Word retrieval difficulty in adult females with the FMR1 premutation: Changes over time and across contexts.

Individuals with a premutation of the fragile X mental retardation (FMR1) gene are at risk for a variety of psychological, physical, and cognitive issues, including difficulty with word retrieval. The present study examined three indicators of word retrieval difficulty; reduced productivity, reduced lexical diversity, and increased errors in word retrieval in a group of 38 female premutation carriers during standard-length speech samples collected over a period of eight years. Our results revealed that as women aged, they produced fewer words, produced fewer different words, and had greater word retrieval errors. In addition, the rate of word retrieval errors was highly correlated between two speaking contexts, indicating that this difficulty was pervasive and not solely the result of speaking in monologue. Our results suggest that subtle areas of cognitive decline emerge at a much earlier age among female premutation carriers than would be expected during healthy aging.

Input sources of third person singular -s inconsistency in children with and without specific language impairment.

We tested four predictions based on the assumption that optional infinitives can be attributed to properties of the input whereby children inappropriately extract non-finite subject-verb sequences (e.g., the girl run) from larger input utterances (e.g., Does the girl run? Let's watch the girl run). Thirty children with specific language impairment (SLI) and thirty typically developing children heard novel and familiar verbs that appeared exclusively either in utterances containing non-finite subject-verb sequences or in simple sentences with the verb inflected for third person singular -s. Subsequent testing showed strong input effects, especially for the SLI group. The results provide support for input-based factors as significant contributors not only to the optional infinitive period in typical development, but also to the especially protracted optional infinitive period seen in SLI.

Sources of Misinterpretation in the Input and Their Implications for Language Intervention With English-Speaking Children.

PURPOSE: In English and related languages, many preschool-age children with developmental language disorder (DLD) have difficulties using tense and agreement consistently. In this review article, we discuss two potential input-related sources of this difficulty and offer several possible strategies aimed at circumventing input obstacles. METHOD: We review a series of studies from English, supplemented by evidence from computational modeling and studies of other languages. Collectively, the studies show that instances of failures to express tense and agreement in DLD resemble portions of larger sentences in everyday input in which tense and agreement marking is appropriately absent. Furthermore, experimental studies show that children's use of tense and agreement can be swayed by manipulating details in fully grammatical input sentences. RESULTS: The available evidence points to two particular sources of input that may contribute to tense and agreement inconsistency. One source is the appearance of subject + nonfinite verb sequences that appear in auxiliary-fronted questions (e.g., Is [the girl running]? Does [the boy like popcorn]?) and as dependent clauses in more complex sentences (e.g., Help [her wash the dishes]; We saw [the frog hopping]). The other source is the frequent appearance of bare stems in the input, whether nonfinite (e.g., go in Make him go fast) or finite (e.g., go in I go, you go). CONCLUSIONS: Although the likely sources of input are a natural part of the language that all children hear, procedures that alter the distribution of this input might be used in the early stages of intervention. Subsequent steps can incorporate more explicit comprehension and production techniques. A variety of suggestions are offered.

Transactional relationships between maternal requesting and responsivity, adolescent compliance, and task demands in fragile X syndrome.

BACKGROUND: Males with fragile X syndrome (FXS) may have difficulty with social communication and language in addition to behavioral concerns such as noncompliance to maternal requests. Mothers vary in how they pose requests to their children, and contextual demands may also be a factor in compliance. This study examined the relationships between maternal requesting behaviors, maternal responsivity, child compliance, and task demands in adolescent males with FXS. METHODS: 35 mother-son dyads completed three interactive tasks during in-home visits (puzzle, iPad games, snack). The adolescents also completed assessments of language and autism symptoms. The three interactive tasks were video-recorded, and behavior-by-behavior coding and transcription was completed. RESULTS: Comparing between tasks, mothers requested a behavior most often during the snack task and used a higher proportion of indirect requests during the iPad task. Adolescents were largely compliant across tasks, with average compliance equal to 70%. Adolescent compliance was predicted by maternal request frequency such that mothers who used more requests had adolescent sons who were less compliant. Maternal responsivity was higher for mothers of adolescents with FXS-only compared to those with FXS and high autism symptoms. CONCLUSIONS AND IMPLICATIONS: Task demands may have influenced maternal requesting, which in turn may have impacted adolescent compliance. Compliance with requests is important for both social and educational aspects of life, and further study of requesting and compliance in FXS is needed to identify effective teaching and behavioral intervention methods.

Examination of the ADOS-2 Expressive Language Score in Fragile X Syndrome.

