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 | |
| Publication status | Published - Feb 1 2002 |
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Keywords
- Cochlear implantation
- Connexin 26
- GJB2
- Language development
- Mutation screening
- Speech perception
ASJC Scopus subject areas
- Otorhinolaryngology
- Pediatrics, Perinatology, and Child Health
- Pulmonary and Respiratory Medicine
- Surgery
Cite this
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
}
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
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 -