Psychometrics and applications of a novel self-report measure of emicizumab adherence: VERITASNexGen.

INTRODUCTION: Treatment adherence is critical to minimize bleeding episodes in persons with haemophilia. Suboptimal adherence increases risk of adverse medical outcomes and negatively impacts quality of life. Assessment of treatment adherence is therefore an integral component of intervention to mitigate the adverse impacts of haemophilia. AIM: To develop and validate a multifactorial, patient (self or caregiver) report adherence measure for emicizumab treatment and report the first patient-report data on adherence to specific components of emicizumab treatment (dosing, timing, injection, planning and bleeds). METHODS: An IRB approved multi-site prospective study enrolled 83 participants with factor VIII deficiency being treated with emicizumab. Participants completed the 25-item VERITAS NexGen (self-report from 50 adults age 18+ years; caregiver-report from 33 parents of children aged 6 months to 17 years) as well as a global adherence rating (GAR) scale. Providers of participants also completed a GAR scale. RESULTS: Most VERITAS-NexGen subscales had good-to-excellent internal consistency reliability, test-retest reliability, and validity. VERITASNexGen scores revealed globally strong patient-reported adherence; however timing and bleed management were reported as greater challenges to adherence compared to dosing and injections. Adults struggled more with timing and planning of injections than caregivers. CONCLUSION: The VERITASNexGen is the first validated multifactorial patient-report measure of adherence designed specifically for emicizumab treatment. Results suggest excellent adherence, with only 4%-13% of participants reporting suboptimal adherence to different components of the treatment regimen. Used in conjunction with other adherence measures, VERITAS-NexGen meets a crucial need for monitoring and understanding patient adherence to emicizumab in clinical and research settings.

Novel Approach to Integrating Mental Health Care into a Primary Care Setting: Development, Implementation, and Outcomes.

It is now widely accepted that there is a growing discrepancy between demand and access to adequate treatment for behavioral or mental health conditions in the United States. This results in immense personal, societal, and economic costs. One rapidly growing method of addressing this discrepancy is to integrate mental health services into the primary care setting, which has become the de facto service provider for these conditions. In this paper, we describe the development and implementation of a novel integrated care program in a large mid-western university-based healthcare system, drawn from the collaborative care model, and describe the benefits in terms of both health care utilization and depression outcomes. Limitations and proposed future directions are discussed.

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.

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 Environment in Children With Hearing Aids and Cochlear Implants: Associations With Spoken Language, Psychosocial Functioning, and Cognitive Development.

OBJECTIVES: To examine differences in family environment and associations between family environment and key speech, language, and cognitive outcomes in samples of children with normal hearing and deaf and hard-of-hearing (DHH) children who use hearing aids and cochlear implants. DESIGN: Thirty families of children with normal hearing (n = 10), hearing aids (n = 10), or cochlear implants (n = 10) completed questionnaires evaluating executive function, social skills, and problem behaviors. Children's language and receptive vocabulary were evaluated using standardized measures in the children's homes. In addition, families were administered a standardized in-home questionnaire and observational assessment regarding the home environment. RESULTS: Family environment overall was similar across hearing level and sensory aid, although some differences were found on parental responsivity and physical environment. The level of supportiveness and enrichment within family relationships accounted for much of the relations between family environment and the psychosocial and neurocognitive development of DHH children. In contrast, the availability of objects and experiences to stimulate learning in the home was related to the development of spoken language. CONCLUSIONS: Whereas broad characteristics of the family environments of DHH children may not differ from those of hearing children, variability in family functioning is related to DHH children's at-risk speech, language, and cognitive outcomes. Results support the importance of further research to clarify and explain these relations, which might suggest novel methods and targets of family-based interventions to improve developmental outcomes.

High-Variability Sentence Recognition in Long-Term Cochlear Implant Users: Associations With Rapid Phonological Coding and Executive Functioning.

