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

Authors

Leyn B. Shakhtour, Division of Otolaryngology-Head and Neck Surgery, The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, USA.
Sophia Song, Division of Otolaryngology-Head and Neck Surgery, The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, USA.
Nicklas C. Orobello, Department of Otolaryngology, Children's National Hospital, Washington, District of Columbia, USA.
Samuel Garrett, Department of Otolaryngology, Walter Reed National Military Medical Center, Bethesda, Maryland, USA.
Tracey Ambrose, Department of Otolaryngology, Children's National Hospital, Washington, District of Columbia, USA.
Hengameh K. Behzadpour, Department of Otolaryngology, Children's National Hospital, Washington, District of Columbia, USA.
Gilbert Vezina, Departments of Radiology and Pediatrics, Division of Neuroradiology, Children's National Medical Center, Washington, District of Columbia, USA.
Diego A. Preciado, Department of Otolaryngology, Children's National Hospital, Washington, District of Columbia, USA.
Brian K. Reilly, Department of Otolaryngology, Children's National Hospital, Washington, District of Columbia, USA.

Document Type

Journal Article

Publication Date

5-20-2024

Journal

Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery

DOI

10.1002/ohn.827

Keywords

cochlear implant; cochlear nerve deficiency; pediatric otolaryngology

Abstract

OBJECTIVE: To 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 DESIGN: retrospective review. SETTING: Cochlear implant program at tertiary care center. METHODS: A 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. RESULTS: Seven 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. CONCLUSION: Our 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.

APA Citation

Shakhtour, Leyn B.; Song, Sophia; Orobello, Nicklas C.; Garrett, Samuel; Ambrose, Tracey; Behzadpour, Hengameh K.; Vezina, Gilbert; Preciado, Diego A.; and Reilly, Brian K., "Evaluation of Cochlear Implantation in Children With Cochlear Nerve Absence or Deficiency" (2024). GW Authored Works. Paper 4910.
https://hsrc.himmelfarb.gwu.edu/gwhpubs/4910

Department

Surgery