The development of an expressive language score for people with autism based on the ADOS-2 was recently reported by Mazurek et al. (2019). The current study examined the construct validity of the ADOS-2 expressive language score (ELS) in a sample of adolescents with fragile X syndrome (n = 45, 10 girls), a neurodevelopmental disorder with high rates of autism symptomology. The ADOS-2 ELS showed strong convergent validity with multiple assessments of expressive language, receptive language, and nonverbal cognition. Divergent validity was demonstrated between the expressive language score and chronological age, symptoms of anxiety/depression, and rule-breaking behaviors. This expressive language score is a promising measure of expressive language ability that can be used in research when other language assessments are unavailable.

Rethinking Measurement Standards of Autism Symptomology in Adolescents with Fragile X Syndrome.

Accurate representation of autism spectrum disorder (ASD) in fragile X syndrome (FXS) is necessary for the field. We examined classifications of ASD using three approaches-Autism Diagnostic Observation Schedule (ADOS-2; Lord et al. 2012), Childhood Autism Rating Scale (CARS2-ST; Schopler et al. 2010), and Vineland Adaptive Behavior Scales (VABS-II; Sparrow et al. 2005)-in 45 adolescents with FXS. Maladaptive items from the VABS-II plus a maternal interview were matched with Diagnostic and Statistical Manual of Mental Disorders, 5th edition (DSM-5; American Psychiatric Association 2013) criteria for ASD. Results indicated discrepant classifications. The ADOS-2 yielded the highest rate of comorbid autism (71%); CARS2-ST and VABS-II/DSM-5 yielded lower rates (38% and 42%, respectively). A singular measure of autism symptomology is insufficient to characterize autism in FXS.

Use of Imitation Training for Targeting Grammar: A Narrative Review.

Purpose The purpose of this narrative review was to examine the evidence for imitation training for targeting grammar in children with developmental language disorder. Method Studies investigating imitation training were compiled from two databases-PsycINFO and Linguistics and Language Behavior Abstracts-as well as from reference lists of several books and articles reviewing language therapy. Results Twenty-one studies were reviewed. We summarized the demographic and methodological characteristics of the included studies. We identified substantial differences in the implementation of imitation training across the studies and noted a lack of studies considering how those differences might affect therapy outcome. Studies showed a rapid increase in the production of grammatical targets during imitation training activities, higher performance relative to a control group that did not receive any therapy, and generalization to untrained exemplars. However, the studies did not unequivocally link the increased performance to the imitation training therapy. Studies also showed limited generalization to conversational speech. Conversationally based therapy resulted in faster or higher usage in conversational speech, while imitation training resulted in faster immediate gains in usage during therapy trials. Conclusion We believe that imitation training can continue to be a useful tool in a speech-language pathologist's toolbox, but with suggested modifications. Specifically, we suggest use of imitation training as a means for rapidly achieving production of previously absent grammatical targets. However, we do not recommend sole or long-term use of imitation training for working on grammar. Further research is needed to refine our use of the procedure.

Language Development From Early Childhood to Adolescence in Youths With Fragile X Syndrome.

Purpose The aim of this study was to investigate language growth in individuals with fragile X syndrome (FXS) from early childhood to adolescence and the influence of maternal responsivity on language growth. Method We conducted a longitudinal analysis of language development in 55 youths (44 males, 11 females) with FXS. Data collection spanned the ages of 11-216 months. We measured expressive and receptive vocabulary with standardized tests. The number of different words and mean length of utterance were obtained from language sample analyses of mother-child interactions. We also measured maternal comments (responsivity indicator) produced during the language samples and child nonverbal IQ. Results Growth models indicated that rates of number of different words and receptive vocabulary were related to maternal commenting. Mean length of utterance did not change significantly over time. Expressive vocabulary measured with a standardized test grew, but the growth was not related to maternal commenting. Nonverbal IQ was related to all language outcomes at age of 10 years and to changes over time in vocabulary. Visual analysis indicated that the highest scores on standardized tests were produced by girls; however, measures derived from language sample analyses appeared similar for boys and girls. Language models for boys only were similar to the total sample models with lower scores at age of 10 years for some outcomes. Conclusion Results of persistent language impairments for most youths with FXS suggest the need for continued, focused interventions aimed at improved language productions in addition to a responsive environment. Supplemental Material https://doi.org/10.23641/asha.13022825.

A randomized trial of longitudinal effects of low-intensity responsivity education/prelinguistic milieu teaching.

