Consequences of severe visual-spatial deficits for reading acquisition: Evidence from Williams syndrome

To further understand the nature of the visual-spatial representations required for successful acquisition of written language skills, we investigated the written language abilities of two individuals with Williams syndrome (WS) a developmental genetic disorder in which the presence of severe visual-spatial developmental delays and deficits has been well established. Using a case study approach, we examined the relationship between reading achievement and general cognitive ability, phonological skills, and visual-spatial skills for the two individuals. We found that, despite the strong similarity between the two individuals in terms of their verbal and non-verbal cognitive abilities and their phonological abilities (as well as chronological age and educational opportunities), their reading and spelling abilities differed by more than 5 grade levels. We present evidence that the difference in written language performance was likely to be due to differences in the severity and nature of their visual-spatial impairment. Moreover, we show that specific difficulty processing the orientation of visual stimuli is related to the reading difficulties of one of the two individuals. These results underscore the contribution of visual-spatial abilities to the reading acquisition process and identify WS as a potential source of valuable information regarding the role of visual-spatial processing in reading development.     

Foundations of phonological awareness in pre-school children with cerebral palsy: the impact of intellectual disability

Background Children with cerebral palsy (CP) and accompanying disabilities are prone to reading difficulties. The aim of the present study was to examine the foundations of phonological awareness in pre‐school children with CP in comparison with a normally developing control group. Rhyme perception was regarded as an early indicator of phonological awareness, whereas non‐verbal reasoning, speech ability, auditory perception, auditory short‐term memory and vocabulary were regarded as foundation measures. Methods A number of tasks were administrated to examine group differences in rhyme perception and its foundation measures. Correlations between the tasks were analysed for both groups followed by multiple regression analyses wherein rhyme perception was predicted by its foundation measures. Results Children with CP scored below their normally developing peers on emergent phonological awareness and its foundation measures. Regarding the prediction of phonological awareness, non‐verbal reasoning followed by pseudoword articulation, were found to predict phonological awareness, i.e. rhyme perception, in the group of children with CP. In the control group, auditory perception was a significant predictor of emergent phonological awareness. The CP group was further split up into two groups according to the children's non‐verbal reasoning skills, i.e. general IQ. The below‐average IQ group scored below the average IQ group on phonological awareness and on most foundation measures. In addition, the average IQ group of the children with CP scored lower than the control group. Conclusion The results of this study indicate that general intelligence and speech ability (i.e. pseudoword articulation) can be seen as important facilitators of emergent phonological awareness in children with CP. These findings support the role of intelligence in the emergence of phonological awareness in children with CP. Children with CP with intellectual disabilities seem to have a disadvantage in acquiring phonological awareness, especially when their speech abilities are also impaired. However, general intelligence is not enough to predict phonological awareness as other foundation measures are also important for phonological awareness independent of general intelligence.     

Visual-perceptual and phonological factors in the acquisition of literacy among children with congenital developmental coordination disorder

Much research has shown that children with congenital developmental coordination disorder (CDCD) have marked impairments in the perception of visual-spatial information, a deficit which has been assumed to be causally related to difficulties that many CDCD children experience when learning to read and spell. However, current research in reading disability suggests that poor reading is mainly related to difficulties with the processing of phonological information or with metaphonological ability, not to visual-perceptual deficits. This study aimed to explore the relationship between reading achievement and the visual-perceptual, phonological and general cognitive ability of CDCD children. Twenty-eight children with a mean age of 8.3 years were tested on motor coordination, language, general ability, visual discrimination, visual-motor integration, phoneme awareness, phoneme discrimination, reading and spelling. Results showed that phoneme awareness was the most strongly related to reading and spelling performance, and that visual discrimination showed no relationship to reading ability, although it was related to spelling performance, phonological awareness, and motor coordination. These results suggest that CDCD children who have difficulty with the acquisition of literacy may suffer from visual-perceptual problems and metaphonological deficits, but these problems. are differentially related to reading and spelling.     

