Children with childhood apraxia of speech (CAS) are likely to experience severe and persistent spoken and written language disorder. There is a scarcity of intervention research, however, investigating techniques to improve the speech and literacy outcomes of this population. The series of 5 experiments reported in this thesis investigated phonological awareness and early reading development in children with CAS and trialled a new intervention designed to advance the spoken and written language development of those affected. In the first experiment (presented in Chapter 2), a comparison of 12 children with CAS, 12 children with inconsistent speech disorder (ISD), and 12 children with typical speech-language development (TD) revealed that children with CAS may be particularly susceptible to phonological awareness and reading deficits. There was no difference in the articulatory consistency and speech severity of the CAS and ISD groups, and no difference in the receptive vocabulary of the CAS, ISD, and TD groups. The children with CAS exhibited poorer phonological awareness scores than the comparison groups and had a greater percentage of participants performing below the expected range for their age on letter knowledge, real word decoding, and phonological awareness normative measures. The children with CAS and ISD performed inferiorly than the children with TD on a receptive phonological representation task. The results showed that the children with CAS had a representational component to their disorder that needed to be addressed in intervention. In the second experiment (presented in Chapter 3), a follow-up pilot study was conducted to examine the long-term effects of a previously conducted intensive integrated phonological awareness programme (7 hours of intervention over 3 weeks) on 2 children with CAS. The children aged 7;3 and 8;3 at follow-up assessment had previously responded positively to the intervention. Results showed that the children were able to maintain their high accuracy in targeted speech repeated measures over the follow-up period. One child was also able to maintain her high accuracy in phonological awareness repeated measures. The children performed superiorly on a standardised phonological awareness measure at follow-up than at pre-intervention. Non-word reading ability showed a sharp increase during the intervention period, while minimal gains were made in this measure over the follow-up period. The findings suggested that an integrated intervention was a potential therapeutic approach for children with CAS. In the third experiment (presented in Chapter 4), the effectiveness of an integrated phonological awareness programme was evaluated for the 12 children (identified in the first experiment) aged 4 to 7 years with CAS. A controlled multiple single-subject design with repeated measures was employed to analyse change in trained and untrained speech and phoneme segmentation targets. A comparative group design was used to evaluate the phonological awareness, reading, and spelling development of the children with CAS compared to their peers with TD over the intervention. The children participated in two 6- week intervention blocks (2-sessions per week) separated by a 6-week withdrawal block. Seven children with CAS made significant gains in their production of trained and untrained speech words with 7 of these children demonstrating transfer of skills to connected speech for at least one target. Ten children showed significant gains in phoneme awareness, and 8 of these children demonstrated transfer of skills to novel phoneme awareness tasks. As a group, the children with CAS demonstrated accelerated development over the intervention period in letter knowledge, phonological awareness, word decoding, and spelling ability compared to their peers with typical development. In the fourth experiment (presented in Chapter 5), the speech, phonological awareness, reading, and spelling skills of children with CAS and TD were re-evaluated 6- months following completion of the intervention programme. A measure of reading accuracy and reading comprehension in a text reading task was administered to the children with CAS. There was no difference in the performance of the children with CAS in post-intervention and follow-up assessments. The children with CAS and children with TD presented with similar relative change in phonological awareness, reading, and decoding measures over the follow-up period. The connected reading performance of children with CAS mirrored their phonological awareness and decoding skills. The findings demonstrated that children with CAS were able to maintain gains achieved during the intervention but may need further support to promote sustained development in written language. In the fifth experiment (presented in Chapter 6), the long-term effects of the integrated phonological awareness programme for identical twin boys who participated in the research intervention at pre-school were examined. The study examined Theo and Jamie's spoken language, phonological awareness, reading, and spelling development during their first year of schooling. The results pointed to the benefit of providing phonological awareness within a preventative framework for children with CAS. Theo and Jamie experienced continued growth in speech and phonological awareness skills. They exhibited age-appropriate reading and spelling development during their first year of formal literacy instruction. It was concluded from this series of experiments that children with CAS are particularly vulnerable to phonological awareness and early reading difficulty, and that an integrated phonological awareness intervention is an effective means of developing speech, phonological awareness, reading, and spelling skills in most children with CAS. The intervention appears to target processes underlying spoken and written language development in this population. The results are discussed within a phonological representation deficit hypothesis of CAS and clinical implications of the findings are highlighted.

