Cogan's syndrome: disease involving inner ear and eye

Living with Cogan’s syndrome

Cogan's syndrome is a rare autoimmune disorder involving the eye and inner ear. It affects both young people and adults; In fact, 80% of patients are between the ages of 14 and 47.

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What are Cogan syndrome's symptoms?

Cogan syndrome may present symptoms that affect either the ocular system in 38% of cases, audiovestibular system in 46%, or both in 15%. In most cases, 75% of patients experience ocular and audiovestibular symptoms after 5 months. Common ocular symptoms include pain, light sensitivity, irritation, and decreased vision. On the other hand, common audiovestibular symptoms include sensorineural hearing loss, tinnitus, dizziness, and a sense of fullness or pressure.

Cogan’s syndrome causes

Cogan syndrome's exact cause remains uncertain. However, it seems to occur due to an autoimmune response that targets an unidentified, diffuse autoantigen found in both the inner ear and cornea.

In some cases, the condition is associated with vasculitis, an inflammation of blood vessels, which may contribute to systemic manifestations.

Who gets Cogan’s syndrome?

Cogan syndrome affects individuals of all genders and occurs in both young people and adults. However, it is more commonly observed in individuals between the ages of 14 and 47, constituting about 80% of patients.

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How is Cogan syndrome diagnosed?

The diagnosis of Cogan syndrome does not have a standard method and is mainly based on the ocular symptoms, which are often the first to appear. It may take several months before the symptoms affecting the ear become apparent. An audiogram is usually performed to determine the extent of hearing loss. Blood tests are also conducted to evaluate any systemic involvement, and a differential diagnosis is conducted to rule out other diseases with similar symptoms.

Cogan’s syndrome treatment

Treatment for Cogan's syndrome involves the same conventional methods used for autoimmune disorders. Immunosuppressants are prescribed to reduce any harmful activity to the immune system and to treat individual symptoms.

Surgical treatments

In some cases, when symptoms are limited to the acoustic part, surgical replacement of the cochlear implant is possible. However, if there is significant involvement of the vascular component, it is best to resort to surgical therapies.

Possibile side effects

The available therapies for Cogan's syndrome are highly specific and vary depending on the patient's symptoms, leading to a wide range of potential side effects that can differ from person to person.

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What is Cogan’s syndrome?