Cochlear implantation for children with GJB2-related deafness

OBJECTIVES/HYPOTHESIS: Mutations in GJB2 are a common cause of congenital sensorineural hearing loss. Many children with these mutations receive cochlear implants for auditory habilitation. The purpose of the study was to compare the speech perception performance of cochlear implant patients with GJB2-related deafness to patients without GJB2-related deafness. STUDY DESIGN: Retrospective case review. METHODS: Pediatric cochlear implant recipients who have been tested for GJB2 mutation underwent chart review. All patients received cochlear implantation at a tertiary referral center, followed by outpatient auditory habilitation. Charts were reviewed for cause and duration of deafness, age at time of cochlear implantation, intraoperative and postoperative complications, duration of use, and current age. Results of standard tests of speech perception administered as a part of the patients' auditory habilitation were reviewed. RESULTS: Twenty patients with GJB2 mutations were compared with 27 patients without GJB2 mutations. There was no statistical difference between patients with and without GJB2-related congenital sensorineural hearing loss with regard to open-set and closed-set speech recognition performance at 12, 24, and 36 months after cochlear implantation. Surgical complications were uncommon. CONCLUSION: Pediatric patients with congenital sensorineural hearing loss without other comorbid conditions (eg, developmental delay, inner ear malformations) perform well when they receive cochlear implantation and auditory habilitation. The presence or absence of GJB2 mutation does not appear to impact speech recognition performance at 12, 24, and 36 months after implantation.     

Auditory perception and speech discrimination after cochlear implantation in patients with connexin 26 (GJB2) gene-related deafness.

HYPOTHESIS: Auditory perception and speech discrimination among pediatric cochlear implantees may vary because of underlying deafness etiology, including connexin 26 (GJB2) gene-related deafness. BACKGROUND: Preliminary data suggest pathologic changes due to GJB2 mutations do not affect the spiral ganglion cells, which are stimulated by the cochlear implant. The survival of the spiral ganglion cells is believed to be an important determinant of outcome after surgery. Patients with GJB2-related deafness may therefore have enhanced prospects for good speech discrimination after implantation. METHODS: In an observational cohort study, GJB2 mutation analysis was performed using polymerase chain reaction amplification and direct sequencing on 31 prelingually deaf pediatric cochlear implantees, of which there were 30 with nonsyndromic deafness of unknown etiology, and one with keratitis-ichthyosis-deafness syndrome. Speech discrmination was assessed prospectively when they had reached postoperative year 3 using the IOWA Matrix Level B Sentences test and Glendonald Auditory Screening Procedure (GASP), with both patients and assessors blind to GJB2 status. RESULTS: Eleven patients had GJB2-related deafness and 20 patients had GJB2-unrelated deafness. IOWA Matrix scores were higher in patients with GJB2-related deafness but did not reach statistical significance. However, GASP scores were statistically significantly higher in patients with GJB2-related deafness (median word score, 92%; median sentence score, 80%), compared with those of patients with GJB2-unrelated deafness (median word score, 63%; median sentence score, 45%; word score, p = 0.037; sentence score, p = 0.045). Ordinal logistic regression analysis on IOWA Matrix and GASP sentence scores found better statistically significant scores in patients with GJB2-related deafness (p < 0.05) after adjustment for confounding variables. CONCLUSION: Pediatric cochlear implantees with GJB2-related deafness appear to have equal or better speech discrimination compared with a group of prelingually deaf children with deafness of unknown etiology.     

Connexin 26 (GJB2) gene-related deafness and speech intelligibility after cochlear implantation.

