Systematically studying the swallowing and speech of children with UCP is new and noteworthy. We found that they demonstrate neuromuscular adaptations for swallowing and speech compared with typically developing peers. We examined swallowing and speech using carefully designed tasks, similar in motor complexity, which allowed us to directly compare patterns. We found shared and distinctive neuromuscular patterns between swallowing and speech.

Health-related quality of life (HRQoL) is difficult to measure in rare diseases, especially in pediatric populations, yet capturing HRQoL is critical for evaluating treatment, including the cost-effectiveness of treatments. Given the ultra-rare nature of AADC deficiency (AADC-d) indirect elicitation of HRQoL data through proxy caregiver/parent ratings is not feasible. In these circumstances HRQoL data may be derived through vignette studies using the general public. The study aim was to generate health utilities for AADC-d. The study was completed online by panel participants from a French representative sample. Five health state vignettes, reflecting key milestones in the eladocagene exuparvovec clinical trials and economic model were presented to the participants: bedridden, head control, sitting unsupported, standing with assistance and walking with assistance. Participants have then valued the vignettes through a revealing preferences method (standard gamble and time trade-off). The vignettes had been previously developed with input from parents of AADC-d patients and expert opinions. A total of 1001 participants (51% females; mean age 46 years) completed the study. Utilities increased linearly as the health state improved for both the time trade-off (TTO), and standard gamble (SG). A significant minority had incongruent responses (high utilities for the bedridden compared to walking health states): 27% for TTO and 34% for SG. When these were removed the mean TTO health utilities (n=729) were: 0.3891 (bedridden state), 0.4839 (head control), 0.5271 (sitting unsupported), 0.5293 (standing with assistance) and 0.5577 (walking with assistance). The SG utilities (n=664) ranged from 0.5534 for bedridden to 0.7093 for walking with assistance. Health utilities derived for AADC-d through vignettes are suitable for use as a cost-effectiveness model of an AADC-d treatment.

Purpose: Surface electromyography (sEMG) is often used for biofeedback during swallowing rehabilitation. However, commercially available sEMG electrodes are not optimized for the head and neck area, have rigid form, and are mostly available in large medical centers. We developed an ultrathin, soft, and flexible sEMG patch, specifically designed to conform to the submental anatomy and which will be ultimately incorporated into a telehealth system. To validate this first-generation sEMG patch, we compared its safety, efficiency, and signal quality in monitoring submental muscle activity with that of widely used conventional sEMG electrodes. Method: A randomized crossover design was used to compare the experimental sEMG patch with conventional (snap-on) sEMG electrodes. Participants completed the same experimental protocol with both electrodes in counterbalanced order. Swallow trials included five trials of 5- and 10-ml water. Comparisons were made on (a) signal-related factors: signal-to-noise ratio (SNR), baseline amplitude, normalized mean amplitude, and sEMG burst duration and (b) safety/preclinical factors: safety/adverse effects, efficiency of electrode placement, and satisfaction/comfort. Noninferiority and equivalence tests were used to examine signal-related factors. Paired t tests and descriptive statistics were used to examine safety/preclinical factors. Results: Forty healthy adults participated (24 women, M-age = 67.5 years). Signal-related factors: SNR of the experimental patch was not inferior to the SNR of the conventional electrodes (p < .0056). Similarly, baseline amplitude obtained with the experimental patch was not inferior to that obtained with conventional electrodes (p < .0001). Finally, normalized amplitude values were equivalent across swallows (5 ml: p < .025; 10 ml: p < .0012), and sEMG burst duration was also equivalent (5 ml: p < .0001; 10 ml: p < .0001). Safety/preclinical factors: The experimental patch resulted in fewer mild adverse effects. Participant satisfaction was higher with the experimental patch (p = .0476, d = 0.226). Conclusions: Our new wearable sEMG patch is equivalent with widely used conventional sEMG electrodes in terms of technical performance. In addition, our patch is safe, and healthy older adults are satisfied with it. With lessons learned from the current COVID-19 pandemic, efforts to develop optimal swallowing telerehabilitation devices are more urgent than ever. Upon further validation, this new technology has the potential to improve rehabilitation and telerehabilitation efforts for patients with dysphagia. Supplemental Material: https://doi.org/10.23641/asha.12915509

