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
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.
Available from: Mancini Patrizia
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To evaluate, with a long-term follow-up, the speech perception and language development in children with cytomegalovirus (CMV)-related deafness after cochlear implantation.
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.
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.
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.
Available from: Ken Chin-Lung Kuo 郭錦龍
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Following cholesteatoma surgery, effective long-term hearing preservation in children is difficult and is not typically expected. Hence, long-term data on hearing outcomes are lacking. The aim of this study was to analyze long-term hearing outcomes in children following cholesteatoma surgery.
For this study, 49 ears in 47 children (≤16 years) with acquired cholesteatomas following atticotomy-limited mastoidectomy with cartilage reconstruction (inside-out approach) during 1986-2010 were included. Pre- and post-operative recidivism-free audiometric results were compared. Hearing success was defined as a post-operative air conduction (AC) threshold of ≤30 dB (serviceable hearing). Logistic regression analyses were used to evaluate potential prognostic factors that independently contributed to the prediction of hearing success. These factors included stapes condition, pre-operative AC threshold, ossicular chain integrity, disease severity, age, and gender.
The mean duration of follow-up was 14.2 years. The post-operative AC (33.55 ± 15.42 dB) and air-bone gap (17.88 ± 12.94 dB) were significantly improved compared with the pre-operative AC (42.90 ± 16.47 dB, p < 0.001) and air-bone gap (30.23 ± 13.68 dB, p < 0.001). The probability of hearing success following surgery (40.8%) was significantly higher than prior to surgery (24.5%, p = 0.008). Multivariate logistic regression analyses revealed a statistically significant correlation between hearing success and stapes integrity only (p = 0.005).
This study provides important information on effective long-term hearing preservation over a mean follow-up of 14 years. In addition, stapes destruction is an independent negative prognostic determinant of achieving hearing success. The prediction model in this study provides otologists with useful pre-operative information to inform patients and parents on expected hearing outcomes and may be useful for post-operative observations.
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To compare the long-term speech perception and production outcomes after cochlear implantation (CI) in children deafened by congenital cytomegalovirus (cCMV) with a matched group of Cx26-CI children by controlling for chronological age and magnetic resonance imaging (MRI) findings.
Retrospective review of 12 cCMV-CI children and matched Cx26-CI children for speech perception and speech production outcomes.
Two trends were seen in our data. First, cCMV-CI children with normal MRI scans perform equally or even slightly better on speech perception tests compared to their Cx26-CI peers during the first three years. The majority of cCMV-CI children with normal MRI scans (5 out of 7), suffered from a delayed-onset SNHL. Their mean age at first implantation (2yr9m, range 15m-82m) was higher compared to their matched Cx26 peers (9m, range 7m-12m). Before being implanted, the majority of these delayed-onset hearing impaired children had benefitted from a certain period of normal hearing (with or without amplification of a hearing aid). Possibly, this input might have led to an advantage the first three years after CI. Second, results between cCMV-CI children with and cCMV-CI children without MRI abnormalities and their matched Cx26-CI counterparts tentatively suggest that, over a 5-yr follow-up period, cCMV-CI children with abnormalities on MRI scans catch up for speech perception, but lag behind for speech production.
cCMV-CI children with normal MRI scans perform equally or even slightly better on speech perception tests compared to their Cx26-CI peers during the first three years, whereas results between cCMV-CI children with and cCMV-CI children without MRI abnormalities and their matched Cx26-CI counterparts tentatively suggest that, over a 5-yr follow-up period, cCMV-CI children with abnormal MRI scans catch up for speech perception, but lag behind for speech production. In future, the inclusion of MRI results may assist in improved counseling of parents with cCMV deafened children seeking CI.
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