Margreet Langereis

Expressive Vocabulary, Morphology, Syntax and Narrative Skills in Profoundly Deaf Children after Early Cochlear Implantation.

Practical experience and research reveal generic spoken language benefits after cochlear implantation. However, systematic research on specific language domains and error analyses are required to probe sub-skills. Moreover, the effect of predictive factors on distinct language domains is unknown. In this study, outcomes of 70 school-aged children with cochlear implants were compared with hearing peers. Approximately half of the children with cochlear implants achieved age-adequate language levels. Results did not reveal systematic strong or weak language domains. Error analyses showed difficulties with morphological and syntactic rules and inefficient narrative skills. Children without additional disabilities who received early intervention were raised with one spoken language, and used a second cochlear implant or contralateral hearing aid were more likely to present good language skills.

Classroom performance of children with cochlear implants in mainstream education.

Objectives: We compared classroom performance of children with a cochlear implant (CI) with that of their normal-hearing peers in mainstream education. Methods: Thirty-two CI children in mainstream education, congenitally or prelingually deaf, participated in this study, as did 37 hearing classmates. Their teachers filled out 2 questionnaires: the Assessment of Mainstream Performance (AMP) and the Screening Instrument for Targeting Educational Risk (SIFTER). A high Fletcher index and open-set speech perception scores were obtained. Results: The children with CIs scored above average on the AMP and sufficiently well in all but one area (communication) of the SIFTER questionnaire. Class rankings did not differ significantly between the CI students and their normal-hearing peers. Overall, the normal-hearing group outperformed the CI group. The classroom performance of CI children correlated negatively with duration of deafness and age at implantation. All longitudinal audiological data of the CI children showed improvement in open-set speech recognition. Conclusions: Although the results are encouraging, the CI group scored significantly less well than their normal-hearing peers on most questionnaire domains of both the AMP and the SIFTER. The most important variables for the outcome in this study were age at implantation and duration of deafness.

Narrative spoken language skills in severely hearing impaired school-aged children with cochlear implants

Cochlear implants have a significant positive effect on spoken language development in severely hearing impaired children. Previous work in this population has focused mostly on the emergence of early-developing language skills, such as vocabulary. The current study aims at comparing narratives, which are more complex and later-developing spoken language skills, of a contemporary group of profoundly deaf school-aged children using cochlear implants (n=66, median age=8 years 3 months) with matched normal hearing peers. Results show that children with cochlear implants demonstrate good results on quantity and coherence of the utterances, but problematic outcomes on quality, content and efficiency of retold stories. However, for a subgroup (n=20, median age=8 years 1 month) of deaf children without additional disabilities who receive cochlear implantation before the age of 2 years, use two implants, and are raised with one spoken language, age-adequate spoken narrative skills at school-age are feasible. This is the first study to set the goals regarding spoken narrative skills for deaf children using cochlear implants.

Classroom performance and language development of CI students placed in mainstream elementary school.

Objective: Investigation of the relation between classroom performance and language development of cochlear implant (CI) students in mainstream education. Structural analyses of assessment of mainstream performance (AMP) and Screening Instrument For Targeting Educational Risk (SIFTER) instruments. Study Design: Cross-sectional instrument and language development analyses. Setting: Tertiary university medical center. Patients: Twenty-six CI children in elementary school with congenital or prelingual deafness were included. At the time of this study, mean period of multichannel CI use was 5.3 years, and childrens ages ranged from 6.5 to 12.8 years. Main Outcome Measure: Assessment of mainstream performance and SIFTER instruments measured classroom performance and language development were measured by means of Reynell and Schlichting tests. Results: Assessment of mainstream performance and SIFTER domains showed good reliability (Cronbach &agr; >0.6), but factor analyses only showed the expected instrument structure in the AMP. In both questionnaires and within all domains, individual variability is detected. Spearmans correlation analyses showed the probable explanation of individual questionnaire variability by language test results (p value mostly <0.01). The AMP and SIFTER instruments showed a predictive capacity for language development, based upon general linear model univariate and linear regression analyses. Conclusion: Individual classroom performance, measured by AMP and SIFTER questionnaires, of CI children in mainstream education varies. Correlation analyses showed strong significant relation between questionnaire results (classroom performance) and both expressive and receptive language test results (Schlichting and Reynell tests). Structural questionnaire analyses of the AMP and SIFTER demonstrated good reliability. The predictive value of the AMP can monitor the actual linguistic functioning of the child.

