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Otolaryngologic features of Laurence-Moon-Bardet-Biedl syndrome.

The most common otolaryngologic features associated with LMBBS include SNHL, speech and language disorders, and oral and dental abnormalities. Early otolaryngologic, audiologic, speech pathology, and dental evaluation of these individuals is recommended. This is the first reported case of bifid epiglottis, a rare congenital laryngeal anomaly, found in association with LMBBS. Most patients with bifid epiglottis have additional congenital anomalies, most commonly polysyndactyly. Polysyndactyly is a feature of both LMBBS and bifid epiglottis and may be an early hallmark for the presence of other congenital anomalies.

Child↗

Assessing morphosyntax in Spanish-speaking children.

Accurate assessment of the morpho-syntactic skills of Spanish-speaking children depends on the clinician's understanding of the morpho-syntactic system and on the development of tasks that obligate the use of structures of interest. In this article, the nature and acquisition of the Spanish morpho-syntactic system is outlined. The aspects of the system that are likely to be difficult for children with language impairments and those that are critical to communicative competence are emphasized, as the clinician must take these into account when planning assessment tasks. The analysis of spontaneous language samples and the use of structured probes are discussed as alternatives for assessment. The naturalness and linguistic demands of assessment tasks are also considered because they are critical to understanding children's performance on morpho-syntactic tasks.

Adult↗

Reading emotion cues: social communication difficulties in pediatric populations.

Speech-language pathologists frequently address social communication difficulties in children with diverse linguistic profiles. Of consequence to effective management of social communication skills is that some children with language disorders may also have difficulty understanding emotional cues. The ability to recognize and comprehend the emotional meaning of messages is accomplished through integration of linguistic cues (e.g., what the speaker says), nonlinguistic cues (e.g., the speaker's facial expressions), and situational cues (e.g., predicting how the speaker is likely to feel about the particular topic). This article explores children's comprehension of emotion as expressed through facial expressions and situational cues. First, development of emotion comprehension in children with normal development is summarized. This is followed by a brief review of studies investigating emotion comprehension in clinical populations. Suggestions for assessment and intervention of children's emotion comprehension skills are presented.

Affect↗

Diagnosing language variations: underlying principles for syntactic assessment.

The assessment of complex aspects of children's syntactic development can be carried out in a dialect-neutral fashion. The item types proposed for this purpose encompass wh-questions, passives, and articles, and test the child's understanding of implicit grammatical relations, the rules governing syntactic movement, and discourse linking. We present the rationale for the items and the specific research supporting them, as well as some suggestions for how to help children who fail on the concepts to gain a better understanding of them.

Child, Preschool↗

Assessing pragmatic skills in elicited production.

In developing a test of pragmatic skills for children ages 4 to 9 years, we focused on a number of functional language skills that are important for children's success in early schooling and for the development of fluent reading and writing. They included (1) wh-question asking, (2) communicative role taking, (3) linking events in a cohesive narrative, and (4) articulating the mental states of the characters in a story. All of the proposed items provide specific referential support and pragmatic motivation for the forms and content to be produced by the child. The pictured materials and elicitation prompts constrain the range of appropriate utterances, so the children's productions are more easily scored than an open-ended spontaneous speech sample. All tasks described show a clear developmental trend, a clear separation between the performance of typically developing and language-impaired children, and no performance differences between African American English- and Mainstream American English-speaking children.

Child↗

Cultural and linguistic fairness in the assessment of semantics.

In creating a semantic assessment that will be linguistically and culturally fair, it is important to avoid the bias of acquired vocabulary tests. This article describes techniques to assess children's processing of new words, their lexical organization and retrieval, which may be more significant than the number of words known. Special properties of the quantifier every also give an idea of the child's level of semantic ability. In addition, we examine how the child's performance on each item-type helps the speech-language pathologist determine the sources of children's problems in this domain.

Cultural Diversity↗

Developing study, organization, and management strategies for adolescents with language disabilities.

Developing strategies for learning is important for all individuals, but even more so for students who have difficulty learning and processing information. Because many students with language disabilities have not developed a repertoire of such strategies or do not actively apply the strategies they know, direct instruction in this area is imperative. It is also important that any strategy taught is functional, easily and directly applied to present learning situations, and easy to generalize. Determining the variables in a learning situation (the script) should provide appropriate choices and direction for strategic teaching and learning. The acquisition of strategies involves direct, explicit teaching and requires time for modeling, practice, and discussion. Using a cognitive strategy model is critical because it supports the later use and generalization of strategies, but it is important to explore various models for strategy instruction and to work collaboratively with students and teachers. The SLP can provide direct instruction, consultation, or training, or can facilitate a collaborative model among professionals. In so doing, her impact may reach throughout a life, because learning strategies are life strategies, and if learned they are tools forever.