PURPOSE: To evaluate the longitudinal effects of a 6-month course of responsivity education (RE)/prelinguistic milieu teaching (PMT) for young children with developmental delay. METHOD: Fifty-one children, age 24-33 months, with fewer than 10 expressive words were randomly assigned to early-treatment/no-treatment groups. All treatment was added as a supplement to services that the children received in the community. Follow-up data were collected 6 and 12 months after the conclusion of the initial 6-month treatment/no-treatment conditions. RESULTS: No effects of this treatment were detected 6 or 12 months after the conclusion of the initial treatment condition. CONCLUSIONS: M. E. Fey et al. (2006) reported that 6 months of RE/PMT led to a significant treatment effect in the use of intentional communication in 1 of 2 communication sampling contexts. This finding, combined with evidence from other studies, suggests that RE/PMT may be applied clinically at low intensity with the expectation of medium-sized effects on children's rate of intentional communication acts over the short term. The results of the present study, however, provide no evidence for the anticipated longer term benefits of this intervention. Further investigation of the approach at higher intensity levels and for longer periods of time is warranted.

Clinician vs. Machine: Estimating Vocalizations Rates in Young Children With Developmental Disorders.

Purpose: The purpose of this study was to investigate the reliability of an automated language analysis system, the Language Environment Analysis (LENA), compared with a human transcriber to determine the rate of child vocalizations during recording sessions that were significantly shorter than recommended for the automated device. Method: Participants were 6 nonverbal male children between the ages of 28 and 46 months. Two children had autism diagnoses, 2 had Down syndrome, 1 had a chromosomal deletion, and 1 had developmental delay. Participants were recorded by the LENA digital language processor during 14 play-based interactions with a responsive adult. Rate of child vocalizations during each of the 84 recordings was determined by both a human transcriber and the LENA software. Results: A statistically significant difference between the 2 methods was observed for 4 of the 6 participants. Effect sizes were moderate to large. Variation in syllable structure did not explain the difference between the 2 methods. Vocalization rates from the 2 methods were highly correlated for 5 of the 6 participants. Conclusions: Estimates of vocalization rates from nonverbal children produced by the LENA system differed from human transcription during sessions that were substantially shorter than the recommended recording length. These results confirm the recommendation of the LENA Foundation to record sessions of at least 1 hr.

A Clinical Evaluation of the Competing Sources of Input Hypothesis.

Purpose: Our purpose was to test the competing sources of input (CSI) hypothesis by evaluating an intervention based on its principles. This hypothesis proposes that children's use of main verbs without tense is the result of their treating certain sentence types in the input (e.g., Wasshe laughing?) as models for declaratives (e.g., She laughing). Method: Twenty preschoolers with specific language impairment were randomly assigned to receive either a CSI-based intervention or a more traditional intervention that lacked the novel CSI features. The auxiliary is and the third-person singular suffix -s were directly treated over a 16-week period. Past tense -ed was monitored as a control. Results: The CSI-based group exhibited greater improvements in use of is than did the traditional group (d = 1.31), providing strong support for the CSI hypothesis. There were no significant between-groups differences in the production of the third-person singular suffix -s or the control (-ed), however. Conclusions: The group differences in the effects on the 2 treated morphemes may be due to differences in their distribution in interrogatives and declaratives (e.g., Ishe hiding/Heishiding vs. Doeshe hide/He hides). Refinements in the intervention could address this issue and lead to more general effects across morphemes.

Early effects of responsivity education/prelinguistic milieu teaching for children with developmental delays and their parents.

PURPOSE: To evaluate the efficacy of a 6-month course of responsivity education/prelinguistic milieu teaching (RE/PMT) for children with developmental delay and RE/PMT's effects on parenting stress in a randomized clinical trial. METHOD: Fifty-one children, age 24-33 months, with no more than 10 expressive words or signs, were randomly assigned to treatment/no-treatment groups. Thirteen children in each group had a diagnosis of Down syndrome. RESULTS: In 1 of 2 multivariate comparisons, the RE/PMT group exhibited superior gains in communication compared with the no-treatment group. The treatment effect for overall use of intentional communication acts in the child-examiner context was significant (d = .68, 95% confidence interval = 0.12-1.24). There were no effects on child outcomes due to presence or absence of Down syndrome. RE/PMT led to modest increases in recoding of child acts by parents of children who did not have Down syndrome. There were no effects on parenting stress associated with the intervention or the presence or absence of Down syndrome. CONCLUSIONS: RE/PMT may be applied clinically with the expectation of medium-size effects on the child's rate of intentional communication acts after 6 months of intervention. The approach warrants further investigation with modifications, such as delivery at higher intensity levels.

Subordinate clause comprehension and tense/agreement inconsistency in children with specific language impairment.

