Perseveration in the connected speech of boys with Fragile X syndrome with and without autism spectrum disorder.

Verbal perseveration is a frequently reported language characteristic of males with Fragile X syndrome and may be a defining feature or hallmark of the syndrome. We compared the verbal perseveration of boys with Fragile X syndrome with (n  =  29) and without (n  =  30) autism spectrum disorder, boys with Down syndrome (n  =  27), and typically developing boys (n  =  25) at similar nonverbal mental ages. During a social interaction, boys with both Fragile X syndrome and autism spectrum disorder produced significantly more topic perseveration than all other groups. In social interaction as compared to narration, boys with Fragile X syndrome (regardless of autism status) produced significantly more topic perseveration. These findings suggest that autism status, as well as language sampling context, affect perseveration in boys with Fragile X syndrome.

Cognitive, environmental, and linguistic predictors of syntax in fragile X syndrome and Down syndrome.

PURPOSE: To examine which cognitive, environmental, and speech-language variables predict expressive syntax in boys with fragile X syndrome (FXS), boys with Down syndrome (DS), and typically developing (TD) boys, and whether predictive relationships differed by group. METHOD: We obtained Index of Productive Syntax ( Scarborough, 1990) scores for 18 boys with FXS only, 20 boys with both FXS and an autism spectrum disorder, 27 boys with DS, and 25 younger TD boys of similar nonverbal mental age. Predictors included group (diagnosis), nonverbal cognition, phonological working memory (PWM), maternal education, speech intelligibility, and expressive vocabulary. The research questions were addressed via hierarchical linear regression. RESULTS: Diagnostic group, nonverbal cognition, and PWM predicted 56% of the variance in syntactic ability, with approximately three-fourths of the predicted variance explained by group membership alone. The other factors did not contribute any additional significant variance in this final model. There was no evidence that predictor effects differed by group. CONCLUSIONS: Nonverbal cognition and PWM have an effect on expressive syntax beyond that of diagnostic group. These effects are estimated to be the same in boys with FXS, boys with DS, and TD boys. Explanations for residual variance and the relative role of different predictors are discussed.

Narrative Skill in Boys with Fragile X Syndrome with and without Autism Spectrum Disorder.

We examined recalled narratives of boys with fragile X syndrome with autism spectrum disorder (FXS-ASD; N=28) and without ASD (FXS-O; N=29), and compared them to those of boys with Down syndrome (DS; N=33) and typically developing boys (TD; N=39). Narratives were scored for mentions of macrostructural Story Grammar elements (Introduction, Relationship, Initiating Events, Internal Response, Attempts/Actions, and Ending). We found that narrative recall is predicted by short-term memory and nonverbal mental age levels in almost all groups (except TD), but not by expressive syntax or caregiver education. After adjusting for these covariates, there were no differences between the three groups with intellectual disability (ID). The FXS-ASD group, however, had significantly poorer performance than the TD group on the overall Story Grammar score, and both the FXS-O and FXS-ASD groups had lower Attempts/Actions scores than the TD group. We conclude that some form of narrative impairment may be associated with FXS, that this impairment may be shared by other forms of ID, and that the presence of ASD has a significantly detrimental effect on narrative recall.

Direct magnitude estimation of articulation rate in boys with fragile X syndrome.

PURPOSE: To compare the perceived articulation rate of boys with fragile X syndrome (FXS) with that of chronologically age-matched (CA) boys and to determine segmental and/or prosodic factors that account for perceived rate. METHOD: Ten listeners used direct magnitude estimation procedures to judge the articulation rates of 7 boys with FXS only, 5 boys with FXS and a diagnosis of autism spectrum disorder (ASD), and 12 CA boys during sentence repetition. Sentences had similar articulation rates in syllables per second as determined acoustically. Four segmental/prosodic factors were used to predict perceived rate: (a) percentage consonants correct, (b) overall fundamental frequency (F(0)) level, (c) sentence-final F(0) drop, and (d) acoustically determined articulation rate with the final word of the sentence excluded. RESULTS: Boys with FXS and ASD were judged to talk faster than CA controls. Multiple linear regression indicated that articulation rate with the final word of the sentence excluded and sentence-final F(0) drop accounted for 91% of the variance for perceived rate. CONCLUSIONS: Descriptions of speakers with FXS as having fast and/or fluctuating articulation rates may be influenced by autism status. Also, atypical sentence-final prosody may be related to perceived rate in boys with FXS and ASD.

