Cogan's Syndrome

Cogan's syndrome is a rare disease. Cogan's syndrome is a kind of rheumatic disease which is usually characterized by inflammation of the eyes and ears. Cogan's syndrome generally results in hearing loss, dizziness and vision difficulty. Cogan's syndrome sometimes may also lead to inflammation of blood vessels i.e. vasculitis resulting in damaging the main organ present in other regions of the body. This may also cause death in very few of the cases. Cogan's syndrome is very common in individuals in the age group of 20 to 30 years. The exact cause of Cogan's syndrome is still not known. The autoimmune disorder is referred to as one of the suspected causes. Autoimmune disorder in this case is a state in which tissues in the eyes and ears get attacked by body's own immune system.

Cogan's Syndrome

Epidemiology of Cogan's Syndrome

Cogan's syndrome is a very rare disease. Only 250 cases have been reported so far. Cogan's syndrome is rare in children. Cogan's syndrome usually affects individuals between 20 to 30 years. Men and women both can get affected with Cogan's syndrome though women are more prone to Cogan's syndrome when compared to men.

Causes and Risk Factors of Cogan's Syndrome

  • Although the exact cause of the Cogan's syndrome is still not known the suspected cause implicates it to be an autoimmune disease which is caused due to inflammation in the ears and eyes. This happens when the body's immune system starts producing antibodies in order to attack tissues present in the eye and inner ear. The suspected reasons for Cogan's syndrome may also include some pathological and immunological factors like plasma cell infiltration in the cochlea and cornea, and antibodies such as immunoglobulin G which act against the inner ear and the cornea. Effects of corticosteroids and immunosupressants and presence of lymphocytes points out towards an autoimmune pathogenesis. Polyarteritis nodosa and collagen diseases are also seen to be a cause in multiple organs as a result of autoimmune disorders.
  • Cogan's syndrome is also found to be caused with bacteria due to the presence of infection occurring from Chlamydia pneumonia bacteria before developing the syndrome. Chlamydia pneumonia bacteria usually cause mild pneumonia although not all the patients infected with Chlamydia pneumonia develop Cogan's syndrome.

Signs and Symptoms of Cogan's Syndrome

  • Painful, light-sensitive, red eyes and blurred vision.
  • Rapid onset of hearing loss that may go on to be profound or permanent.
  • Poor balance.
  • Dizziness or vertigo.
  • Nausea and vomiting.
  • Weight loss.
  • Fever.
  • Fatigue.

Rare Symptoms of Cogan's Syndrome May Include

  • Chest pain.
  • Arm pain.
  • Enlarged lymph nodes.
  • Shortness of breath.
  • Rash.

Treatment for Cogan's Syndrome

  • Anti-Inflammatory Medications are used for the treatment of mild eye disease. Application of steroids and nonsteroidal anti-inflammatory drugs are helpful for the eye. Oral antibiotics are usually prescribed only when steroids and NSAIDs fail to work properly. Oral antibiotics like tetracycline may be prescribed to treat eye inflammation caused from an infection.
  • Oral Corticosteroids are used in very severe cases when steroids are taken in very large amounts. Other immunosuppressive medications are also prescribed. Immunosuppressive drugs may include cyclosporine, azathioprine, methotrexate and cyclophosphamide. These medications may also be used in combination in few of the cases. In some rare cases, when the damage is caused to the blood vessels, surgery is performed in order to correct the problem.
  • Diuretic Medications are often prescribed to treat in case of balance problems caused due to excess fluid in the inner ear. Diuretic medications help by increasing urination in order to drain the fluid from the body. Balance problem may be treated with benzodiazepines or antihistamines like clonazepam or diazepam and bed rest.
  • Cochlear Implants are performed when medical treatment fails to work properly for improvement in hearing. Cochlear implants are electronic devices that help in improving the hearing ability of a person.
  • A Corneal Transplant is done when the front side of the eye severely gets damaged due to inflammation. A corneal transplant helps in improving vision. A corneal transplant is surgery which is performed in order to replace scarred cornea with a healthy one from another person.

Investigations for Cogan's Syndrome

Rapid onset of eye inflammation, hearing loss, and vasculitis are all indications of Cogan's syndrome and form the basis for investigation.

A thorough subjective and physical examination is performed to rule out other causes before confirming the diagnosis of Cogan's syndrome. Cogan's syndrome cannot be diagnosed directly and requires ruling out all the other possible diseases that may cause the symptoms.

Blood Tests Helpful in Diagnosing Cogan's Syndrome May Include:

  • ESR (sedimentation rate).
  • Ferritin level.
  • Complete blood count (CBC).
  • C-reactive protein.
  • Liver function tests.
  • Urinalysis.
  • ANA test and rheumatoid factor.

Other Tests for Diagnosing Other Specific Disease Processes May Include:

  • Angiogram.
  • CT scan.
  • X-rays.
  • HIV testing.
  • Abdominal ultrasound.

Written, Edited or Reviewed By:

, MD, FFARCSI

Last Modified On: May 2, 2016

Pain Assist Inc.

Pramod Kerkar
  Note: Information provided is not a substitute for physician, hospital or any form of medical care. Examination and Investigation is necessary for correct diagnosis.

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