Cochlear Implants for Children with Severe-to-Profound Hearing Loss

A 4-month-old baby girl whose parents and 6-year-old brother have severe-to-profound sensorineural hearing loss is referred for consideration for cochlear implantation. Each of the baby’s parents had previously received a cochlear implant in adulthood, and her older brother had received a cochlear implant at 18 months of age. The family uses oral communication in the English language. The baby had been diagnosed with sensorineural hearing loss during newborn screening, and she was fitted with bilat-eral hearing aids without clinically significant benefit. Bilateral profound sensori-neural hearing loss, with pure-tone thresholds of 100 dB or higher, is confirmed and a homozygous mutation in the connexin 26 gene (GJB2) is identified. It is recommended that the child undergo auditory-verbal therapy followed by implantation of a unilat-eral cochlear implant at 12 months of age. THE CL INIC A L PROBLEM Sensorineural hearing loss affects 1 to 3 of every 1000 children born in the United States and other developed countries1,2; the rate is probably higher in the developing world.3 In most cases, the hearing loss is nonsyndromic (i.e., it is not associated wit