A Síndrome Velocardiofacial (SVCF) é uma afecção genética caracterizada por múltiplas anomalias congênitas tais como, fissura de palato submucosa e/ou disfunção velofaríngea, malformações cardiovasculares, face típica, distúrbio de aprendizagem envolvendo a capacidade de abstração, raciocínio matemático e habilidade viso-motora, dentre outros. As alterações da fala e linguagem, estão entre as manifestações clínicas mais comuns dessa síndrome. A aquisição e desenvolvimento da linguagem e aprendizagem requerem processos neuropsicolingüísticos dentre os quais destacam-se a atenção e a memória. Com base na literatura estudada e na observação clínica desses indivíduos, este estudo tem por objetivo caracterizar as habilidades de atenção auditiva e de memória de trabalho fonológica (MTF) em 6 crianças com a SVCF, de ambos os sexos com faixa etária de 9,2 a 11,7 anos por meio de testes específicos. de atenção e MTF. Os resultados demonstram alterações nos seguintes testes; THAAS (Teste de Habilidade de Atenção Auditiva Sustentada) 3(50%) alterado, 3(50%) normal; DNVSA (dicótico nãoverbal sem atenção direcionada) 2 (33%) normal, 4 (67%) alterado; DNVA (dicótico não-verbal com atenção à direita) 1(17%) normal, 5(83%) alterado, DNVE (dicótico não-verbal com atenção à esquerda), 2 (33%) normal, 4 (67%) alterado, DD (Teste Dicótico de Dígitos) 1(17%) normal, 5(83%) alterado, OD (Dígitos na ordem direta) 6 (100%) normal, OI (Dígitos Ordem Inversa) 3 (50%) alterado, BCPR (Teste de Repetição de Pseudopalavras para Crianças Brasileiras), 6(100%) normal. Como conclusão pode-se verificar que as crianças com SCVF apresentam alterações de atenção no que se refere as habilidades de sustentar e selecionar a atenção, porém a capacidade de selecionar a atenção parece estar mais afetada para esse grupo. / Velocardiofacial syndrome ( VCFS) is a disorder presenting multiple congenital anomalies and the major one are cleft palate and/or velopharyngeal dysfunction, cardiac malformations, facial dysmorphism, learning disability including visual-motor ability, abstract and mathematical reasoning. Speech and language disorders are among the most common VCFS clinical features. Both acquisition and development of language and learning domains require neuropsycholinguistic processes being attention and memory highlighted. Based on the literature review and on the clinical observation of VCFS individuals, this paper aims at presenting auditory attention and phonological working memory (FWM) abilities of 6 VCFS children aged 9, 2 to 11,7. Specific tests were administered in order to evaluate FWM and attention abilities. The results have shown alterations of the sustained attention test in 3 (50%) individuals; NVDNDA (non-verbal dichotic, non-directed attention) 2 (33%) normal, 4 (67%) altered; NVDAR (non-verbal dichotic, attention to the/at the right) 1 (17%) normal, 5 (83%) altered; NVDAL ( non-verbal dichotic, attention to left) 2 (33%) normal, 4 (67%) altered, DDT (Digit Dichotic Test) 1 (17%) normal 5 (83%) altered, DOD (Direct Order Digit ) 6 (100%) normal, I O D (Inverse Order Digit ) 3 (50%) altered, BCPR (Brazilian Children Pseudoword Repetition), 6 (100%) normal. In summary, it is possible to show that children with known VCFS present with disorders of attention concerning their abilities to select and sustain attention; nonetheless, the VCFS group selection ability seems to be more affected.