Background: In our Dynamic Pathways, account, we hypothesized that childhood stuttering reflects an impairment in speech sensorimotor control that is conditioned by cognitive, linguistic, and emotional factors. The purpose of this study was to investigate potential differences in levels of sympathetic arousal during performance of speech and non-speech tasks between children who do and do not stutter. Methods: Seventy-two preschool-aged children participated in the study, 47 children who stutter (CWS; 38 boys) and 25 children who do not stutter (CWNS; 18 boys). We recorded skin conductance and blood pulse volume (BPV) signals, indices of sympathetic arousal, during higher/lower load speech tasks (structured sentence production and picture description) and non-speech tasks (jaw wagging and forceful blowing). We included a measure that reflects children's attitudes about their communication skills and a parent-report assessment of temperament. Results: We found no significant differences between preschool CWS and CWNS in phasic skin conductance response amplitude or frequency, BPV, and pulse rate for any of the experimental tasks. However, compared to CWNS, CWS had, on average, significantly higher skin conductance levels (SCL), indexing slowly changing tonic sympathetic activity, across both speech and non-speech experimental conditions. We found distinctive task-related profiles of sympathetic arousal in both groups of preschool children. Most children produced the highest levels of sympathetic arousal in the physically demanding blowing task rather than in speech, as seen in previous studies of adults. We did not find differences in temperament between the two groups of preschool children nor a relationship among behavioral indices of temperament and communication attitude and physiological measures of sympathetic arousal. Conclusion: We did not find that atypically high, speech-related sympathetic arousal is a significant factor in early childhood stuttering. Rather, CWS had higher, on average, task-related tonic SCLs across speech and non-speech tasks. A relationship among behavioral measures of temperament and physiological measures of sympathetic arousal was not confirmed. Key questions for future experiments are how the typical coupling of sympathetic and speech sensorimotor systems develops over childhood and adolescence and whether task related developmental profiles follow a different course in children who continue to stutter.

Successful rehabilitation of oropharyngeal swallowing disorders (i.e., dysphagia) requires frequent performance of head/neck exercises that primarily rely on expensive biofeedback devices, often only available in large medical centers. This directly affects treatment compliance and outcomes, and highlights the need to develop a portable and inexpensive remote monitoring system for the telerehabilitation of dysphagia. Here, we present the development and preliminarily validation of a skin-mountable sensor patch that can fit on the curvature of the submental (under the chin) area noninvasively and provide simultaneous remote monitoring of muscle activity and laryngeal movement during swallowing tasks and maneuvers. This sensor patch incorporates an optimal design that allows for the accurate recording of submental muscle activity during swallowing and is characterized by ease of use, accessibility, reusability, and cost-effectiveness. Preliminary studies on a patient with Parkinson's disease and dysphagia, and on a healthy control participant demonstrate the feasibility and effectiveness of this system.

Over the past 10 years, we (the Purdue Stuttering Project) have implemented longitudinal studies to examine factors related to persistence and recovery in early childhood stuttering. Stuttering develops essentially as an impairment in speech sensorimotor processes that is strongly influenced by dynamic interactions among motor, language, and emotional domains. Our work has assessed physiological, behavioral, and clinical features of stuttering within the motor, linguistic, and emotional domains. We describe the results of studies in which measures collected when the child was 4 to 5 years old are related to eventual stuttering status. We provide supplemental evidence of the role of known predictive factors (e.g., sex and family history of persistent stuttering). In addition, we present new evidence that early delays in basic speech motor processes (especially in boys), poor performance on a nonword repetition test, stuttering severity at the age of 4 to 5 years, and delayed or atypical functioning in central nervous system language processing networks are predictive of persistent stuttering.

Purpose The purpose of this study was to determine if indices of speech motor coordination during the production of sentences varying in sentence length and syntactic complexity were associated with stuttering persistence versus recovery in 5- to 7-year-old children. Methods We compared children with persistent stuttering (CWS-Per) with children who had recovered (CWS-Rec), and children who do not stutter (CWNS). A kinematic measure of articulatory coordination, lip aperture variability (LAVar), and overall movement duration were computed for perceptually fluent sentence productions varying in length and syntactic complexity. Results CWS-Per exhibited higher LAVar across sentence types compared to CWS-Rec and CWNS. For the participants who successfully completed the experimental paradigm, the demands of increasing sentence length and syntactic complexity did not appear to disproportionately affect the speech motor coordination of CWS-Per compared to their recovered and fluent peers. However, a subset of CWS-Per failed to produce the required number of accurate utterances. Conclusions These findings support our hypothesis that the speech motor coordination of school-age CWS-Per, on average, is less refined and less mature compared to CWS-Rec and CWNS. Childhood recovery from stuttering is characterized, in part, by overcoming an earlier occurring maturational lag in speech motor development.