OBJECTIVES: The objective of the present study was to determine whether long-term cochlear implant (CI) users would show greater variability in rapid phonological coding skills and greater reliance on slow-effortful compensatory executive functioning (EF) skills than normal-hearing (NH) peers on perceptually challenging high-variability sentence recognition tasks. We tested the following three hypotheses: First, CI users would show lower scores on sentence recognition tests involving high speaker and dialect variability than NH controls, even after adjusting for poorer sentence recognition performance by CI users on a conventional low-variability sentence recognition test. Second, variability in fast-automatic rapid phonological coding skills would be more strongly associated with performance on high-variability sentence recognition tasks for CI users than NH peers. Third, compensatory EF strategies would be more strongly associated with performance on high-variability sentence recognition tasks for CI users than NH peers. DESIGN: Two groups of children, adolescents, and young adults aged 9 to 29 years participated in this cross-sectional study: 49 long-term CI users (≥7 years) and 56 NH controls. All participants were tested on measures of rapid phonological coding (Children's Test of Nonword Repetition), conventional sentence recognition (Harvard Sentence Recognition Test), and two novel high-variability sentence recognition tests that varied the indexical attributes of speech (Perceptually Robust English Sentence Test Open-set test and Perceptually Robust English Sentence Test Open-set test-Foreign Accented English test). Measures of EF included verbal working memory (WM), spatial WM, controlled cognitive fluency, and inhibition concentration. RESULTS: CI users scored lower than NH peers on both tests of high-variability sentence recognition even after conventional sentence recognition skills were statistically controlled. Correlations between rapid phonological coding and high-variability sentence recognition scores were stronger for the CI sample than for the NH sample even after basic sentence perception skills were statistically controlled. Scatterplots revealed different ranges and slopes for the relationship between rapid phonological coding skills and high-variability sentence recognition performance in CI users and NH peers. Although no statistically significant correlations between EF strategies and sentence recognition were found in the CI or NH sample after use of a conservative Bonferroni-type correction, medium to high effect sizes for correlations between verbal WM and sentence recognition in the CI sample suggest that further investigation of this relationship is needed. CONCLUSIONS: These findings provide converging support for neurocognitive models that propose two channels for speech-language processing: a fast-automatic channel that predominates whenever possible and a compensatory slow-effortful processing channel that is activated during perceptually-challenging speech processing tasks that are not fully managed by the fast-automatic channel (ease of language understanding, framework for understanding effortful listening, and auditory neurocognitive model). CI users showed significantly poorer performance on measures of high-variability sentence recognition than NH peers, even after simple sentence recognition was controlled. Nonword repetition scores showed almost no overlap between CI and NH samples, and correlations between nonword repetition scores and high-variability sentence recognition were consistent with greater reliance on engagement of fast-automatic phonological coding for high-variability sentence recognition in the CI sample than in the NH sample. Further investigation of the verbal WM-sentence recognition relationship in CI users is recommended. Assessment of fast-automatic phonological processing and slow-effortful EF skills may provide a better understanding of speech perception outcomes in CI users in the clinical setting.

Psychosocial Outcomes in Long-Term Cochlear Implant Users.

OBJECTIVES: The objectives of this study were to investigate psychosocial outcomes in a sample of prelingually deaf, early-implanted children, adolescents, and young adults who are long-term cochlear implant (CI) users and to examine the extent to which language and executive functioning predict psychosocial outcomes. DESIGN: Psychosocial outcomes were measured using two well-validated, parent-completed checklists: the Behavior Assessment System for Children and the Conduct Hyperactive Attention Problem Oppositional Symptom. Neurocognitive skills were measured using gold standard, performance-based assessments of language and executive functioning. RESULTS: CI users were at greater risk for clinically significant deficits in areas related to attention, oppositional behavior, hyperactivity-impulsivity, and social-adaptive skills compared with their normal-hearing peers, although the majority of CI users scored within average ranges relative to Behavior Assessment System for Children norms. Regression analyses revealed that language, visual-spatial working memory, and inhibition-concentration skills predicted psychosocial outcomes. CONCLUSIONS: Findings suggest that underlying delays and deficits in language and executive functioning may place some CI users at a risk for difficulties in psychosocial adjustment.

Verbal Learning and Memory After Cochlear Implantation in Postlingually Deaf Adults: Some New Findings with the CVLT-II.

