Joanne Roberts - US grants

Otitis media with effusion (OME) is one of the most common diseases of early childhood. Considerable controversy surrounds whether a history of OME and associated hearing loss causes language, auditory processing, and later learning problems. Children with similar OME and hearing loss histories have been shown to have different language and academic outcomes, and the differential role of the environment in these relationships may account for some of these discrepancies. Indeed, it is increasingly clear that the quality of the caregiving environment (e.g., parent-child interaction, classroom interactions) impacts children's development. Whether OME/hearing loss has a direct effect, underlying auditory processes mediate, and/or the environment moderates the linkage between early OME history and language and academic sequelae, is still undetermined. This project is designed to examine the extent to which OME with accompanying hearing loss during early childhood and subsequent auditory processing relate to language and academic performance during the first three years of elementary school. In this broad context, we will examine the role of the child's home and classroom environments in moderating any effects. The specific aims of this study are to examine: a) the amount of OME with accompanying hearing loss from birth to three years in relationship to children's later language (i.e., vocabulary, syntax, and discourse), attention, auditory processing, working memory, and academic achievement during the first three years of elementary school; b) how the quality of the home and classroom environments moderate the association between OME/hearing loss and children's later language development and school performance; and c) how children's auditory processes serve as intervening variables in the association between OME history and later language, attention, and classroom performance. We will follow 179 children from geographically-diverse lower and middle socioeconomic status families whose OME and hearing histories, language development, and home and child care environments have been prospectively documented since infancy. In the proposed study, new measures will be applied to the cohort including: a) auditory electrophysiology, binaural processing, and central auditory processing; b) vocabulary, syntax, discourse, memory, attention, and academic achievement; and c) classroom and family environments. Patterns of language, attention, working memory, and school performance over time will be studied in relation to early OME/hearing loss, auditory processes, and the environment. Growth curve methods will be used to quantify development of each attribute, and indices of these individual growth curves will be related to measures of OME with associated hearing loss. Since OME is a nearly ubiquitous condition of early childhood, factors that mediate or moderate children's ability to function optimally in the academic environment are important to delineate. Resources then can be focused on those children at highest risk for developmental sequelae.

DESCRIPTION: Fragile X syndrome is the most common inherited cause of mental retardation. In addition to intellectual impairments which tend to become more apparent in later childhood, communication deficits are common although variable among males with fragile X syndrome (FXS). Currently, very little is known about the speech and language development of young males with FXS and the impact of genetic variation on their language development. The proposed research will examine longitudinally the communication development of preschool and elementary age males with FXS, focusing on language characteristics common in older males with FXS (e.g., perseveration of words and topics, declines in language growth), and examine how variations in genotype may explain these communication differences. This research also will compare the language development of males with FXS to males with Down syndrome (DS) and to typically developing matched controls. It is anticipated that males with FXS and DS will show overall communication delays and different profiles of communication development compared with typically developing males. In these communication profiles, nonverbal cognitive skills will be a strength for males with FXS and DS, followed by speech in isolated words and vocabulary, with syntax, particularly morphosyntax, being the weakest. However, the investigators expect that males with FXS and DS will differ in discourse skills; males with FXS will initiate interactions more frequently, maintain topics less often, and perseverate on words and topics more often. In addition, they hypothesize that FMR1 protein (FMRP) will contribute to the rate of change and profiles of communication skills of young males with FXS. Sixty preschool and elementary school age males with FXS, 40 males with DS, and 40 typically developing males matched for nonverbal intelligence age will be followed for five years. Vocabulary size and diversity, utterance length and complexity, topic maintenance and perseveration, speech accuracy and intelligibility, and overall receptive and expressive language will be compared for the three groups. Fragile X DNA testing and FMRP analysis from blood samples will be done only on males with FXS to determine the methylation status of the FMR1 gene and the percentage of lymphocytes producing FMRP. Growth curve methods will be used to quantify patterns of change over time in the overall level and rate of communication development, to develop profiles of cognitive, language, and speech development for each group, to determine the extent to which profiles differ among the groups over time, and to determine for males with FXS if variation in communication development can be accounted for by their genotype. Findings will contribute to a better understanding of the patterns of communication development in young males with FXS and provide an essential knowledge base for early communication intervention.