HYPOTHESIS: Speech intelligibility in children after cochlear implantation may depend on their deafness cause, including connexin 26 (GJB2) gene-related deafness. BACKGROUND: There is significant variability in the degree of intelligibility, or clarity, of children's speech after cochlear implantation. GJB2 gene-related deafness may be a factor, as preliminary data suggest that pathologic changes do not affect the spiral ganglion cells, which are the neural elements stimulated by the implant, thus favoring better results. METHODS: In an observational retrospective cohort study of pediatric cochlear implantees, 38 patients with nonsyndromic deafness of unknown cause and 1 with keratitisichthyosis-deafness syndrome underwent GJB2 mutation analysis using polymerase chain reaction amplification and direct sequencing. The primary outcome measure assessed was Speech Intelligibility Rating score from postoperative Year 1 (n = 39) to Year 5 (n = 17). Educational setting was considered as a secondary outcome measure. Statistical analysis was double-blinded, with patients and assessors of outcome unaware of GJB2 status. RESULTS: Fourteen patients had GJB2-related deafness and 25 had GJB2-unrelated deafness. Comparisons at Year 3 (n = 31) revealed intelligible speech achieved by 9 of 11 with GJB2-related deafness, compared with only 6 of 20 with GJB2-unrelated deafness (p = 0.017). Ordinal logistic regression analysis on Speech Intelligibility Rating scores found statistically significantly better scores in children with GJB2-related deafness (p < 0.05) both before and after adjustment for confounding variables. A larger proportion with GJB2-related deafness also attended mainstream school (p = 0.01). CONCLUSION: In pediatric cochlear implantees, GJB2-related deafness is a predictor of good speech intelligibility.     

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.     

Audiological performance after cochlear implantation: a 2-year follow-up in children with inner ear malformations

CONCLUSIONS: Open-set speech perception in children with an inner ear malformation is equal to that of other congenitally deaf children after an average of 2 years follow-up. OBJECTIVE: To analyze audiological performance after cochlear implantation in a sample of children with radiographically detectable malformations of the inner ear compared to performance in prelingually deafened children at large. MATERIALS AND METHODS: Nine children with osseous inner ear malformations were compared to 22 congenitally deaf children, all of whom underwent cochlear implantation. All subjects were tested on their electrical evoked compound action potential. Speech perception tests were performed using the monosyllabic trochee polysyllabic test without visual support and the open-set monosyllabic wordlist. RESULTS: In all, 20% of the congenitally deaf children in our center study have inner ear abnormalities. Inner ear malformations were limited to incomplete partition of the cochlea; none of the subjects had common cavity malformations. Electrical compound action potentials were successfully recorded in both groups intraoperatively. Speech perception tests on open-set speech yielded an average of 48.8% (SD 21.2%) in the group of children with inner ear malformations vs 54.5% (SD 21.1%) in congenitally deaf children. In four of nine cases with an inner ear malformation we encountered a minor CSF leak.     

The effect of GJB2 and SLC26A4 gene mutations on rehabilitative outcomes in pediatric cochlear implant patients

To analyze the treatment outcomes in pediatric cochlear implant patients with mutations in GJB2 or SLC26A4 and to determine these mutations’ impact on rehabilitative outcomes. The study included 41 children who received unilateral cochlear implantations. Fifteen of these children had GJB2-related deafness, 10 had SLC26A4-related deafness, and 16 had deafness of unknown etiology. Speech perception and language development evaluations, including the Meaningful Auditory Integration Scale (MAIS), categories of auditory performance (CAP), speech intelligibility rating (SIR) and babbling spurt, were conducted before and after the implantation. Better results for the GJB2 group (vs. the control group) were observed regarding MAIS, CAP and SIR at 24 months after implantation (P < 0.05). The performance of GJB2 group was better than SLC26A4 group, expressed by a significant difference in the variance of CAP and SIR at 24 months postoperatively (P < 0.05). A trend towards earlier babbling spurt onset could be observed for the GJB2 group, intergroup comparison did not reveal any significant difference among the three groups (P > 0.05). The SLC26A4 group performed better than the control group at 12 and 24 months postoperatively, although without a statistically significant difference (P > 0.05). The GJB2 gene mutations had a significantly positive impact on the outcome of cochlear implantation. Patients with SLC26A4-related deafness were shown to benefit from cochlear implantation.     

