What is Cogan Syndrome?

J.L. Drede

Cogan syndrome is a disorder that causes inflammation of the eyes and hearing problems. It takes its name from Dr. David Cogan, who first identified the condition in the 1940s. It is a very rare disorder, with only approximately 1,000 cases reported since it was first diagnosed. In addition to eye inflammation and hearing loss, other symptoms of Cogan syndrome can include bouts of vertigo and poor balance, nausea with vomiting, and flu-like symptoms such as fever and fatigue. In some cases, sufferers of Cogan syndrome may also experience rashes, chest and arm pain, and enlarged lymph nodes.

Cogan syndrome causes imflammation of the eye.
Cogan syndrome causes imflammation of the eye.

In most cases the onset of symptoms is staggered, with either just the ear or eye becoming affected first. The effects are often staggered, and it can take months or even years for both to be affected and a diagnosis of Cogan syndrome to be made.

Cochlear implants can be used to restore permanent hearing loss.
Cochlear implants can be used to restore permanent hearing loss.

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There is no way to prevent Cogan syndrome, but there are many different forms of treatment that can help manage or even eliminate the effects of the condition. Anti-inflammatory medication such as steroids can often help to alleviate the eye inflammation and help restore vision. Antibiotics may also be prescribed if an eye infection is suspected.

Hearing loss and balance problems caused by Cogan syndrome are usually caused by an excess of fluid buildup in the inner ear. Many times, prescribed diuretics that increase urination will help the body remove these fluids and restore normal hearing and balance. Antihistamines and benzodiazepines may also be prescribed to help restore balance if diuretics are not helping enough.

Surgery may be needed in some cases. Many times the disease will cause damage to the blood vessels around the eye, which can only be corrected with surgery. In especially severe cases where damage to eyesight is permanent, a corneal transplant may be needed to restore vision. Cochlear implants may also be used to restore permanent hearing loss.

The cause of Cogan syndrome is unknown, although researchers do not believe that the condition is hereditary. It most commonly affects adults around the age of 30, but cases in children as young as 10 have also been reported. Most reported cases were in Caucasians.

While Cogan syndrome is rarely fatal, many of its effects are irreversible. Even with treatment and surgery, nearly 90 percent of those with the condition suffer severe or complete hearing loss. Its effects on sight are usually less severe and respond more favorably to treatment, even though many times they are recurring and require regular treatment in order to be kept in check.

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