Charge Association: Cochlear Implant
At 14 months of age, Emma was brought to Children's Memorial Hospital for a second opinion regarding her medical and audiological management. The parents had observed no auditory responses during their child's 10 months of hearing aid use. Her medical history was notable for many conditions related to CHARGE, including visual impairment; congenital heart disease; choanal atresia requiring tracheotomy; developmental delay with fine, gross, and oral motor dysfunction; kidney and urologic abnormalities; malformation of auricles; and middle ear dysfunction, for which tubes had been placed. Computed tomography of the temporal bones had revealed the presence of bilateral cochlear malformations notable for absence of the semicircular canals and hypoplasia of the vestibules bilaterally. The left cochlear aperture, the opening through which the cochlear nerve bundle enters the cochlea, appeared severely narrowed on the left. The turns of the cochlea and the internal auditory canals were well formed bilaterally.