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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

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

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.[1]


  1. Better speech performance in cochlear implant patients with GJB2-related deafness. Fukushima, K., Sugata, K., Kasai, N., Fukuda, S., Nagayasu, R., Toida, N., Kimura, N., Takishita, T., Gunduz, M., Nishizaki, K. Int. J. Pediatr. Otorhinolaryngol. (2002) [Pubmed]
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