Adolescent↗

Neural deficits in children with dyslexia ameliorated by behavioral remediation: evidence from functional MRI.

Developmental dyslexia, characterized by unexplained difficulty in reading, is associated with behavioral deficits in phonological processing. Functional neuroimaging studies have shown a deficit in the neural mechanisms underlying phonological processing in children and adults with dyslexia. The present study examined whether behavioral remediation ameliorates these dysfunctional neural mechanisms in children with dyslexia. Functional MRI was performed on 20 children with dyslexia (8-12 years old) during phonological processing before and after a remediation program focused on auditory processing and oral language training. Behaviorally, training improved oral language and reading performance. Physiologically, children with dyslexia showed increased activity in multiple brain areas. Increases occurred in left temporo-parietal cortex and left inferior frontal gyrus, bringing brain activation in these regions closer to that seen in normal-reading children. Increased activity was observed also in right-hemisphere frontal and temporal regions and in the anterior cingulate gyrus. Children with dyslexia showed a correlation between the magnitude of increased activation in left temporo-parietal cortex and improvement in oral language ability. These results suggest that a partial remediation of language-processing deficits, resulting in improved reading, ameliorates disrupted function in brain regions associated with phonological processing and produces additional compensatory activation in other brain regions.

Brain↗

Multiple domains define the expression and regulatory properties of Foxp1 forkhead transcriptional repressors.

The Foxp subfamily of forkhead/HNF3 transcription factors has recently been recognized because of its involvement in autoimmune disease, speech and language disorders, and lung development. Domains unique to this subfamily include a divergent DNA-binding winged helix, a leucine zipper, a zinc finger, and a polyglutamine tract. Little is known about the properties of these proteins that are fundamental to their function as transcription factors nor how the Foxp sequence motifs regulate their transcriptional regulatory properties. We report here a structure/function analysis of the Foxp1 protein. We have analyzed the alternative splice isoforms 1A and 1C and also report the cloning and characterization of a novel isoform Foxp1D that lacks the polyglutamine domain. We have isolated the preferred DNA-binding sites for Foxp1 transcription factors. Foxp1A, C, and D isoforms and the related Foxp2 protein repress gene transcription via binding to this consensus site or to a naturally occurring site within the SV40 and the interleukin-2 promoters. In some cases the strength of Foxp1 repression is mediated by the polyglutamine domain. Unlike previously characterized forkhead factors, Foxp1 proteins can form homodimers or heterodimers with subfamily members. The dimerization domain was localized to an evolutionarily conserved C2H2 zinc finger and leucine zipper motif. Finally, we demonstrate that Foxp1, although broadly expressed, is further regulated by tissue-specific alternative splicing of these functionally important sequence domains. These results suggest that Foxp1 proteins have diverse functional roles in different cell and tissue types.

Alternative Splicing↗

Normative data for clustering and switching on verbal fluency tasks.

Normative data for clustering and switching on verbal fluency tasks are provided. Four hundred and eleven healthy adults between the ages of 18 and 91 were given tests of phonemic fluency (FAS or CFL) and semantic fluency (Animals and Supermarket). Raw scores were corrected for demographic (i.e., age, education, and sex) and test (i.e., fluency form) variables that were determined to make sizable contributions to fluency performance. These normative data should be useful for clinicians and researchers in determining the nature of the fluency impairment in any given individual.

Adult↗

Language functions in patients with multiple sclerosis.

Few studies have demonstrated language impairment in patients with multiple sclerosis (MS) compared to healthy controls. The purpose of this investigation was to examine language functions, specifically naming, comprehension, and verbal fluency, in patients with MS and healthy controls to (1) determine if patients with MS demonstrate language impairment, and (2) provide clarification of MS-related language disturbance, particularly in patients with MS of differing courses. Results showed that both chronic-progressive and relapsing-remitting patients with MS performed significantly more poorly than controls on naming, aural comprehension, letter fluency, and category fluency, as well as other language-based cognitive measures. Chronic-progressive patients obtained significantly lower scores than relapsing-remitting patients on the latter three tests. The language disturbance in this MS sample may be partly explained by impairment in other verbal cognitive functions. These findings highlight the importance of assessing language abilities in patients with MS.

Adult↗

Digit span in dyslexia: variations according to language comprehension and mathematics skills.

