Better speech performance in cochlear implant patients with GJB2-related deafness

Kunihiro Fukushima; Kennichi Sugata; Norio Kasai; Shouichirou Fukuda; Rie Nagayasu; Naomi Toida; Nobuhiko Kimura; Teruaki Takishita; Mehmet Gunduz; Kazunori Nishizaki

doi:10.1016/S0165-5876(01)00619-X

Better speech performance in cochlear implant patients with GJB2-related deafness

Kunihiro Fukushima, Kennichi Sugata, Norio Kasai, Shouichirou Fukuda, Rie Nagayasu, Naomi Toida, Nobuhiko Kimura, Teruaki Takishita, Mehmet Gunduz, Kazunori Nishizaki

Graduate School of Medicine Dentistry and Pharmaceutical Sciences

Research output: Contribution to journal › Article › peer-review

71 Citations (Scopus)

Abstract

Objective: We applied mutation screening in seven cochlear implant users to identify those persons with GJB2-related deafness to determine whether etiology of deafness was predictive of speech performance after implantation. Methods: Direct sequence of GJB2 was conducted over seven cochlear implant users with prelingual hearing impairment and their speech, language and cognitive performance was examined. Results: The three persons with GJB2-related deafness had a mean vocabulary of 1243 words compared to a mean vocabulary of 195 words in the four children with GJB2-unrelated deafness, although the number of patients examined here was limited. The developmental quotient (DQ) of cognitive ability also was higher in those children with GJB2-related deafness. Conclusions: These preliminary results suggest that better speech performance after cochlear implantation may be observed in persons with GJB2-related deafness. In the future, detailed phenotypic studies and mutation screening for non-syndromic hearing loss may play an important role in the preoperative assessment of prelingually-deafened children.

Original language	English
Pages (from-to)	151-157
Number of pages	7
Journal	International Journal of Pediatric Otorhinolaryngology
Volume	62
Issue number	2
DOIs	https://doi.org/10.1016/S0165-5876(01)00619-X
Publication status	Published - Feb 1 2002

Keywords

Cochlear implantation
Connexin 26
GJB2
Language development
Mutation screening
Speech perception

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Otorhinolaryngology

Access to Document

10.1016/S0165-5876(01)00619-X

Cite this

@article{c8b9a0495b9946df89fefbba6ff6cbe2,

title = "Better speech performance in cochlear implant patients with GJB2-related deafness",

abstract = "Objective: We applied mutation screening in seven cochlear implant users to identify those persons with GJB2-related deafness to determine whether etiology of deafness was predictive of speech performance after implantation. Methods: Direct sequence of GJB2 was conducted over seven cochlear implant users with prelingual hearing impairment and their speech, language and cognitive performance was examined. Results: The three persons with GJB2-related deafness had a mean vocabulary of 1243 words compared to a mean vocabulary of 195 words in the four children with GJB2-unrelated deafness, although the number of patients examined here was limited. The developmental quotient (DQ) of cognitive ability also was higher in those children with GJB2-related deafness. Conclusions: These preliminary results suggest that better speech performance after cochlear implantation may be observed in persons with GJB2-related deafness. In the future, detailed phenotypic studies and mutation screening for non-syndromic hearing loss may play an important role in the preoperative assessment of prelingually-deafened children.",

keywords = "Cochlear implantation, Connexin 26, GJB2, Language development, Mutation screening, Speech perception",

author = "Kunihiro Fukushima and Kennichi Sugata and Norio Kasai and Shouichirou Fukuda and Rie Nagayasu and Naomi Toida and Nobuhiko Kimura and Teruaki Takishita and Mehmet Gunduz and Kazunori Nishizaki",

note = "Funding Information: We would like to thank to all of the participants of this study program. This project is partly supported by the grant from Ministry of Health, Labor and Welfare and the grant for young scientists, Ministry of Education, Culture, Sports, Science and Technology. ",

year = "2002",

month = feb,

day = "1",

doi = "10.1016/S0165-5876(01)00619-X",

language = "English",

volume = "62",

pages = "151--157",

journal = "International Journal of Pediatric Otorhinolaryngology",

issn = "0165-5876",

publisher = "Elsevier Ireland Ltd",

number = "2",

}

TY - JOUR

T1 - Better speech performance in cochlear implant patients with GJB2-related deafness

AU - Fukushima, Kunihiro

AU - Sugata, Kennichi

AU - Kasai, Norio

AU - Fukuda, Shouichirou

AU - Nagayasu, Rie

AU - Toida, Naomi

AU - Kimura, Nobuhiko

AU - Takishita, Teruaki

AU - Gunduz, Mehmet

AU - Nishizaki, Kazunori

N1 - Funding Information: We would like to thank to all of the participants of this study program. This project is partly supported by the grant from Ministry of Health, Labor and Welfare and the grant for young scientists, Ministry of Education, Culture, Sports, Science and Technology.

PY - 2002/2/1

Y1 - 2002/2/1

N2 - Objective: We applied mutation screening in seven cochlear implant users to identify those persons with GJB2-related deafness to determine whether etiology of deafness was predictive of speech performance after implantation. Methods: Direct sequence of GJB2 was conducted over seven cochlear implant users with prelingual hearing impairment and their speech, language and cognitive performance was examined. Results: The three persons with GJB2-related deafness had a mean vocabulary of 1243 words compared to a mean vocabulary of 195 words in the four children with GJB2-unrelated deafness, although the number of patients examined here was limited. The developmental quotient (DQ) of cognitive ability also was higher in those children with GJB2-related deafness. Conclusions: These preliminary results suggest that better speech performance after cochlear implantation may be observed in persons with GJB2-related deafness. In the future, detailed phenotypic studies and mutation screening for non-syndromic hearing loss may play an important role in the preoperative assessment of prelingually-deafened children.

AB - Objective: We applied mutation screening in seven cochlear implant users to identify those persons with GJB2-related deafness to determine whether etiology of deafness was predictive of speech performance after implantation. Methods: Direct sequence of GJB2 was conducted over seven cochlear implant users with prelingual hearing impairment and their speech, language and cognitive performance was examined. Results: The three persons with GJB2-related deafness had a mean vocabulary of 1243 words compared to a mean vocabulary of 195 words in the four children with GJB2-unrelated deafness, although the number of patients examined here was limited. The developmental quotient (DQ) of cognitive ability also was higher in those children with GJB2-related deafness. Conclusions: These preliminary results suggest that better speech performance after cochlear implantation may be observed in persons with GJB2-related deafness. In the future, detailed phenotypic studies and mutation screening for non-syndromic hearing loss may play an important role in the preoperative assessment of prelingually-deafened children.

KW - Cochlear implantation

KW - Connexin 26

KW - GJB2

KW - Language development

KW - Mutation screening

KW - Speech perception

UR - http://www.scopus.com/inward/record.url?scp=0036467473&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0036467473&partnerID=8YFLogxK

U2 - 10.1016/S0165-5876(01)00619-X

DO - 10.1016/S0165-5876(01)00619-X

M3 - Article

C2 - 11788148

AN - SCOPUS:0036467473

VL - 62

SP - 151

EP - 157

JO - International Journal of Pediatric Otorhinolaryngology

JF - International Journal of Pediatric Otorhinolaryngology

SN - 0165-5876

IS - 2

ER -

Better speech performance in cochlear implant patients with GJB2-related deafness

Abstract

Keywords

ASJC Scopus subject areas

Access to Document

Other files and links

Fingerprint

Cite this