Evaluation of Cochlear Implantation in Children With Cochlear Nerve Absence or Deficiency

ObjectiveTo identify associations between cochleovestibular anatomy findings and hearing outcomes found in children with imaging evidence of an absent or hypoplastic cochlear nerve treated with cochlear implantation (CI).Study Designretrospective review.SettingCochlear implant program at tertiary care center.MethodsA retrospective review was performed to identify children with imaging evidence of cochlear nerve absence or deficiency who underwent CI evaluation. High-resolution 3-dimensional T2-weighted magnetic resonance imaging in the oblique sagittal and axial planes were reviewed by a neuroradiologist to identify cochleovestibular anatomy. Hearing was assessed pre and postoperatively with Speech Perception Category scores.ResultsSeven CI recipients were identified (n = 10 ears) who had bilateral severe to profound sensorineural hearing loss with lack of auditory development with binaural hearing aid trial and imaging evidence of cochlear nerve aplasia/hypoplasia. All ears had 2 nerves in the cerebellopontine angle (100%, n = 10), half of the ears had evidence of 2 or less nerves in the internal auditory canal (IAC). All children showed large improvement in speech perception after CI.ConclusionOur experience with CIs for children with absent or hypoplastic cochlear nerves demonstrates that CI can be a viable option in select patients who satisfy preoperative audiological criteria. Radiological identification of a hypoplastic or aplastic cochlear nerve does not preclude auditory innervation of the cochlea. CI recipients in this subgroup must be counseled on difficulty in predicting postimplantation language and speech outcomes, and cautioned about facial nerve stimulation.