The aim of this study was to investigate digit span performance in dyslexia. It was hypothesised that differences would be found in accordance with subgrouping by language comprehension and mathematic skills, and by analyses of how the digit span scores were attained. Two digit span tasks were given to a group of dyslexic children and controls (n = 57), mean age 12.62 (SD = 1.43). The tasks were "Digit Span" of the WISC-R, and "Digit Span 2," where the use of back-up strategies like finger counting and lip reading were restricted. As expected, the digit span scores were significantly lower in the dyslexia group than in the control group. Restrictions of back-up strategies did not alter the scores in the control group, while the scores were lowered in the dyslexia group. Further analyses of longest digit span, serial recall, and serial position indicated different retrieval patterns in the subgroups. The subgroup with good language comprehension and mathematic skills (n = 12), showed impaired serial recall especially in backward recall. The subgroup with good language comprehension skills, but with mathematics impairment (n = 9), showed impaired serial recall in both forward and backward recall. The subgroup with language impairments (n = 16), recalled fewer digits than the two other subgroups. The findings were discussed in relation to the "Phonological loop" of the Multi Component Model of Working Memory, and implications for intervention were discussed.

Adolescent↗

Test performance of language-handicapped children with familiar and unfamiliar examiners.

The importance of examiner familiarity to children's performance on tasks requiring high or low levels of symbolic mediation was investigated. Thirty-four handicapped preschool children were examined within a repeated measures crossover design, once by one of two familiar classroom teachers and once by one of four strange teachers. Subjects performed significantly better with familiar than with unfamiliar examiners on high symbolic mediation tasks; no such differential performance was obtained on low symbolic mediation items. Findings are related to current efforts to identify procedural and situational variables in assessment, uncontrolled by present standard test administrations, that may preclude children's optimal performance.

Child, Preschool↗

Profile of handicap situations in children with cerebral palsy.

The purposes of this study were to establish a profile of handicap situations in children with cerebral palsy and to identify some variables associated with the occurrence of these situations. Ninety-eight children with a diagnosis of cerebral palsy (mean age +/- 1 SD, 10.5 +/- 3.5 years) were recruited on a voluntary basis. The Life Habits Assessment (LIFE-H, version 1.0) was used to measure the degree of accomplishment in 12 categories of life situations (activities of daily living and social roles). Significant disruptions in the accomplishment of all life habit categories were revealed. The highest disruptions were observed in the following categories: recreation, community, personal care, education, mobility, housing and nutrition. Disruption progressed significantly with increased severity of cerebral palsy. Impairment type, severity, speech and language disorders, and comprehension difficulties explained a high percentage of the total variance (> 60%) in the accomplishment of life habits. The results suggest that life habits related to school and social integration are greatly disturbed.

Activities of Daily Living↗

Evaluation of a typology of reading disability.

A typology of reading disability was derived from a cluster analysis of psychological and educational tests administered to 63 moderately to severely reading-disabled children and adolescents. The quality of the cluster solution was evaluated through the use of statistics designed to measure the degree to which the clusters were compact and well separated. The values of the evaluation statistics for the reading clusters were compared to the distribution of these statistics in a sample of 100 cluster solutions from comparable simulated data sets. The results of this comparison suggest that the derived reading-disability clusters represent true subtypes rather than a random partitioning of a homogeneous group of subjects. The commonalities among subjects in the same clusters appear to be such as to permit hypotheses about treatment based on cluster membership. The clusters included a language disorder with associated deficits in auditory memory and sound blending, an auditory processing disorder, a mixed auditory and visual processing disorder, and an auditory memory and visual processing disorder. This study did not find the "normal" and visual-perceptual subtypes reported in some other recent investigations.

Adolescent↗

Effects of a cycled morphological intervention on selected suppletive BE forms.

The purpose of this study was to demonstrate the efficacy of a cycled morphological intervention involving copula is, by comparison to a control group, and to examine the effectiveness of this intervention in producing change in untreated auxiliary is and am. Twenty-two preschool children, who produced copula is but no other suppletive forms of BE as targets, received six sessions of naturalistic intervention cycled over 24 weeks. Production accuracy levels for treated copula and untreated auxiliary is and am were obtained from spontaneous language samples elicited after 12 and 24 weeks of intervention. The cycled intervention resulted in significant improvement in copula is in comparison to the no-treatment control group at the 12 week sampling point; similar gains were observed for the untreated auxiliary is, but not am. After 24 weeks, however, gains in untreated am were significantly greater than for copula is, providing new data to suggest that generalization may also be observed for the suppletive am.

Child↗