Evaluation of Cochlear Implantation in Children With Cochlear Nerve Deficiency

Monday, October 2, 2023

8:24 AM - 8:30 AM East Coast USA Time

Location: 207CD

Presenting Author(s)

Leyn B. Shakhtour, BS

Medical Student
George Washington University School of Medicine and Health Sciences
Washington, District of Columbia, United States

Co-Author(s)

Leyn B. Shakhtour, BS

Medical Student
George Washington University School of Medicine and Health Sciences
Washington, District of Columbia, United States
NO

Nicklas C. Orobello, MD

Pediatric otolaryngologist
Department of Otolaryngology, Children's National Hospital
Tampa, FL, United States
Sameul L. Garrett, MD

Resident Physician
Walter Reed National Military Medical Center - Otolaryngology Department
Silver Spring, Maryland, United States
TA

Tracey E. Ambrose, AuD

Lead Audiologist
Department of Otolaryngology, Children's National Hospital
Washington , DC, United States
HB

Hengameh K. Behzadpour, MSHS

Clinical Research Coordinator
Children's National Medical Center
Washington, District of Columbia, United States

Senior Author(s)

DP

Diego A. Preciado, MD, PHD

Chief of Otolaryngology
Department of Otolaryngology–Head & Neck Surgery, George Washington University, Children's National Hospital, Washington, DC, USA; Editorial Board JAMA Otolaryngol Head Neck Surg
Washington, DC, United States
Brian K. Reilly, MD

Member
Childrens National Medical Center
Washington, DC, United States

Disclosure(s):

Leyn B. Shakhtour, BS: No relevant relationships to disclose.

Introduction: There is a lack of clarity regarding which children will have effective outcomes post cochlear implantation (CI) when there is imaging evidence of an absent or hypoplastic cochlear nerve. Our study evaluated the preoperative candidacy and postoperative performance of CI recipients with absent or hypoplastic cochlear nerves.

Methods: A retrospective case review was performed to identify children with cochlear nerve absence or deficiency who underwent CI evaluation. High-resolution three-dimensional T2-weighted magnetic resonance imaging in the oblique sagittal and axial planes were used to identify absent or hypoplastic cochlear nerves. CI candidacy was determined by test results from the auditory brainstem response and behavioral audiometry. Neural response telemetry (NRT) and behavioral audiometry were used to measure audiological performance after cochlear implantation.

Results: Seven children underwent cochlear implantation with imaging evidence of an absent or hypoplastic cochlear nerve. Based on aided behavioral test results indicating speech awareness thresholds of greater than 25 dB HL, all children were deemed candidates for CIs. Two patients experienced post-implantation facial nerve stimulation, prompting further re-programming of implant. Evaluation of auditory rehabilitation status indicated significant and appropriate benefit from CI in all seven patients who underwent our candidacy screening and testing. Due to developmental delays, one patient’s performance was determined from post-CI NRT findings and demonstrated sound awareness through exhibited behavioral responses.

Conclusions: 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 pre-operative audiological criteria. Radiological identification of a hypoplastic of aplastic cochlear nerve does not preclude auditory innervation of the cochlea. CI recipients in this subgroup must be counseled on difficulty in predicting post-implantation language /speech outcomes, and cautioned about facial nerve stimulation.