No description available.

Children with childhood apraxia of speech (CAS) are likely to experience severe and persistent spoken and written language disorder. There is a scarcity of intervention research, however, investigating techniques to improve the speech and literacy outcomes of this population. The series of 5 experiments reported in this thesis investigated phonological awareness and early reading development in children with CAS and trialled a new intervention designed to advance the spoken and written language development of those affected. In the first experiment (presented in Chapter 2), a comparison of 12 children with CAS, 12 children with inconsistent speech disorder (ISD), and 12 children with typical speech-language development (TD) revealed that children with CAS may be particularly susceptible to phonological awareness and reading deficits. There was no difference in the articulatory consistency and speech severity of the CAS and ISD groups, and no difference in the receptive vocabulary of the CAS, ISD, and TD groups. The children with CAS exhibited poorer phonological awareness scores than the comparison groups and had a greater percentage of participants performing below the expected range for their age on letter knowledge, real word decoding, and phonological awareness normative measures. The children with CAS and ISD performed inferiorly than the children with TD on a receptive phonological representation task. The results showed that the children with CAS had a representational component to their disorder that needed to be addressed in intervention. In the second experiment (presented in Chapter 3), a follow-up pilot study was conducted to examine the long-term effects of a previously conducted intensive integrated phonological awareness programme (7 hours of intervention over 3 weeks) on 2 children with CAS. The children aged 7;3 and 8;3 at follow-up assessment had previously responded positively to the intervention. Results showed that the children were able to maintain their high accuracy in targeted speech repeated measures over the follow-up period. One child was also able to maintain her high accuracy in phonological awareness repeated measures. The children performed superiorly on a standardised phonological awareness measure at follow-up than at pre-intervention. Non-word reading ability showed a sharp increase during the intervention period, while minimal gains were made in this measure over the follow-up period. The findings suggested that an integrated intervention was a potential therapeutic approach for children with CAS. In the third experiment (presented in Chapter 4), the effectiveness of an integrated phonological awareness programme was evaluated for the 12 children (identified in the first experiment) aged 4 to 7 years with CAS. A controlled multiple single-subject design with repeated measures was employed to analyse change in trained and untrained speech and phoneme segmentation targets. A comparative group design was used to evaluate the phonological awareness, reading, and spelling development of the children with CAS compared to their peers with TD over the intervention. The children participated in two 6- week intervention blocks (2-sessions per week) separated by a 6-week withdrawal block. Seven children with CAS made significant gains in their production of trained and untrained speech words with 7 of these children demonstrating transfer of skills to connected speech for at least one target. Ten children showed significant gains in phoneme awareness, and 8 of these children demonstrated transfer of skills to novel phoneme awareness tasks. As a group, the children with CAS demonstrated accelerated development over the intervention period in letter knowledge, phonological awareness, word decoding, and spelling ability compared to their peers with typical development. In the fourth experiment (presented in Chapter 5), the speech, phonological awareness, reading, and spelling skills of children with CAS and TD were re-evaluated 6- months following completion of the intervention programme. A measure of reading accuracy and reading comprehension in a text reading task was administered to the children with CAS. There was no difference in the performance of the children with CAS in post-intervention and follow-up assessments. The children with CAS and children with TD presented with similar relative change in phonological awareness, reading, and decoding measures over the follow-up period. The connected reading performance of children with CAS mirrored their phonological awareness and decoding skills. The findings demonstrated that children with CAS were able to maintain gains achieved during the intervention but may need further support to promote sustained development in written language. In the fifth experiment (presented in Chapter 6), the long-term effects of the integrated phonological awareness programme for identical twin boys who participated in the research intervention at pre-school were examined. The study examined Theo and Jamie's spoken language, phonological awareness, reading, and spelling development during their first year of schooling. The results pointed to the benefit of providing phonological awareness within a preventative framework for children with CAS. Theo and Jamie experienced continued growth in speech and phonological awareness skills. They exhibited age-appropriate reading and spelling development during their first year of formal literacy instruction. It was concluded from this series of experiments that children with CAS are particularly vulnerable to phonological awareness and early reading difficulty, and that an integrated phonological awareness intervention is an effective means of developing speech, phonological awareness, reading, and spelling skills in most children with CAS. The intervention appears to target processes underlying spoken and written language development in this population. The results are discussed within a phonological representation deficit hypothesis of CAS and clinical implications of the findings are highlighted.