A Síndrome Velocardiofacial (SVCF) é uma afecção genética caracterizada por múltiplas anomalias congênitas tais como, fissura de palato submucosa e/ou disfunção velofaríngea, malformações cardiovasculares, face típica, distúrbio de aprendizagem envolvendo a capacidade de abstração, raciocínio matemático e habilidade viso-motora, dentre outros. As alterações da fala e linguagem, estão entre as manifestações clínicas mais comuns dessa síndrome. A aquisição e desenvolvimento da linguagem e aprendizagem requerem processos neuropsicolingüísticos dentre os quais destacam-se a atenção e a memória. Com base na literatura estudada e na observação clínica desses indivíduos, este estudo tem por objetivo caracterizar as habilidades de atenção auditiva e de memória de trabalho fonológica (MTF) em 6 crianças com a SVCF, de ambos os sexos com faixa etária de 9,2 a 11,7 anos por meio de testes específicos. de atenção e MTF. Os resultados demonstram alterações nos seguintes testes; THAAS (Teste de Habilidade de Atenção Auditiva Sustentada) 3(50%) alterado, 3(50%) normal; DNVSA (dicótico nãoverbal sem atenção direcionada) 2 (33%) normal, 4 (67%) alterado; DNVA (dicótico não-verbal com atenção à direita) 1(17%) normal, 5(83%) alterado, DNVE (dicótico não-verbal com atenção à esquerda), 2 (33%) normal, 4 (67%) alterado, DD (Teste Dicótico de Dígitos) 1(17%) normal, 5(83%) alterado, OD (Dígitos na ordem direta) 6 (100%) normal, OI (Dígitos Ordem Inversa) 3 (50%) alterado, BCPR (Teste de Repetição de Pseudopalavras para Crianças Brasileiras), 6(100%) normal. Como conclusão pode-se verificar que as crianças com SCVF apresentam alterações de atenção no que se refere as habilidades de sustentar e selecionar a atenção, porém a capacidade de selecionar a atenção parece estar mais afetada para esse grupo. / Velocardiofacial syndrome ( VCFS) is a disorder presenting multiple congenital anomalies and the major one are cleft palate and/or velopharyngeal dysfunction, cardiac malformations, facial dysmorphism, learning disability including visual-motor ability, abstract and mathematical reasoning. Speech and language disorders are among the most common VCFS clinical features. Both acquisition and development of language and learning domains require neuropsycholinguistic processes being attention and memory highlighted. Based on the literature review and on the clinical observation of VCFS individuals, this paper aims at presenting auditory attention and phonological working memory (FWM) abilities of 6 VCFS children aged 9, 2 to 11,7. Specific tests were administered in order to evaluate FWM and attention abilities. The results have shown alterations of the sustained attention test in 3 (50%) individuals; NVDNDA (non-verbal dichotic, non-directed attention) 2 (33%) normal, 4 (67%) altered; NVDAR (non-verbal dichotic, attention to the/at the right) 1 (17%) normal, 5 (83%) altered; NVDAL ( non-verbal dichotic, attention to left) 2 (33%) normal, 4 (67%) altered, DDT (Digit Dichotic Test) 1 (17%) normal 5 (83%) altered, DOD (Direct Order Digit ) 6 (100%) normal, I O D (Inverse Order Digit ) 3 (50%) altered, BCPR (Brazilian Children Pseudoword Repetition), 6 (100%) normal. In summary, it is possible to show that children with known VCFS present with disorders of attention concerning their abilities to select and sustain attention; nonetheless, the VCFS group selection ability seems to be more affected.

京都大学 / 0048 / 新制・課程博士 / 博士(医学) / 甲第20967号 / 医博第4313号 / 新制||医||1026(附属図書館) / 京都大学大学院医学研究科医学専攻 / (主査)教授 村井 俊哉, 教授 松田 秀一, 教授 安達 泰治 / 学位規則第4条第1項該当 / Doctor of Medical Science / Kyoto University / DFAM