Purpose:We advanced a multifactorial, dynamic account of the complex, nonlinear interactions of motor, linguistic, and emotional factors contributing to the development of stuttering. Our purpose here is to update our account as the multifactorial dynamic pathways theory.Method:We review evidence related to how stuttering develops, including genetic/epigenetic factors; motor, linguistic, and emotional features; and advances in neuroimaging studies. We update evidence for our earlier claim: Although stuttering ultimately reflects impairment in speech sensorimotor processes, its course over the life span is strongly conditioned by linguistic and emotional factors.Results:Our current account places primary emphasis on the dynamic developmental context in which stuttering emerges and follows its course during the preschool years. Rapid changes in many neurobehavioral systems are ongoing, and critical interactions among these systems likely play a major role in determining persistence of or recovery from stuttering.Conclusion:Stuttering, or childhood onset fluency disorder (Diagnostic and Statistical Manual of Mental Disorders, 5th edition; American Psychiatric Association [APA], 2013), is a neurodevelopmental disorder that begins when neural networks supporting speech, language, and emotional functions are rapidly developing. The multifactorial dynamic pathways theory motivates experimental and clinical work to determine the specific factors that contribute to each child's pathway to the diagnosis of stuttering and those most likely to promote recovery.

Remarkable progress has been made over the past two decades in expanding our understanding of the behavioral, peripheral physiologic, and central neurophysiologic bases of stuttering in early childhood. It is clear that stuttering is a neurodevelopmental disorder characterized by atypical development of speech motor planning and execution networks. The speech motor system must interact in complex ways with neural systems mediating language and other cognitive and emotional processes. During the time when stuttering typically appears and follows its path to either recovery or persistence, all of these neurobehavioral systems are undergoing rapid and dramatic developmental changes. We summarize our current understanding of the various developmental trajectories relevant for the understanding of stuttering in early childhood. We also present theoretical and experimental approaches that we believe will be optimal for even more rapid progress toward developing better and more targeted treatment for stuttering in the preschool children who are more likely to persist in stuttering.

PURPOSE Stuttering involves a breakdown in the speech motor system. We address whether stuttering in its early stage is specific to the speech motor system or whether its impact is observable across motor systems. METHOD As an extension of Olander, Smith, and Zelaznik (2010), we measured bimanual motor timing performance in 115 children: 70 children who stutter (CWS) and 45 children who do not stutter (CWNS). The children repeated the clapping task yearly for up to 5 years. We used a synchronization-continuation rhythmic timing paradigm. Two analyses were completed: a cross-sectional analysis of data from the children in the initial year of the study (ages 4;0 [years;months] to 5;11) compared clapping performance between CWS and CWNS. A second, multiyear analysis assessed clapping behavior across the ages 3;5-9;5 to examine any potential relationship between clapping performance and eventual persistence or recovery of stuttering. RESULTS Preschool CWS were not different from CWNS on rates of clapping or variability in interclap interval. In addition, no relationship was found between bimanual motor timing performance and eventual persistence in or recovery from stuttering. The disparity between the present findings for preschoolers and those of Olander et al. (2010) most likely arises from the smaller sample size used in the earlier study. CONCLUSION From the current findings, on the basis of data from relatively large samples of stuttering and nonstuttering children tested over multiple years, we conclude that a bimanual motor timing deficit is not a core feature of early developmental stuttering.

BACKGROUND:Five to eight percent of preschool children develop stuttering, a speech disorder with clearly observable, hallmark symptoms: sound repetitions, prolongations, and blocks. While the speech motor processes underlying stuttering have been widely documented in adults, few studies to date have assessed the speech motor dynamics of stuttering near its onset. We assessed fundamental characteristics of speech movements in preschool children who stutter and their fluent peers to determine if atypical speech motor characteristics described for adults are early features of the disorder or arise later in the development of chronic stuttering.METHODS:Orofacial movement data were recorded from 58 children who stutter and 43 children who do not stutter aged 4;0 to 5;11 (years; months) in a sentence production task. For single speech movements and multiple speech movement sequences, we computed displacement amplitude, velocity, and duration. For the phrase level movement sequence, we computed an index of articulation coordination consistency for repeated productions of the sentence.RESULTS:Boys who stutter, but not girls, produced speech with reduced amplitudes and velocities of articulatory movement. All children produced speech with similar durations. Boys, particularly the boys who stuttered, had more variable patterns of articulatory coordination compared to girls.CONCLUSIONS:This study is the first to demonstrate sex-specific differences in speech motor control processes between preschool boys and girls who are stuttering. The sex-specific lag in speech motor development in many boys who stutter likely has significant implications for the dramatically different recovery rates between male and female preschoolers who stutter. Further, our findings document that atypical speech motor development is an early feature of stuttering.

