Outcome of cochlear implantation in children with congenital cytomegalovirus infection or GJB2 mutation

Abstract

Conclusions: Outcomes following cochlear implantation in children with congenital cytomegalovirus (CMV) infection were almost equivalent to those of children with GJB2 mutation-related sensorineural hearing loss (SNHL). Although our patients with developmental disorder showed poor auditory performance and speech and language skills after cochlear implantation, SNHL with developmental disorder should not be a contraindication for the procedure. Objective: Congenital CMV infection accounts for approximately 20% of all cases of neonatal hearing loss, while the GJB2 mutation accounts for 30–50% of all cases of profound nonsyndromic hearing loss. Here, outcomes for auditory behavior and speech and language skills were compared in children with congenital CMV infection or GJB2 mutation who received cochlear implantation for profound SNHL. Methods: Five children with asymptomatic congenital CMV infection and seven children with GJB2 mutation-related SNHL, with and without developmental disorder, underwent cochlear implantation. Hearing level and speech and language development were evaluated post-implantation using IT-MAIS, MUSS, and S-S method. Results: The IT-MAIS and MUSS scores of the congenital CMV infection group and the GJB2 mutation group continued to increase for 4 years after implantation. The S-S method score in both groups gradually increased, although the scores for children with mental retardation were low.

Keywords::

• Congenital cytomegalovirus infection
• GJB2 mutation
• cochlear implantation
• sensorineural hearing loss
• developmental disorder

Acknowledgments

We thank all the children who participated in the study and their parents. This work was supported by a Grant-in-Aid for Young Scientists (B) (21791630) from the Ministry of Education, Culture, Sports, Science and Technology (MEXT).

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.