Cogan syndrome affects young adults, with 80% of patients between 14 and 47 years. The disease appears to result from an autoimmune reaction directed against an unknown common autoantigen in the cornea and inner ear. About 10 to 30% of patients also have severe systemic vasculitis, which may include life-threatening aortitis Aortitis Aortitis is inflammation of the aorta, sometimes causing aneurysm or occlusion. Aortitis is rare, but potentially life threatening. Its reported incidence is 1 to 3 per one million/year. Aortitis... read more .
Symptoms and Signs of Cogan Syndrome
The presenting symptoms involve the ocular system in 38% of patients, the vestibuloauditory system in 46%, and both in 15%. By 5 months, 75% of patients have both ocular and vestibuloauditory symptoms. Nonspecific systemic complaints include fever, headache, joint pain, and myalgia.
Ocular involvement includes any combination of the following:
Episcleritis Episcleritis Episcleritis is self-limiting, recurring, usually idiopathic inflammation of the episcleral tissue that does not threaten vision. Symptoms are a localized area of hyperemia of the globe, irritation... read more or scleritis Scleritis Scleritis is a severe, destructive, vision-threatening inflammation involving the deep episclera and sclera. Symptoms are moderate to marked pain, hyperemia of the globe, lacrimation, and photophobia... read more
Other orbital inflammation (eg, vitritis, choroiditis)
Ocular symptoms include irritation, pain, photophobia, and decreased vision. Ocular examination shows a patchy corneal stromal infiltrate typical of interstitial keratitis Interstitial Keratitis Interstitial keratitis is chronic, nonulcerative inflammation of the mid-stroma (the middle layers of the cornea) that is sometimes associated with uveitis. The cause is usually infectious.... read more , ocular redness, optic nerve edema, proptosis, or a combination of these symptoms.
Vestibuloauditory symptoms can include sensorineural hearing loss Pathophysiology , tinnitus Tinnitus Tinnitus is a noise in the ears. It is experienced by 10 to 15% of the population. Subjective tinnitus is perception of sound in the absence of an acoustic stimulus and is heard only by the... read more , vertigo Dizziness and Vertigo Dizziness is an imprecise term patients often use to describe various related sensations, including Faintness (a feeling of impending syncope) Light-headedness Feeling of imbalance or unsteadiness... read more , and/or a sensation of fullness or pressure.
A diastolic heart murmur may be present when aortitis is significant. Claudication may be present if limb vessels are affected.
Diagnosis of Cogan Syndrome
Urgent evaluation by an ophthalmologist and otolaryngologist is indicated when Cogan syndrome is suspected. Diagnosis is based on clinical findings and exclusion of other causes of stromal keratitis (eg, syphilis, Lyme disease, Epstein-Barr virus infection) by appropriate serologic tests. Antibodies to inner ear antigens, anti-Hsp70 (associated with sensorineural hearing loss), and antineutrophil cytoplasmic antibodies (associated with vasculitis) have been found in patients with Cogan syndrome. CT and MRI should be done and may be normal or demonstrate labyrinthitis, inflammation, or calcification of the semicircular canals, vestibule, or cochlea.
Treatment of Cogan Syndrome
Initially topical and sometimes systemic corticosteroids for ocular involvement
Untreated disease may lead to corneal scarring and vision loss and, in 60 to 80% of patients, permanent hearing loss Hearing Loss Worldwide, about half a billion people (almost 8% of the world's population) have hearing loss ( 1). More than 10% of people in the US have some degree of hearing loss that compromises their... read more . Keratitis (and symptoms of corneal involvement), episcleritis Episcleritis Episcleritis is self-limiting, recurring, usually idiopathic inflammation of the episcleral tissue that does not threaten vision. Symptoms are a localized area of hyperemia of the globe, irritation... read more , and anterior uveitis Overview of Uveitis Uveitis is defined as inflammation of the uveal tract—the iris, ciliary body, and choroid. However, the retina and fluid within the anterior chamber and vitreous are often involved as well.... read more can usually be treated with topical prednisolone acetate 1%, 1 drop 4 times daily up to once every hour while awake, for 2 to 3 weeks. To treat deeper ocular inflammation and to treat vestibuloauditory symptoms before they become permanent, prednisone 1 mg/kg orally once a day is begun as soon as possible and continued for 2 to 6 months. Some clinicians add cyclophosphamide, methotrexate, cyclosporine, infliximab, or rituximab for recalcitrant cases.
Cogan syndrome is a rare autoimmune syndrome affecting mainly the ocular and vestibuloauditory systems.
Life-threatening aortitis sometimes occurs.
If untreated, permanent vision and hearing loss can result.
Urgent evaluation by an ophthalmologist and otolaryngologist is indicated.
Treatment is typically with corticosteroids (topical and systemic).