Jessica Beer

Neurocognitive Risk in Children With Cochlear Implants

IMPORTANCE Children who receive a cochlear implant (CI) for early severe to profound sensorineural hearing loss may achieve age-appropriate spoken language skills not possible before implantation. Despite these advances, reduced access to auditory experience may have downstream effects on fundamental neurocognitive processes for some children with CIs. OBJECTIVE To determine the relative risk (RR) of clinically significant executive functioning deficits in children with CIs compared with children with normal hearing (NH). DESIGN, SETTING, AND PARTICIPANTS In this prospective, cross-sectional study, 73 children at a hospital-based clinic who received their CIs before 7 years of age and 78 children with NH, with average to above average mean nonverbal IQ scores, were recruited in 2 age groups: preschool age (age range, 3-5 years) and school age (age range, 7-17 years). No children presented with other developmental, cognitive, or neurologic diagnoses. INTERVENTIONS Parent-reported checklist measures of executive functioning were completed during psychological testing sessions. MAIN OUTCOMES AND MEASURES Estimates of the RR of clinically significant deficits in executive functioning (≥1 SDs above the mean) for children with CIs compared with children with NH were obtained based on 2 parent-reported child behavior checklists of everyday problems with executive functioning. RESULTS In most domains of executive functioning, children with CIs were at 2 to 5 times greater risk of clinically significant deficits compared with children with NH. The RRs for preschoolers and school-aged children, respectively, were greatest in the areas of comprehension and conceptual learning (RR [95% CI], 3.56 [1.71-7.43] and 6.25 [2.64-14.77]), factual memory ( 4.88 [1.58-15.07] and 5.47 [2.03-14.77]), attention (3.38 [1.03-11.04] and 3.13 [1.56-6.26]), sequential processing (11.25 [1.55-81.54] and 2.44 [1.24-4.76]), working memory (4.13 [1.30-13.06] and 3.64 [1.61-8.25] for one checklist and 1.77 [0.82-3.83] and 2.78 [1.18-6.51] for another checklist), and novel problem-solving (3.93 [1.50-10.34] and 3.13 [1.46-6.67]). No difference between the CI and NH samples was found for visual-spatial organization (2.63 [0.76-9.03] and 1.04 [0.45-2.40] on one checklist and 2.86 [0.98-8.39] for school-aged children on the other checklist). CONCLUSIONS AND RELEVANCE A large proportion of children with CIs are at risk for clinically significant deficits across multiple domains of executive functioning, a rate averaging 2 to 5 times that of children with NH for most domains. Screening for risk of executive functioning deficits should be a routine part of the clinical evaluation of all children with deafness and CIs.

Executive function in everyday life: implications for young cochlear implant users

Cochlear implants (CIs) provide significant speech and language benefits to many prelingually deaf children (Geers et al., 2008). However, CIs do not provide optimal benefits to all children who receive them. Identification of pre-implant predictors of CI outcomes is critical to providing appropriate expectations to parents and in decision making regarding intervention, assessment, management, and focused habilitation throughout childhood. A growing body of research findings suggests that deafness and degraded auditory experience may affect not only speech and language skills, but also other neurocognitive functions (Pisoni et al., 2010). We adopt the working hypothesis (supported by recent findings from our center) that many profoundly deaf children who use CIs, especially the low-performing users, may have other neural, cognitive, and affective sequelae resulting from a period of auditory deprivation combined with a delay in language development before implantation. For example, compared to hearing peers, young CI users show differences in short-term and Working Memory capacity and have slower processing speeds for rehearsing and retrieving verbal information (Pisoni et al., 2010). Robust speech perception and spoken language processing are highly dependent on these underlying core neurocognitive building blocks. Executive function (EF) is an umbrella construct from research in neuropsychology and cognitive neuroscience representing a complex set of cognitive processes such as organization, planning, working memory, inhibition, and flexibility, involved in the control of thought, action, and emotion (Gioia et al., 2001). There is general agreement among cognitive psychologists and neuroscientists that several different aspects of EF play important roles in language perception and production via top-down feedback and control of processing activities in a wide range of behavioral tasks (Pisoni et al., 2010). In addition, language itself serves an EF because through verbal mediation processes it can be used by the child to exercise control over thoughts, actions, and emotions with self-directed speech (Muller et al., 2009). For these reasons, knowledge of the developmental relations between EF and language processes in children with CIs is critical to understanding not only outcomes proximal to language, but also more distal outcomes such as learning, self-regulation, and social cognition. The two objectives of this study were to identify areas of EF vulnerability in children with CI’s using a behavior rating scale completed by parents, and to determine if these areas of vulnerability are associated with conventional speech and language outcomes.

