Cogan syndrome

What is it?

Cogan syndrome is defined as non-syphilitic interstitial keratitis associated with audiovistibular lesions with progressive hearing loss to complete deafness during the first 2 years of life. The syndrome affects mainly young people and affects another organ in 64% of cases, causing a clinical picture of systemic vasculitis in 33% of patients. The etiology is unknown, but numerous studies suggest an autoimmune pathogenesis.

Which are the symptoms?

In the beginning, Cogan syndrome usually presents with nonsyphilitic interstitial keratitis associated with bouts of dizziness, tinnitus, and hearing loss. Although it can present as a single symptom, most patients have both eyes and hearing affected within one year of diagnosis. Interstitial keratitis has a recurring pattern, even if only 5% of patients have severe visual impairment. Additional examinations reveal an inflammatory syndrome, and sometimes immunological disorders. There is no specific biological test to diagnose this disease. Cogan syndrome can also affect organs other than the eye and ear with vasculitic manifestations; the most common symptoms are cardiovascular, musculoskeletal, neurological, gastrointestinal and mucocutaneous symptoms. The prognosis is due to the risk of complete deafness and cardiovascular complications, in particular aortic insufficiency.

How is it diagnosed?

Suggested exams

How is it treated?

Timely diagnosis of Cogan syndrome is important because it is one of the few treatable causes of deafness if recognized and treated early. If the disease is left untreated, most people develop deafness within three years of the first symptoms of the disease. Treatment mainly consists of corticosteroids. Eye symptoms usually improve, but deafness is rarely reversible. In case of corticosteroids or cortic dependence, immunosuppressants are prescribed.

Where do we treat it?

Within the San Donato Group, you can find Cogan syndrome specialists at these departments:

Are you interested in receiving the treatment?

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