Cochlear implantation in late-implanted prelingually deafened adults: changes in quality of life.

Background With expanding inclusion criteria for cochlear implantation, the number of prelingually deafened persons who are implanted as adults increases. Compared with postlingually deafened adults, this group shows limited improvement in speech recognition. In this study, the changes in health-related quality of life in late-implanted prelingually deafened adults are evaluated and related to speech recognition. Methods Quality of life was measured before implantation and 1 year after implantation in a group of 28 prelingually deafened adults, who had residual hearing and who used primarily oral communication. Patients completed 3 questionnaires (Nijmegen Cochlear Implant Questionnaire, Glasgow Benefit Inventory, and Health Utility Index 3). Postoperative scores were compared with preoperative scores. Additionally, phoneme recognition scores were obtained preimplantation and 1 year postimplantation. Results Quality of life improved after implantation: scores on the Nijmegen Cochlear Implant Questionnaire improved significantly in all subdomains (basic speech perception, advanced speech perception, speech production, self-esteem, activity, and social interaction), the total Glasgow Benefit Inventory score improved significantly, and the Health Utility Index 3 showed a significant improvement in the utility score and in the subdomains “hearing” and “emotion.” Additionally, a significant improvement in speech recognition scores was found. No significant correlations were found between gain in quality of life and speech perception scores. Conclusion The results suggest that quality of life and speech recognition in prelingually deafened adults significantly improved as a result of cochlear implantation. Lack of correlation between quality of life and speech recognition suggests that in evaluating performance after implantation in prelingually deafened adults, measures of both speech recognition and quality of life should be used.

Lexical access in children with hearing loss or specific language impairment, using the cross-modal picture–word interference paradigm

In this study we compared lexical access to spoken words in 25 deaf children with cochlear implants (CIs), 13 hard-of-hearing (HoH) children and 20 children with specific language impairment (SLI). Twenty-one age-matched typically developing children served as controls. The children with CIs and the HoH children in the present study had good speech perception abilities. We used a cross-modal picture-word interference paradigm to examine lexical access. Results showed that children with SLI revealed overall slower reaction times and produced more errors than the children with CIs, the HoH children, and the control children. Reaction times of children with CIs and the HoH children did not differ from those of the control children. Thus, problems with spoken language processing, as is the case in children with SLI, seem to affect lexical access more than limitations in auditory perception, as is the fundamental problem in children with hearing loss. We recommend that improvement of lexical access in children with SLI deserves specific attention in therapy and education.

School performance and wellbeing of children with CI in different communicative-educational environments

OBJECTIVES This study aimed to evaluate the long term effects of CI on auditory, language, educational and social-emotional development of deaf children in different educational-communicative settings. METHODS The outcomes of 58 children with profound hearing loss and normal non-verbal cognition, after 60 months of CI use have been analyzed. At testing the children were enrolled in three different educational settings; in mainstream education, where spoken language is used or in hard-of-hearing education where sign supported spoken language is used and in bilingual deaf education, with Sign Language of the Netherlands and Sign Supported Dutch. Children were assessed on auditory speech perception, receptive language, educational attainment and wellbeing. RESULTS Auditory speech perception of children with CI in mainstream education enable them to acquire language and educational levels that are comparable to those of their normal hearing peers. Although the children in mainstream and hard-of-hearing settings show similar speech perception abilities, language development in children in hard-of-hearing settings lags significantly behind. Speech perception, language and educational attainments of children in deaf education remained extremely poor. Furthermore more children in mainstream and hard-of-hearing environments are resilient than in deaf educational settings. Regression analyses showed an important influence of educational setting. CONCLUSIONS Children with CI who are placed in early intervention environments that facilitate auditory development are able to achieve good auditory speech perception, language and educational levels on the long term. Most parents of these children report no social-emotional concerns.

