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Cochlear implantation in Children with CHARGE syndrome: therapeutic decisions and outcomes
Biana G Lanson1, Janet E Green, J Thomas Roland
1Department of Otolaryngology, NYU School of Medicine, New York, NY 10016, USA.
Insights
Cochlear implantation in children with CHARGE syndrome offers limited auditory benefits and does not improve oral language skills. Careful planning is crucial for managing hearing loss in these patients.
Area of Science:
- Otolaryngology
- Genetics
- Pediatrics
Background:
- CHARGE syndrome is a genetic disorder associated with congenital anomalies, including ear abnormalities and deafness.
- Sensorineural hearing loss is a common and significant challenge in children with CHARGE syndrome.
- Cochlear implantation is a potential intervention for severe to profound hearing loss.
Purpose of the Study:
- To evaluate the effectiveness of cochlear implantation in children diagnosed with CHARGE syndrome.
- To assess the auditory outcomes and surgical complications associated with cochlear implantation in this population.
- To explore the relationship between cochlear deformities and implantation outcomes.
Main Methods:
- Retrospective chart review of eleven children with CHARGE syndrome and sensorineural hearing loss.
- Pre- and postoperative audiological assessments, including routine audiometry and the Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS).
- Measurement and correlation of cochlear deformity with implantation outcomes.
Main Results:
- Ten out of eleven eligible children with CHARGE syndrome underwent successful cochlear implantation without complications.
- Implanted children demonstrated varying degrees of auditory benefit, as indicated by audiological tests and IT-MAIS scores.
- One child was not implanted due to severe developmental retardation.
Conclusions:
- Cochlear implantation in children with CHARGE syndrome can provide some auditory benefits when carefully planned.
- The procedure did not lead to an increase in surgical complications.
- While improving environmental sound awareness, cochlear implants did not significantly enhance oral language development in this cohort.
Objectives:
Ear anomalies and deafness are associated with CHARGE syndrome, which also presents with a cluster of features including coloboma of the eye, heart defects, atresia of the choanae, developmental retardation, and genitourinary abnormalities. The aim of this study is to explore the viability of cochlear implantation in children with CHARGE syndrome and to assess the outcome.
Study Design:
Retrospective chart review.
Methods:
Eleven children presenting with severe to profound sensorineural hearing loss associated with CHARGE syndrome were the subjects of this study. Routine audiometric measurements and the Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS) were performed pre- and postoperatively. In addition, the degree of the subjects' cochlear deformity were measured and correlated to outcome.
Results:
All patients had varying degrees of ear anomalies, seven patients suffered from coloboma of the eyes, two had heart defects, five exhibited choanal atresia, eleven showed developmental retardation, and six had genitourinary abnormalities. Ten of the children underwent cochlear implantation with complete insertion of the electrode array without complication and were followed over a 3-month to a 7-year period. The eleventh child was not implanted because of severe retardation. All of the implanted children showed varying, but limited degrees, of auditory benefit as measured by routine audiometry and the IT-MAIS.
Conclusions:
Careful treatment planning for children with sensorineural hearing loss and CHARGE syndrome can lead to varying, but limited degrees, of auditory benefit with no increase in surgical complications. Although the implant enhanced the children's 'connectivity' to the environment, it did not promote the development of oral language skills in this population.