The profile of very preterm children on academic achievement. A cross-population comparison with children with specific learning disorders

BackgroundVery preterm (VPT) children showed delays in reading, spelling and maths, but their academic achievement profile is not clearly understood.AimsVPT children were compared with children with specific learning disorders (SLD) and typically developing (TD) children on academic achievement, considering cognitive and linguistic phenotypic markers. A learning profile analysis was also performed.MethodsWe included 170 10-year old monolingual Italian-speaking children (37 VPT, 28 SLD, 105 TD) assessing cognitive, linguistic and academic skills.ResultsOn academic achievements VPT children fell behind TD peers in some reading (text speed, comprehension), spelling (non-word), and math (number knowledge, written calculations and problem-solving) tasks. SLD children underperformed in all academic tasks with respect to VPT and TD peers. Concerning cognitive and linguistic phenotypic markers, compared to TD peers, VPT children showed lower scores in verbal IQ and phonological fluency, SLD children in phonological processing and rapid automatized naming. VPT children showed a higher rate of at-risk performance in reading compared to TD group, but a minor percentage of impaired profiles and comorbidity among learning areas compared to SLD group.Conclusions and implicationsThe academic achievement profile of VPT children shows persistent delays, but it differs to that of SLD children, since delays are less widespread and severe, and differences were found in phenotypic markers and comorbidity. Follow-up programs and effective interventions are needed for VPT children.     

Phonological awareness in children with Down syndrome

Research in the area of phonological awareness has mainly focused on the nature of the relationship between reading ability and awareness of phonemes. However, a recent study of phonological awareness in children with Down syndrome questioned the existence of any necessary relationship (Cossu, Rossini & Marshall, 1993). This paper describes a study of phonological awareness in children with Down syndrome with varying levels of reading ability. The sample consisted of 10 male and 7 female children with Down syndrome (aged 9 years 2 months to 14 years 5 months). All children received a battery of tests which consisted of assessments of: 1) phonological awareness, 2) reading and spelling competence, 3) non-word reading and spelling ability, and 4) non-verbal measures. Children with Down syndrome demonstrated measurable levels of phonological awareness. Significant positive correlations were found between phonological awareness and: reading and spelling competence, ability to spell non-words and non-verbal measures.     

The influence of manifest strabismus and stereoscopic vision on non-verbal abilities of visually impaired children

This research was conducted in order to examine the influence of manifest strabismus and stereoscopic vision on non-verbal abilities of visually impaired children aged between 7 and 15. The sample included 55 visually impaired children from the 1st to the 6th grade of elementary schools for visually impaired children in Belgrade. RANDOT stereotest and polaroid glasses were used for the examination of stereoscopic vision, while Cover test and Hirschberg's pupils reflex test were used for the evaluation of strabismus. In the area of non-verbal abilities was evaluated visual discrimination, visuomotor integration, constructive praxia, visual memory, strategy formation, non-verbal reasoning and the representational dimension of drawings. Subtests of ACADIA test of developmental abilities were used for the evaluation of non-verbal abilities (Atkinson et al., 1972). Statistically significant relations between strabismus and constructive praxia (p = 0.009), visual memory (p = 0.037), strategy formation (0.040) and the quality of drawings were determined by the results analysis. According to our findings, children with divergent strabismus achieve the best results. Children with stereoscopic vision generally achieve better results in all the examined areas of non-verbal abilities, and statistically significant relations were determined in the areas of visuomotor coordination (0.002), constructive praxia (0.026) and non-verbal reasoning (0.015), which are directly connected to visuospatial abilities. Children with convergent strabismus achieve significantly lower results in the areas of constructive praxia, visual memory, strategy formation and representational dimension of drawings, and children with the lack of stereoscopic vision - in the areas of visuomotor integration, constructive praxia and non-verbal reasoning.     

