Neuro-ophthalmologic and blood biomarker responses in ADHD following subconcussive head impacts: a case-control trial.

Introduction: This clinical trial aimed to determine the influence of attention-deficit/hyperactivity disorder (ADHD) on neuro-ophthalmologic function and brain-derived blood biomarkers following acute subconcussive head impacts. Methods: The present trial consisted of age- and sex-matched samples with a ratio of 1:1 between two groups with a total sample size of 60 adults (age ± SD; 20.0 ± 1.8 years). Soccer players diagnosed with and medicated daily for ADHD were assigned into an ADHD group (n = 30). Soccer players without ADHD were assigned into a non-ADHD group (n = 30). Participants performed 10 soccer headers with a soccer ball projected at a velocity of 25mph. King-Devick test (KDT), near point of convergence (NPC), and serum levels of NF-L, tau, GFAP, and UCH-L1 were assessed at baseline (pre-heading) and at 2 h and 24 h post-heading. Results: There were no statistically significant group-by-time interactions in outcome measures. However, at baseline, the ADHD group exhibited lower neuro-ophthalmologic functions compared to the non-ADHD group (NPC: p = 0.019; KDT: p = 0.018), and persisted at 2 h-post (NPC: p = 0.007; KDT: p = 0.014) and 24 h-post heading (NPC: p = 0.001). NPC significantly worsened over time in both groups compared to baseline [ADHD: 2 h-post, 1.23 cm, 95%CI:(0.77, 1.69), p < 0.001; 24 h-post, 1.68 cm, 95%CI:(1.22, 2.13), p = 0.001; Non-ADHD: 2 h-post, 0.96 cm, 95%CI:(0.50, 1.42), p < 0.001; 24 h-post, 1.09 cm, 95%CI:(0.63, 1.55), p < 0.001]. Conversely, improvements in KDT time compared to baseline occurred at 2 h-post in the non-ADHD group [-1.32 s, 95%CI:(-2.55, -0.09), p = 0.04] and at 24 h-post in both groups [ADHD: -4.66 s, 95%CI:(-5.89, -3.43), p < 0.001; Non-ADHD: -3.46 s, 95%CI:(-4.69, -2.23), p < 0.001)]. There were no group-by-time interactions for GFAP as both groups exhibited increased levels at 2 h-post [ADHD: 7.75 pg./mL, 95%CI:(1.41, 14.10), p = 0.019; Non-ADHD: 7.91 pg./mL, 95%CI:(1.71, 14.14), p = 0.015)] that returned to baseline at 24 h-post. NF-L levels increased at 2 h-post heading in the ADHD group [0.45 pg./mL, 95%CI:(0.05, 0.86), p = 0.032], but no significant NF-L changes were observed in the non-ADHD group over time. Discussion: Ten soccer headers elevated GFAP levels and NPC impairment in both groups. However, persisting group difference in NPC, blunted KDT performance, and increased NF-L levels in the ADHD group suggest that ADHD may reduce neuro-ophthalmologic function and heighten axonal response to soccer headers. Clinical trial registration: ClinicalTrials.gov, identifier ID: (NCT04880304).

A post hoc analysis of Projected Retained Ability Scores (PRAS) for the longitudinal assessment of cognitive functioning in patients with neuronopathic mucopolysaccharidosis II receiving intrathecal idursulfase-IT.

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).

Extraordinary Speech and Language Outcomes After Auditory Brainstem Implantation: Guidance From a Case Study.

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 measures to include more sensitive and robust measures of speech recognition as well as neurocognitive measures such as executive function, working memory, and lexical access.

Speed of Information Processing and Verbal Working Memory in Children and Adolescents With Cochlear Implants.

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.

Verbal Fluency in Prelingually Deaf, Early Implanted Children and Adolescents With Cochlear Implants.

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.

Contribution of Verbal Learning & Memory and Spectro-Temporal Discrimination to Speech Recognition in Cochlear Implant Users.

