Audra Sterling

Maternal Responsivity Predicts Language Development in Young Children With Fragile X Syndrome

The relationship between early maternal responsivity and later child communication outcomes in young children with fragile X syndrome was investigated. Data were obtained from 55 mother-child dyads over a 36-month period. Performance data were obtained at each measurement point from video observations of four different contexts. These were coded for (a) child communication behaviors, (b) parent responsivity, and (c) behavior management behaviors. Results indicate that early maternal responsivity predicts the level of four important child language outcomes at 36 months of age after controlling for child developmental level and autism symptomology.

Interventions Aimed at Improving Child Language by Improving Maternal Responsivity.

Maternal responsivity, or the ways in which mothers provide for, interact with, and respond to their children, helps to shape their childrens development, including language development. In this chapter, we describe maternal responsivity as a multilevel construct with different measures appropriate for each level. Molar responsivity refers to aspects of interaction style such as affect that can best be measured with rating scales. Molecular responsivity refers to contingent maternal behaviors that occur in response to child behaviors; and are best reflected by the frequencies of occurrence of these contingent behaviors. Results of many studies have demonstrated that both molar and molecular responsivity are related to important child outcomes such as language development. Children of more responsive mothers tend to have better outcomes. Based on these findings, interventions aimed at improving maternal responsivity and thereby child language outcomes have been developed and investigated through a number of studies. Results have shown positive outcomes for maternal responsivity and lesser secondary benefits to child language outcomes. Some of the qualities that appear associated with better outcomes include timing the interventions to co-occur with specific developments in child behaviors, teaching over a span of approximately 10-12 sessions, and designing lessons to be culturally sensitive to individual families.

Discriminating Down Syndrome and Fragile X Syndrome based on language ability

ABSTRACT This study compared the receptive and expressive language profiles of verbally expressive children and adolescents with Down Syndrome (DS) and those with Fragile X syndrome (FXS) and examined the extent to which these profiles reliably differentiate the diagnostic groups. A total of twenty-four verbal participants with DS (mean age: 12 years), twenty-two verbal participants with FXS (mean age: 12 years), and twenty-seven participants with typical development (TD; mean age = 4 years) completed standardized measures of receptive and expressive vocabulary and grammar, as well as a conversational language sample. Study results indicate that there are distinct DS and FXS language profiles, which are characterized by differences in grammatical ability. The diagnostic groups were not differentiated based on vocabulary performance. This study supports the existence of unique language profiles associated with DS and FXS.

Language dysfluencies in females with the FMR1 premutation.

Recent evidence suggests that there are age-related neurocognitive implications for fragile X premutation carriers, including deficits in executive function, and that such deficits are more common in male than female premutation carriers. The purpose of the current study is to examine one aspect of executive function, language dysfluencies, in a group of 193 women with the premutation, and to contrast them with a comparison group (mothers of children with autism spectrum disorders). Our results demonstrate a linguistic profile in the female premutation carriers characterized by dysfluencies associated with deficits in organization and planning, with a clear impact of age. The comparison group, matched on both age and education level, did not demonstrate the age effect. Our results suggest dysfluencies could be an early indicator of cognitive aging in some female premutation carriers, and could be used to target early intervention.

Development of an expressive language sampling procedure in fragile X syndrome: a pilot study.

