Course of auditory impairment in Cogan's syndrome

Abstract

Purpose Cogan's syndrome (CS), characterized by interstitial keratitis, hearing loss, and vestibular impairment, rarely occurs in children. Hearing loss is commonly bilateral and permanent in 37%–67% of patients. To date, long-term evaluation of hearing impairment in children with CS has been reported in only 3 patients. We describe the 35-month course of hearing impairment in a teenaged boy with Cogan's syndrome. Materials and methods The medical record of a 15-year-old boy with Cogan's syndrome was reviewed. Data included relevant history and physical examination, diagnostic workup, and management. Results The patient was diagnosed with bilateral uveitis at age 12 and was placed on oral steroid and methotrexate. He developed sudden sensorineural hearing loss, intermittent tinnitus, and no vestibular dysfunction approximately 9 months after the ophthalmic disease onset. The initial audiogram revealed mild to moderate right-sided high-frequency sensorineural hearing loss and profound left-sided sensorineural hearing loss. Steroid dosage was increased, and the patient exhibited right-side hearing improvement within 2 months. Hearing thresholds reached within normal limits on the right side at 4 months and continued to improve up to 12 months on the left side. Conclusions In a teenager with Cogan's syndrome, the severity and course of hearing impairment showed interaural differences. Improvement of hearing thresholds was slower and incomplete on the left ear. Further studies examining the course of cochleovestibular impairment in a larger group of patients with Cogan's syndrome potentially improve management and counseling.