UNLABELLED: Several recent studies have suggested that the production errors of children with specific language impairment (SLI) such as The girl singing may be explained by a misinterpretation of grammatical adult input containing a similar structure (e.g., The boy hears the girl singing). Thirteen children with SLI and 13 younger typically developing children with comparable sentence comprehension test scores (TD-COMP) completed a comprehension task to assess their understanding of sentences involving a nonfinite subject-verb sequence in a subordinate clause such as The dad sees the boy running. TD-COMP children were more accurate on subordinate clause items than children with SLI despite similar performance on simple transitive (e.g., The horse sees the cow) and simple progressive (e.g., The cow is eating) items. However, no relationship was found between the SLI group's specific subordinate clause comprehension level and their specific level of auxiliary is production, casting some doubt on this type of structure as a source for inconsistent use of auxiliary is. LEARNING OUTCOMES: The reader will learn that children with specific language impairment (SLI): (1) have difficulty understanding complex sentences that include nonfinite subject-verb sequences; (2) that this difficulty is apparent in comparison to younger typically developing peers who have similar scores not only on a sentence comprehension test, but also on simple sentences that correspond to the component parts of the complex sentences; and (3) that this weakness is concurrent with these children's inconsistent use of auxiliary is in production. Although novel verb studies show a clear connection between how children with SLI hear new verbs and how they use them, we do not yet have evidence that this connection is tied to a poor understanding of the input sentences that house the verbs.

Children's responses to telegraphic and grammatically complete prompts to imitate.

PURPOSE: The purpose of this study was to determine whether children in the early stage of combining words are more likely to respond to imitation prompts that are telegraphic than to prompts that are grammatically complete and whether they produce obligatory grammatical morphemes more reliably in response to grammatically complete imitation prompts than to telegraphic prompts. METHOD: Five children between 30 and 51 months of age with language delay participated in a single-case alternating treatment design with 14 sessions split between a grammatical and a telegraphic condition. Alternating orders of the 14 sessions were randomly assigned to each child. Children were given 15 prompts to imitate a semantic relation that was either grammatically complete or telegraphic. RESULTS: No differences between conditions were found for the number of responses that contained a semantic relation. In contrast, 3 of the 5 children produced significantly more grammatical morphemes when presented with grammatically complete imitation prompts. Two children did not include a function word in either condition. CONCLUSION: Providing a telegraphic prompt to imitate does not offer any advantage as an intervention technique. Children are just as likely to respond to a grammatically complete imitation prompt. Further, including function words encourages children who are developmentally ready to imitate them.

Sentence comprehension in specific language impairment: a task designed to distinguish between cognitive capacity and syntactic complexity.

PURPOSE: This study examined sentence comprehension in children with specific language impairment (SLI) in a manner designed to separate the contribution of cognitive capacity from the effects of syntactic structure. METHOD: Nineteen children with SLI, 19 typically developing children matched for age (TD-A), and 19 younger typically developing children (TD-Y) matched according to sentence comprehension test scores responded to sentence comprehension items that varied in either length or their demands on cognitive capacity, based on the nature of the foils competing with the target picture. RESULTS: The TD-A children were accurate across all item types. The SLI and TD-Y groups were less accurate than the TD-A group on items with greater length and, especially, on items with the greatest demands on cognitive capacity. The types of errors were consistent with failure to retain details of the sentence apart from syntactic structure. CONCLUSIONS: The difficulty in the more demanding conditions seemed attributable to interference. Specifically, the children with SLI and the TD-Y children appeared to have difficulty retaining details of the target sentence when the information reflected in the foils closely resembled the information in the target sentence.

Is more better? Milieu communication teaching in toddlers with intellectual disabilities.

PURPOSE: The authors sought to determine whether a program of 5 weekly doses of milieu communication teaching (MCT) would yield improvements in children's communication and word use compared with a once-weekly delivery of the same treatment. METHOD: Sixty-four children with intellectual and communication delay were randomly assigned to receive 60-min sessions of MCT either 1 time or 5 times per week over a 9-month treatment. Growth curves were fit to data collected at 5 points before, during, and after the MCT was delivered. RESULTS: With groups collapsed, significant growth across the experimental period was observed on all measures, but this was not associated unconditionally with treatment intensity. Children who played with 9 or more objects during a standard play assessment, an empirically identified cut-point, benefited more from the high- than from the low-intensity treatment on lexical measures (Hedges's g range = .49 to .65). CONCLUSIONS: More MCT is not always better for all children. Clinicians can expect that increasing the frequency of MCT sessions will yield moderate enhancement of outcomes if the child has high interest in objects.