OBJECTIVES: Existing cochlear implant (CI) outcomes research demonstrates a high degree of variability in device effectiveness among experienced CI users. Increasing evidence suggests that verbal learning and memory (VL&M) may have an influence on speech recognition with CIs. This study examined the relations in CI users between visual measures of VL&M and speech recognition in a series of models that also incorporated spectro-temporal discrimination. Predictions were that (1) speech recognition would be associated with VL&M abilities and (2) VL&M would contribute to speech recognition outcomes above and beyond spectro-temporal discrimination in multivariable models of speech recognition. METHODS: This cross-sectional study included 30 adult postlingually deaf experienced CI users who completed a nonauditory visual version of the California Verbal Learning Test-Second Edition (v-CVLT-II) to assess VL&M, and the Spectral-Temporally Modulated Ripple Test (SMRT), an auditory measure of spectro-temporal processing. Participants also completed a battery of word and sentence recognition tasks. RESULTS: CI users showed significant correlations between some v-CVLT-II measures (short-delay free- and cued-recall, retroactive interference, and "subjective" organizational recall strategies) and speech recognition measures. Performance on the SMRT was correlated with all speech recognition measures. Hierarchical multivariable linear regression analyses showed that SMRT performance accounted for a significant degree of speech recognition outcome variance. Moreover, for all speech recognition measures, VL&M scores contributed independently in addition to SMRT. CONCLUSION: Measures of spectro-temporal discrimination and VL&M were associated with speech recognition in CI users. After accounting for spectro-temporal discrimination, VL&M contributed independently to performance on measures of speech recognition for words and sentences produced by single and multiple talkers. LEVEL OF EVIDENCE: 3 Laryngoscope, 133:661-669, 2023.

Development and Validation of the Patient/Caregiver Reported Hydroxyurea Evaluation of Adherence for Life (HEAL) Scale.

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.

Executive functioning and spoken language skills in young children with hearing aids and cochlear implants: Longitudinal findings.

Deaf or hard-of-hearing (DHH) children who use auditory-oral communication display considerable variability in spoken language and executive functioning outcomes. Furthermore, language and executive functioning skills are strongly associated with each other in DHH children, which may be relevant for explaining this variability in outcomes. However, longitudinal investigations of language and executive functioning during the important preschool period of development in DHH children are rare. This study examined the predictive, reciprocal associations between executive functioning and spoken language over a 1-year period in samples of 53 DHH and 59 typically hearing (TH) children between ages 3-8 years at baseline. Participants were assessed on measures of receptive spoken language (vocabulary, sentence comprehension, and following spoken directions) and caregiver-completed executive functioning child behavior checklists during two in-person home visits separated by 1 year. In the sample of DHH children, better executive functioning at baseline (Time 1) was associated with better performance on the higher-order language measures (sentence comprehension and following spoken directions) 1 year later (Time 2). In contrast, none of the Time 1 language measures were associated with better executive functioning in Time 2 in the DHH sample. TH children showed no significant language-executive functioning correlations over the 1-year study period. In regression analyses controlling for Time 1 language scores, Time 1 executive functioning predicted Time 2 language outcomes in the combined DHH and TH samples, and for vocabulary, that association was stronger in the DHH than in the TH sample. In contrast, after controlling for Time 1 executive functioning, none of the regression analyses predicting Time 2 executive functioning from Time 1 language were statistically significant. These results are the first findings to demonstrate that everyday parent-rated executive functioning behaviors predict basic (vocabulary) and higher-order (comprehension, following directions) spoken language development 1 year later in young (3-8 year old) DHH children, even after accounting for initial baseline language skills.

Exceptional Speech Recognition Outcomes After Cochlear Implantation: Lessons From Two Case Studies.

