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Updated: Sep 28, 2025

Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
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Cochlear Nerve Deficiency in Pediatric Unilateral Hearing Loss and Asymmetric Hearing Loss

Teresa G Vos1, Lisa R Park1, Amy S Noxon2

  • 1Department of Otolaryngology/Head & Neck Surgery, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.

Audiology & Neuro-Otology
|March 28, 2022
PubMed

Insights

Pediatric unilateral hearing loss (UHL) shows a high incidence of cochlear nerve deficiency (CND), affecting nearly 30% of UHL cases. MRI is crucial for diagnosing CND in children with UHL or asymmetric hearing loss (AHL).

Area of Science:

  • Pediatric audiology
  • Neuroradiology
  • Genetics of hearing loss

Background:

  • Conflicting literature exists regarding cochlear nerve abnormalities in pediatric unilateral hearing loss (UHL).
  • Accurate diagnosis is essential for effective management and intervention strategies.

Purpose of the Study:

  • To determine the incidence of cochlear nerve deficiency (CND) in pediatric subjects with UHL or asymmetric hearing loss (AHL).
  • To evaluate the role of advanced imaging in diagnosing inner ear malformations.

Main Methods:

  • Retrospective chart review of 291 pediatric patients (<18 years) with UHL or AHL.
  • Fine-cut heavily T2-weighted MRI and CT temporal bone imaging were analyzed.
  • Cochlear nerve status, inner ear malformations, and audiometric thresholds (PTA) were assessed.

Main Results:

  • CND was identified in 29.9% of UHL cases and 11.5% of AHL cases.
  • Ipsilateral cochlear malformations occurred in 12.3% of UHL and 12.6% of AHL.
  • Higher median PTA was observed in ears with CND compared to normal nerves.

Conclusions:

  • A high incidence of CND and inner ear malformations exists in pediatric UHL.
  • CND cannot be excluded based on hearing thresholds alone.
  • MRI is recommended for all pediatric UHL/AHL patients to guide management and cochlear implantation candidacy.
Abstract

Keywords:
Cochlear nerve aplasiaCochlear nerve hypoplasiaSingle-sided deafness

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