We report a case of bilateral cochlear implant surgery performed for profound sensorineural hearing loss in a 2-year-old girl with Wolfram syndrome and osteogenesis imperfecta type I. Wolfram syndrome is a rare autosomal-recessive genetic disorder characterized by juvenile diabetes mellitus and visual impairment. The hearing loss in Wolfram syndrome is typically a slowly progressive sensorineural hearing loss, primarily in the high-frequency range. Genetic testing of our patient showed mutations in WFS1 and COL1A1. Bilateral cochlear implant surgery was performed, because adequate wearing thresholds could not be achieved with hearing aids. This report is the first case of a patient with Wolfram syndrome with profound congenital hearing loss who benefited from cochlear implant surgery. The patient had a visual-auditory dual disability and lacked external stimuli. The cochlear implant provided valuable auditory compensation to ensure auditory stimulation. In the future, it is necessary to continue comprehensive rehabilitation for the patient with collaboration among the parents, speech-language pathologists, and rehabilitation facilities.