OBJECTIVES: Despite the importance of verbal learning and memory in speech and language processing, this domain of cognitive functioning has been virtually ignored in clinical studies of hearing loss and cochlear implants in both adults and children. In this article, we report the results of two studies that used a newly developed visually based version of the California Verbal Learning Test-Second Edition (CVLT-II), a well-known normed neuropsychological measure of verbal learning and memory. DESIGN: The first study established the validity and feasibility of a computer-controlled visual version of the CVLT-II, which eliminates the effects of audibility of spoken stimuli, in groups of young normal-hearing and older normal-hearing (ONH) adults. A second study was then carried out using the visual CVLT-II format with a group of older postlingually deaf experienced cochlear implant (ECI) users (N = 25) and a group of ONH controls (N = 25) who were matched to ECI users for age, socioeconomic status, and nonverbal IQ. In addition to the visual CVLT-II, subjects provided data on demographics, hearing history, nonverbal IQ, reading fluency, vocabulary, and short-term memory span for visually presented digits. ECI participants were also tested for speech recognition in quiet. RESULTS: The ECI and ONH groups did not differ on most measures of verbal learning and memory obtained with the visual CVLT-II, but deficits were identified in ECI participants that were related to recency recall, the buildup of proactive interference, and retrieval-induced forgetting. Within the ECI group, nonverbal fluid IQ, reading fluency, and resistance to the buildup of proactive interference from the CVLT-II consistently predicted better speech recognition outcomes. CONCLUSIONS: Results from this study suggest that several underlying foundational neurocognitive abilities are related to core speech perception outcomes after implantation in older adults. Implications of these findings for explaining individual differences and variability and predicting speech recognition outcomes after implantation are discussed.

Verbal learning and memory in prelingually deaf children with cochlear implants.

OBJECTIVE: Deaf children with cochlear implants (CIs) show poorer verbal working memory compared to normal-hearing (NH) peers, but little is known about their verbal learning and memory (VLM) processes involving multi-trial free recall. DESIGN: Children with CIs were compared to NH peers using the California Verbal Learning Test for Children (CVLT-C). STUDY SAMPLE: Participants were 21 deaf (before age 6 months) children (6-16 years old) implanted prior to age 3 years, and 21 age-IQ matched NH peers. RESULTS: Results revealed no differences between groups in number of words recalled. However, CI users showed a pattern of increasing use of serial clustering strategies across learning trials, whereas NH peers decreased their use of serial clustering strategies. In the CI sample (but not in the NH sample), verbal working memory test scores were related to resistance to the build-up of proactive interference, and sentence recognition was associated with performance on the first exposure to the word list and to the use of recency recall strategies. CONCLUSIONS: Children with CIs showed robust evidence of VLM comparable to NH peers. However, their VLM processing (especially recency and proactive interference) was related to speech perception outcomes and verbal WM in different ways from NH peers.

Relations of Social Maturity, Executive Function, and Self-Efficacy Among Deaf University Students.

This study explored possible associations of social maturity, executive function (EF), self-efficacy, and communication variables among deaf university students, both cochlear implant (CI) users and nonusers. Previous studies have demonstrated differences between deaf and hearing children and young adults in EF and EF-related social and cognitive functioning. EF differences also have been demonstrated between hearing children and deaf children who use CIs. Long-term influences of cochlear implantation in the social domain largely have not been explored, but were examined in the present study in terms of social maturity, as it might be related to EF and communication variables. Replicating and extending recent findings, social maturity was found to be related to somewhat different aspects of EF in CI users, deaf nonusers, and hearing students, but unrelated to hearing status, CI use, or deaf students' use of sign language versus spoken language. Self-efficacy proved a predictor of self-reported socially mature and immature behaviours for all groups. Individuals' beliefs about their parents' views of such behaviours was a potent predictor of behaviours for deaf CI users and those deaf students who reported sign language as their best form of communication.

Some Neurocognitive Correlates of Noise-Vocoded Speech Perception in Children With Normal Hearing: A Replication and Extension of ).

