Cogan syndrome

A rare disorder characterized by inflammation of the eyes and ears

Cogan syndrome
Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Keratitis, hearing loss, vertigo, tinnitus
Complications	Aortic insufficiency, Aneurysm
Onset	Typically young adults
Duration	Chronic
Types	N/A
Causes	Unknown, possibly autoimmune
Risks
Diagnosis	Clinical evaluation, audiometry, slit lamp examination
Differential diagnosis	N/A
Prevention	N/A
Treatment	Corticosteroids, immunosuppressive drugs
Medication	N/A
Prognosis	Variable
Frequency	Rare
Deaths	N/A

Cogan syndrome is a rare autoimmune disease characterized by inflammation of the eyes and ears, leading to keratitis and audiovestibular dysfunction. It primarily affects young adults and can lead to serious complications if not treated promptly.

Clinical Presentation

Cogan syndrome typically presents with two main symptoms: interstitial keratitis and audiovestibular symptoms.

Ocular Manifestations

The most common eye-related symptom is interstitial keratitis, which involves inflammation of the cornea. Patients may experience redness, pain, photophobia, and decreased vision. Other ocular symptoms can include scleritis, uveitis, and conjunctivitis.

Audiovestibular Symptoms

Patients often experience sudden onset of hearing loss, tinnitus, and vertigo. These symptoms are similar to those seen in Meniere's disease, but in Cogan syndrome, they are associated with systemic inflammation.

Pathophysiology

The exact cause of Cogan syndrome is unknown, but it is believed to be an autoimmune disorder. The immune system mistakenly attacks the tissues of the eyes and ears, leading to inflammation and damage. Some studies suggest a possible link to viral infections as a trigger for the autoimmune response.

Diagnosis

Diagnosis of Cogan syndrome is primarily clinical, based on the characteristic symptoms of keratitis and audiovestibular dysfunction. Additional tests may include:

• Slit lamp examination to assess corneal inflammation.
• Audiometry to evaluate hearing loss.
• Blood tests to rule out other autoimmune conditions.

Treatment

The mainstay of treatment for Cogan syndrome is the use of corticosteroids to reduce inflammation. In cases where corticosteroids are insufficient, immunosuppressive drugs such as methotrexate or cyclophosphamide may be used. Early treatment is crucial to prevent permanent damage to the eyes and ears.

Prognosis

The prognosis for Cogan syndrome varies. With prompt and appropriate treatment, many patients can achieve good control of symptoms. However, some may experience chronic symptoms or complications such as aortic insufficiency or aneurysm formation.

Also see

• Autoimmune disease
• Keratitis
• Meniere's disease
• Uveitis

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