Cogans Syndrome

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Cogans 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 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.


  • Reddening of eyes, eyes becoming sensitive to light, or hazy eyesight
  • Loss of hearing, sometimes even constant and serious
  • Vertigo
  • Failure in balancing
  • Vomiting or nausea
  • Fever, loss of weight, weakness

  • More rarely, Cogan's syndrome can cause enlarged lymph nodes, rash, chest pain, arm pain and shortness of breath.

    Mild eye disease may be treated with anti-inflammatory medications, including steroids and nonsteroidal anti-inflammatory drugs, that are applied to the eye. If these medications do not work well enough, oral antibiotics, such as tetracycline, 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), cyclophosphamide (Cytoxan, Neosar), cyclosporine (Neoral, Sandimmune) or azathioprine (Imuran). In some cases, combinations of these 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 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.

    There is no way to prevent Cogans syndrome.


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