Cogans Syndrome

Timothy C. Hain, MD
Last update: 10/2012

What is Cogans syndrome?

What causes Cogans syndrome?

How is Cogans syndrome treated?

References

What is Cogans syndrome?

Cogans syndrome is defined as nonsyphilitic interstitial keratitis (an inflammation of the eye)
and bilateral audiovestibular deficits (hearing problems and dizziness). It is more common in
Caucasians than in other races.
Onset of the disease is generally a brief episode of inflammatory eye disease, most commonly
interstitial keratitis. Other inflammatory eye conditions that may occur include uveitis,
episcleritis or scleritis. These eye conditions cause pain, lacrimation (tearing of the eye) and
photophobia (eye pain with exposure to light).
Shortly following these ocular symptoms, patients develop bilateral audiovestibular symptoms,
including hearing loss, vertigo (dizziness) and tinnitus (ringing in the ears). Approximately half
of patients ultimately develop complete hearing loss, but only a minority experience permanent
visual loss.
Systemic symptoms that may occur include headache, fever, arthralgia (joint pain), and systemic
vasculitis (inflammation of the blood vessels). Vasculitis may affect any blood vessels, including
the aorta. Aortitis placed patients at an increased risk of aortic aneurysm, a potentially fatal
complication.
The symptoms typically deteriorate progressively within days. While the ESR, WBC, and Creactive protein test may be abnormal and there may be thrombocytosis or anemia, none of these
findings are reliable indicators of the disease. A slit-lamp examination is essential. Recent work
has suggested that high-resolution MRI and antibodies to inner ear antigens may be helpful. Very
little has been published in recent years on Cogans syndrome.

What causes Cogans syndrome?

It is currently thought that Cogans syndrome is an autoimmune disease. The inflammation in the
eye and ear are due to the patients own immune system producing antibodies that attack the
inner ear and eye tissue. Autoantibodies can be demonstrated in the blood of some patients, and
these antibodies have been shown to attack inner ear tissue in laboratory studies. Infection with
the bacteria Chlamydia pneumoniae has been demonstrated in some patients prior to the
development of Cogans syndrome, leading some researchers to hypothesis that the autoimmune
disease may be initiated by the infection. Chlamydia pneumoniae is a common cause of mild
pneumonia, and the vast majority of patients who are infected with the bacteria do not develop
Cogans syndrome.

How is Cogans syndrome treated?

Immediate treatment for Cogans syndrome is with steroids, which reduce the inflammatory
response. TNF-alpha is powerful anti-inflammatory agent that has been used in a small number
of patients who do not respond to steroid treatment. Once hearing loss has occurred, antiinflammatory medication may not improve hearing. Cochlear implantation may be used in some
patients, and studies have shown it to produce significant improvement in hearing loss in some
patients.
Learn about a patient with Cogans syndrome.
See also: Autoimmune Inner Ear Disease