Related Experiment Video

Updated: Jul 30, 2025

Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
06:04

Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages

Published on: March 24, 2023

424

Programming Levels and Speech Perception in Pediatric Cochlear Implant Recipients With Enlarged Vestibular Aqueduct

Kelly N Jahn1, Charlotte Morse-Fortier2, Amanda M Griffin

  • 1Department of Speech, Language, and Hearing, University of Texas at Dallas, Richardson, Texas.

Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [And] European Academy of Otology and Neurotology
|May 11, 2023
PubMed

Insights

Pediatric cochlear implant (CI) recipients with enlarged vestibular aqueduct (EVA) showed different device programming needs compared to those with GJB2 mutations. However, speech perception depended more on CI experience than the cause of hearing loss.

Area of Science:

  • Audiology and Otolaryngology
  • Pediatric Medicine
  • Genetics and Hearing Loss

Background:

  • Cochlear implants (CIs) are crucial for hearing restoration in children.
  • Understanding how hearing loss etiology affects CI outcomes is vital for personalized care.
  • Enlarged vestibular aqueduct (EVA) and GJB2 (Connexin-26) mutations are common causes of pediatric hearing loss.

Purpose of the Study:

  • To investigate the relationship between hearing loss causes (EVA vs. GJB2 mutation), CI programming parameters, and speech perception in children.
  • To identify factors influencing speech understanding in pediatric CI users.

Main Methods:

  • Retrospective chart review of 136 pediatric CI recipients (218 ears) with confirmed EVA or GJB2 mutations.
  • Analysis of electrode impedances, CI programming levels (thresholds and upper stimulation levels), and speech perception scores.
  • Comparison of outcomes based on hearing loss etiology and duration of CI use.

Main Results:

  • Children with EVA exhibited significantly higher electrode impedances and CI programming levels than those with GJB2 mutations.
  • Speech perception scores were comparable between etiologies but positively correlated with the duration of CI use.
  • These findings suggest differences in the electrode-neuron interface based on hearing loss etiology.

Conclusions:

  • Hearing loss etiology influences CI device programming requirements in pediatric patients.
  • Duration of cochlear implant experience is a stronger predictor of speech perception than the underlying cause of hearing loss.
  • Pediatric CI recipients demonstrate adaptability to CI stimulation over time, highlighting the importance of consistent audiological management.
Abstract

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