DESCRIPTION: (Adapted from the applicant's Description) This project proposes to conduct two pilot studies on the hearing and speech intelligibility skills of young males with fragile X syndrome. The specific objectives are to determine if atypical peripheral hearing, auditory brainstem responses, and higher auditory processing skills are displayed in males with fragile X syndrome, and to determine the phonologic, prosodic, segmental, and oral-motor factors that affect the speech intelligibility of young males with fragile X syndrome. In Study 1, the investigators hypothesize that males with fragile X syndrome, compared to typically developing controls, will display normal peripheral and brainstem measures of hearing, and will show poorer higher auditory processing for recognizing speech in noise than in quiet. In Study II, the investigators hypothesize that males with fragile X syndrome will show poorer speech intelligibility in conversational speech due to phonological errors, prosodic features, segmental features, and oral-motor skills.

DESCRIPTION (provided by applicant): Fragile X syndrome is the most common inherited cause of mental retardation, and unintelligible speech is a very common characteristics of young males with fragile X syndrome (FXS). It is unclear what aspects of speech (e.g., rate, prosody, oral structure/function, articulation, phonological processes) cause poor intelligibility. Furthermore, it has not been determined what is the role of motor speech/FMR1 protein (FMRP) levels, cognitive/linguistic factors, and the communicative context in affecting intelligibility of conversational speech of males with FXS. Identifying these factors is important because the ability to be understood is critical for effective communication, and poor speech intelligibility compromises all aspects of daily verbal interactions. The proposed study builds on a currently funded R0-3 pilot study Hearing and Speech of Young with Males with Fragile X Syndrome, funded by the RFA "Neurobiology and Genetics of Fragile X Syndrome" in April of 2001, which examined at one point in time the speech and hearing skills of young males with fragile X syndrome. The proposed research will expand this research by examining longitudinally over a five year period the factors that affect changes in the speech intelligibility of 8 to 12 year old males with FXS in comparison to developmental age matched males with Down syndrome (DS) and males who are typically developing (TD). It will identify if motor speech/FMRP levels, cognitive/linguistic factors, and the social demands of the communicative context affect speech intelligibility. The specific objectives of this study are to: a) compare the development of phonological, prosodic, and segmental factors in the speech of males with FXS, males with DS, and TD males; b) identify the phonological, prosodic, and segmental factors affecting the speech intelligibility in conversational speech of males with FXS and determine if similar patterns of association are observed among males with DS and TD males; and c) to identify the motor speech/FMRP, cognitive/linguistic, and communicative contextual factors associated with speech intelligibility in conversational speech among males with FXS, and determine if similar patterns of association are observed among males with DS and TD males. Sixty males with FXS between 8 and 12 years of age, 40 males with Down syndrome between 8 and 12 years of age and 40 typically developing males matched for nonverbal intelligence age will be followed for five years. Speech production in isolated words, imitated sentences, spontaneous conversational speech, and narratives will be examined for prosodic and segmental features, phonological processes, and speech intelligibility for the three groups. In addition, children's oral motor skills, phonological processing, and selective listening will be examined. Fragile X DNA testing and FMRP analysis from blood samples will be done only on males with FXS. Growth curve methods will be used to quantify patterns of change over time in the overall level and rate of speech development including speech intelligibility. Of particular interest will be longitudinal analyses of speech intelligibility designed to determine the degree to which motor speech/FMRP, cognitive/linguistic, and contextual factors predict speech intelligibility among males with FXS. Subsequent analyses will ask whether factors associated with intelligibility among males with FXS are also associated among males with DS and TD males. Findings will contribute to a better understanding of the factors that affect speech intelligibility in young males with FXS and provide an essential knowledge base for speech intervention.

children; African American; elementary school; academic achievement; racial /ethnic difference; performance; clinical research; human subject;