PURPOSE: Individual differences and variability in outcomes following cochlear implantation (CI) in patients with hearing loss remain significant unresolved clinical problems. Case reports of specific individuals allow for detailed examination of the information processing mechanisms underlying variability in outcomes. Two adults who displayed exceptionally good postoperative CI outcomes shortly after activation were administered a novel battery of auditory, speech recognition, and neurocognitive processing tests. METHOD: A case study of two adult CI recipients with postlingually acquired hearing loss who displayed excellent postoperative speech recognition scores within 3 months of initial activation. Preoperative City University of New York sentence testing and a postoperative battery of sensitive speech recognition tests were combined with auditory and visual neurocognitive information processing tests to uncover their strengths, weaknesses, and milestones. RESULTS: Preactivation CUNY auditory-only (A) scores were < 5% correct while the auditory + visual (A + V) scores were > 74%. Acoustically with their CIs, both participants' scores on speech recognition, environmental sound identification and speech in noise tests exceeded average CI users scores by 1-2 standard deviations. On nonacoustic visual measures of language and neurocognitive functioning, both participants achieved above average scores compared with normal hearing adults in vocabulary knowledge, rapid phonological coding of visually presented words and nonwords, verbal working memory, and executive functioning. CONCLUSIONS: Measures of multisensory (A + V) speech recognition and visual neurocognitive functioning were associated with excellent speech recognition outcomes in two postlingual adult CI recipients. These neurocognitive information processing domains may underlie the exceptional speech recognition performance of these two patients and offer new directions for research explaining variability in postimplant outcomes. Results further suggest that current clinical outcome measures should be expanded beyond the conventional speech recognition measures to include more sensitive robust tests of speech recognition as well as neurocognitive measures of working memory, vocabulary, lexical access, and executive functioning.

Preoperative Visual Measures of Verbal Learning and Memory and their Relations to Speech Recognition After Cochlear Implantation.

OBJECTIVES: This study examined the performance of a group of adult cochlear implant (CI) candidates (CIC) on visual tasks of verbal learning and memory. Preoperative verbal learning and memory abilities of the CIC group were compared with a group of older normal-hearing (ONH) control participants. Relations between preoperative verbal learning and memory measures and speech recognition outcomes after 6 mo of CI use were also investigated for a subgroup of the CICs. DESIGN: A group of 80 older adult participants completed a visually presented multitrial free recall task. Measures of word recall, repetition learning, and the use of self-generated organizational strategies were collected from a group of 49 CICs, before cochlear implantation, and a group of 31 ONH controls. Speech recognition outcomes were also collected from a subgroup of 32 of the CIC participants who returned for testing 6 mo after CI activation. RESULTS: CICs demonstrated poorer verbal learning performance compared with the group of ONH control participants. Among the preoperative verbal learning and memory measures, repetition learning slope and measures of self-generated organizational clustering strategies were the strongest predictors of post-CI speech recognition outcomes. CONCLUSIONS: Older adult CI candidates present with verbal learning and memory deficits compared with older adults without hearing loss, even on visual tasks that are independent from the direct effects of audibility. Preoperative verbal learning and memory processes reflecting repetition learning and self-generated organizational strategies in free recall were associated with speech recognition outcomes 6 months after implantation. The pattern of results suggests that visual measures of verbal learning may be a useful predictor of outcomes in postlingual adult CICs.

Family Environmental Dynamics Differentially Influence Spoken Language Development in Children With and Without Hearing Loss.

PURPOSE: The aim of this study was to evaluate whether families of children with sensorineural hearing loss (SNHL) are organized similarly to those of typically developing, typically hearing (TH) children and whether the dimensions of family dynamics and environment are related to spoken language development similarly in children with and without SNHL. METHOD: Primary caregivers of children with SNHL (n = 63) or TH (n = 65) completed the Family Environment Scale-Fourth Edition (FES-4) to assess multiple dimensions of family environment. Children's receptive vocabulary was assessed with the Peabody Picture Vocabulary Test-Fourth Edition, and their receptive language was assessed by an age-appropriate version of the Concepts and Following Directions subtest of the Clinical Evaluation of Language Fundamentals and the Sentence Comprehension subscale of the Comprehensive Assessment of Spoken Language-Second Edition. Principal component analysis was used to examine the dimensional structure of the family environment. RESULTS: Three higher order components were derived from FES-4 subscales for both families of children with SNHL and with TH: Supportive, Controlling, and Conflicted. However, the composition of the factors themselves differed between the two groups. For the TH group, most family environment measures on the FES-4 were not associated with language outcomes. In contrast, for children with SNHL, families who were more supportive, less controlling, and less conflicted had children with better language skills. CONCLUSIONS: Three well-accepted dimensions of family dynamics and functioning apply to families of children with SNHL, but their composition differs from those of families with TH children. Family environmental dynamics were much more strongly associated with language outcomes in children with SNHL than in their TH peers. The spoken language development of children with SNHL, in particular, is better in families that provide high levels of support for each other and, in particular, low levels of control, disorganization, and conflict, reflecting the fragile nature of their spoken language development.