Executive Functioning Skills in Preschool-Age Children With Cochlear Implants

PURPOSE The purpose of this study was to determine whether deficits in executive functioning (EF) in children with cochlear implants (CIs) emerge as early as the preschool years. METHOD Two groups of children ages 3 to 6 years participated in this cross-sectional study: 24 preschoolers who had CIs prior to 36 months of age and 21 preschoolers with normal hearing (NH). All were tested on normed measures of working memory, inhibition-concentration, and organization-integration. Parents completed a normed rating scale of problem behaviors related to EF. Comparisons of EF skills of children with CIs were made to peers with NH and to published nationally representative norms. RESULTS Preschoolers with CIs showed significantly poorer performance on inhibition-concentration and working memory compared with peers with NH and with national norms. No group differences were found in visual memory or organization-integration. When data were controlled for language, differences in performance measures of EF remained, whereas differences in parent-reported problems with EF were no longer significant. Hearing history was generally unrelated to EF. CONCLUSIONS This is the first study to demonstrate that EF deficits found in older children with CIs begin to emerge as early as preschool years. The ability to detect these deficits early has important implications for early intervention and habilitation after cochlear implantation.

The ear is connected to the brain: some new directions in the study of children with cochlear implants at Indiana University.

Since the early 1980s, the DeVault Otologic Research Laboratory at the Indiana University School of Medicine has been on the forefront of research on speech and language outcomes in children with cochlear implants. This paper highlights work over the last decade that has moved beyond collecting speech and language outcome measures to focus more on investigating the underlying cognitive, social, and linguistic skills that predict speech and language outcomes. This recent work reflects our growing appreciation that early auditory deprivation can affect more than hearing and speech perception. The new directions include research on attention to speech, word learning, phonological development, social development, and neurocognitive processes. We have also expanded our subject populations to include infants and children with additional disabilities.

Auditory skills, language development, and adaptive behavior of children with cochlear implants and additional disabilities

Objective: The objective of this study was to evaluate the development of functional auditory skills, language, and adaptive behavior in deaf children with cochlear implants (CI) who also have additional disabilities (AD). Design: A two-group, pre-test versus post-test design was used. Study sample: Comparisons were made between 23 children with CIs and ADs, and an age-matched comparison group of 23 children with CIs without ADs (No-AD). Assessments were obtained pre-CI and within 12 months post-CI. Results: All but two deaf children with ADs improved in auditory skills using the IT-MAIS. Most deaf children in the AD group made progress in receptive but not expressive language using the preschool language scale, but their language quotients were lower than the No-AD group. Five of eight children with ADs made progress in daily living skills and socialization skills; two made progress in motor skills. Children with ADs who did not make progress in language, did show progress in adaptive behavior. Conclusions: Children with deafness and ADs made progress in functional auditory skills, receptive language, and adaptive behavior. Expanded assessment that includes adaptive functioning and multi-center collaboration is recommended to best determine benefits of implantation in areas of expected growth in this clinical population.

Developmental Effects of Family Environment on Outcomes in Pediatric Cochlear Implant Recipients