This research will examine the production and development of African American English (AAE) through early adolescence, the potential impact of vernacular dialect on the literacy acquisition of African Americans from entry to school through middle school, and the youth, family, and school factors that may affect this linkage. Even though youth have acquired most of their primary linguistic structures prior to adolescence, they make important adjustments to their language based on social context and peer relations during this critical life phase. These adjustments include the choice of vernacular or standard dialect and the development of style shifting ability. This study builds on a unique, longitudinal database collected over the past 14 years for a group of 70 African-American adolescents from low- and middle-income families whose language skills, literacy skills, and family and school environments, have been progressively documented since infancy. This research will also include an additional sample of 70 friends of the original study participants. During these middle school years, AAE production in formal and informal activities with friends and adult examiners and reading skills will be measured. Additional measures of the youth (e.g., metalinguistic skills, school attitudes), family (e.g., responsiveness of the home environment), and school (e.g., SES of school, teacher-student relationship) characteristics will also be collected. Hypotheses will be tested with respect to the trajectory of AAE variants from the elementary though middle school years, the relationship of formality of context to vernacular variations in the middle school years, the relationship of vernacular dialect to the literacy acquisition of African Americans from entry to school through middle school, and the specific youth characteristics that may explain these vernacular literacy associations, including school attitudes and metalinguistic skills. Growth curve methods will be used to quantify patterns of change in the development of specific aspects of youths' vernacular dialect to determine their relation to literacy development. Such data address the possible role of vernacular dialect prominence in literacy achievement during those periods of schooling when the gap in achievement between Caucasian- and African-Americans widens greatly.

This project will describe the longitudinal development of African American English (AAE) from school entry through middle school, the youth, family, school factors that influence these patterns, and the impact of these factors on African American youth literacy. The study will address the social and educational implications of the most significant sociocultural variation of American English. Understanding the sociocultural factors that affect the vernacular variations and literacy achievement of African-American adolescents is critical for addressing the academic achievement gaps between African Americans and their non-minority peers in American society. The findings will be of great interest to parents, students, teachers, other educators, and researchers concerned with issues related to the school success of African-American youth and risk factors for school failure.

[unreadable] DESCRIPTION (provided by applicant): This study compares the developmental trajectories of pragmatic skills, the use of language in social contexts, among girls and boys with fragile X syndrome (FXS), Down syndrome (DS), and typical development (TD) and boys with autism spectrum disorder only (ASD-O) to determine whether individual differences in conversational discourse and narrative skills relate to FXS specifically or to either mental retardation (MR) or autism in general. The study will also compare potential mechanisms underlying these individual differences in pragmatic skills in FXS in comparison to DS and ASD-O. The specific aims of the study are to identify differences in pragmatic skills in conversation and narration that are syndrome specific to FXS or DS and determine the extent to which co-morbidity between FXS and autism may account for some apparent FXS group differences. Further, we will examine the extent to which pragmatic skills among boys and girls with FXS differ, specifically whether gender differences vary depending on the presence of MR or whether they also exist in children with DS or TD. Finally, we seek to identify possible mediators that account for anticipated group differences in the acquisition of conversational and narrative skills among children with FXS (who do and do not have ASD) in comparison to children with DS, ASD-O, or TD. Study participants will be 80 boys with FXS (40 with and 40 without ASD) who are 6-12 years, 60 girls with FXS (30 6-12 years with MR and 30 3-6 years without MR), 40 girls and 40 boys with DS 6-12 years, 40 boys with ASD and MR 6-12 years, and 30 TD girts and 30 TD boys 3-6 years. All participants will have nonverbal mental ages between 3 and 6 years. Children's pragmatic skills, including their initiations, contingency, perseveration, and repair strategies during conversation and their informativeness and cohesiveness during narration will be assessed annually for four years. Children's language content and structure (receptive vocabulary and expressive syntax), cognition (nonverbal cognitive level and attention), social-emotional behavior (anxiety and autistic characteristics), and family environment (responsiveness and support of home environment and maternal contingency) will also be examined annually. In addition, Fragile X Mental Retardation Protein (FMRP) analysis from blood samples will be completed on the girls and boys with FXS, and activation ratios will be computed for the girts with FXS. Growth curve methods will be used to quantify patterns of change over time in the overall level and rate of growth in pragmatic development. Pragmatic skills are essential for effective communication and pragmatic difficulties can compromise all aspects of communicative competence in daily interactions. Determining whether a unique pragmatic phenotype exists for FXS that is syndrome specific or related to gender, MR, or autism as well as identifying the potential mechanisms underlying pragmatic skills, have critically important implications for defining treatment protocols. [unreadable] [unreadable] [unreadable]