Verbal Working Memory Error Patterns and Speech-Language Outcomes in Youth With Cochlear Implants.

PURPOSE: Verbal working memory (VWM) delays are commonly found in prelingually deaf youth with cochlear implants (CIs), albeit with considerable interindividual variability. However, little is known about the neurocognitive information-processing mechanisms underlying these delays and how these mechanisms relate to spoken language outcomes. The goal of this study was to use error analysis of the letter-number sequencing (LNS) task to test the hypothesis that VWM delays in CI users are due, in part, to fragile, underspecified phonological representations in short-term memory. METHOD: Fifty-one CI users aged 7-22 years and 53 normal hearing (NH) peers completed a battery of speech, language, and neurocognitive tests. LNS raw scores and error profiles were compared between samples, and a hierarchical regression model was used to test for associations with measures of speech, language, and hearing. RESULTS: Youth with CIs scored lower on the LNS test than NH peers and committed a significantly higher number of errors involving phonological confusions (recalling an incorrect letter/digit in place of a phonologically similar one). More phonological errors were associated with poorer performance on measures of nonword repetition and following spoken directions but not with hearing quality. CONCLUSIONS: Study findings support the hypothesis that poorer VWM in deaf children with CIs is due, in part, to fragile, underspecified phonological representations in short-term/working memory, which underlie spoken language delays. Programs aimed at strengthening phonological representations may improve VWM and spoken language outcomes in CI users.

Differential At-Risk Pediatric Outcomes of Parental Sensitivity Based on Hearing Status.

Purpose The aim of this study was to investigate the role of parental sensitivity in language and neurocognitive outcomes in children who are deaf and/or hard of hearing (DHH). Method Sixty-two parent-child dyads of children with normal hearing (NH) and 64 of children who are DHH (3-8 years) completed parent and child measures of inhibitory control/executive functioning and child measures of sentence comprehension and vocabulary. The dyads also participated in a video-recorded, free-play interaction that was coded for parental sensitivity. Results There was no evidence of associations between parental sensitivity and inhibitory control or receptive language in children with NH. In contrast, parental sensitivity was related to children's inhibitory control and all language measures in children who are DHH. Moreover, inhibitory control significantly mediated the association between parental sensitivity and child language on the Clinical Evaluation of Language Fundamentals-Fifth Edition Following Directions subscale (6-8 years)/Clinical Evaluation of Language Fundamentals Preschool-Second Edition Concepts and Following Directions subscale (3-5 years). Follow-up analyses comparing subgroups of children who used hearing aids (n = 29) or cochlear implants (CIs; n = 35) revealed similar correlational trends, with the exception that parental sensitivity showed little relation to inhibitory control in the group of CI users. Conclusions Parental sensitivity is associated with at-risk language outcomes and disturbances in inhibitory control in young children who are DHH. Compared to children with NH, children who are DHH may be more sensitive to parental behaviors and their effects on emerging inhibitory control and spoken language. Specifically, inhibitory control, when scaffolded by positive parental behaviors, may be critically important for robust language development in children who are DHH.

Principles of Executive Functioning Interventions for Children With Cochlear Implants: Guidance From Research Findings and Clinical Experience.

: Children with cochlear implants (CIs) are at risk for experiencing deficits in the development of neurocognitive processes known as executive functions (EF). Such deficits likely arise as a consequence of early-onset deafness, the degraded auditory input provided by CIs, and delays in spoken language development. Interventions specifically designed for pediatric CI users are needed to address challenges and delays in EF because of the unique influences of hearing loss and language delay on EF development, which have deleterious, cascading effects on speech and language development and subsequent reading and academic achievement. Because patterns of EF weakness emerge even in the preschool years, interventions to improve EF should be implemented at early ages, while children are young and neuroplasticity is high. Drawing on previous research findings and clinical experience, this paper highlights 10 principles to guide the development of EF interventions for children with CIs.

Recognizing spoken words in semantically-anomalous sentences: Effects of executive control in early-implanted deaf children with cochlear implants.

