Development of auditory skills after cochlear implantation in children with inner ear malformations

CI improves hearing thresholds and auditory skills in children with most types of inner ear malformations. However, the development of sound detection skills is not as good as it is in children without inner ear malformations.

To investigate the influence of inner ear malformations on development of auditory skills after cochlear implantation (CI).

Records of 20 children with inner ear malformations who underwent cochlear implantation before 4 years of age and followed up for more than 2 years were retrospectively reviewed. Hearing thresholds, the Meaningful Auditory Integration Scale (MAIS), and Meaningful Use of Speech Scale (MUSS) scores before and after CI were analyzed and compared with 20 age-matched deaf children who underwent CI.

The children with inner ear malformations showed significant improvements in hearing thresholds and the MAIS and MUSS scores 1 year after CI (p < 0.01). However, their development of the MAIS scores was significantly delayed compared to children without inner ear malformations (p < 0.05), while there was no significant difference in development of the MUSS scores between them. Significant improvements in hearing thresholds and the MAIS and MUSS scores were observed in cochlear hypoplasia and incomplete partition (p < 0.05).