Influence of DFNB1 status on expressive language in deaf children with cochlear implants

Simon I Angeli, Hamlet Suarez, Alina Lopez, Thomas J. Balkany, Xue Z Liu

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

Objective: The objective of this study was to compare the language growth of children with connexin-related deafness (DFNB1) who received cochlear implants versus the language growth of implanted children with non-DFNB1 deafness. Study Design: A prospective longitudinal observational study and analysis. Seting: Two tertiary referral centers. Patients: There were 37 children with severe-to-profound hearing loss who received cochlear implants before the age of 5 years. Interventions: A standardized language measure, the section for expressive language of the Reynell Developmental Language Scale was used to assess expressive language skills at 2 times postimplantation (14 and 57 mo postimplantation). Molecular screening for DFNB1 gene variants. Main Outcome Measures Language quotient (LQ) scores (i.e., age-equivalent score obtained on the Reynell Developmental Language Scale divided by the child's chronological age), results of genotyping. Results: The mean language age at the second time interval (mean ± standard deviation, 51.8 ± 13 mo) was greater than at the first testing session (mean ± standard deviation, 19 ± 8 mo, p < 0.001, Wilcoxon signed rank test). When divided by genotype, DFNB1 children exhibited a higher LQ and less variability in scores than non-DFNB1 children at the second testing interval (Wilcoxon sign rank test, p = 0.0034). A regression analysis (linear-fit by least squares) conducted on 26 children with preimplantation audiometric data showed that DFNB1 status was the independent variable with greater predictive effect on LQ at the second testing interval, followed by age at implantation (R = 0.35, p = 0.0479). Conclusion: Deaf children who received cochlear implants before the age of 5 years and use oral communication show substantial improvement in language abilities. In this study, DFNB1 children who use cochlear implants show greater gains in expressive language than non-DFNB1 children, independent of residual hearing, age at implantation, and duration of implant use.

Original languageEnglish
Pages (from-to)1437-1443
Number of pages7
JournalOtology and Neurotology
Volume32
Issue number9
DOIs
StatePublished - Dec 1 2011

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Cochlear Implants
Language
Deafness
Nonparametric Statistics
Child Language
Connexins
Aptitude
Growth
Least-Squares Analysis
Hearing Loss
Tertiary Care Centers
Hearing
Observational Studies
Longitudinal Studies
Communication
Genotype
Regression Analysis
Outcome Assessment (Health Care)

ASJC Scopus subject areas

  • Otorhinolaryngology
  • Clinical Neurology
  • Sensory Systems
  • Medicine(all)

Cite this

Influence of DFNB1 status on expressive language in deaf children with cochlear implants. / Angeli, Simon I; Suarez, Hamlet; Lopez, Alina; Balkany, Thomas J.; Liu, Xue Z.

In: Otology and Neurotology, Vol. 32, No. 9, 01.12.2011, p. 1437-1443.

Research output: Contribution to journalArticle

Angeli, Simon I ; Suarez, Hamlet ; Lopez, Alina ; Balkany, Thomas J. ; Liu, Xue Z. / Influence of DFNB1 status on expressive language in deaf children with cochlear implants. In: Otology and Neurotology. 2011 ; Vol. 32, No. 9. pp. 1437-1443.
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abstract = "Objective: The objective of this study was to compare the language growth of children with connexin-related deafness (DFNB1) who received cochlear implants versus the language growth of implanted children with non-DFNB1 deafness. Study Design: A prospective longitudinal observational study and analysis. Seting: Two tertiary referral centers. Patients: There were 37 children with severe-to-profound hearing loss who received cochlear implants before the age of 5 years. Interventions: A standardized language measure, the section for expressive language of the Reynell Developmental Language Scale was used to assess expressive language skills at 2 times postimplantation (14 and 57 mo postimplantation). Molecular screening for DFNB1 gene variants. Main Outcome Measures Language quotient (LQ) scores (i.e., age-equivalent score obtained on the Reynell Developmental Language Scale divided by the child's chronological age), results of genotyping. Results: The mean language age at the second time interval (mean ± standard deviation, 51.8 ± 13 mo) was greater than at the first testing session (mean ± standard deviation, 19 ± 8 mo, p < 0.001, Wilcoxon signed rank test). When divided by genotype, DFNB1 children exhibited a higher LQ and less variability in scores than non-DFNB1 children at the second testing interval (Wilcoxon sign rank test, p = 0.0034). A regression analysis (linear-fit by least squares) conducted on 26 children with preimplantation audiometric data showed that DFNB1 status was the independent variable with greater predictive effect on LQ at the second testing interval, followed by age at implantation (R = 0.35, p = 0.0479). Conclusion: Deaf children who received cochlear implants before the age of 5 years and use oral communication show substantial improvement in language abilities. In this study, DFNB1 children who use cochlear implants show greater gains in expressive language than non-DFNB1 children, independent of residual hearing, age at implantation, and duration of implant use.",
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