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Updated: Apr 17, 2026
Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
Published on: March 24, 2023
CHARGE syndrome and Cochlear implantation: difficulties and outcomes in the paediatric population
Catherine S Birman1, Jane A Brew2, William P R Gibson3
1Discipline of Paediatrics and Child Health, Sydney Medical School, University of Sydney, Sydney, Australia; Sydney Children's Hospital Network (Children's Hospital at Westmead), Hawkesbury Road, Westmead 2145, NSW, Australia; The Sydney Cochlear Implant Centre, Royal Institute for Deaf and Blind Children, PO Box 188, Gladesville 1675, NSW, Australia; Department of Linguistics, Faculty of Human Sciences, Macquarie University, North Ryde, Australia.
Insights
Cochlear implants in children with CHARGE syndrome show varied outcomes. Congenital profound hearing loss with absent auditory nerves presents challenges, while progressive hearing loss cases achieve good verbal language results.
Area of Science:
- Otolaryngology
- Pediatric audiology
- Medical genetics
Background:
- CHARGE syndrome is a genetic disorder causing complex congenital abnormalities.
- Auditory nerve hypoplasia or absence is a common feature in CHARGE syndrome, impacting hearing.
- Cochlear implantation is a potential intervention for hearing loss in these children.
Purpose of the Study:
- To evaluate preoperative factors and outcomes for pediatric cochlear implant recipients with CHARGE syndrome.
- To improve surgical preparation and family counseling for cochlear implantation in CHARGE syndrome.
- To analyze the impact of auditory nerve status and middle ear anatomy on cochlear implant success.
Main Methods:
- Retrospective database search of pediatric cochlear implant recipients with CHARGE syndrome (age ≤16).
- Data collection included clinical history, hearing assessments, imaging (MRI/CT), electrophysiological tests (ABR), and language outcomes (CAP scores).
- Surgical findings and intraoperative electrophysiological measures were also recorded.
Main Results:
- Seven children with congenital profound hearing loss had bilateral auditory nerve hypoplasia/absence; three required revision surgery.
- Abnormal middle ear anatomy complicated cochlear implant insertion in several cases.
- Children with progressive hearing loss and normal auditory nerves achieved excellent verbal language outcomes (CAP scores of 6).
Conclusions:
- CHARGE syndrome with congenital profound hearing loss and auditory nerve anomalies presents significant challenges for cochlear implantation.
- CT image-guided surgery may aid in cases with complex middle ear anatomy.
- A bilingual early intervention approach (sign and verbal language) is recommended for optimal language development in these children.
Objectives:
CHARGE syndrome is a complex cluster of congenital abnormalities, these children may have absent or hypoplastic auditory nerves. Our objective was to assess preoperative factors and outcomes for paediatric cochlear implant recipients with CHARGE syndrome, to enable better surgical preparation and family counselling.
Methods:
The Sydney Cochlear Implant Centre database was searched for children with CHARGE syndrome who had received a cochlear implant at ages 16 and less. Data were collected regarding clinical history; hearing assessments; MRI and CT scan findings; preoperative transtympanic electrical Auditory Brainstem Response (ABR); intraoperative findings and intraoperative electrical ABR and Neural Response Telemetry; and language outcomes in terms of main language used and Categories of Auditory Performance scores (0-7 ranking).
Results:
Ten children were identified. All seven prelingual profoundly deaf children with CHARGE syndrome had hypoplastic or absent auditory nerves bilaterally on MRI scans. Middle ear anatomy was often abnormal, affecting surgical landmarks and making identification of the cochlea very difficult in some cases. Three cases required repeated surgery to obtain successful cochlear implant insertion, one under CT scan image guided technique. All seven children used sign language, or simpler gestures, as their main mode of communication. Two children of of these children, who were implanted early, also attained some spoken language. CAP scores ranged from 0 to 6. The three children with CHARGE syndrome and progressive sensorineural hearing loss had a normal auditory nerve in at least one ear on MRI scans. All had preoperative verbal language, with CAP scores of 6, and continued with CAP scores of 6 following receipt of the cochlear implant.
Conclusion:
Children with CHARGE and congenital profound hearing loss all had hypoplasia or absent auditory nerves, affecting their outcomes with cochlear implants. They often had markedly abnormal middle ear anatomy and CT image guided surgery can be helpful. These children should be offered a bilingual early intervention approach, using sign language and verbal language, to ensure best language outcomes. Children with CHARGE syndrome and progressive profound hearing loss did well with cochlear implants and continue to be able to use verbal language.
