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To assess a group of children who consecutively received implants more than 10 years after implantation with regard to speech perception, speech intelligibility, receptive language level, and academic/occupational status.A prospective longitudinal study.Pediatric referral center for cochlear implantation.Eighty-two prelingually deafened children received the Nucleus multichannel cochlear implant.Cochlear implantation with Cochlear Nucleus CI22 implant.The main outcome measures were open-set Phonetically Balanced Kindergarten word test, discrimination of sentences in noise, connective discourse tracking (CDT) using voice and telephone, speech intelligibility rating (SIR), vocabulary knowledge measured using the Peabody Picture Vocabulary Test (Revised), academic performance on French language, foreign language, and mathematics, and academic/occupational status.After 10 years of implant experience, 79 children (96%) reported that they always wear the device; 79% (65 of 82 children) could use the telephone. The mean scores were 72% for the Phonetically Balanced Kindergarten word test, 44% for word recognition in noise, 55.3 words per minute for the CDT, and 33 words per minute for the CDT via telephone. Thirty-three children (40%) developed speech intelligible to the average listener (SIR 5), and 22 (27%) developed speech intelligible to a listener with little experience of deaf person's speech (SIR 4). The measures of vocabulary showed that most (76%) of children who received implants scored below the median value of their normally hearing peers. The age at implantation was the most important factor that may influence the postimplant outcomes. Regarding educational/vocational status, 6 subjects attend universities, 3 already have a professional activity, 14 are currently at high school level, 32 are at junior high school level, 6 additional children are enrolled in a special unit for children with disability, and 3 children are still attending elementary schools. Seventeen are in further noncompulsory education studying a range of subjects at vocational level.This long-term report shows that many profoundly hearing-impaired children using cochlear implants can develop functional levels of speech perception and production, attain age-appropriate oral language, develop competency level in a language other than their primary language, and achieve satisfactory academic performance.

Objective: To update a 15-year-old study of 800 postlinguistically deaf adult patients showing how duration of severe to profound hearing loss, age at cochlear implantation (CI), age at onset of severe to profound hearing loss, etiology and CI experience affected CI outcome. Study Design: Retrospective multicenter study. Methods: Data from 2251 adult patients implanted since 2003 in 15 international centers were collected and speech scores in quiet were converted to percentile ranks to remove differences between centers. Results: The negative effect of long duration of severe to profound hearing loss was less important in the new data than in 1996; the effects of age at CI and age at onset of severe to profound hearing loss were delayed until older ages; etiology had a smaller effect, and the effect of CI experience was greater with a steeper learning curve. Patients with longer durations of severe to profound hearing loss were less likely to improve with CI experience than patients with shorter duration of severe to profound hearing loss. Conclusions: The factors that were relevant in 1996 were still relevant in 2011, although their relative importance had changed. Relaxed patient selection criteria, improved clinical management of hearing loss, modifications of surgical practice, and improved devices may explain the differences.

Objective: To test the influence of multiple factors on cochlear implant (CI) speech performance in quiet and in noise for postlinguistically deaf adults, and to design a model of predicted auditory performance with a CI as a function of the significant factors.Study Design: Retrospective multi-centre study.Methods: Data from 2251 patients implanted since 2003 in 15 international centres were collected. Speech scores in quiet and in noise were converted into percentile ranks to remove differences between centres. The influence of 15 pre-, per- and postoperative factors, such as the duration of moderate hearing loss (mHL), the surgical approach (cochleostomy or round window approach), the angle of insertion, the percentage of active electrodes, and the brand of device were tested. The usual factors, duration of profound HL (pHL), age, etiology, duration of CI experience, that are already known to have an influence, were included in the statistical analyses.Results: The significant factors were: the pure tone average threshold of the better ear, the brand of device, the percentage of active electrodes, the use of hearing aids (HAs) during the period of pHL, and the duration of mHL.Conclusions: A new model was designed showing a decrease of performance that started during the period of mHL, and became faster during the period of pHL. The use of bilateral HAs slowed down the related central reorganization that is the likely cause of the decreased performance. Citation: Lazard DS, Vincent C, Venail F, Van de Heyning P, Truy E, et al. (2012) Pre-, Per- and Postoperative Factors Affecting Performance of Postlinguistically Deaf Adults Using Cochlear Implants: A New Conceptual Model over Time. PLoS ONE 7(11):e48739. doi:10.1371/journal.pone.0048739

Objectives: We compare the evolution of electrode impedance values (IVs) following either conventional cochlear implantation or implantation by the soft surgery (SS) technique. Methods: We performed a retrospective chart review of 20 consecutive adult patients who underwent implantation with the Nucleus CA 24 device between 2004 and 2007. Five patients with preoperative residual hearing at the frequencies 256, 512, and 1,024 Hz underwent implantation by an SS cochlear implantation technique (SS group), and the 15 other patients underwent a conventional implantation technique (conventional cochleostomy [CC] group). The active electrodes were classified as distal (17 to 22), middle (10 to 16), or proximal (3 to 9) according to their position in relation to the tip of the electrode array. Their IVs were collected at 1, 3, 12, 24, and 36 months after implantation. Changes in auditory thresholds at 3 and 24 months were reported for patients in the SS group. Results: The postoperative IVs of both the CC and SS groups decreased significantly between 1 and 3 months after implantation (p < 0.05) and then remained stable. The IVs after 12 months were significantly lower (p < 0.05) in the SS group than in the CC group. Conclusions: Patients who underwent the SS technique displayed lower long-term electrode IVs than did their counterparts in the CC group. If electrode IVs are indeed an indirect representation of cochlear fibrosis, the use of the SS technique in lieu of the CC technique could reduce fibrotic development.

