What Is Cogan Syndrome?

  • Medical Editor: William C. Shiel Jr., MD, FACP, FACR
    William C. Shiel Jr., MD, FACP, FACR

    William C. Shiel Jr., MD, FACP, FACR

    Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.

Ask the experts

Is it possible to have arteritis of the ear? What are the signs of this?

Doctor's response

Arteritis (also referred to as vasculitis) can involve the ear. This condition is called Cogan syndrome after the doctor that first described it.

The syndrome features not only problems of the hearing and balance portions of the ear, but also inflammation of the front of the eye (cornea) and often fever, fatigue, and weight loss. Joint and muscle pains can also be present. Less frequently the arteritis can involve blood vessels elsewhere in the body and affect the skin, kidneys, nerves, or other organs.

Cogan syndrome can lead to deafness or blindness.

Treatment of Cogan syndrome is directed toward stopping the inflammation of the blood vessels. Cortisone-related medications, such as prednisone, are often used. Some patients with severe disease can require immune suppression medications, such as cyclophosphamide/Cytoxan.

Cogan syndrome is extremely rare and its cause is not known.

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Reviewed on 1/11/2018