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Vestibular function in children with vestibulocochlear nerve aplasia/hypoplasia

Niki Karpeta1,2, Filip Asp1,2, Kaijsa Edholm3

  • 1Division of Ear, Nose and Throat Diseases, Department of Clinical Science, Intervention and Technology, Karolinska Institute, Stockholm, Sweden.

Acta Oto-Laryngologica
|December 8, 2023
PubMed

Insights

Vestibular tests in infants with vestibulocochlear nerve (VCN) hypoplasia/aplasia show functioning pathways. Normal vestibular responses predict better hearing outcomes after cochlear implantation (CI).

Area of Science:

  • Otolaryngology
  • Neuroscience
  • Pediatric Audiology

Background:

  • Infants with vestibulocochlear nerve (VCN) hypoplasia/aplasia experience severe hearing loss, making them candidates for cochlear implantation (CI).
  • The relationship between vestibular function and CI outcomes, and the utility of vestibular tests in surgical decision-making, remain unclear.

Purpose of the Study:

  • To characterize vestibular function in pediatric patients diagnosed with VCN hypoplasia/aplasia prior to potential CI.
  • To investigate if vestibular function assessment can inform CI candidacy and predict audiological outcomes.

Main Methods:

  • Utilized bone-conducted cervical vestibular evoked myogenic potentials (BCcVEMP), video head impulse test (vHIT), and mini ice-water caloric tests (mIWC).
  • Tested 42 ears across 23 pediatric patients between 2019 and 2022.
  • Achieved high testability rates, with 83% of ears assessed using multiple vestibular methods.

Main Results:

  • A significant portion of patients (61%) demonstrated normal vestibular function on at least one test, despite radiological VCN abnormalities.
  • The presence of any normal vestibular response was associated with a twofold increase in the likelihood of achieving a measurable hearing threshold post-CI.
  • BCcVEMP and vHIT emerged as the strongest predictors of favorable audiological outcomes.

Conclusions:

  • Vestibular testing reveals functional pathways in many infants with VCN hypoplasia/aplasia, challenging purely radiological interpretations.
  • Canal function appears to be a valuable predictor of auditory pathway integrity and potential audiological success following cochlear implantation.
Abstract

Keywords:
Vestibular functionchildrencochlear implantsvestibular evoked myogenic potentialsvestibulocochlear nerve aplasiavideo head impulse test

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