Successful cochlear implantation in prelingual profound deafness resulting from the common 233delC mutation of the GJB2 gene in the Japanese

OBJECTIVES: Recently, we identified three novel mutations of the GJB2 gene in Japanese families with autosomal-recessive non-syndromic deafness.1 Seven of 11 mutated chromosomes (63.6%) contained a 233delC allele, suggesting that the 233delC mutation is the most common mutation of the GJB2 gene in the Japanese population. After it was recognized that cochlear implantation (CI) is of benefit to children with prelingual deafness, we have had a number of prelingual pediatric CI patients. Because children carrying the homozygous 233delC mutation show bilateral prelingual profound deafness, they could be enrolled in the CI program at Osaka University Graduate School of Medicine. The purposes of this study were 1) to analyze the occurrence of the GJB2 mutations in our 15 prelingual pediatric CI patients in whom the cause of non-syndromic deafness was unknown, and 2) to evaluate the auditory function and postoperative speech perception with CI of those GJB2-related deaf subjects. STUDY DESIGN: Retrospective analysis. METHODS: Mutation analysis of the GJB2 gene by direct sequencing was performed with genomic DNA from 15 children born profoundly deaf as a result of unknown causes and implanted with CI. Intraoperative electrically evoked auditory brainstem response (EABR) and intra-/postoperative EAP were measured. The speech perception was evaluated with Infants and Toddlers Meaningful Auditory Integration Scale (IT-MAIS). RESULTS AND CONCLUSIONS: We identified 4 CI patients (26.7%) out of 15 children carrying the homozygous 233delC mutation. Intra- and postoperative evaluation of the auditory system revealed almost intact cochlear and retrocochlear auditory function in these 4 patients. Postoperative auditory testing indicates that their speech perception had become significantly higher in comparison with that of other prelingual CI patients. These results suggest that prelingual deaf children carrying the homozygous 233delC mutation of the GJB2 gene can benefit from CI.     

Partial deafness cochlear implantation in children

OBJECTIVE: Partial deafness cochlear implantation and electric-acoustic stimulation have proven to be a useful method of treating adults with a ski-slope type hearing loss. Good hearing preservation and speech perception outcomes have been reported. This study aims to assess partial deafness cochlear implantation in children. METHOD: Nine children, ranging in age from 4.2 to 12 years, received a cochlear implant following the round window surgical technique for partial deafness cochlear implantation. Hearing preservation was assessed by pure-tone audiometry and speech perception outcomes were measured using monosyllable word tests in quiet and noise. Data are available for most children up to a period of 1 year. RESULTS: Hearing could be preserved partially in all cases, however, one child does not have sufficient preservation to make use of electric-acoustic stimulation. The eight children with sufficiently preserved hearing either use the natural low frequency hearing in combination with a cochlear implant to hear or use the DUET combined hearing system. Speech perception tests showed improvement in quiet and noise over time. CONCLUSION: Results suggest that partial deafness cochlear implantation is a viable treatment method in children. However, surgery should only be conducted by an experienced surgeon and parents need to be carefully counselled about the risks and benefits of partial deafness cochlear implantation.     

Cochlear implants in young children: the relationship between speech perception and speech intelligibility.

OBJECTIVE: To determine the relationship between measures of speech perception and speech production after cochlear implantation of young children with profound congenital and prelingual deafness. DESIGN: A prospective study was undertaken on a consecutive group of children with profound deafness. There were 126 children at the preimplantation interval and 71, 50, 26, and 20 children, respectively, at the 2, 3, 4, and 5 yr follow-up after implantation. Speech perception and speech intelligibility were assessed using hierarchical rating scales. Spearman rank correlation coefficients were used to determine the statistical correlations. All patients were either congenitally deaf or deafened before the age of 3 yr and were implanted before age 7 yr. The patients all received the Nucleus multichannel cochlear implant system with the most appropriate speech encoding strategy. RESULTS: Speech intelligibility at 5 yr was strongly correlated with speech perception at the 2, 3, 4, and 5 yr intervals after implantation (Spearman coefficients 0.77, 0.81, 0.58, 0.58; p < or = 0.01). Speech intelligibility at the 2, 3, and 4 yr intervals also correlated in a similar manner with earlier speech perception abilities (p < or = 0.01). CONCLUSIONS: The results suggest that speech intelligibility between 2 and 5 yr after implantation in young children with congenital and prelingual profound deafness can be predicted by measures of earlier speech perception.     