Benefits of simultaneous bilateral cochlear implantation on verbal reasoning skills in prelingually deaf children

BACKGROUND Impaired auditory speech perception abilities in deaf children with hearing aids compromised their verbal intelligence enormously. The availability of unilateral cochlear implantation (CI) auditory speech perception and spoken vocabulary enabled them to reach near ageappropriate levels. This holds especially for children in spoken language environments. However, speech perception in complex listening situations and the acquisition of complex verbal skills remains difficult. Bilateral CI was expected to enhance the acquisition of verbal intelligence by improved understanding of speech in noise. METHODS This study examined the effect of bilateral CI on verbal intelligence of 49 deaf children (3;5-8;0 years). Relations between speech perception in noise, auditory short-term memory and verbal intelligence were analysed with multiple linear regressions. In addition, the interaction of educational setting, mainstream or special, on these relations was analysed. RESULTS Children with bilateral CI obtained higher scores on verbal intelligence. Significant associations were present between speech perception in noise, auditory short-term memory and verbal intelligence. CONCLUSION Children with simultaneous bilateral CIs showed better speech perception in noise than children with unilateral CIs, which mediated by the auditory short-term memory capacity, enhanced the ability to acquire more complex verbal skills for BICI children in mainstream education.

Suggestions for a Guideline for Cochlear Implantation in CHARGE Syndrome

Objective: Identifying aspects for establishing cochlear implantation guidelines for patients with ocular coloboma, heart defects, atresia of the choanae, retardation (of growth and/or of development), genital anomalies, and ear anomalies (CHARGE) syndrome (CS). Study Design: Explorative retrospective study. Setting: Cochlear implant (CI)-centers of tertiary referral centers in The Netherlands. Patients: Ten patients with CS who received a CI between 2002 and 2012. Interventions: Describing the challenges and benefits of cochlear implantation in CS. Main Outcome Measures: Imaging and surgical findings, language development, and Quality-of-life (QoL), compared with two control groups: 1) 34 non-syndromic CI-users and 2) 13 patients with CS without CI because of sufficient hearing. Results: Subjective and objective audiometry and magnetic resonance imaging were necessary to confirm the presence of the cochlear nerve. Surgery in CS was challenging because of enlarged emissary veins, semi-circular-canal aplasia, aberrant facial nerve, and dysplastic cochlear windows, making computed tomography indispensable in surgical preparations. No major intraoperative complications occurred. Despite additional handicaps, all patients showed auditory benefit and improvement in disease-specific QoL. Patients implanted at a relatively young age (⩽37 months) followed by a long period of CI-use (>5 years) and with minor additional problems, developed spoken language at a basic level comparable to that of the control group of CS patients. Conclusion: A CI should be considered in all patients with CS and severe sensorineural hearing loss. A careful work-up is required, comprising computed tomography, magnetic resonance imaging, objective, and subjective audiometry and assessment by a specialized multidisciplinary team. Cochlear implantation in CS might be complicated by syndrome-related temporal-bone anatomy, and the outcome of the CI is more individually determined. Early implantation should be aimed for.

Linguistic Profiles of Children with CI as Compared with Children with Hearing or Specific Language Impairment.

BACKGROUND The spoken language difficulties of children with moderate or severe to profound hearing loss are mainly related to limited auditory speech perception. However, degraded or filtered auditory input as evidenced in children with cochlear implants (CIs) may result in less efficient or slower language processing as well. To provide insight into the underlying nature of the spoken language difficulties in children with CIs, linguistic profiles of children with CIs are compared with those of hard-of-hearing (HoH) children with conventional hearing aids and children with specific language impairment (SLI). AIMS To examine differences in linguistic abilities and profiles of children with CIs as compared with HoH children and children with SLI, and whether the spoken language difficulties of children with CIs mainly lie in limited auditory perception or in language processing problems. METHODS & PROCEDURE Differences in linguistic abilities and differential linguistic profiles of 47 children with CI, 66 HoH children with moderate to severe hearing loss, and 127 children with SLI are compared, divided into two age cohorts. Standardized Dutch tests were administered. Factor analyses and cluster analyses were conducted to find homogeneous linguistic profiles of the children. OUTCOMES & RESULTS The children with CIs were outperformed by their HoH peers and peers with SLI on most linguistic abilities. Concerning the linguistic profiles, the largest group of children with CIs and HoH children shared similar profiles. The profiles observed for most of the children with SLI were different from those of their peers with hearing loss in both age cohorts. CONCLUSIONS & IMPLICATIONS Results suggest that the underlying nature of spoken language problems in most children with CIs manifests in limited auditory perception instead of language processing difficulties. However, there appears to be a subgroup of children with CIs whose linguistic profiles resemble those of children with SLI.