OBJECTIVES: To investigate differences in speech, language, and neurocognitive functioning in normal hearing (NH) children and deaf children with cochlear implants (CIs) using anomalous sentences. Anomalous sentences block the use of downstream predictive coding during speech recognition, allowing for investigation of rapid phonological coding and executive functioning.Methods: Extreme groups were extracted from samples of children with CIs and NH peers (ages 9 to 17) based on the 7 highest and 7 lowest scores on the Harvard-Anomalous sentence test (Harvard-A). The four groups were compared on measures of speech, language, and neurocognitive functioning.Results: The 7 highest-scoring CI users and the 7 lowest-scoring NH peers did not differ in Harvard-A scores but did differ significantly on measures of neurocognitive functioning. Compared to low-performing NH peers, highperforming children with CIs had significantly lower nonword repetition scores but higher nonverbal IQ scores, greater verbal WM capacity, and excellent EF skills related to inhibition, shifting attention/mental flexibility and working memory updating.Discussion: High performing deaf children with CIs are able to compensate for their sensory deficits and weaknesses in automatic phonological coding of speech by engaging in a slow effortful mode of information processing involving inhibition, working memory and executive functioning.

Remote Assessment of Verbal Memory in Youth With Cochlear Implants During the COVID-19 Pandemic.

Purpose Youth with cochlear implants (CIs) are at risk for delays in verbal short-term memory (STM)/working memory (WM), which adversely affect language, neurocognitive, and behavioral outcomes. Assessment of verbal STM/WM is critical for identifying and addressing these delays, but standard assessment procedures require face-to-face (FTF) administration. The purpose of this study was to determine the feasibility and validity of remote testing methods (teleassessment) of verbal STM/WM in youth with CIs as a method of addressing COVID-19-related restrictions on FTF test administration. Method Tests of verbal STM/WM for nonwords, digit spans, letter-number sequences, sentences, and stories were individually administered by speech-language pathologists over a teleassessment platform to 28 youth (aged 9-22 years) with CIs and 36 same-aged normal-hearing peers. Examiners, parents, and participants completed quality and satisfaction ratings with the teleassessment procedure. Teleassessment scores were compared to results of tests obtained at FTF visits an average of 1.6 years earlier. Results Quality and satisfaction ratings for teleassessment were high and in almost all cases did not differ between the CI and normal-hearing samples. Youth with CIs scored lower than normal-hearing peers on measures of verbal STM/WM, and scores for digit span and letter-number sequencing did not differbetween teleassessment and FTF methods. Correlations across teleassessment and FTF visits were strong for digit span, letter-number sequencing, and sentence memory, but were more modest for nonword repetition. Conclusion With some caveats, teleassessment of verbal STM/WM was feasible and valid for youth with CIs.

Projected Retained Ability Score (PRAS): A New Methodology for Quantifying Absolute Change in Norm-Based Psychological Test Scores Over Time.

A limitation of norm-based ability test scores is that they can only be used to evaluate relative change (compared with change in the norm sample), as opposed to absolute (raw) change in performance from Time 1 to Time 2. To address this limitation, a novel method (Projected Retained Ability Score [PRAS]) was developed to characterize absolute change in norm-based ability test scores. The PRAS method was applied to Differential Ability Scales®-Second Edition (DAS-II) General Conceptual Ability (GCA) scores in three cases of children with the neurodegenerative condition mucopolysaccharidosis type II (MPS II) who were assessed at two visits, 16 to 23 months apart. Although all three cases showed declines in norm-based GCA scores, the PRAS method revealed differences in absolute change in performance. The PRAS method allows for differentiation of slower-than-average improvement or stabilization versus deterioration of cognitive ability when norm-based scores decline from Time 1 to Time 2.

Functional hearing quality in prelingually deaf school-age children and adolescents with cochlear implants.