Audiological performance after cochlear implantation: a 2-year follow-up in children with inner ear malformations

Conclusions: Open-set speech perception in children with an inner ear malformation is equal to that of other congenitally deaf children after an average of 2 years follow-up. Objective: To analyze audiological performance after cochlear implantation in a sample of children with radiographically detectable malformations of the inner ear compared to performance in prelingually deafened children at large. Materials and methods: Nine children with osseous inner ear malformations were compared to 22 congenitally deaf children, all of whom underwent cochlear implantation. All subjects were tested on their electrical evoked compound action potential. Speech perception tests were performed using the monosyllabic trochee polysyllabic test without visual support and the open-set monosyllabic wordlist. Results: In all, 20% of the congenitally deaf children in our center study have inner ear abnormalities. Inner ear malformations were limited to incomplete partition of the cochlea; none of the subjects had common cavity malformations. Electrical compound action potentials were successfully recorded in both groups intraoperatively. Speech perception tests on open-set speech yielded an average of 48.8% (SD 21.2%) in the group of children with inner ear malformations vs 54.5% (SD 21.1%) in congenitally deaf children. In four of nine cases with an inner ear malformation we encountered a minor CSF leak.     

Speech perception and speech intelligibility in children after cochlear implantation

OBJECTIVE: This study aimed to evaluate the long-term speech perception and speech intelligibility of congenitally and prelingually deaf children after cochlear implantation. It was a longitudinal study following 63 congenitally or prelingually deaf children up to 5 years after implantation. They each received a nucleus multichannel cochlear implant before they were 10 years old. METHODS: Perception is evaluated using the Test for the Evaluation of Voice Perception and Production (TEPP) and concerns closed- and open-set word and sentence perception without lip-reading. The intelligibility is classified according to the Speech Intelligibility Rating (SIR). The evaluations have been made every 3 months for 1 year, then at 18 months, 2 years, 3 years and 5 years after the cochlear implantation. RESULTS: After 5 years of implantation, the median percentage of closed-words speech perception (CSW) is 95.5%-93.67% for closed-sentence speech perception (CSS) and 76.3% for open-sentence speech perception (OSS); the median Speech Intelligibility Rating is 3.83. CONCLUSIONS: Congenitally and prelingually deaf children who receive cochlear implant before the age of 10 years develop speech perception and speech intelligibility abilities. The closed-set perception progresses quickly and seems to reaching a plateau at 5 years post implantation. The improvement of open-sentence perception is not significant until the first year post implantation. The speech intelligibility improves regularly the five first year post implantation.     

Effect of age at cochlear implantation on open-set word recognition in Mandarin speaking deaf children

OBJECTIVE: The purpose of this study was to determine whether age at cochlear implantation influences open-set speech perception in children after long-term use of the implant device. METHOD: Twenty-eight congenitally deafened children, receiving implants of Nucleus CI24M devices, were divided into two groups: (1) CI < 3: those who received implants before 3 years of age and (2) CI > 3: those who received implants after 3 years of age. We compared open-set speech perception in CI < 3 and CI > 3 after 4-5 years of device use. Speech perception tests were conducted using the Mandarin Lexical Neighborhood Test (M-LNT). Unpaired t-test was applied for statistical analysis, and p < 0.05 was considered significant. RESULTS: In CI < 3, the average of percent correct was 80.0 +/- 8.8 and 70.5 +/- 9.2% on, respectively, the easy and hard versions of the M-LNT. By contrast, in CI > 3, the average percent correct was 62.5 +/- 19.9 and 59.1 +/- 15.2%, respectively. Regardless of the M-LNT version used, CI < 3 performed significantly better than CI > 3 (easy, p = 0.005 versus hard, p = 0.022). CONCLUSION: The present investigation demonstrated that age at implantation influences open-set speech perception of cochlear implanted children 4-5 years after device connection. Implantation before 3 years of age promotes the development of open-set speech perception abilities in congenitally deafened children.     

