Cogan Syndrome

Medically reviewed by Ann Marie Griff, O.D. on November 10, 2017Written by Lana Bandoim on November 10, 2017

What is Cogan syndrome?

Cogan syndrome is a rare condition that causes inflammation in your eyes and ears. Researchers aren’t sure about its exact origin, but many consider it an autoimmune disease. Autoimmune diseases cause your immune system to attack healthy cells in your body.

In the case of Cogan syndrome, it’s believed that your immune system created antibodies that attack the tissue in your eyes and ears. Cogan syndrome is also referred to in the possessive form as Cogan’s syndrome.

What are the symptoms?

Cogan syndrome often starts as an inflammatory eye disease, usually interstitial keratitis. This causes inflammation in the tissue of your cornea, the clear film that covers the front of your eye.

Additional symptoms of interstitial keratitis include:

  • watering eyes
  • red, painful eyes
  • sensitivity to light

You might start noticing signs of inflammation in your ears. This usually happens within about two years of having inflammatory eye disease.

These symptoms include:

  • hearing loss
  • dizziness
  • ringing in your ears
  • trouble balancing

As time goes on, you may also notice:

  • fatigue
  • headaches
  • joint pain
  • rashes
  • arm pain
  • shortness of breath
  • enlarged lymph nodes

What causes it?

The exact cause of Cogan syndrome is unknown, but it’s likely an autoimmune condition. It’s also closely related to vasculitis, which refers to inflammation of your blood vessels. However, researchers aren’t clear on the relationship between the two conditions. There’s no evidence that Cogan syndrome is hereditary.

Cogan syndrome affects both men and women. It tends to start between the ages of 20 and 40.

How is it diagnosed?

There’s no specific test doctors use to diagnose Cogan syndrome. Instead, your doctor will review your symptoms, especially when they each started, and give you a physical exam. They’ll also likely give you a thorough eye exam to check for signs of inflammation and test your hearing. In some cases, they might also use an MRI scan or CT scan to get a better look at your eyes and ears.

If possible, keep track of:

  • when you first noticed symptoms in your eyes
  • the time between when you noticed symptoms in your eyes and when you noticed them in your ears
  • whether the symptoms in your ears changed from one ear to the other

This information may help your doctor rule out any other possible causes of your symptoms.

How is it treated?

There are several treatment options for Cogan syndrome. Treatments depends on your symptoms and how severe they are. However, keep in mind that you may continue to have periodic flare-ups for many years.

Your doctor might start by prescribing antibiotics or diuretics to make sure your symptoms aren’t due to an infection or extra fluid in your ears.

Next, they may suggest medications to deal with the inflammation. These may include:

These medications usually start working within 10 days. If you don’t notice any improvement by then, contact your doctor. You may need a higher dosage.

If these options don’t work, you may need immunosuppressant medications to prevent your immune system from attacking your eyes and ears.

If your symptoms are more advanced, and you’re having ongoing vision and hearing problems, your doctor might also suggest:

  • a corneal transplant to improve vision
  • a cochlear implant to improve hearing
  • surgery to repair damaged blood vessels

Are there any complications?

Many people with Cogan syndrome end up losing some of their vision or hearing. However, if you start treatment early, you can often avoid significant vision or hearing loss.

Living with Cogan syndrome

Cogan syndrome is a rare condition that affects your eyes and ears. Over time, it can lead to permanent vision and hearing loss. Be sure to talk to your doctor about any symptoms you have as soon as possible. While there are several effective treatment options, you may continue to have occasional flare-ups for many years.

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