Language Characteristics of Individuals with Down Syndrome.

On average, language and communication characteristics of individuals with Down syndrome (the most common genetic cause of intellectual disability) follow a consistent profile. Despite considerable individual variability, receptive language is typically stronger than expressive language, with particular challenges in phonology and syntax. We review the literature on language and literacy skills of individuals with Down syndrome, with emphasis on the areas of phonology, vocabulary, syntax, and pragmatics. We begin by describing the hearing, oral-motor, cognitive, social, and prelinguistic and early nonverbal communication characteristics of individuals with Down syndrome. We conclude with a discussion of clinical implications and research directions.

Racial identity, social context, and race-related social cognition in African Americans during middle childhood.

This study examined the effect of changes in racial identity, cross-race friendships, same-race friendships, and classroom racial composition on changes in race-related social cognition from 3rd to 5th grade for 73 African American children. The goal of the study was to determine the extent to which preadolescent racial identity and social context predict expectations of racial discrimination in cross-race social interactions (social expectations). Expectations of racial discrimination were assessed using vignettes of cross-race social situations involving an African American child in a social interaction with European Americans. There were 3 major findings. First, expectations for discrimination declined slightly from 3rd to 5th grade. Second, although racial composition of children's classrooms, number of European American friends, gender, and family poverty status were largely unrelated to social expectations, having more African American friends was associated with expecting more discrimination in cross-racial interactions from 3rd to 5th grade. Third, increases in racial centrality were related to increases in discrimination expectations, and increases in public regard were associated with decreases in discrimination expectations. These data suggest that as early as 3rd grade, children are forming attitudes about their racial group that have implications for their cross-race social interactions.

Social risk and protective factors for African American children's academic achievement and adjustment during the transition to middle school.

The transition to middle school is often marked by decreased academic achievement and increased emotional stress, and African American children exposed to social risk may be especially vulnerable during this transition. To identify mediators and protective factors, the authors related severity and timing of risk exposure to academic achievement and adjustment between 4th and 6th grade in 74 African American children. Longitudinal analyses indicated that severity more than timing of risk exposure was negatively related to all outcomes and that language skills mediated the pathway from risk for most outcomes. Transition to middle school was related to lower math scores and to more externalizing problems when children experienced higher levels of social risk. Language skills and parenting served as protective factors, whereas expectations of racial discrimination was a vulnerability factor. Results imply that promoting parenting and, especially, language skills, and decreasing expectations of racial discrimination provide pathways to academic success for African American children during the transition from elementary to middle school, especially those exposed to adversity.

Syntactic complexity during conversation of boys with fragile X syndrome and Down syndrome.

PURPOSE: This study compared the syntax of boys who have fragile X syndrome (FXS) with and without autism spectrum disorder (ASD) with that of (a) boys who have Down syndrome (DS) and (b) typically developing (TD) boys. METHOD: Thirty-five boys with FXS only, 36 boys with FXS with ASD, 31 boys with DS, and 46 TD boys participated. Conversational language samples were evaluated for utterance length and syntactic complexity (i.e., Index of Productive Syntax; H. S. Scarborough, 1990). RESULTS: After controlling for nonverbal mental age and maternal education levels, the 2 FXS groups did not differ in utterance length or syntactic complexity. The FXS groups and the DS group produced shorter, less complex utterances overall and less complex noun phrases, verb phrases, and sentence structures than did the TD boys. The FXS with ASD group and the DS group, but not the FXS-only group, produced less complex questions/negations than did the TD group. Compared with the DS group, both FXS groups produced longer, more complex utterances overall, but on the specific complexity measures, they scored higher only on questions/negations. CONCLUSION: Boys with FXS and DS have distinctive language profiles. Although both groups demonstrated syntactic delays, boys with DS showed greater delays.

Language and communication development in Down syndrome.

Although there is considerable variability, most individuals with Down syndrome have mental retardation and speech and language deficits, particularly in language production and syntax and poor speech intelligibility. This article describes research findings in the language and communication development of individuals with Down syndrome, first briefly describing the physical and cognitive phenotype of Down syndrome, and two communication related domains-hearing and oral motor skills. Next, we describe language development in Down syndrome, focusing on communication behaviors in the prelinguistic period, then the development of language in children and adolescents, and finally language development in adults and the aging period. We describe language development in individuals with Down syndrome across four domains: phonology, semantics, syntax, and pragmatics. Wethen suggest strategies for intervention and directions for research relating to individuals with Down syndrome.