Auditory processing and speech perception in children with specific language impairment: relations with oral language and literacy skills

This longitudinal study investigated temporal auditory processing (frequency modulation and between-channel gap detection) and speech perception (speech-in-noise and categorical perception) in three groups of 6 years 3 months to 6 years 8 months-old children attending grade 1: (1) children with specific language impairment (SLI) and literacy delay (n = 8), (2) children with SLI and normal literacy (n = 10) and (3) typically developing children (n = 14). Moreover, the relations between these auditory processing and speech perception skills and oral language and literacy skills in grade 1 and grade 3 were analyzed. The SLI group with literacy delay scored significantly lower than both other groups on speech perception, but not on temporal auditory processing. Both normal reading groups did not differ in terms of speech perception or auditory processing. Speech perception was significantly related to reading and spelling in grades 1 and 3 and had a unique predictive contribution to reading growth in grade 3, even after controlling reading level, phonological ability, auditory processing and oral language skills in grade 1. These findings indicated that speech perception also had a unique direct impact upon reading development and not only through its relation with phonological awareness. Moreover, speech perception seemed to be more associated with the development of literacy skills and less with oral language ability.     

Early phonological awareness and reading skills in children with Down syndrome

Increasingly, children with Down syndrome receive literacy instruction with the expectation of acquiring functional reading skills. Unfortunately, little is known about the processes underlying literacy skills in this special population. Phonological awareness contributes to literacy development in typically developing children, however, there is inconclusive evidence about these skills in younger children with Down syndrome. 9 children with Down syndrome (5.6-8.10 years) participated in this investigation. Due to the paucity of standardised phonological awareness measures for children with special needs, in particular children with Down syndrome, a variety of tasks were adapted from the literature. The assessment battery examined the skills of phonological awareness, literacy, speech production, expressive language, hearing acuity, speech perception, and auditory-visual memory. The results suggest that children with Down syndrome are at risks for reading acquisition difficulties due to reduced phonological awareness skills. These deficits are in addition to delays caused by reduced cognitive skills. Only one of the participants was able to demonstrate rhyme awareness, which may have been due to task effects. Written word recognition ability was correlated with tests of phonemic awareness, and error analysis of the spelling and non-word reading tasks suggested grapheme-phoneme connections deficits. Further research is needed to determine the best methods of assessment and intervention for phonological awareness in children with Down syndrome.     

Language skills and nonverbal cognitive processes associated with reading comprehension in deaf children

The main aim of this study was to examine the relationship between language skills (vocabulary knowledge and phonological awareness), nonverbal cognitive processes (attention, memory and executive functions) and reading comprehension in deaf children. Participants were thirty prelingually deaf children (10.7 ± 1.6 years old; 18 boys, 12 girls), who were classified as either good readers or poor readers by their scores on two reading comprehension tasks. The children were administered a rhyme judgment task and seven computerized neuropsychological tasks specifically designed and adapted for deaf children to evaluate vocabulary knowledge, attention, memory and executive functions in deaf children. A correlational approach was also used to assess the association between variables. Although the two groups did not show differences in phonological awareness, good readers showed better vocabulary and performed significantly better than poor readers on attention, memory and executive functions measures. Significant correlations were found between better scores in reading comprehension and better scores on tasks of vocabulary and non-verbal cognitive processes. The results suggest that in deaf children, vocabulary knowledge and nonverbal cognitive processes such as selective attention, visuo-spatial memory, abstract reasoning and sequential processing may be especially relevant for the development of reading comprehension.     

Visual dysfunction is associated with cognitive impairment in Parkinson's disease

