PubMed · 2314799
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S J Karmy. 1990. Audiometry: why and how.. https://pubmed.ncbi.nlm.nih.gov/2314799/
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OBJECTIVE: The purpose of this study was to examine the speech perception skills of prelingually deafened French children with preoperative residual hearing who received multichannel cochlear implants. DESIGN: The design of the study incorporated a within-subject, repeated measures design for assessing speech perception skills. SETTING: Montpellier, Toulouse and Lyon Pediatric Cochlear Implant Centers. SUBJECTS: Seven prelingually deafened children demonstrating marginal benefit from conventional amplification prior to implantation with a Nucleus multichannel cochlear implant, served as subjects for the speech perception assessment (a speech recognition score less than 30% defines marginal benefit from acoustic amplification on open set materials). The mean age at implantation was 7 years, 9 months. OUTCOME MEASURES: Speech perception skills were assessed using open set materials and the MUSS and MAIS questionnaires. RESULTS: Open-set speech recognition averaged 21.4% before implantation, and 83.6% after 1 year's cochlear implant experience. All children demonstrated an open-set score over 60% after 12 months of CI use. MAIS test scores averaged 18.1/40 before implantation and 35.1/40 after 9 months of CI use. MUSS test scores averaged 24.4/40 before implantation and 34.1/40 after 9 months of CI use. CONCLUSIONS: Cochlear implantation should be considered for prelingually hearing impaired children demonstrating marginal benefit from hearing aids, with a speech recognition score less than 30% on open set materials, in order to improve their speech discrimination skills.
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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.