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

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

68 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

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Better speech performance in cochlear implant patients with GJB2-related deafness. / Fukushima, Kunihiro; Sugata, Kennichi; Kasai, Norio; Fukuda, Shouichirou; Nagayasu, Rie; Toida, Naomi; Kimura, Nobuhiko; Takishita, Teruaki; Gunduz, Mehmet; Nishizaki, Kazunori.

In: International Journal of Pediatric Otorhinolaryngology, Vol. 62, No. 2, 01.02.2002, p. 151-157.

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

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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.",

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AU - Toida, Naomi

AU - Kimura, Nobuhiko

AU - Takishita, Teruaki

AU - Gunduz, Mehmet

AU - Nishizaki, Kazunori

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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.

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