Expressive language during conversational speech in boys with fragile X syndrome.

We compared the expressive syntax and vocabulary skills of 35 boys with fragile X syndrome and 27 younger typically developing boys who were at similar nonverbal mental levels. During a conversational speech sample, the boys with fragile X syndrome used shorter, less complex utterances and produced fewer different words than did the typically developing boys after controlling for their nonverbal MA, speech intelligibility, and mother's education. The boys with fragile X used less complex noun phrases, verb phrases, and sentence structure, but did not use fewer questions and negations. These findings suggest that the language difficulties in boys with fragile X reflect an overall expressive language delay and not a specific syntactic or vocabulary delay.

Articulation rate and vowel space characteristics of young males with fragile X syndrome: preliminary acoustic findings.

PURPOSE: Increased speaking rate is a commonly reported perceptual characteristic among males with fragile X syndrome (FXS). The objective of this preliminary study was to determine articulation rate-one component of perceived speaking rate-and vowel space characteristics of young males with FXS. METHOD: Young males with FXS (n = 38), developmental age (DA)-matched males (n = 21), and chronological age (CA)-matched males (n = 16) were audiotaped while engaged in spontaneous conversation and a picture-naming task. Articulation rate in syllables per second during intelligible utterances and vowel space area/dispersion measures were acoustically determined for each speaker. RESULTS: Males with FXS did not articulate significantly faster than CA-matched males. Area and dispersion of the acoustic vowel space also were similar between the 2 groups. Males with FXS, however, used significantly shorter utterances and had a tendency to pause less often than CA-matched males. In addition, males with FXS exhibited greater intraspeaker variability of formants associated with the vowel /a/. CONCLUSIONS: These preliminary findings suggest that articulation rate may not be a primary factor contributing to perceived speaking rate of males with FXS. Limitations of the study relative to speech production tasks and utterance intelligibility are discussed.

The relationship of otitis media in early childhood to attention dimensions during the early elementary school years.

This study examined the impact of otitis media with effusion (OME) and associated hearing loss between 6 and 48 months of age on attention dimensions (i.e., selective/focus, sustained) during the elementary school years. A prospective cohort design in which 74 African American infants were recruited between ages 6 and 12 months. Ear examinations were done repeatedly using both otoscopy and tympanometry, and hearing was assessed using standard audiometric procedures between 6 and 48 months. Multiple measures of attention (i.e., direct assessment, behavioral observations, parent/teacher ratings) were administered from kindergarten through second grade to assess two theoretical dimensions of attention: selective/focused and sustained. The home environment was assessed annually. Results indicated that neither early childhood OME nor hearing loss showed significant correlations with any of the longitudinal or cross-sectional measures of selective/focused attention and sustained attention. In contrast, children with mothers who had fewer years of education and who lived in less responsive and supportive home environments scored higher on both parent and teacher ratings of sustained attention (i.e., hyperactivity) through the second grade of elementary school. For NEPSY Auditory Attention in second grade, a significant interaction between the Home Observation for Measurement of the Environment and hearing loss was uncovered. This interaction showed that children with hearing loss from poor home environments experienced greater difficulties on the NEPSY Auditory Attention task than those with hearing loss from good home environments. These findings do not support a direct linkage of a history of OME and associated hearing loss to difficulties in selective/focused attention or sustained attention in early elementary school children. Relationships between sociodemographic variables and attention-related functions appear stronger and should be considered as mediators in any examination of the linkages between early OME and subsequent attention functions.

Structural development of the fictional narratives of African American preschoolers.

PURPOSE: This study examined the structural development of African American preschoolers' narratives. It also investigated the effect of background variables (e.g., gender, maternal education, stimulation and responsiveness of the home environment, and whether or not the child lived in poverty) on the children's narratives. METHOD: Sixty-five children completed a story-retelling task at age 4 and again at kindergarten entry. Narratives were then coded for story grammar elements. RESULTS: Four-year-olds narrated some attempts to solve the problem and some elements of the story ending. At kindergarten entry, children had higher total narrative scores and included more of every type of story grammar element except relationship. Overall, narratives were not related to background variables. CLINICAL IMPLICATIONS: The Bus Story Language Test (C. Renfrew, 1991) appears to be an assessment tool that is sensitive to structural growth in African American children's narratives from 4 years to kindergarten entry.

