Outcome of cochlear implantation in children with congenital cytomegalovirus infection and GJB2 mutation in relation to developmental disorder

✍ Scribed by T Matsui; H Ogawa; N Yamada; Y Baba; M Nomoto; K Omori

• Publisher: John Wiley and Sons
• Year: 2011
• Tongue: English
• Weight: 252 KB
• Volume: 121
• Category: Article
• ISSN: 0023-852X
• DOI: 10.1002/lary.22282

✦ Synopsis

Objective: Congenital cytomegalovirus (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 in many populations. Here, outcomes for auditory behavior and speech and language skills were compared between children with congenital CMV infection or GJB2 mutation who received cochlear implantation for profound sensorineural hearing loss (SNHL).

Study Design: Longitudinal study

Methods:

Five children with asymptomatic congenital CMV infection and 7 children with GJB2 mutation-related SNHL, with and without developmental disorder, underwent cochlear implantation. Hearing level and speech and language development were evaluated postimplantation using the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS), the Meaningful Use of Speech Scale (MUSS), and the Test for Language Retardation Based on Sign-Significate Relations (S-S method). Results: The average hearing level with cochlear implantation was 36.5 dB in the congenital CMV infection group and 36.8 dB in the GJB2 mutation group. 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.

Conclusions:

Outcomes following cochlear implantation in children with congenital CMV infection were almost equivalent to those of children with GJB2 mutation-related SNHL. Because our patients with developmental disorder showed poor but auditory performance and speech and language skills after cochlear implantation, SNHL with developmental disorder should not be a contraindication for the procedure.