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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Authors | Kunihiro Fukushima, Kennichi Sugata, Norio Kasai, Shouichirou Fukuda, Rie Nagayasu, Naomi Toida, Nobuhiko Kimura, Teruaki Takishita, Mehmet Gunduz, Kazunori Nishizaki |
Journal | International journal of pediatric otorhinolaryngology
(Int J Pediatr Otorhinolaryngol)
Vol. 62
Issue 2
Pg. 151-7
(Feb 01 2002)
ISSN: 0165-5876 [Print] Ireland |
PMID | 11788148
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Connexins
- GJB2 protein, human
- Connexin 26
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Topics |
- Audiometry
- Child
- Child Development
- Child, Preschool
- Cochlear Implantation
- Cognition
- Connexin 26
- Connexins
(genetics)
- DNA Mutational Analysis
- Deafness
(genetics, therapy)
- Humans
- Infant
- Language Development
- Male
- Mutation
(genetics)
- Predictive Value of Tests
- Speech Intelligibility
- Vocabulary
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