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Cochlear Implant Surgery and Electrically-evoked Auditory Brainstem Response Recordings in C57BL/6 Mice
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Auditory Skills following Cochlear Implantation in Children with the Charge Syndrome

Paula Aragón-Ramos1, Maria-Fernanda Pedrero-Escalas2, Javier Gavilán2

  • 1Department of Otorhinolaryngology, La Paz University Hospital, Madrid, Spain, p.aragon.ramos@gmail.com.

Audiology & Neuro-Otology
|July 11, 2019
PubMed

Insights

Cochlear implantation (CI) in children with CHARGE syndrome is safe and improves auditory skills. Inner ear malformations present surgical challenges but do not prevent sound detection and identification.

Area of Science:

  • Otolaryngology
  • Pediatric audiology
  • Genetics

Background:

  • CHARGE syndrome is a complex genetic disorder associated with congenital anomalies, including inner ear malformations.
  • Auditory development is frequently impaired in children with CHARGE syndrome, necessitating auditory habilitation strategies.

Purpose of the Study:

  • To evaluate auditory outcomes in pediatric cochlear implant (CI) users with CHARGE syndrome.
  • To investigate the impact of inner ear malformations on surgical procedures and speech understanding in this population.

Main Methods:

  • Retrospective observational study analyzing data from pediatric CI users with CHARGE syndrome.
  • Data collected included imaging, auditory testing, intraoperative findings, complications, and postoperative auditory skills.

Main Results:

  • Eight ears from 6 children with CHARGE syndrome were analyzed; 5 had prelingual deafness.
  • Cochlear malformations were present in 6 ears (hypoplasia type III most common).
  • All children demonstrated improved auditory skills, with 2 developing verbalization and 1 using oral language.

Conclusions:

  • Cochlear implantation is a safe procedure for pediatric patients with CHARGE syndrome when performed by experienced surgeons with careful planning.
  • While inner ear malformations pose surgical challenges, CI can lead to variable but significant improvements in auditory skills.
Abstract

Keywords:
Auditory skillsCHARGE syndromeCochlear implantation

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