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Updated: May 4, 2026
Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
Published on: March 24, 2023
Outcome of cochlear implantation in children with cochlear malformations
Jesper Bille1, Vibeke Fink-Jensen, Therese Ovesen
1Department of Otorhinolaryngology, Head and Neck Surgery, Aarhus University Hospital, Noerrebrogade 44, 8000, Aarhus C, Denmark.
Insights
Cochlear implantation (CI) in children with inner ear malformations yields outcomes comparable to those without malformations. Key factors for success in pediatric hearing restoration include implantation age and duration of hearing loss.
Area of Science:
- Otolaryngology
- Pediatric Audiology
- Neurosurgery
Background:
- Cochlear malformations present unique challenges for cochlear implantation (CI) in children.
- Evaluating long-term auditory and speech outcomes is crucial for this patient population.
Purpose of the Study:
- To assess the effectiveness of cochlear implantation in children with inner ear malformations.
- To compare auditory and speech performance between children with and without cochlear malformations post-CI.
Main Methods:
- Retrospective case-control study of children undergoing cochlear implantation.
- High-resolution computed tomography and magnetic resonance imaging used for diagnosis of inner ear malformations.
- Standard perimodiolar electrode utilized in all cases with a minimum 3-year follow-up.
Main Results:
- No significant differences in Category of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) scores between groups.
- Children with cochlear malformations performed similarly to controls in the long term.
- Age at implantation and duration of hearing loss were identified as critical outcome determinants.
Conclusions:
- Cochlear implantation is effective for children with cochlear malformations, achieving comparable results to non-malformed peers.
- Standard perimodiolar electrodes are suitable for use in these cases.
- Early implantation and minimizing duration of hearing loss are paramount for optimal outcomes.
Abstract:
The objective of the study was the evaluation of outcomes of cochlear implantation (CI) in children with cochlear malformations. A retrospective case-control study was conducted in a tertiary referral centre. The patients were children with inner ear malformation judged by high-resolution computed tomography and magnetic resonance imaging treated with uni- or bilateral CI and a follow-up period of at least 3 years. They were matched with a control group of children operated for other reasons. The patients were operated by one of two surgeons using similar techniques including a standard perimodiolar electrode in all cases. The intervention was therapeutic and rehabilitative. The main outcome measures were category of auditory performance (CAP) and speech intelligibility rating (SIR). Eighteen children were diagnosed with cochlear malformations (12 % of children receiving CI). No statistical differences regarding CAP and SIR scores were found between the two groups. Only one child was diagnosed with a common cavity and performed below average. Children with auditory neuropathy performed beyond average. Children with cochlear malformations performed equally to children without malformation in the long term. Standard perimodiolar electrodes can be used despite cochlear malformations. The most important factors determining the outcome is the age of the child at the time of implantation and duration of hearing loss before CI. Awareness towards an increased risk of complications in case of inner ear malformations is recommended.