IntroductionVisual dysfunction and cognitive impairment are common in Parkinson's disease (PD) but the precise contribution of lower-level visual impairment to visual-input based cognitive performance has not been extensively characterized in PD.MethodsWe included 49 PD patients and 22 healthy controls (HC). Lower-level visual function tests [high and low contrast visual acuity (HCVA and LCVA) and contrast sensitivity (CS)] and a neuropsychological battery (involving visual cognition) were performed. Pairwise correlations between lower-level visual functions and visual cognition were computed and stepwise linear regressions were fitted introducing age, Geriatric Depression Scale, and lower-level visual functions in the model to calculate their predicted effect on visual cognition.ResultsCompared to controls, patients presented a significant impairment in all cognitive domains (visual attention, visual processing speed and visual perception, visuospatial abilities, visuoconstructive abilities, and visual memory), and lower-level visual functions. HCVA and LCVA were significantly associated with visual cognition in PD. HCVA explained up to 49.3% and 34.2% of the variability in visual perception and visuospatial abilities, respectively, whereas LCVA was mainly associated with short- and long-term visual memory and visuospatial abilities.ConclusionLower-level visual dysfunction is highly associated with cognitive performance in PD, when cognitive tests are based on visual input. Our results support that lower-level visual functions should be considered when assessing cognitive status of PD patients and might be useful for predicting cognitive deterioration.     

Acquisition of new words and phonological working memory in Williams syndrome: A case study

Abstract

We report the case of a 20-year-old woman, CS, with Williams syndrome, who showed particularly well developed language abilities (especially lexical abilities) in spite of severe visuospatial and visuoconstructive deficits. In addition, CS demonstrated a (quasi-)normal functioning of phonological short-term memory. Finally, she performed normally on a paired-associate word-non-word learning task, while being dramatically impaired in the memorization of word-word pairs. These findings corroborate the existence of a link between the phonological working memory and long-term phonological learning of new words. They also confirm that the acquisition of the phonological form of new words may occur even in the presence of a defective verbal episodic memory.     

Inflectional spelling deficits in developmental dyslexia

The goal of this study was to examine past-tense spelling deficits in developmental dyslexia and their relationship to phonological abilities, spoken morphological awareness and word specific orthographic memory. Three groups of children (28 9-year-old dyslexic, 28 chronological age-matched and 28 reading/spelling age-matched children) completed a battery of tests including spelling regularly inflected words (e.g., kissed) and matched one-morpheme words (e.g., wrist). They were also assessed on a range of tests of reading and spelling abilities and associated linguistic measures. Dyslexic children were impaired in relation to chronological age-matched controls on all measures. Furthermore, they were significantly poorer than younger reading and spelling age-matched controls at spelling inflected verbs, supporting the existence of a specific deficit in past-tense spelling in dyslexia. In addition to under-using the -ed spelling on inflected verbs, the dyslexic children were less likely to erroneously apply this spelling to one-morpheme words than younger controls. Dyslexics were also poorer than younger controls at using a consistent spelling for stems presented in isolation versus as part of an inflected word, indicating that they make less use of the morphological relations between words to support their spelling. In line with this interpretation, regression analyses revealed another qualitative difference between the spelling and reading age-matched group and the dyslexic group: while both spoken morphological awareness and orthographic word specific memory were significant predictors of the accuracy of past-tense spelling in the former group, only orthographic memory (irregular word reading and spelling) was a significant factor in the dyslexic group. Finally, we identified a subgroup of seven dyslexic children who were severely deficient in past-tense spelling. This subgroup was also significantly worse than other dyslexics and than younger controls on scores of orthographic memory. The implications of our findings for teaching and remediation strategies are discussed.     

Early reading and spelling abilities in children with severe speech and physical impairment: a cross-linguistic comparison

Early reading and spelling abilities in children from Ireland and Sweden with severe speech and physical impairment (SSPI) were studied and compared. The aim was to look for similarities as well as for differences that could be related to the different linguistic environments. Both group consisted of 15 children, and were matched on linguistic age. Reading and spelling as well as phonological awareness and memory were tested. Both groups performed fairly well on the phonological awareness tasks, but the Swedish children did not seem to manage to use this ability successfully for reading and spelling. Overall the Irish children showed a slightly stronger performance, and were significantly better at the reading tasks. There were some signs that children were affected by the structure of their individual languages. The Irish children revealed the greatest problems with pseudoword spelling which is in line with results from speaking children with English as their mother tongue and could be a result of using a strategy based on larger linguistic units. The Swedish children had particular problems with tasks presented without oral support, which may be an effect of memory problems due to their lack of articulatory ability.     

