William G. Kronenberger

Purpose: Deaf children with cochlear implants (CIs) experience challenges in early development of hearing and language skills that may place them at risk for externalizing behavior problems, such as aggression, hyperactivity–impulsivity, and oppositional behavior. This study is a longitudinal investigation of (a) between-groups differences in externalizing behavior problems between preschool-aged children with CIs and normal-hearing (NH) peers, and (b) within-group factors that may explain variability in externalizing problems within the sample of CI users. Method: Parents of 26 children with CIs and 30 NH peers completed externalizing behavior checklists at two visits separated by 1 year, starting at ages 3 or 4 years. Demographic/hearing history variables, language (vocabulary), nonverbal intelligence, and coping flexibility were assessed for concurrent and predictive associations with externalizing problems within the CI sample. Results: Results showed significantly greater externalizing behavior problems in CI users compared to NH peers at Time 1, although these differences were less pronounced 1 year later. Poorer residual hearing and better coping flexibility at Time 1 were associated with fewer externalizing behavior problems in CI users at Time 2. CI users who showed improvement in coping flexibility over the 1-year period also showed improvement in externalizing behaviors during that period. Nonverbal intelligence and language were not associated with externalizing behavior problems. Conclusions: Preschool-aged CI users may be at greater risk than NH peers for the early development of externalizing behavior problems. Improved coping flexibility may offer the potential for improvement in externalizing behavior problems for young CI users.

Purpose: Many child-, hearing-, and device-related factors contribute to spoken language outcomes in children who are deaf and hard of hearing (DHH). Recently, the family environment has been implicated as another contributing factor in language development. However, most studies on the role of families in language outcomes of children who are DHH are cross-sectional in nature and fail to draw directional conclusions. This study investigated the bidirectional relationship between attributes of the family environment and the spoken language outcomes of DHH children by examining data collected over approximately 1 year. Method: Caregiver–child dyads composed of 57 typically hearing (TH) children and 53 DHH children and their primary caregiver completed a standardized, self-report family environment assessment, along with three standardized measures of receptive language at two separate home visits, separated by approximately 1 year. Results: Family environments of DHH and TH children did not differ significantly. In the DHH and TH groups, families that reported placing a greater emphasis on intellectual and cultural pursuits at the initial visit (T1) had children with better language outcomes at the second visit (T2), above and beyond the contribution of T1 language. Additionally, in the DHH group only, families that reported a greater emphasis on achievement and competition at T1 had children with poorer language skills at T2. Conclusions: Families that emphasize intellectual–cultural environments had DHH and TH children with better language skills 1 year later, whereas those that deemphasized competition and perfection had DHH children with better language skills 1 year later. These findings highlight the importance of family environment and dynamics in contributing to language development in all children, but particularly DHH children, who are at a heightened risk for language delays.

Purpose: Large individual differences and poor speech recognition outcomes are routinely observed in most patients who have received auditory brainstem implants (ABIs). A case report of an ABI recipient with exceptionally good speech recognition outcomes presents an opportunity to better understand the core information processing mechanisms that underlie variability and individual differences in outcomes.Method: A case study is reported of an adult ABI recipient (ID-006) with postlingually acquired, Neurofibromatosis Type 2 (NF2)-related hearing loss who displayed exceptional postoperative speech recognition scores. A novel battery of assessment measures was used to evaluate ID-006's auditory, cognitive, and linguistic information processing skills.Results: Seventeen years following ABI activation, ID-006 scored 77.6% correct on the AzBio Sentences in quiet. On auditory processing tasks, ID-006 scored higher on tasks with meaningful sentences and much lower on tasks that relied exclusively on audibility. ID-006 also demonstrated exceptionally strong abilities on several cognitive and linguistic information processing tasks. Conclusions: Results from a novel battery of information processing tests suggest that ID-006 relies extensively on top-down predictive processing and cognitive control strategies to efficiently encode and process auditory information provided by his ABI. Results suggest that current measures of outcomes and benefits should be expanded beyond conventional speech recognition mea-sures to include more sensitive and robust measures of speech recognition as well as neurocognitive measures such as executive function, working memory, and lexical access.

Background: Norm-based scores used to assess cognitive ability have clinical value when describing functioning of patients with neuronopathic disorders compared with unaffected, same-age peers. However, they have limitations when used to assess change in cognitive ability between two timepoints, especially in children with severe cognitive decline. Calculation of Projected Retained Ability Scores (PRAS) is a novel method developed to characterize absolute change in norm-based ability test scores. In this analysis, PRAS were calculated post hoc for children with mucopolysaccharidosis II (MPS II; Hunter syndrome) and early cognitive impairment in a 52-week phase 2/3 randomized controlled trial (RCT) and its extension study of intrathecal idursulfase (idursulfase-IT). Patients completing the first year of the extension after receiving idursulfase-IT in the RCT and extension (n = 32 of 34 enrolled) or the extension only (n = 15 of 15 enrolled) were categorized according to changes in Differential Ability Scales, Second Edition, General Conceptual Ability (DAS-II GCA) scores and PRAS at 1 and 2 years. Analyses were conducted in the overall population and a subpopulation aged < 6 years at baseline (idursulfase-IT in the RCT and extension [n = 27] and extension only [n = 12]). Results: PRAS methodology differentiated patients with decreases in DAS-II GCA scores into three separate categories reflecting below-average cognitive growth rates, plateauing cognitive development, and deteriorating cognitive functioning. After 1 year in the RCT, 72.4% of patients who initiated idursulfase-IT had above-average or average cognitive growth rates in DAS-II GCA scores compared with 53.3% of those who did not receive idursulfase-IT; 6.9% versus 20.0% experienced deteriorating cognitive functioning. Similar results were seen in children aged < 6 years: 76% (idursulfase-IT group) versus 50% (no idursulfase-IT) had above-average or average cognitive growth rates in DAS-II GCA scores; 4% versus 17% had deteriorating cognitive functioning. The difference in the distributions of cognitive categories at 1 year in children aged < 6 years was significant (p = 0.048). At 2 years, the proportions of patients in different cognitive categories were more similar between treatment groups. Conclusions: PRAS methodology may help to differentiate changes in cognitive development in MPS II, and therefore may represent a valuable addition to existing approaches for interpreting changes in cognitive scores over time. Trial Registration: ClinicalTrials.gov NCT02055118 (registration date: 4 February 2014) and NCT02412787 (registration date: 9 April 2015).