PURPOSE: The purpose of this qualitative descriptive study was to explore the experiences of emerging adults with idiopathic CAS, as they reflected on their transitions through childhood, adolescence, and young adulthood. SPECIFIC AIMS: Describe the experiences of emerging adults with idiopathic CAS as they reflect on developmental stages of childhood, adolescence, and young adulthood, including the situational experiences of transition occurring between elementary, middle, high school, and post-secondary education, training, or work. Identify strategies and the effectiveness of the strategies utilized by emerging adults with idiopathic CAS to manage experiences during different developmental stages and situational experiences of transition occurring between elementary, middle, high school, and post-secondary education, training, or work. FRAMEWORK: Meleis’ Transitions Theory. DESIGN: A qualitative descriptive design with purposive sampling was used. Data was analyzed using thematic analysis. RESULTS: Findings support the use of Transitions Theory. Three major themes were identified: The Child’s Environment, Implications of CAS, and Strategies. The school environment contributed to many implications for children. Older children were able to develop strategies to overcome challenges. In the school setting, children did not access nurses for concerns related to their CAS. CONCLUSIONS: CAS creates many challenges for children. Emerging adults with CAS report that environments in which people are knowledgeable, patient, understanding, accepting, and supportive help them express themselves freely despite their speech impairment. The nurse’s role in supporting children with CAS during grade school is untapped as they were largely invisible to the children as a potential resource for anything other than an injury or illness. To better facilitate supportive environments in which children with CAS can flourish, nursing assessment and interventions are needed.

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No description available.

abstract: Childhood Apraxia of Speech (CAS) is a severe motor speech disorder that is difficult to diagnose as there is currently no gold-standard measurement to differentiate between CAS and other speech disorders. In the present study, we investigate underlying biomarkers associated with CAS in addition to enhanced phenotyping through behavioral testing. Cortical electrophysiological measures were utilized to investigate differences in neural activation in response to native and non-native vowel contrasts between children with CAS and typically developing peers. Genetic analysis included full exome sequencing of a child with CAS and his unaffected parents in order to uncover underlying genetic variation that may be causal to the child’s severely impaired speech and language. Enhanced phenotyping was completed through extensive behavioral testing, including speech, language, reading, spelling, phonological awareness, gross/fine motor, and oral and hand motor tasks. Results from cortical electrophysiological measures are consistent with previous evidence of a heightened neural response to non-native sounds in CAS, potentially indicating over specified phonological representations in this population. Results of exome sequencing suggest multiple genetic variations contributing to the severely affected phenotype in the child and provide further evidence of heterogeneous genomic pathways associated with CAS. Finally, results of behavioral testing demonstrate significant impairments evident across tasks in CAS, suggesting underlying sequential processing deficits in multiple domains. Overall, these results have the potential to delineate functional pathways from genetic variations to the brain to observable behavioral phenotypes and motivate the development of preventative and targeted treatment approaches. / Dissertation/Thesis / Doctoral Dissertation Speech and Hearing Science 2018