Because they present with uneven cognitive profiles, neurodevelopmental disorders have often been characterized in terms of intact vs impaired modules. The availability of eye-tracking techniques as well as a variety of neuroimaging technologies enabling researchers to examine the spatial and temporal dynamics of brain activity, has challenged such dichotomies, suggesting that even when participants achieve “scores in the normal range”, this does not necessarily indicate intactness, because the behavioural scores can be shown to be underpinned by atypical neural processes. This holds for a wide range of cognitive domains (e.g., face processing, language, number, attention, inhibition, memory) and across a wide range of neurodevelopmental disorders (e.g., Williams syndrome, Down syndrome, Autism Spectrum Disorders, Foetal Alcohol syndrome, Obstructive Sleep Apnea), as well as low/high SES.

Purpose To assess autonomic arousal associated with speech and nonspeech tasks in school-age children and young adults. Method Measures of autonomic arousal (electrodermal level, electrodermal response amplitude, blood pulse volume, and heart rate) were recorded prior to, during, and after the performance of speech and nonspeech tasks by twenty 7- to 9-year-old children and twenty 18- to 22-year-old adults. Results Across age groups, autonomic arousal was higher for speech tasks compared with nonspeech tasks, based on peak electrodermal response amplitude and blood pulse volume. Children demonstrated greater relative arousal, based on heart rate and blood pulse volume, for nonspeech oral motor tasks than adults but showed similar mean arousal levels for speech tasks as adults. Children demonstrated sex differences in autonomic arousal; specifically, autonomic arousal remained high for school-age boys but not girls in a more complex open-ended narrative task that followed a simple sentence production task. Conclusions Speech tasks elicit greater autonomic arousal than nonspeech tasks, and children demonstrate greater autonomic arousal for nonspeech oral motor tasks than adults. Sex differences in autonomic arousal associated with speech tasks in school-age children are discussed relative to speech-language differences between boys and girls.

Purpose The study was aimed at characterizing age-related changes in speech motor performance on a nonword repetition task as a function of practice and nonword length and complexity. Method Nonword repetition accuracy, lip aperture coordination, and nonword production durations were assessed on 2 consecutive days for 16 young and 16 elderly participants for the production of 6 novel nonwords increasing in length and complexity. Results The effect of age on the ability to accurately and rapidly repeat long, complex nonwords was significant. However, the authors found no differences between the speech motor coordinative patterns of young and elderly adults. Further, the authors demonstrated age- and nonword-specific within- and between-session gains in speech motor performance. Conclusions The authors speculate that cognitive, sensory, and motor factors interact in complex ways in elderly individuals to produce individual differences in nonword repetition ability at the levels of both behavioral and speech motor performance.

Purpose To investigate the potential effects of increased sentence length and syntactic complexity on the speech motor control of children who stutter (CWS). Method Participants repeated sentences of varied length and syntactic complexity. Kinematic measures of articulatory coordination variability and movement duration during perceptually fluent speech were analyzed for 16 CWS and 16 typically developing children (CTD) between 4 and 6 years of age. Behavioral data from a larger pool of children were also examined. Results For both groups, articulatory coordination variability increased with sentence length. For syntactically simple sentences, CWS had higher coordination variability than CTD. There was no group difference in coordination variability for complex sentences. Coordination variability increased significantly with complexity for CTD, whereas that of CWS remained at the high level demonstrated for simple sentences. There was a trend for higher overall coordination variability in CWS compared with CTD. For both groups, movement duration was greater for syntactically complex, as compared with simple, sentences. Conclusions Results indicate more variable speech motor coordination during fluent speech production in many CWS as compared with CTD. Disproportionate effects of length and complexity on coordination variability and duration were not found for CWS. Considerable individual differences in performance were observed.