Cogan Syndrome

Cogan syndrome is a rare inflammatory disease of the eye and inner ear, with large vessel vasculitis in a proportion of cases.

• Typical Cogan: non-syphilitic interstitial keratitis plus vestibuloauditory dysfunction (Meniere-like), onset within 2 years of each other; atypical: other ocular inflammation (scleritis, uveitis) or audiovestibular symptoms.

• Young adults (mean age 30); no sex predominance.

• Systemic vasculitis in 15 to 30%: aortitis with aortic insufficiency, medium vessel and large vessel disease, arteritis.

• Autoimmune pathogenesis (antibodies to inner ear antigens); association with infections (chlamydia).

• Eye: photophobia, pain, redness, tearing from interstitial keratitis, scleritis, uveitis.

• Ear: sudden vertigo, tinnitus, nausea, rapidly progressive sensorineural hearing loss, often bilateral, leading to deafness within months.

• Systemic: fever, weight loss, arthralgia, myalgia, aortic regurgitation, limb claudication, abdominal pain, neurological manifestations.

• Clinical diagnosis (slit lamp examination for keratitis, audiometry for sensorineural loss, vestibular tests).

• CRP/ESR elevation, anaemia, leucocytosis; exclude syphilis (VDRL/TPHA, FTA-ABS), Lyme disease, tuberculosis, sarcoidosis, Susac, granulomatosis with polyangiitis (ANCA), Behçet, relapsing polychondritis.

• Imaging: CT/MR angiography for large vessel vasculitis, echocardiography for aortic regurgitation, MRI inner ear/brain.

• Autoimmune markers (ANA, ANCA, anti-inner ear antibodies are non-specific).

• Early high dose glucocorticoids is urgent to preserve hearing: prednisolone 1 mg/kg (60 to 80 mg) daily, or methylprednisolone 1 g IV for 3 days, tapered over months; topical steroid (prednisolone acetate 1%) and cycloplegic for keratitis.

• Steroid sparing or refractory: methotrexate 15 to 25 mg weekly, azathioprine 2 mg/kg, cyclophosphamide 2 mg/kg for systemic vasculitis, ciclosporin, TNF inhibitors (infliximab 5 mg/kg, adalimumab), tocilizumab, rituximab.

• Intratympanic dexamethasone may rescue residual hearing.

• Cochlear implantation for profound deafness (best results with early implantation before cochlear ossification).

• Aortic insufficiency or large vessel disease: surgical repair as indicated.

• Monitor audiometry every 1 to 3 months initially, ESR/CRP, eye examination.

• Rehabilitation: hearing aids, vestibular therapy.