OBJECTIVE: This study investigated differences in functional hearing quality between youth with cochlear implants (CIs) and normal hearing (NH) peers, as well as associations between functional hearing quality and audiological measures, speech perception, language and executive functioning (EF). DESIGN: Youth with CIs and NH peers completed measures of audiological functioning, speech perception, language and EF. Parents completed the Quality of Hearing Scale (QHS), a questionnaire measure of functional hearing quality. STUDY SAMPLE: Participants were 43 prelingually-deaf, early-implanted, long-term CI users and 43 NH peers aged 7-17 years. RESULTS: Compared to NH peers, youth with CIs showed poorer functional hearing quality on the QHS Speech, Localization, and Sounds subscales and more hearing effort on the QHS Effort subscale. QHS scores did not correlate significantly with audiological/hearing history measures but were significantly correlated with most speech perception, language and EF scores in the CI sample. In the NH sample, QHS scores were uncorrelated with speech perception and language and were inconsistently correlated with EF. CONCLUSIONS: The QHS is a valid measure of functional hearing quality that is distinct from office-based audiometric or hearing history measures. Functional hearing outcomes are associated with speech-language and EF outcomes in CI users.

Family-Level Executive Functioning and At-Risk Pediatric Hearing Loss Outcomes.

Purpose Using a new measure of family-level executive functioning (EF; the Family Characteristics Scale [FCS]), we investigated associations between family-level EF, spoken language, and neurocognitive skills in children with hearing loss (HL), compared to children with normal hearing. Method Parents of children with HL (n = 61) or children with normal hearing (n = 65) completed the FCS-Parent, and clinicians evaluated families using the FCS-Examiner. Children completed an age-appropriate version of the Concepts and Following Directions subtest of the Clinical Evaluation of Language Fundamentals and the Peabody Picture Vocabulary Test-Fourth Edition. Child EF was assessed via the parent report Behavior Rating Inventory of Executive Function. Results Two higher order components were derived from FCS subscales: Family Inhibition and Family Organization. For both samples, Family Inhibition was positively associated with child inhibition, child shifting, and child language comprehension skills. Family Organization was differentially associated with child inhibition, working memory, and planning/organization skills across the samples. Additionally, Family Inhibition was associated with child planning and organization skills for children with HL. Conclusions Results support the FCS as a measure of family-level EF. Family-level inhibition related to better child inhibition, flexibility/shifting, and language comprehension across both samples and to better planning and organization skills in children with HL. As children with HL experienced greater difficulties in EF, families demonstrated greater organization, possibly as a compensatory measure. Results suggest that inhibition and organization at a family level may be important targets for the development of novel interventions to promote EF and language outcomes for children with HL.

Word and Nonword Reading Efficiency in Postlingually Deafened Adult Cochlear Implant Users.

HYPOTHESIS: This study tested the hypotheses that 1) experienced adult cochlear implants (CI) users demonstrate poorer reading efficiency relative to normal-hearing controls, 2) reading efficiency reflects basic, underlying neurocognitive skills, and 3) reading efficiency relates to speech recognition outcomes in CI users. BACKGROUND: Weak phonological processing skills have been associated with poor speech recognition outcomes in postlingually deaf adult CI users. Phonological processing can be captured in nonauditory measures of reading efficiency, which may have wide use in patients with hearing loss. This study examined reading efficiency in adults CI users, and its relation to speech recognition outcomes. METHODS: Forty-eight experienced, postlingually deaf adult CI users (ECIs) and 43 older age-matched peers with age-normal hearing (ONHs) completed the Test of Word Reading Efficiency (TOWRE-2), which measures word and nonword reading efficiency. Participants also completed a battery of nonauditory neurocognitive measures and auditory sentence recognition tasks. RESULTS: ECIs and ONHs did not differ in word (ECIs: M = 78.2, SD = 11.4; ONHs: M = 83.3, SD = 10.2) or nonword reading efficiency (ECIs: M = 42.0, SD = 11.2; ONHs: M = 43.7, SD = 10.3). For ECIs, both scores were related to untimed word reading with moderate to strong effect sizes (r = 0.43-0.69), but demonstrated differing relations with other nonauditory neurocognitive measures with weak to moderate effect sizes (word: r = 0.11-0.44; nonword: r = (-)0.15 to (-)0.42). Word reading efficiency was moderately related to sentence recognition outcomes in ECIs (r = 0.36-0.40). CONCLUSION: Findings suggest that postlingually deaf adult CI users demonstrate neither impaired word nor nonword reading efficiency, and these measures reflect different underlying mechanisms involved in language processing. The relation between sentence recognition and word reading efficiency, a measure of lexical access speed, suggests that this measure may be useful for explaining outcome variability in adult CI users.