Background: Verbal working memory delays are found in many deaf children with cochlear implants compared with normal-hearing peers, but the factors contributing to these delays are not well understood. This study investigated differences between cochlear implant users and normal-hearing peers in memory scanning speed during a challenging verbal working memory task. To better understand variability in verbal working memory capacity within each sample, associations between memory scanning speed, speech recognition, and language were also investigated. Methods: Twenty-five prelingually deaf, early implanted children (age, 8–17 yr) with cochlear implants and 25 normal-hearing peers completed the Wechsler Intelligence Scale for Children, Fifth Edition, Letter-Number Sequencing (LNS) working memory task. Timing measures were made for response latency and average pause duration between letters/numbers recalled during the task. Participants also completed measures of speech recognition, vocabulary, and language comprehension. Results: Children with cochlear implants had longer pause durations than normal-hearing peers during three-span LNS sequences, but the groups did not differ in response latencies or in pause durations during two-span LNS sequences. In the sample of cochlear implant users, poorer speech recognition was correlated with longer pause durations during two-span sequences, whereas poorer vocabulary and weaker language comprehension were correlated with longer response latencies during two-span sequences. Response latencies and pause durations were unrelated to language in the normal-hearing sample. Conclusion: Children with cochlear implants have slower verbal working memory scanning speed than children with normal hearing. More robust phonological-lexical representations of language in memory may facilitate faster memory scanning speed and better working memory in cochlear implant users.

Purpose: Verbal fluency tasks assess the ability to quickly and efficiently retrieve words from the mental lexicon by requiring subjects to rapidly generate words within a phonological or semantic category. This study investigated differences between cochlear implant users and normal-hearing peers in the clustering and time course of word retrieval during phonological and semantic verbal fluency tasks.Method: Twenty-eight children and adolescents (aged 9–17 years) with cochlear implants and 33 normal-hearing peers completed measures of verbal fluency, nonverbal intelligence, speech perception, and verbal short-term/working memory. Phonological and semantic verbal fluency tests were scored for total words generated, words generated in each 10-s interval of the 1-min task, latency to first word generated, number of word clusters, average cluster size, and number of word/cluster switches.Results: Children and adolescents with cochlear implants generated fewer words than normal-hearing peers throughout the entire 60-s time interval of the phonological and semantic fluency tasks. Cochlear implant users also had slower start latency times and produced fewer clusters and switches than normal-hearing peers during the phonological fluency task. Speech perception and verbal working memory scores were more strongly associated with verbal fluency scores in children and adolescents with cochlear implants than in normal-hearing peers.Conclusions: Cochlear implant users show poorer phonological and semantic verbal fluency than normal-hearing peers, and their verbal fluency is significantly associated with speech perception and verbal working memory. These findings suggest deficits in fluent retrieval of phonological and semantic information from long-term lexical memory in cochlear implant users.

Introduction: Hydroxyurea reduces the incidence of vaso-occlusive episodes, stroke, and respiratory, cardiac, and renal damage in sickle cell disease by increasing fetal hemoglobin. However, because suboptimal adherence to hydroxyurea limits its effectiveness, understanding patient-specific barriers to hydroxyurea adherence could help improve adherence and health outcomes in patients with sickle cell disease. The aim of this single-site, prospective, IRB-approved study was to validate a 24-item patient- and caregiver-reported hydroxyurea treatment adherence questionnaire, the Hydroxyurea Evaluation of Adherence for Life (HEAL) scale. Methods: A sample of 24 adults with sickle cell disease and 16 caregivers of children with sickle cell disease completed the HEAL scale, and a subset of the original sample provided a second HEAL scale for test-retest reliability. HEAL scale results were validated against global adherence ratings from participants and health-care providers, records of access to pill bottles, and laboratory values for fetal hemoglobin and absolute neutrophil count. Results and Discussion: Results demonstrated excellent internal consistency for the HEAL Total score and eight (3-item) subscale scores (Dose, Remember, Plan, Cost, Understand, Effectiveness, Laboratory, and Pharmacy), as well as strong test-retest reliability for all HEAL scores except the Cost subscale. HEAL Total scores correlated significantly with validity measures, including global adherence ratings and lab values. The HEAL scale offers significant clinical potential for understanding adherence in individual sickle cell disease patients and significant research potential for characterizing adherence in persons with sickle cell disease who are treated with hydroxyurea.