Objective To examine and compare the family environment of preschool- and school-age children with cochlear implants and assess its influence on children’s executive function and spoken language skills. Study Design Retrospective between-subjects design. Setting Outpatient research laboratory. Patients Prelingually deaf children with cochlear implants and no additional disabilities and their families. Intervention(s) Cochlear implantation and speech-language therapy. Main Outcome Measures Parents completed the Family Environment Scale and the Behavior Rating Inventory of Executive Function (or the preschool version). Children were tested using the Peabody Picture Vocabulary Test-4 and either the Preschool Language Scales-4 or the Clinical Evaluation of Language Fundamentals–4. Results The family environments of children with cochlear implants differed from normative data obtained from hearing children, but average scores were within 1 standard deviation of norms on all subscales. Families of school-age children reported higher levels of control than those of preschool-age children. Preschool-age children had fewer problems with emotional control when families reported higher levels of support and lower levels of conflict. School-age children had fewer problems with inhibition but more problems with shifting of attention when families reported lower levels of conflict. School-age children’s receptive vocabularies were enhanced by families with lower levels of control and higher levels of organization. Conclusion Family environment and its relation to language skills and executive function development differed across the age groups in this sample of children with cochlear implants. Because family dynamics is one developmental/environmental factor that can be altered with therapy and education, the present results have important clinical implications for family-based interventions for deaf children with cochlear implants.

Preschool speech intelligibility and vocabulary skills predict long-term speech and language outcomes following cochlear implantation in early childhood

Abstract Speech and language measures during grade school predict adolescent speech-language outcomes in children who receive cochlear implants (CIs), but no research has examined whether speech and language functioning at even younger ages is predictive of long-term outcomes in this population. The purpose of this study was to examine whether early preschool measures of speech and language performance predict speech-language functioning in long-term users of CIs. Early measures of speech intelligibility and receptive vocabulary (obtained during preschool ages of 3–6 years) in a sample of 35 prelingually deaf, early-implanted children predicted speech perception, language, and verbal working memory skills up to 18 years later. Age of onset of deafness and age at implantation added additional variance to preschool speech intelligibility in predicting some long-term outcome scores, but the relationship between preschool speech-language skills and later speech-language outcomes was not significantly attenuated by the addition of these hearing history variables. These findings suggest that speech and language development during the preschool years is predictive of long-term speech and language functioning in early-implanted, prelingually deaf children. As a result, measures of speech-language functioning at preschool ages can be used to identify and adjust interventions for very young CI users who may be at long-term risk for suboptimal speech and language outcomes.

Concept Formation Skills in Long-Term Cochlear Implant Users

This study investigated if a period of auditory sensory deprivation followed by degraded auditory input and related language delays affects visual concept formation skills in long-term prelingually deaf cochlear implant (CI) users. We also examined if concept formation skills are mediated or moderated by other neurocognitive domains (i.e., language, working memory, and executive control). Relative to normally hearing (NH) peers, CI users displayed significantly poorer performance in several specific areas of concept formation, especially when multiple comparisons and relational concepts were components of the task. Differences in concept formation between CI users and NH peers were fully explained by differences in language and inhibition-concentration skills. Language skills were also found to be more strongly related to concept formation in CI users than in NH peers. The present findings suggest that complex relational concepts may be adversely affected by a period of early prelingual deafness followed by access to underspecified and degraded sound patterns and spoken language transmitted by a CI. Investigating a unique clinical population such as early-implanted prelingually deaf children with CIs can provide new insights into foundational brain-behavior relations and developmental processes.

Early Expressive Language Skills Predict Long-Term Neurocognitive Outcomes in Cochlear Implant Users: Evidence from the MacArthur–Bates Communicative Development Inventories

PURPOSE The objective of the present article was to document the extent to which early expressive language skills (measured using the MacArthur-Bates Communicative Development Inventories [CDI; Fenson et al., 2006]) predict long-term neurocognitive outcomes in a sample of early-implanted prelingually deaf cochlear implant (CI) users. METHOD The CDI was used to index the early expressive language skills of 32 pediatric CI users after an average of 1.03 years (SD = 0.56, range = 0.39-2.17) of CI experience. Long-term neurocognitive outcomes were assessed after an average of 11.32 (SD = 2.54, range = 7.08-16.52) years of CI experience. Measures of long-term neurocognitive outcomes were derived from gold-standard performance-based and questionnaire-based assessments of language, executive functioning, and academic skills. RESULT Analyses revealed that early expressive language skills, collected on average 1.03 years post cochlear implantation, predicted long-term language, executive functioning, and academic skills up to 16 years later. CONCLUSION These findings suggest that early expressive language skills, as indexed by the CDI, are clinically relevant for identifying CI users who may be at high risk for long-term neurocognitive delays and disturbances.