Objective: There is a great need for valid outcome measures of functional improvement for impending clinical trials of targeted interventions for fragile X syndrome (FXS). Families often report conversational language improvement during clinical treatment, but no validated measures exist to quantify this outcome. This small-scale study sought to determine the feasibility, reproducibility, and clinical validity of highly structured expressive language sampling as an outcome measure reflecting language ability. Methods: Narrative and conversation tasks were administered to 36 verbal participants (25 males and 11 females) with FXS (aged 5–36 years, mean, 18 ± 7 years). Alternate versions were used with randomized task order at 2- to 3-week intervals (mean, 19.6 ± 6.4 days). Audio recordings of sessions were transcribed and analyzed. Dependent measures reflected talkativeness (total number of utterances), utterance planning (proportion of communication [C] units with mazes), articulation (proportion of unintelligible/partly unintelligible C-units), vocabulary (number of different word roots), and syntactic ability (mean length of utterance [MLU] in words). Reproducibility of measures was evaluated with intraclass correlation coefficients (ICC). Results: All participants could complete the tasks. Coded data were highly reproducible with Pearsons correlations at p < .01 for all measures and ICC values of .911 to .966 (conversation) and .728 to .940 (narration). Some measures including MLU and different word roots were correlated with expressive language subscale scores from the Vineland Adaptive Behavior Scale. Conclusions: These expressive language sampling tasks appear to be feasible, reproducible, and clinically valid and should be further validated in a larger cohort, as a promising means of assessing functional expressive language outcomes during clinical trials in FXS.

Language development in school‐age girls with fragile X syndrome

BACKGROUND Girls with fragile X syndrome (FXS) have a wide range of cognitive and language abilities. The range of language outcomes experienced by girls with FXS, however, has been relatively unexplored. The purpose of this exploratory study was to examine receptive and expressive language, with a focus on vocabulary and syntax, in a group of school-age girls with FXS. METHOD Twenty-one girls with FXS aged 7-15 years participated in the study. The girls completed a receptive vocabulary test, non-verbal IQ test and an expressive language sample. RESULTS The mean IQ for this group of girls was at the cut-off for intellectual disability. Vocabulary was an area of strength relative to non-verbal cognition. Age and non-verbal IQ were significant predictors of vocabulary performance. CONCLUSIONS The data suggest that a substantial portion of the sample would qualify for speech and language services. This study highlights the need for continued research in the area of language and cognitive development in girls with the full mutation of fragile X.

Influences on maternal responsivity in mothers of children with fragile X syndrome

This study investigated the influence of maternal and child variables on the maternal responsivity of 55 mothers with young children with fragile X syndrome. Data included video observations of mother-child interactions in four different contexts, standardized assessments with the children, and standardized questionnaires for the mothers. The video observations were coded for child communication acts; maternal responsivity was coded at two levels: a more general measure and a behavior-by-behavior measure. Results indicated that child developmental level and language ability strongly influenced behavior-by-behavior responsivity, while maternal IQ was the strongest predictor of both general and behavior-by-behavior responsivity, after controlling for child developmental level.

Parenting Young Children with and without Fragile X Syndrome

The purpose of this study was to examine maternal parenting styles across age-matched siblings using a within-family design, in which one child has Fragile X syndrome. Thirteen families participated; children were aged 16 to 71 months. Mothers completed several videotaped activities with each child separately as well as an interview. Mothers used a consistent, responsive style with both children, using the same degree of positive affect and warmth. Differences included using more behavior management strategies with the child with Fragile X and a conversational style of interaction with the sibling. Differences in approaches suggest the mothers adapted to the developmental differences between the children. The interview data supported these findings; mothers were aware of the changes made to accommodate the developmental differences.

Maternal responsivity in mothers of young children with Down syndrome.

Objective: The purpose of this study was to examine maternal responsivity and directive behaviors in mothers of children with Down syndrome (DS). Methods: Participants included 22 mothers with a young child with DS compared to 22 mothers of chronologically age-matched typically developing (TD) children using a cross-sectional design. The dyads participated in videotaped structured activities that were coded for responsive and directive behaviors. Results: Results indicated that the mothers of children with DS used a more facilitative style with the older children while these behaviors decreased with older children with TD; one directive behavior, request for behavioral comply, increased with the older children with DS. Conclusion: The mothers of children with DS adapted their parenting style to be facilitative of their childrens linguistic development.

The inconvenience of data of convenience: Computational research beyond post-mortem analyses

The inconvenience of data of convenience: computational research beyond post-mortem analyses