Cochlear implants for children with severe-to-profound hearing loss.

Copyright © 2007 Massachusetts Medical Society. 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 bilateral hearing aids without clinically significant benefit. Bilateral profound sensorineural 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 unilateral cochlear implant at 12 months of age.