OBJECTIVES: Noise-vocoded speech is a valuable research tool for testing experimental hypotheses about the effects of spectral degradation on speech recognition in adults with normal hearing (NH). However, very little research has utilized noise-vocoded speech with children with NH. Earlier studies with children with NH focused primarily on the amount of spectral information needed for speech recognition without assessing the contribution of neurocognitive processes to speech perception and spoken word recognition. In this study, we first replicated the seminal findings reported by ) who investigated effects of lexical density and word frequency on noise-vocoded speech perception in a small group of children with NH. We then extended the research to investigate relations between noise-vocoded speech recognition abilities and five neurocognitive measures: auditory attention (AA) and response set, talker discrimination, and verbal and nonverbal short-term working memory. DESIGN: Thirty-one children with NH between 5 and 13 years of age were assessed on their ability to perceive lexically controlled words in isolation and in sentences that were noise-vocoded to four spectral channels. Children were also administered vocabulary assessments (Peabody Picture Vocabulary test-4th Edition and Expressive Vocabulary test-2nd Edition) and measures of AA (NEPSY AA and response set and a talker discrimination task) and short-term memory (visual digit and symbol spans). RESULTS: Consistent with the findings reported in the original ) study, we found that children perceived noise-vocoded lexically easy words better than lexically hard words. Words in sentences were also recognized better than the same words presented in isolation. No significant correlations were observed between noise-vocoded speech recognition scores and the Peabody Picture Vocabulary test-4th Edition using language quotients to control for age effects. However, children who scored higher on the Expressive Vocabulary test-2nd Edition recognized lexically easy words better than lexically hard words in sentences. Older children perceived noise-vocoded speech better than younger children. Finally, we found that measures of AA and short-term memory capacity were significantly correlated with a child's ability to perceive noise-vocoded isolated words and sentences. CONCLUSIONS: First, we successfully replicated the major findings from the ) study. Because familiarity, phonological distinctiveness and lexical competition affect word recognition, these findings provide additional support for the proposal that several foundational elementary neurocognitive processes underlie the perception of spectrally degraded speech. Second, we found strong and significant correlations between performance on neurocognitive measures and children's ability to recognize words and sentences noise-vocoded to four spectral channels. These findings extend earlier research suggesting that perception of spectrally degraded speech reflects early peripheral auditory processes, as well as additional contributions of executive function, specifically, selective attention and short-term memory processes in spoken word recognition. The present findings suggest that AA and short-term memory support robust spoken word recognition in children with NH even under compromised and challenging listening conditions. These results are relevant to research carried out with listeners who have hearing loss, because they are routinely required to encode, process, and understand spectrally degraded acoustic signals.

Speech Intelligibility and Psychosocial Functioning in Deaf Children and Teens with Cochlear Implants.

Deaf children with cochlear implants (CIs) are at risk for psychosocial adjustment problems, possibly due to delayed speech-language skills. This study investigated associations between a core component of spoken-language ability-speech intelligibility-and the psychosocial development of prelingually deaf CI users. Audio-transcription measures of speech intelligibility and parent reports of psychosocial behaviors were obtained for two age groups (preschool, school-age/teen). CI users in both age groups scored more poorly than typically hearing peers on speech intelligibility and several psychosocial scales. Among preschool CI users, five scales were correlated with speech intelligibility: functional communication, attention problems, atypicality, withdrawal, and adaptability. These scales and four additional scales were correlated with speech intelligibility among school-age/teen CI users: leadership, activities of daily living, anxiety, and depression. Results suggest that speech intelligibility may be an important contributing factor underlying several domains of psychosocial functioning in children and teens with CIs, particularly involving socialization, communication, and emotional adjustment.

Social Maturity and Executive Function Among Deaf Learners.

Two experiments examined relations among social maturity, executive function, language, and cochlear implant (CI) use among deaf high school and college students. Experiment 1 revealed no differences between deaf CI users, deaf nonusers, and hearing college students in measures of social maturity. However, deaf students (both CI users and nonusers) reported significantly greater executive function (EF) difficulties in several domains, and EF was related to social maturity. Experiment 2 found that deaf CI users and nonusers in high school did not differ from each other in social maturity or EF, but individuals who relied on sign language reported significantly more immature behaviors than deaf peers who used spoken language. EF difficulties again were associated with social maturity. The present results indicate that EF and social maturity are interrelated, but those relations vary in different deaf subpopulations. As with academic achievement, CI use appears to have little long-term impact on EF or social maturity. Results are discussed in terms of their convergence with findings related to incidental learning and functioning in several domains.

Long-term musical experience and auditory and visual perceptual abilities under adverse conditions.