Cognitive functions in children with benign childhood epilepsy with centrotemporal spikes (BECTS).

Benign childhood epilepsy with centrotemporal spikes (BECTS) is regarded as a benign form of epilepsy because of its usually favorable outcome, in terms of seizures. Eighteen children with BECTS were studied in terms of neuropsychological and learning abilities: intellectual quotient, oral language (phonological production, naming skills, verbal fluency and syntactic comprehension), drawing and visuo-spatial skills, visual and selective attention, verbal and visuo-spatial memory, reading, numeracy and spelling. The mean IQ of the population was within the normal range, but individual results were heterogeneous. Verbal functions and memory were normal. In contrast, drawing and visuo-spatial skills, attention and visuo-spatial memory were significantly weak compared to the normal range for age. Reading, numeracy and/or spelling ability were significantly delayed by one academic year or more in ten of the children. In conclusion, despite its benign outcome in terms of epilepsy, BECTS can be accompanied by specific cognitive disorders and low academic achievement.     

Reduced sensitivity to slow-rate dynamic auditory information in children with dyslexia

The etiology of developmental dyslexia remains widely debated. An appealing theory postulates that the reading and spelling problems in individuals with dyslexia originate from reduced sensitivity to slow-rate dynamic auditory cues. This low-level auditory deficit is thought to provoke a cascade of effects, including inaccurate speech perception and eventually unspecified phoneme representations. The present study investigated sensitivity to frequency modulation and amplitude rise time, speech-in-noise perception and phonological awareness in 11-year-old children with dyslexia and a matched normal-reading control children. Group comparisons demonstrated that children with dyslexia were less sensitive than normal-reading children to slow-rate dynamic auditory processing, speech-in-noise perception, phonological awareness and literacy abilities. Correlations were found between slow-rate dynamic auditory processing and phonological awareness, and speech-in-noise perception and reading. Yet, no significant correlation between slow-rate dynamic auditory processing and speech-in-noise perception was obtained. Together, these results indicate that children with dyslexia have difficulties with slow-rate dynamic auditory processing and speech-in-noise perception and that these problems persist until sixth grade.     

Electrophysiological study of the basal temporal language area: A convergence zone between language perception and production networks

ObjectiveRegions involved in language processing have been observed in the inferior part of the left temporal lobe. Although collectively labelled ‘the Basal Temporal Language Area’ (BTLA), these territories are functionally heterogeneous and are involved in language perception (i.e. reading or semantic task) or language production (speech arrest after stimulation). The objective of this study was to clarify the role of BTLA in the language network in an epileptic patient who displayed jargonaphasia.MethodsIntracerebral evoked related potentials to verbal and non-verbal stimuli in auditory and visual modalities were recorded from BTLA. Time–frequency analysis was performed during ictal events.ResultsEvoked potentials and induced gamma-band activity provided direct evidence that BTLA is sensitive to language stimuli in both modalities, 350 ms after stimulation. In addition, spontaneous gamma-band discharges were recorded from this region during which we observed phonological jargon.ConclusionThe findings emphasize the multimodal nature of this region in speech perception. In the context of transient dysfunction, the patient’s lexical semantic processing network is disrupted, reducing spoken output to meaningless phoneme combinations.SignificanceThis rare opportunity to study the BTLA “in vivo” demonstrates its pivotal role in lexico-semantic processing for speech production and its multimodal nature in speech perception.     

Maximising recovery from aphasia with central and peripheral agraphia: The benefit of sequential treatments

