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Scientific Oral Presentation

Pediatric Otolaryngology

Cochlear Implant Outcomes in Children with Cochlear Nerve Aplasia versus Hypoplasia: A Systematic Review

Sunday, October 18, 2026
8:00 AM - 8:06 AM PT
Location: 409AB

    Presenting Author(s)

  • Jasmine L. Leahy, MD

    Resident
    SUNY Health Science Center At Brooklyn (Downstate)
    Brooklyn, NY, United States

    • Co-Author(s)

  • Nancy Deng, BA

    Medical Student
    SUNY Downstate Health Sciences University
    Woodhaven, NY, United States

  • MA

    Matthew D. Adams, MD

    Resident Physician
    SUNY Health Science Center At Brooklyn (Downstate)
    Brooklyn, New York, United States

  • Richard M. Rosenfeld, MD, MPH, MBA

    Distinguished Professor and Past Chairman of Otolaryngology
    SUNY Downstate Health Sciences University
    Brooklyn, New York, United States

    • Senior Author(s)

  • AP

    Ann W. Plum, MD

    Chief of Pediatric Otolaryngology
    SUNY Downstate Medical Center
    Brooklyn, New York, United States

Disclosure(s):
Jasmine L. Leahy, MD:

faculty for this accredited education activity has no relationship(s) with ineligible companies to disclose.

Introduction: Recent studies have investigated the possible benefits of CIs in children with cochlear nerve deficiency, a population once considered ineligible for implantation. Many of these studies are hindered by small sample sizes and heterogenous outcome measures. This is the first systematic review directly comparing audiometric and speech-language outcomes after CI between children with cochlear nerve hypoplasia and aplasia.

Methods: A systematic review was performed of PubMed, EMBASE, and Web of Science from January 2003 to October 2025 for peer-reviewed studies of pediatric patients with radiographically confirmed cochlear nerve deficiency who underwent CI. Sixteen studies were included for review. Primary outcomes included pure-tone average (PTA) and speech-language measures (e.g. Speech Intelligibility Rating (SIR), Categories of Auditory Performance (CAP)), comparing hypoplasia versus aplasia with subgroup analyses by age at implantation ( < 2, 2-5, and >5 years old).

Results: There were 364 patients total: 78 with cochlear nerve hypoplasia and 286 with aplasia. Mean follow-up was 30 months. Post-operative PTA improved by –44 dB for aplasia and –43.4 dB for hypoplasia. Children under two years old showed the greatest improvement (–45.2 dB and –49 dB for aplasia and hypoplasia, respectively), while those over five showed the least (–19.5 dB and –23.53 dB, respectively). Both groups had similar speech-language outcomes: aplasia patients improved 1.37 points on SIR and 3.85 on CAP, while hypoplasia patients improved 1.42 on SIR and 3.89 on CAP.

Conclusions: Our systematic review demonstrates that children with cochlear nerve aplasia and hypoplasia may achieve similar audiometric and speech-language outcomes following CI, contradicting the notion that aplasia confers inferior results. Benefit was greatest in children under two years old. Clinicians may counsel families that cochlear nerve deficiency alone should not preclude CI candidacy, and that age of implantation can be critical for implant success.