Long term speech perception after cochlear implant in pediatric patients with GJB2 mutations

To determine the long term effect of cochlear implant (CI) in children with GJB2-related deafness in Japan.

Genetic testing was performed on 29 children with CI. The speech perception in 9 children with GJB2 gene-related deafness fitted with CI was compared with those in matched 10 children who were diagnosed as having no genetic loci. The average follow-up period after CI was 55.9 months and 54.6 months, respectively.

A definitive inherited hearing impairment could be confirmed in 12 (41.4%) of the 29 CI children, including 10 with GJB2-related hearing impairment and 2 with SLC26A4-related hearing impairment. The results of IT-MAIS, word or speech perception testing under the noise, and development of speech perception and production testing using the Enjoji scale were slightly better for the GJB2 group after CI than for the control group without statistical significant difference.

The long-term results of this study show that CI is also effective in the development of speech performance after CI in Japanese children with GJB2-related hearing impairments as HL due to other etiologies.