Cogan's Syndrome

Overview of Cogan's Syndrome

Cogan syndrome is a rare rheumatic autoimmune disorder in which the immune system automatically invades the healthy cells of the ear and eye condition. That results in inflammation of the eyes and ears. Due to this, it subsequently causes hearing loss, vision loss, or dizziness.

If the inflammatory effects are with eyes only, then it is referred to as Cogan's rees syndrome. If the condition is accompanied by inflammation of blood vessels in other areas of the body then it is referred to as Cogan's vasculitis, which may cause organ damage or even death.
Many researchers couldn’t find the exact mode of action of the disease but reported it as an autoimmune disorder.

Cogan’s Syndrome Prevalence

Cogan's syndrome is mostly present in young adults particularly the age between 20 and 30 years. Almost 20% of cases have been reported of the atypical Cogan's syndrome (audiovestibular dysfunction with other types of inflammatory eye disease and vasculitis). There are also 10% of life-threatening cases of aortic insufficiency due to Cogan's syndrome vasculitis.

Signs and Symptoms of Cogan's Syndrome

Cogan’s syndrome normally stimulates whether in a form of eye inflammation or inner ear inflammation, but often becomes progressive to cause both infections. The symptoms of Cogan’s vasculitis syndrome can be observed at the start of the syndrome.

Some general symptoms of Cogan’s syndrome include:

  • Eye redness and pain, blurred vision, light sensitivity, and tearing of eyes
  • Hearing loss, associated with ear pressure and ringing in the ears (tinnitus)
  • Vertigo 
  • Dizziness
  • Poor balance

Cogan's vasculitis symptoms are as follows:

  • Muscle pain
  • Cramping
  • Joint pain
  • Headache
  • Fever
  • Weight loss
  • Heart murmurs 

Types of Cogan's Syndrome

Causes of Cogan's Syndrome

Researchers are still finding the cause of Cogan’s syndrome other than autoimmune dysfunction that mistakenly kills the normal cells related, the ear, eye, or blood vessels (mainly the heart, and joints). In some cases, people suffer Cogan's syndrome after infection, but can't inherit it from their families.


Risk Factors of Cogan's Syndrome





A general physician examines the affected area of patients whether it is affected on the eye, ear, or blood vessels, depending upon their symptoms or any associated disease with it. As such, there is no specific test for Cogan's syndrome, but a general physician recommends the test as per inflamed area for example if the ear inflammation is noted then a hearing test is recommended to patients. Sometimes, a doctor will suggest an MRI scan or CT scan to check the detailed picture of the eyes or ears.

Typically the general physician evaluates Cogan's syndrome associated with the infection (especially syphilis, tuberculosis, viral infection, and chlamydia) or another rheumatic disease (including rheumatoid arthritis, sarcoidosis, and granulomatosis with polyangiitis) as the causing factor of the symptoms.  On the basis of diagnosis, the general physician will send the patients to specialized doctors. 

Treatment of Cogan's Syndrome | When to Consult a Doctor

The choice of Cogan’s syndrome treatment depends on the affected area or severity of the disease If the eye disease is acute then treat it with anti-inflammatory medications, including steroids and NSAIDs that are applied to the eye. 

If these medications do not treat properly then the doctor may recommend oral antibiotics, such as doxycycline to reduce the eye inflammation

For severe cases, oral corticosteroids may be necessary. If steroidal therapy doesn’t respond well, immunosuppressive medications are prescribed to the patients. These immunosuppressive drugs include:

  • Methotrexate (Rheumatrex)
  • Cyclophosphamide (Cytoxan, Neosar)
  • Cyclosporine (Neoral, Sandimmune) 
  • Azathioprine (Imuran)

Sometimes, drugs are given in form of a combination, according to the affected area. Along with it, a corneal transplant of the eye is also required when the eye is damaged from the front to improve the vision of the eye. 

Surgery is recommended when immune system attacks on the blood vessels lead to Cogan syndrome vasculitis. Then, this is the main technique to rectify it.

Another affected area is the inner ear where the accumulation of a large amount of fluid causes balance problems; a doctor may prescribe diuretic medications to increase the flow of urine and remove fluid from the body. Along with it, antihistamines are used to treat vertigo such as:

  • Meclizine (Antivert)
  • Benzodiazepines (Diazepam (Valium) or Clonazepam (Klonopin) )

There are other techniques like cochlear implants that are used to treat impaired hearing. These implants are electronic devices to convert sounds into electrical signals that directly transmit to the brain by bypassing the malfunctioning part of the ear.

If the Cogan’s syndrome doesn’t get treated by antibiotics or if the symptoms get worse then consult a medical professional.