Related Experiment Video
Updated: Jul 16, 2026
Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
Published on: March 24, 2023
Audiological performance after cochlear implantation: a 2-year follow-up in children with inner ear malformations
Gijs K A Van Wermeskerken1, Erwin A Dunnebier, Adriaan F Van Olphen
1Department of Otorhinolaryngology - Head and Neck Surgery, University Medical Center Utrecht, Utrecht, The Netherlands. gijs@vanwermeskerken.com
Conclusions:
Open-set speech perception in children with an inner ear malformation is equal to that of other congenitally deaf children after an average of 2 years follow-up.
Objective:
To analyze audiological performance after cochlear implantation in a sample of children with radiographically detectable malformations of the inner ear compared to performance in prelingually deafened children at large.
Materials And Methods:
Nine children with osseous inner ear malformations were compared to 22 congenitally deaf children, all of whom underwent cochlear implantation. All subjects were tested on their electrical evoked compound action potential. Speech perception tests were performed using the monosyllabic trochee polysyllabic test without visual support and the open-set monosyllabic wordlist.
Results:
In all, 20% of the congenitally deaf children in our center study have inner ear abnormalities. Inner ear malformations were limited to incomplete partition of the cochlea; none of the subjects had common cavity malformations. Electrical compound action potentials were successfully recorded in both groups intraoperatively. Speech perception tests on open-set speech yielded an average of 48.8% (SD 21.2%) in the group of children with inner ear malformations vs 54.5% (SD 21.1%) in congenitally deaf children. In four of nine cases with an inner ear malformation we encountered a minor CSF leak.