Musicians have been shown to have enhanced speech perception in noise skills. It is unclear whether these improvements are limited to the auditory modality, as no research has examined musicians' visual perceptual abilities under degraded conditions. The current study examined associations between long-term musical experience and visual perception under noisy or degraded conditions. The performance of 11 musicians and 11 age-matched nonmusicians was compared on several auditory and visual perceptions in noise measures. Auditory perception tests included speech-in-noise tests and an environmental sound in noise test. Visual perception tasks included a fragmented sentences task, an object recognition task, and a lip-reading measure. Participants' vocabulary knowledge and nonverbal reasoning abilities were also assessed. Musicians outperformed nonmusicians on the speech perception in noise measures as well as the visual fragmented sentences task. Musicians also displayed better vocabulary knowledge in comparison to nonmusicians. Associations were found between perception of speech and visually degraded text. The findings show that long-term musical experience is associated with modality-general improvements in perceptual abilities. Possible systems supporting musicians' perceptual abilities are discussed.

Short-Term and Working Memory Impairments in Early-Implanted, Long-Term Cochlear Implant Users Are Independent of Audibility and Speech Production.

OBJECTIVE: To determine whether early-implanted, long-term cochlear implant (CI) users display delays in verbal short-term and working memory capacity when processes related to audibility and speech production are eliminated. DESIGN: Twenty-three long-term CI users and 23 normal-hearing controls each completed forward and backward digit span tasks under testing conditions that differed in presentation modality (auditory or visual) and response output (spoken recall or manual pointing). RESULTS: Normal-hearing controls reproduced more lists of digits than the CI users, even when the test items were presented visually and the responses were made manually via touchscreen response. CONCLUSIONS: Short-term and working memory delays observed in CI users are not due to greater demands from peripheral sensory processes such as audibility or from overt speech-motor planning and response output organization. Instead, CI users are less efficient at encoding and maintaining phonological representations in verbal short-term memory using phonological and linguistic strategies during memory tasks.

Understanding Language, Hearing Status, and Visual-Spatial Skills.

It is frequently assumed that deaf individuals have superior visual-spatial abilities relative to hearing peers and thus, in educational settings, they are often considered visual learners. There is some empirical evidence to support the former assumption, although it is inconsistent, and apparently none to support the latter. Three experiments examined visual-spatial and related cognitive abilities among deaf individuals who varied in their preferred language modality and use of cochlear implants (CIs) and hearing individuals who varied in their sign language skills. Sign language and spoken language assessments accompanied tasks involving visual-spatial processing, working memory, nonverbal logical reasoning, and executive function. Results were consistent with other recent studies indicating no generalized visual-spatial advantage for deaf individuals and suggested that their performance in that domain may be linked to the strength of their preferred language skills regardless of modality. Hearing individuals performed more strongly than deaf individuals on several visual-spatial and self-reported executive functioning measures, regardless of sign language skills or use of CIs. Findings are inconsistent with assumptions that deaf individuals are visual learners or are superior to hearing individuals across a broad range of visual-spatial tasks. Further, performance of deaf and hearing individuals on the same visual-spatial tasks was associated with differing cognitive abilities, suggesting that different cognitive processes may be involved in visual-spatial processing in these groups.

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.

Association of television violence exposure with executive functioning and white matter volume in young adult males.

Prior research has indicated that self-reported violent media exposure is associated with poorer performance on some neuropsychological tests in adolescents. This study aimed to examine the relationship of executive functioning to violent television viewing in healthy young adult males and examine how brain structure is associated with media exposure measures. Sixty-five healthy adult males (ages 18-29) with minimal video game experience estimated their television viewing habits over the past year and, during the subsequent week, recorded television viewing time and characteristics in a daily media diary. Participants then completed a battery of neuropsychological laboratory tests quantifying executive functions and underwent a magnetic resonance imaging (MRI) scan. Aggregate measures of executive functioning were not associated with measures of overall television viewing (any content type) during the past week or year. However, the amount of television viewing of violent content only, as indicated by both past-year and daily diary measures, was associated with poorer scores on an aggregate score of inhibition, interference control and attention, with no relationship to a composite working memory score. In addition, violent television exposure, as measured with daily media diaries, was associated with reduced frontoparietal white matter volume. Future longitudinal work is necessary to resolve whether individuals with poor executive function and slower white matter growth are more drawn to violent programming, or if extensive media violence exposure modifies cognitive control mechanisms mediated primarily via prefrontal cortex. Impaired inhibitory mechanisms may be related to reported increases in aggression with higher media violence exposure.