Article

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

Department of Otolaryngology, Fukushima Medical University, Fukushima, Japan.
Acta oto-laryngologica (Impact Factor: 1.1). 02/2012; 132(6):597-602. DOI: 10.3109/00016489.2011.653445
Source: PubMed

ABSTRACT

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.
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.
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.
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.

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    ABSTRACT: Objective: To evaluate, with a long-term follow-up, the speech perception and language development in children with cytomegalovirus (CMV)-related deafness after cochlear implantation. Study design: A retrospective study on CMV-related profound deafness and cochlear implantation was performed from 1995 to 2010. Six children with an average follow-up of 10 years were included in this research. Medical history, imaging, cognitive delay, speech perception and production data were reviewed. Results: Two of the 6 patients developed a functional language with the use of phrases and word sequences based on morphological and syntactic rules; the others demonstrated the development of a preverbal or transitional language with the use of single words only. Conclusion: Patients with CMV-related deafness benefit from cochlear implantation; however, the expectations of the parents must be evaluated in a series of counseling efforts prior to the surgery.
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