Early otitis media with effusion, hearing loss, and auditory processes at school age.

OBJECTIVES: To examine the effect of conductive hearing loss (HL) secondary to otitis media with effusion (OME) in the first 3 years of life on physiologic, peripheral, and higher-order behavioral auditory measures examined at school age. METHODS: Peripheral hearing sensitivity for conventional and extended high-frequency audiometric ranges, physiologic (distortion product otoacoustic emissions, contralateral and ipsilateral acoustic middle ear muscle reflexes), auditory brain stem response (ABR), and higher-order auditory processing measures (masking level difference; Virtual Auditory Localization, Speech Intelligibility Gain; adaptive Pediatric Speech Intelligibility task) were examined at the end of the second grade of elementary school in two cohorts (North Carolina, N = 73, and New York, N = 59). All participants (mean age, 8 years) were followed prospectively in infancy and early childhood (7 to 39 months) for middle ear status and hearing loss (using pneumatic otoscopy/tympanometry and repeated conditioned behavioral audiometric response procedures). Multivariate analyses were conducted to address whether early OME and early conductive HL were related to physiologic, peripheral, and higher-order auditory processes. RESULTS: Early hearing loss and OME were significantly associated with peripheral hearing at school age; extended high-frequency thresholds accounted for the result. Similarly, hearing loss in early life and OME were significantly associated with the acoustic middle ear muscle reflex: The contralateral stimulation condition accounted for the association. Significant associations with both early OME and early HL were also found for the auditory brain stem response measure and were explained by the correlations between early hearing loss and the ABR Wave V latency but not other ABR indices. There were no reliable associations between either early OME or early HL on any other auditory processes evaluated at the end of second grade. CONCLUSIONS: Extended high-frequency hearing and brain stem auditory pathway measures in childhood were significantly associated with children's experiences with OME and hearing loss from 7 to 39 months of age. However, no significant associations were found for psychoacoustic measures of binaural processing or a behavioral adaptive speech-in-noise test at school age.

Otitis media and speech and language: a meta-analysis of prospective studies.

OBJECTIVE: Considerable controversy surrounds whether a history of otitis media with effusion (OME) in early childhood causes later speech and language problems. We conducted a meta-analysis of prospective studies to determine: 1) whether a history of OME in early childhood is related to receptive language, expressive language, vocabulary, syntax, or speech development in children 1 to 5 years old and 2) whether hearing loss caused by otitis media in early childhood is related to children's receptive language or expressive language through 2 years of age. METHODS: We searched online databases and bibliographies of OME studies and reviews for prospective or randomized clinical trials published between January 1966 and October 2002 that examined the relationship of OME or OME-associated hearing loss in early childhood to children's later speech and language development. The original search identified 38 studies, of which 14 had data suitable for calculating a pooled correlation coefficient (correlational studies) or standard difference between parallel groups (group studies). Random-effects meta-analysis was used to pool data when at least 3 studies had usable data for a particular outcome. RESULTS: We performed 11 meta-analyses. There were no significant findings for the analyses of OME during early childhood versus receptive or expressive language during the preschool years in the correlation studies. Similarly, there were no significant findings for OME versus vocabulary, syntax, or speech during the preschool years. Conversely, there was a significant negative association between OME and preschoolers' receptive and expressive language (lower language) (0.24 and 0.25 standard difference, respectively) in the group studies. Additionally, hearing was also related to receptive and expressive language in infancy (3%-9% of variance). CONCLUSIONS: Our results indicate no to very small negative associations of OME and associated hearing loss to children's later speech and language development. These findings may overestimate the impact of OME on outcomes, because most studies did not adjust for known confounding variables (such as socioeconomic status) and excluded data not suitable for statistical pooling, especially from methodologically sound studies. Although some OME language differences were detectable by meta-analysis due to increased statistical power, the clinical relevance for otherwise healthy children is uncertain.

Early Intervention Practices and Communication Intervention Strategies for Young Males With Fragile X Syndrome.

