Related Experiment Video
Updated: Jul 31, 2026
Robotic Cochlear Implantation for Direct Cochlear Access
Published on: June 16, 2022
Cochlear implants in children with craniofacial syndromes: assessment and outcomes
B M MacArdle1, C Bailey, P D Phelps
1Cochlear Implant Programme, Great Ormond Street Hospital NHS Trust, London, UK. breege.macardle@rfh.nthames.nhs.u
Insights
Cochlear implants offer benefits for children with craniofacial syndromes, improving sound awareness and language skills. However, these children require specialized care and longer rehabilitation periods for optimal outcomes.
Area of Science:
- Otolaryngology
- Pediatric Audiology
- Medical Genetics
Background:
- Cochlear implantation in children with craniofacial syndromes presents unique challenges.
- Understanding outcomes is crucial for guiding clinical practice and parental counseling.
Observation:
- This retrospective study analyzed four pediatric patients with craniofacial syndromes (CHARGE association, Goldenhar syndrome, brachio-oculo-facial syndrome) who received cochlear implants.
- All patients underwent full electrode insertion.
- Follow-up data was collected on auditory perception and spoken language development.
Findings:
- Three out of four children showed improvements in environmental sound detection, recognition, and identification.
- Receptive spoken language skills advanced, but intelligible speech was not achieved in any participant.
- Patients required higher electrical stimulation levels and careful audiologic mapping compared to typically developing children.
- Facial nerve stimulation occurred in one case.
Implications:
- Cochlear implantation can be beneficial for select children with craniofacial syndromes, enhancing auditory access and language development.
- Extensive pre-operative assessment, including interdisciplinary evaluation and radiological imaging, is essential.
- Realistic parental counseling regarding long-term commitment and habilitation timelines is critical.
- Post-implantation management necessitates a coordinated, multidisciplinary approach.
Abstract:
The aim of this retrospective study was to review the outcomes for children with craniofacial syndromes who had received a cochlear implant. The group comprised four children (three girls, one boy) aged between 3.3 and 10.1 years (mean 6.3 years) at time of implantation with the Cochlear CI-22M device. Two children had the CHARGE association. one had Goldenhar's syndrome and one had brachio-oculo-facial syndrome. All had full electrode insertion at time of surgery. At follow-up, three of the children demonstrated benefit in detection, recognition and identification of environmental sounds, and they continued to gain receptive spoken language skills, although none had intelligible speech. The group required careful mapping and higher levels of electrical stimulation of the implant compared to normal child implantees. Stimulation of the facial nerve was a problem with one child. The pre-implantation assessment of these children requires extensive interdisciplinary discussion and careful radiological investigation. Cases should be carefully selected. Parents should receive realistic counselling about outcomes and the time commitment necessary, as habilitation of these children can take twice as long as that of children without additional special needs. Post-implantation, these children continue to require well-coordinated medical and interdisciplinary management.
More Related Videos
06:04Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
Published on: March 24, 2023
07:00Electrically Evoked Stapedius Reflex Measurements in Cochlear Implantation and Its Application in the Postoperative Fitting Process
Published on: June 21, 2024