ABSTRACT

Maximal recovery from acquired language impairment may require progression from one behavioural treatment protocol to the next in order to build upon residual and relearned cognitive-linguistic and sensory-motor processes. We present a five-stage treatment sequence that was initiated at one year post stroke in a woman with acquired impairments of spoken and written language. As is typical of individuals with left perisylvian damage, she demonstrated marked impairment of phonological retrieval and sublexical phonology, but she also faced additional challenges due to impaired letter shape knowledge and visual attention. The treatment sequence included (1) written spelling of targeted words, (2) retraining sublexical sound-to-letter correspondences and phonological manipulation skills, (3) training strategic approaches to maximise interactive use of lexical, phonological, and orthographic knowledge, (4) lexical retrieval of spoken words, and finally (5) sentence-level stimulation to improve grammatical form of written narratives. This Phase II clinical study documented positive direct treatment outcomes along with evidence of a significant reduction in the underlying deficits and generalisation to untrained items and language tasks. Improvements on a comprehensive assessment battery were realised as functional gains in everyday written and spoken communication, including improved lexical retrieval and grammatical complexity of written narratives. This case provides a valuable example of the cumulative therapeutic benefit of sequential application of theoretically motivated treatment protocols.     

Verbal and academic skills in children with early‐onset type 1 diabetes

Aim Basic verbal and academic skills can be adversely affected by early‐onset diabetes, although these skills have been studied less than other cognitive functions. This study aimed to explore the mechanism of learning deficits in children with diabetes by assessing basic verbal and academic skills in children with early‐onset diabetes and in comparison children. In addition, the incidence of dyslexia (≤10th centile in reading speed or reading–spelling accuracy) was studied. Method The performance of 51 children with early‐onset diabetes (25 females, 26 males; mean age 9y 11mo, SD 4mo; range 9–10y) was compared with that of 92 children without diabetes (40 females, 52 males; mean age 9y 10mo, SD 3mo; range 9–10y) in the tasks of phonological processing, short‐term memory, rapid automatized naming, reading, spelling, and mathematics. Results The performance of children with diabetes was poorer than that of the comparison children in phonological processing (p=0.001), spelling accuracy (p<0.001), and mathematics (p=0.024). They learned to read later (p=0.013), but reading performance and the incidence of dyslexia in the third grade (aged 9–10y) were similar in the two groups. Interpretation Children with early‐onset diabetes are prone to minor learning difficulties in their early school years as a result of deficits in phonological processing.     

Auditory and verbal memory predictors of spoken language skills in children with cochlear implants

BackgroundLarge variability in individual spoken language outcomes remains a persistent finding in the group of children with cochlear implants (CIs), particularly in their grammatical development.AimsIn the present study, we examined the extent of delay in lexical and morphosyntactic spoken language levels of children with CIs as compared to those of a normative sample of age-matched children with normal hearing. Furthermore, the predictive value of auditory and verbal memory factors in the spoken language performance of implanted children was analyzed.Methods & proceduresThirty-nine profoundly deaf children with CIs were assessed using a test battery including measures of lexical, grammatical, auditory and verbal memory tests. Furthermore, child-related demographic characteristics were taken into account.Outcomes & resultsThe majority of the children with CIs did not reach age-equivalent lexical and morphosyntactic language skills. Multiple linear regression analyses revealed that lexical spoken language performance in children with CIs was best predicted by age at testing, phoneme perception, and auditory word closure. The morphosyntactic language outcomes of the CI group were best predicted by lexicon, auditory word closure, and auditory memory for words.ConclusionsQualitatively good speech perception skills appear to be crucial for lexical and grammatical development in children with CIs. Furthermore, strongly developed vocabulary skills and verbal memory abilities predict morphosyntactic language skills.     

Neuropsychologic differences between bilateral dyskinetic and spastic cerebral palsy

This study compared the neuropsychologic performance of 30 adolescents and adults with bilateral dyskinetic, mixed, and spastic cerebral palsy aged between 16 and 38 years. The sample was relatively homogeneous in terms of motor severity; no patients were able to walk unaided. In all subjects, we evaluated the general function of nonverbal reasoning and the following specific neuropsychologic areas: language, visual perception, memory, praxis, and frontal functions. Individuals with dyskinetic cerebral palsy had better auditory comprehension, visuospatial abilities, immediate visual memory, and working verbal memory than those with spastic cerebral palsy. Frontal function was the only cognitive function on which subjects with dyskinetic cerebral palsy had lower scores. We conclude that the neuropsychologic profiles of dyskinetic and spastic cerebral palsy are different.