PURPOSE: This study describes speech-language pathologists' impressions of the communication difficulties of young males with fragile X syndrome (FXS) and the need for both syndrome-specific and individualized interventions. The findings of a regional study that identified speech-language pathologists' impressions of the speech, language, and behavioral difficulties experienced by males with FXS and an array of interventions used by speech-language pathologists to improve communication skills for these children are reported. METHODS: Fifty-one speech-language pathologists providing intervention for males with FXS ranging in age from 2 to 9 years (mean age=6;3 [years;months]) were interviewed. RESULTS: The majority of the speech-language pathologists reported that boys with FXS exhibit a visually based, experiential or wholistic learning preference. They emphasized the necessity of making environmental accommodations for limited attention span, difficulties with topic and activity transitions, sensory deficits, and low threshold for anxiety. They reported that speech goals focused on slowing rate and increasing precision for verbal children and both low and high levels of assistive technology for nonverbal or minimally verbal children. Language goals focused on listening, auditory comprehension, and narrative/conversation skills. Pragmatic goals emphasized social dialogue, role playing, and topic maintenance. CLINICAL IMPLICATIONS: This study suggests that young males with FXS present the clinician with a constellation of behaviors and communication impairments that are both syndrome specific and symptom familiar. The specific communication strengths and deficits described by clinicians working with these children are common to many children with speech and language impairments compounded by cognitive deficits. Intervention programs for young boys with FXS should also attend closely to the specific behavioral (e.g., increased anxiety, attention deficits) and sensory "overload" problems they often exhibit before designing a tailored intervention program.

Otitis media in early childhood in relation to children's school-age language and academic skills.

OBJECTIVE: To examine whether otitis media with effusion (OME) and associated hearing loss during the first 4 years of life are related to the language development and academic achievement of children between 4 years of age and second grade. METHODS: In a prospective cohort study, 83 black children, primarily from low-income families and recruited from community-based child care programs, were examined repeatedly between the ages of 6 months and 4 years for the presence of OME and hearing loss, both when well and when ill with OME. Children's child-rearing environments at home were assessed annually from infancy through second grade, whereas children's language and academic skills were assessed repeatedly between 4 years of age and second grade. RESULTS: We did not find in our longitudinal analyses a relationship between OME and hearing loss during the first 4 years of life and later academic skills in early reading and recognition of words heard. We did find that children with greater incidence of OME and hearing loss during the first 4 years of life scored lower in verbal math problems between kindergarten and second grade, even after partialing out important background factors. Children with more OME tended to score lower in math at the younger ages but caught up once they entered school. Follow-up analyses indicated also that children with more OME during the first 2 years of life scored lower in expressive language during the preschool and early elementary school years but caught up by second grade. In contrast, children from homes that were rated as more stimulating and responsive scored higher on every measure of language and academic skills than did children from less responsive homes. The home environment was related more strongly to all of the outcomes examined than was OME or hearing loss. CONCLUSIONS: There was no evidence of a significant relationship between a history of OME or hearing loss and children's later academic skills in reading or word recognition during the early elementary school years. Children with greater incidence of OME and hearing loss scored lower in math and expressive language at the younger ages but caught up in math with their peers on entering school and in expressive language by second grade. Furthermore, a child's home environment was more related to early math and expressive language skills than was OME or hearing loss, and the home environment continued to be predictive of all of the language and academic outcomes through second grade. These study results should be interpreted cautiously when generalizing to other populations.

A longitudinal study of risk factors for otitis media in African American children.

OBJECTIVE: Otitis media with effusion (OME) is a common health care problem for children. The purpose of this study was to examine factors that place children at risk for OME such as age, type of child care, number of people in the household, and smoking in the household. METHODS: Eighty-six African American children, enrolled in center-based child care in infancy, entered the study at a mean age of 8.2 months and were followed prospectively until 48 months of age. Ear status was documented biweekly using pneumatic otoscopy and tympanometry. Data on risk factors were collected every 6 months. RESULTS: Results indicated that children had a marked decrease in the proportion of time with OME between 6 and 48 months. The rate of OME decline was faster in the first 2.5 years than in subsequent years. Children in center-based child care showed a slightly slower rate of decline than did children in non-center-based care. Longitudinal analysis indicated that the age of the child and the number of other children in the household were significant predictors of OME. For each additional child under 12 years of age in the home, there was a 2% increase in the proportion of time with OME. CONCLUSION: While attendance in group child care predicted a risk for OME, children's age and the number of other children in the household were still contributing risk factors for OME.