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Cogan Syndrome

(Cogan's Syndrome)

By

Melvin I. Roat

, MD, FACS, Sidney Kimmel Medical College at Thomas Jefferson University

Medically Reviewed Aug 2022 | Modified Sep 2022
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Cogan syndrome is a rare autoimmune disease involving the eye and the inner ear.

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 Aortitis .

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

Ocular involvement includes any combination of the following:

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 Interstitial Keratitis , ocular redness, optic nerve edema, proptosis, or a combination of these symptoms.

Vestibuloauditory

Vascular

A diastolic heart murmur may be present when aortitis is significant. Claudication may be present if limb vessels are affected.

Diagnosis of Cogan Syndrome

  • Clinical evaluation

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 Hearing Loss . 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 Episcleritis , 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 Overview of Uveitis 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.

Key Points

  • 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).

Drugs Mentioned In This Article

Drug Name Select Trade
AK-Pred, AsmalPred, Econopred, Econopred Plus, Flo-Pred, Hydeltrasol, Inflamase Forte, Inflamase Mild, Millipred , Millipred DP, Millipred DP 12-Day, Millipred DP 6 Day, Ocu-Pred , Ocu-Pred A, Ocu-Pred Forte, Omnipred, Orapred, Orapred ODT, Pediapred, Pred Mild, Predalone, Pred-Forte, Prednoral, Pred-Phosphate , Prelone, Veripred-20
Cyclophosphamide, Cytoxan, Neosar
Otrexup, Rasuvo, RediTrex, Rheumatrex, Trexall, Xatmep
Cequa, Gengraf , Neoral, Restasis, Sandimmune, SangCya, Verkazia
AVSOLA, INFLECTRA, Remicade, RENFLEXIS
RIABNI, Rituxan, RUXIENCE, truxima
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