The effect of GJB2 allele variants on performance after cochlear implantation

OBJECTIVES/HYPOTHESIS: It has been hypothesized that etiology of hearing loss may serve as an independent variable in performance after cochlear implantation. To test this hypothesis, the authors identified pediatric cochlear implant recipients with gap junction protein beta2 (GJB2)-related deafness. The study examines performance outcomes associated with GJB2 deafness-causing allele variants. STUDY DESIGN: Pediatric cochlear implant patients were screened for GJB2 allele variants; statistical comparisons were made with prospectively obtained performance measures. METHODS: From 181 children who participated in a nationwide cochlear implant research program, 122 children were identified with congenital nonsyndromic sensorineural hearing loss and invited to participate. Screening for GJB2 allele variants was completed for 55 children. The children were homogeneous with respect to age (8 or 9 y) and age at implant (before age 5 y). All patients have previously undergone a prospective regimented battery of performance measures. RESULTS: Performance measures were compared between 22 children with and 33 children without mutations to determine whether GJB2 status was a significant predictor of cochlear implant outcomes. Reading and cognitive outcomes were significantly dependent on connexin status. The group of children who tested positive for GJB2-related deafness scored significantly higher on a nonverbal cognitive measure, Block Design, and on a measure of reading comprehension. CONCLUSION: The isolated insult to the cochlea created by GJB2 allele variants allows for preservation of central cognitive function. Better reading performance is seen in children with GJB2-related deafness.     

Cochlear implants for children with significant residual hearing

BACKGROUND: Previous research suggests that children with pure-tone averages of greater than 90 dB hearing level and/or open-set sentence perception of less than 30% may derive significant benefit from cochlear implantation. OBJECTIVE: To evaluate postoperative speech perception benefit and bilateral-bimodal benefit for 16 children whose preimplant speech perception scores exceeded conservative candidacy guidelines. STUDY DESIGN: Preimplant and postimplant repeated-measure design. METHODS: Sixteen child subjects who obtained 30% or greater on preimplant open-set sentence material, presented live voice audition alone, were selected for this study. Preimplant pure-tone averages ranged from 73 to 110 dB in the better aided ear. Preimplant and postimplant open-set word and sentence testing was completed in quiet and with competing background noise for separate ear and binaural conditions. RESULTS: Fourteen of 16 subjects had improved speech perception scores across all test materials after implantation. Group means were significantly higher for all test materials. Results in the bimodal-bilateral condition were significantly higher than implant alone for open-set word tests (scored for phonemes) and open-set sentences in quiet. CONCLUSION: The results of this study suggest that, with appropriate counseling and management, some children with significant residual hearing benefit from cochlear implantation, in particular improved speech understanding due to bimodal-bilateral hearing.     

Usher type I syndrome in children: genotype/phenotype correlation and cochlear implant benefits

OBJECTIVE: To assess the benefit of cochlear implant in children presenting an Usher type 1 syndrome (speech understanding, speech production intelligibility, academic performance) and to search any correlation between the phenotype and the genotype in this population. MATERIALS AND METHODS: Retrospective case series analysis about 13 implanted Usher type I children. Cochlear implantation was performed from 1995 to 2005. Our population was divided in three groups: group 1 (implantation between 1 and 3 years of age); group 2 (implantation between 4 and 7 years of age) and group 3 (implantation between 14 and 17 years of age). Postoperative speech perception, speech production intelligibility and education settings were evaluated. RESULTS: Molecular genetic analysis was performed in 11 patients and pathogenic mutations were identified in all cases: (mutation in myosin 7A gene in 5 cases; mutation in cadherin 23 gene in 6 cases). Four new mutations 2 in the MYO7A gene and 2 in the CDH23 gene never reported before were found. Walking delay and hearing level were not statistically correlated with the genotype abnormalities found. The speech discrimination skills, the speech production intelligibility and the academic performance were better in the group 1 children than the group 2 children after cochlear implantation. All the children of group 1 but one were in mainstreaming education. Specific language impairment was identified in two children of group 1. The group 3 children could not achieve open-set perceptive tasks after implantation--only closed-set word test can be done and their speech production remained unintelligible after cochlear implantation. CONCLUSION: Molecular analysis of Usher type I syndrome can ascertain the diagnosis in spite of the genetic heterogeneity. In this study, clinical symptoms weren't correlated with genotypic mutations. Speech discrimination skills, speech production quality, and academic performance were correlated with the age at implant.     

Cochlear implants in children, adolescents, and prelinguistically deafened adults: speech perception.