Objective The purpose of this study was to examine prognostic factors of cochlear implantation and to evaluate the impact of early implantation on linguistic development in deaf children. Study design Retrospective study. Setting Tertiary referral center. Patients Seventy-four prelingually deafened children implanted before the age of 5 years. Intervention Annual follow-up after cochlear implantation. Main outcome measures Speech perception, intelligibility, and expressive and receptive language scores from age 3 to 8 years were globally compared between 4 subgroups of children. Significant differences were further explored by intergroup comparisons. Stepwise logistic linear regression was performed using the following variables: age at implantation, duration of cochlear implant (CI) use, preoperative hearing levels, age of hearing aid (HA) fitting, and age at time of the evaluation. Preoperative data were not available. Results Between group comparisons displayed significant differences according to age at implantation. Multivariate analysis demonstrated the positive impact of early implantation on receptive language. Moreover, duration of CI use and preoperative hearing levels were statistically correlated with performance on speech perception, intelligibility, and expressive and receptive language. Age of HA fitting was associated with speech intelligibility. Conclusion Age at implantation, duration of CI, preoperative hearing levels, and age of HA fitting may each be useful in predicting linguistic success after cochlear implantation. Other factors such as preoperative linguistic development may also influence postoperative outcomes, but the lack of tests suitable for use with very young children makes such a hypothesis difficult to confirm.

International audience; OBJECTIVES: To assess device failures as well as early postoperative, late postoperative, and medical complications occurring after cochlear implantation and to discuss their causes and treatments. Study DESIGN: Retrospective study of 500 consecutive cochlear implantations. SETTING: Tertiary referral center. PATIENTS: All patients receiving cochlear implants at our institution between 1989 and 2006. MAIN OUTCOME MEASURES: All complications and treatments were systematically reviewed with a maximum follow-up of 18 years. The number of reimplantations was calculated according to follow-up duration to determine the cochlear implant survival rate. Specific risks of reimplantation were calculated for groups with differing durations of implantation. RESULTS: The overall rate of complications was 16.0% (79 of 500), with minor complications accounting for 5.6%; major complications, 3.2%; and reimplantations, 7.2%. Reasons for revision surgery were device failure, infection, trauma, and "soft device failure" (failure despite normal results from integrity testing). Revision surgery was performed in 51 of the 500 cases (10.2%), and other complications were managed medically (28 of 500; 5.6%). The rate of hard and soft device failures was 6% (30 of 500). Seventy-two percent of reimplantations occurred within 5 years. The risk of severe infection (eventually requiring explantation) was 1.4% (7 of 500). There was 1 case of transient facial palsy following surgery (0.2%), and the incidence of postsurgical meningitis was 0. CONCLUSIONS: Cochlear implantation is a safe technique with a relatively low complication rate; however, certain complications may require specific attention to prevent or correct them.

Background Mutations in the GJB2 gene have been established as a major cause of inherited non syndromic deafness in different populations. A high number of sequence variations have been described in the GJB2 gene and the associated pathogenic effects are not always clearly established. The prevalence of a number of mutations is known to be population specific, and therefore population specific testing should be a prerequisite step when molecular diagnosis is offered. Moreover, population studies are needed to determine the contribution of GJB2 variants to deafness. We present our findings from the molecular diagnostic screening of the GJB2 and GJB6 genes over a three year period, together with a population-based study of GJB2 variants. Methods and results Molecular studies were performed using denaturing High Performance Liquid Chromatograghy (DHPLC) and sequencing of the GJB2 gene. Over the last 3 years we have studied 159 families presenting sensorineural hearing loss, including 84 with non syndromic, stable, bilateral deafness. Thirty families were genotyped with causative mutations. In parallel, we have performed a molecular epidemiology study on more than 3000 dried blood spots and established the frequency of the GJB2 variants in our population. Finally, we have compared the prevalence of the variants in the hearing impaired population with the general population. Conclusion Although a high heterogeneity of sequence variation was observed in patients and controls, the 35delG mutation remains the most common pathogenic mutation in our population. Genetic counseling is dependent on the knowledge of the pathogenicity of the mutations and remains difficult in a number of cases. By comparing the sequence variations observed in hearing impaired patients with those sequence variants observed in general population, from the same ethnic background, we show that the M34T, V37I and R127H variants can not be responsible for profound or severe deafness.

To investigate the educational and employment achievements of prelingually deaf children who undergo cochlear implantation.Prospective study. Data were examined within groups defined by current age and additional disabilities. Multivariate analyses were used to identify variables influencing grade failure and communication mode.Tertiary referral center.One hundred prelingually deaf children who received cochlear implants before 6 years of age and who also had at least 4 years of follow-up.Interview of parents using a standardized questionnaire.The type of schooling, form of communication, employment status, age at which the child learned to read and write, number of grade failures, and educational support required.Mainstream schooling, regardless of educational level, was the standard experience for children without additional disabilities (16 of 24 [67%] in the 12- to 15-year age group to 20 of 24 [83%] in the 8- to 11-year age group). Four of 8 participants older than 18 years (50%) had a university-level education; the remainder had vocational training. Delayed reading and writing skills were experienced by 19 of 74 participants (26%) and, depending on the age group, 42% to 61% of participants (10 of 24 in the 8- to 11-year age group to 11 of 18 in the 16- to 18-year age group) had failed a grade. The number of grade failures was associated with communication mode at the time of the survey. Age at implantation, preoperative communication mode, and educational support influenced the final communication mode. In children with additional disabilities, the level of academic achievement and employment status varied.Despite significant differences in the grade failure rate between the children with cochlear implants and the general population, the participants in the present study ultimately achieved educational and employment levels similar to those of their normal-hearing peers. To minimize these delays and improve academic success in mainstream education, early oral education and early cochlear implantation are important.