Background: This study formed part of a larger longitudinal research study by Jordaan (2009), who tracked the development of language for academic purposes in grade 1- 3 English Additional Language (EAL) and English First Language (EFL) learners attending English only programmes in Gauteng over a period of three years. These learners attended schools in two different educational contexts where there is a marked heterogeneity in the linguistic backgrounds of both the learners and teachers investigated. Results from Jordaan’s (2009) study revealed that some EAL and EFL learners appeared to be slow in the development of their academic language abilities (as no progress was evident over the period of three years), relative to their peers, and thus these learners may have a language impairment. Purpose: This study investigated in detail, these “slow to learn” EAL and EFL learners in the foundation phase, in order to determine whether they have a language impairment and to determine how the language impairment manifests in these learners. Participants: Sixteen learners (5 EFL learners and 16 EAL learners) who demonstrated no improvement in their academic language abilities from grade one to grade two as determined by their performance on the Diagnostic Evaluation of Language Variation Criterion Referenced (DELV-CR) test were the participants of this study. Method: The participants were assessed on the Automated Working Memory Assessment (AWMA) test, a Non Word Repetition test (Dollaghan & Campbell, 1998), a Sentence Repetition test (Redmond, 2005) and the Gray Oral Reading Test (GORT-4). Educators were also asked to rate these learners’ oral language, written language and reading comprehension abilities on a scale of 0- 5. The research design utilized was a non experimental, descriptive quantitative design, involving both correlational and comparative components. The data obtained was then analysed using descriptive and inferential statistics. Pearson correlation coefficients were calculated to establish whether there was a relationship between the cognitive processing and the language proficiency measures as well as the teacher ratings in order to provide information regarding these tests as assessment tools for EAL learners as well as to further enhance the validity of this study. Independent sample t-tests were also conducted to determine whether there were any significant differences between the EFL and EAL learners’ performance in the two different educational contexts, so as to establish whether bilingual learners with language impairment are more severely impaired than monolingual learners with language impairment. Results: Based on the analysis of these learners’ performance on the DELV-CR test, results indicated that all sixteen participants presented with SLI and were not just “slow to learn”. The EAL-SLI learners in both contexts performed poorly on the reading comprehension test and were rated lower than their aged matched peers on the teacher rating scales. Furthermore, when comparing these EAL-SLI learners’ performance on the DELV-CR test to the performance of the EFL-SLI learners, the EAL-SLI learners as a group appeared to have performed more poorly than the EFL-SLI learners on all three subtests. As significant differences were found between the EAL and EFL learners’ performance on the DELV-CR test, the results suggested that bilingual learners with SLI, who acquire a second language sequentially, are more impaired than monolingual learners with SLI. The EAL-SLI learners also presented with visuo-spatial short term and working memory deficits and even though a large majority of the learners presented with verbal short term and working memory difficulties, not all the learners presented with cognitive processing difficulties. This finding has implications for the theories of SLI. However, the sentence repetition task was found to be a useful tool in differentiating between the “slow to learn” EAL learners and EAL-SLI learners and furthermore this test also positively correlated with various sections of the DELV-CR test which adds to the value of this test as an assessment tool in EAL learners. Positive correlations were also found between the teacher ratings of the EAL-SLI learners and the subtests of the DELV-CR test which indicates that teachers have the ability to correctly identify learners with language learning difficulties. Positive correlations were also found between the digit repetition subtest, the non word repetition test and the sentence repetition test which adds to the validity of this study. Conclusion: The results obtained from this study demonstrated that bilingual learners with SLI who acquire a second language sequentially are additionally disadvantaged compared to their EFL-SLI peers in the acquisition of certain aspects of academic language. Furthermore, although research has shown that cognitive processing measures are less biased in the assessment of linguistically diverse learners, results indicated that the DELV-CR test identified more accurately, learners with language impairment whereas the cognitive processing measures provided contradictory and biased results with the verbal working memory subtest over identifying learners “at risk” for language impairment. Finally, the use of sentence repetition tasks in the differentiation between “slow to learn” and language impaired EAL learners proves to be promising.

The presented diploma thesis is focused on a special pedagogical topic and deals with the phonological abilities of preschool children. The diploma thesis is structured into theoretical and practical part. In the theoretical part the reader is introduced with the characteristics of preschool children, principles of the school education system in the Czech Republic based on current legislation, the school's maturity and readiness and the diagnosis of preschool children. The work also presents various divisions of the ontogenesis of speech, language and communication and characterizes individual language levels. The work also describes the development of phonological abilities and introduces phonological abilities in the context of reading literacy. A separate subchapter is devoted to possible diagnostic tools for the diagnosis of phonological abilities and another subchapter is about the Batteries of tests of phonological abilities by Gabriela Seidlová Málková and Markéta Caravolas which was performed in the empirical part. In the empirical part we deal with the analysis of phonological abilities of preschool children. A qualitatively oriented research was made in two classes of two kindergartens. The chosen methods of thesis were observation, analysis of the results of activities and analysis of...

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