Your doctor also will want to evaluate your nervous system (for example, reflexes, strength and sensation), and check your ears by testing hearing, balance and inner ear function.

Expected duration of Cogan's syndrome

Cogan's syndrome is a chronic (long-lasting) disease. Symptoms come and go, or respond well to treatment, but usually the disease is prolonged or even lifelong.

Preventing Cogan's syndrome

There is no way to prevent Cogan's syndrome.

Treating Cogan's syndrome

Mild eye disease may be treated with anti-inflammatory medications, including steroids and NSAIDs that are applied to the eye. If these medications do not work well enough, oral antibiotics, such as doxycycline (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 if the disease is severe and is not responding to steroid therapy, other immunosuppressive medications often are recommended. These immunosuppressive drugs include methotrexate (Rheumatrex), infliximab (Remicade), or azathioprine (Imuran). In some cases, other immunosuppressive medications or combinations of medicines are prescribed. Occasionally, if the disease has damaged blood vessels, surgery may need to be done to correct the problem.

If excess fluid in the inner ear is causing balance problems, your doctor may prescribe diuretic medications, which increase urination and removal of fluid from the body. A sense of imbalance may be treated with antihistamines, such as meclizine (Antivert), or benzodiazepines, such as diazepam (Valium) or clonazepam (Klonopin) and bed rest.

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. Part of the device is implanted in the ear, and part is worn outside 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.

When to call a professional

If you experience any significant visual or auditory problems or difficulties with balance, contact your health care professional to be evaluated, especially if these symptoms continue.

Prognosis

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

Additional info

National Organization for Rare Disorders (NORD)
http://rarediseases.org/

American College of Rheumatology
http://www.rheumatology.org/

Arthritis Foundation
http://www.arthritis.org/