abstract: Speech sound disorders (SSDs) are the most prevalent type of communication disorder in children. Clinically, speech-language pathologists (SLPs) rely on behavioral methods for assessing and treating SSDs. Though clients typically experience improved speech outcomes as a result of therapy, there is evidence that underlying deficits may persist even in individuals who have completed treatment for surface-level speech behaviors. Advances in the field of genetics have created the opportunity to investigate the contribution of genes to human communication. Due to the heterogeneity of many communication disorders, the manner in which specific genetic changes influence neural mechanisms, and thereby behavioral phenotypes, remains largely unknown. The purpose of this study was to identify genotype-phenotype associations, along with perceptual, and motor-related biomarkers within families displaying SSDs. Five parent-child trios participated in genetic testing, and five families participated in a combination of genetic and behavioral testing to help elucidate biomarkers related to SSDs. All of the affected individuals had a history of childhood apraxia of speech (CAS) except for one family that displayed a phonological disorder. Genetic investigation yielded several genes of interest relevant for an SSD phenotype: CNTNAP2, CYFIP1, GPR56, HERC1, KIAA0556, LAMA5, LAMB1, MDGA2, MECP2, NBEA, SHANK3, TENM3, and ZNF142. All of these genes showed at least some expression in the developing brain. Gene ontology analysis yielded terms supporting a genetic influence on central nervous system development. Behavioral testing revealed evidence of a sequential processing biomarker for all individuals with CAS, with many showing deficits in sequential motor skills in addition to speech deficits. In some families, participants also showed evidence of a co-occurring perceptual processing biomarker. The family displaying a phonological phenotype showed milder sequential processing deficits compared to CAS families. Overall, this study supports the presence of a sequential processing biomarker for CAS and shows that relevant genes of interest may be influencing a CAS phenotype via sequential processing. Knowledge of these biomarkers can help strengthen precision of clinical assessment and motivate development of novel interventions for individuals with SSDs. / Dissertation/Thesis / Doctoral Dissertation Communication Disorders 2020

Purpose: This study primarily investigates the effects and influence of frustration in children with childhood apraxia of speech (CAS) in the setting of intensive treatment. Additionally, the study examines the interrater reliability of the frustration rating scale used in an intensive CAS treatment study. Methods: Frustration and treatment data obtained from 17 participants (between 4;0-9;11 years) with CAS in an intensive treatment research study were retrospectively used to determine potential relationships related to frustration in treatment (target complexity, temporal conditions, session number, CAS severity). Interrater reliability of the frustration rating scale was assessed with 34 randomly selected treatment session videos scored by a blinded second rater and compared to original scores. Results: Interrater reliability of the scale was poor to fair but had relatively close agreement within one scale point. Frustration levels were observed to decrease over the course of the treatment period but were typically greater in the afternoon sessions compared to morning. Participants in the complex target treatment condition with lower frustration also exhibited better outcomes than those with greater frustration. No other relationships were observed. Conclusions: Due to relatively poor interrater reliability of frustration scoring system used to obtain data used in the current study, results of the study should be interpreted cautiously. There may be a relationship between frustration levels in children with CAS and treatment conditions and outcomes, but other factors may influence both variables and further investigation into frustration is necessary to draw stronger conclusions. / Public Health

Childhood apraxia of speech (CAS) is a motor-based disorder that often results in long-term communication challenges, as well as adverse academic and psychosocial consequences. Response to CAS treatment is variable, with some children achieving slow or limited progress. A better understanding of factors associated with treatment response is needed to generate accurate prognoses and guide future development of efficient, targeted treatments. This dissertation research includes two studies. Study 1 examined speech perception skills among children with CAS using a discrimination (AX) task and a mispronunciation detection (MPD) task. Study 2 examined the association between four individual-level characteristics—age, speech accuracy, inconsistency, and perception—and CAS treatment response. Participants included 27 children (Study 2; subset of 12 children for Study 1) involved in an ongoing clinical trial for CAS (i.e., the “parent trial;” ClinicalTrials.gov ID = NCT03903120). Research questions were addressed using nonparametric correlation and t-tests, as well as multivariable linear regression. Results from Study 1 indicated that speech perception ability (1) did not differ by task, (2) did not differ by consonant and vowel conditions, (3) was positively correlated with age and speech accuracy, and negatively associated with speech inconsistency and nonverbal cognition, and (4) did not change after integral stimulation treatment. Results from Study 2 indicated that speech perception ability was the sole significant predictor of CAS treatment response, such that children with better perceptual skills at baseline made greater treatment gains. Further research is needed to replicate and extend these findings. / Communication Sciences