A group of 10 children, adolescents, and prelinguistically deafened adults were implanted with the 22-electrode cochlear implant (Cochlear Pty Ltd) at the University of Melbourne Cochlear Implant Clinic and have used the prosthesis for periods from 12 to 65 months. Postoperative performance on the majority of closed-set speech perception tests was significantly greater than chance, and significantly better than preoperative performance for all of the patients. Five of the children have achieved substantial scores on open-set speech tests using hearing without lipreading. Phoneme scores in monosyllabic words ranged from 30% to 72%; word scores in sentences ranged from 26% to 74%. Four of these 5 children were implanted during preadolescence (aged 5:5 to 10:2 years) and the fifth, who had a progressive loss, was implanted during adolescence (aged 14:8 years). The duration of profound deafness before implantation varied from 2 to 8 years. Improvements were also noted over postoperative data collection times for the younger children. The remaining 5 patients who did not demonstrate open-set recognition were implanted after a longer duration of profound deafness (aged 13:11 to 20:1 years). The results are discussed with reference to variables that may affect implant performance, such as age at onset of loss, duration of profound loss, age at implantation, and duration of implantation. They are compared with results for similar groups of children using hearing aids and cochlear implants.     

Speech perception performance in prelingually deaf children with cochlear implants

The goals of this study were to report on mean values of speech perception performance in prelingually deaf children implanted with a Combi 40/40 + cochlear implant. A total of 31 patients were included in the study. The time span ranged over 3 years, during which time the Evaluation of Auditory Responses to Speech (EARS) test battery was used with the children. The EARS battery contains four measures of closed-set speech perception and three open-set tests. The mean test results exhibited steady improvement on all parts of the EARS test battery, even up to 3 years post-implantation. The preoperative scores for the Listening Progress Profile (LiP) were 4%, rising to 93% at 36 months post-implantation. Results for the open-set testing measures were most encouraging, with some children reaching fairly high levels of speech perception, receiving scores as high as 100%, by the 36-month evaluation. The congenitally and prelingually deaf children in our study showed continuous improvement in both closed and open set speech perception following cochlear implantation, although variability in individual performance among the children was noted.     

Speech Perception Performance in Prelingually Deaf Children with Cochlear Implants

The goals of this study were to report on mean values of speech perception performance in prelingually deaf children implanted with a Combi 40/40+ cochlear implant. A total of 31 patients were included in the study. The time span ranged over 3 years, during which time the Evaluation of Auditory Responses to Speech (EARS) test battery was used with the children. The EARS battery contains four measures of closed-set speech perception and three open-set tests. The mean test results exhibited steady improvement on all parts of the EARS test battery, even up to 3 years post-implantation. The preoperative scores for the Listening Progress Profile (LiP) were 4%, rising to 93% at 36 months post-implantation. Results for the open-set testing measures were most encouraging, with some children reaching fairly high levels of speech perception, receiving scores as high as 100% by the 36-month evaluation. The congenitally and prelingually deaf children in our study showed continuous improvement in both closed and open set speech perception following cochlear implantation, although variability in individual performance among the children was noted.     

Cochlear implantation in prelingually deafened children with residual hearing

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.     

Speech perception of children using Nucleus, Clarion or Med-El cochlear implants

OBJECTIVE: The purpose of this study was to present speech perception achievements of implanted children using commercially available cochlear implant devices: Nucleus, Clarion or Med-El. STUDY DESIGN: A retrospective analysis. METHODS: Speech perception data of 96 hearing-impaired children: 27 with Clarion, 49 with Nucleus and 20 with Med-El were collected. Speech tests included the Hebrew Infant Toddlers Meaningful Auditory Integration Scale (HIT-MAIS) for the infants, the Hebrew Early Speech Perception (HESP) closed-set word-identification test and Hebrew Arthur Boothroyd (HAB) open-set one-syllable word recognition test were used for the older children. RESULTS: I HIT-MAIS: (1) Infants showed similar rate of progress, regardless of device. (2) Children implanted under two years of age reached performance within normal development on this test. II HAB: (1) Most children achieved open set results with mean HAB between 40 and 50%, within 1-1.5 years post implantation. (2) Linear regression analyses revealed no statistical differences between the Clarion the Nucleus and the Med-El devices on the mean final measurement of this test. (3) Age of implantation and mode of communication were significant covariate variables: (a) the younger the child is implanted the better the results and (b) oral communication prior to implantation results in better performance than sign language. CONCLUSIONS: There are no apparent differences in speech perception performance between implant devices when considering background variables. The data have important implications on the rehabilitation process of hearing impaired children with cochlear implants in relation to device selection, age at implantation and mode of communication prior to implantation.