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.

[1]  Darielle Greenberg Woodcock Reading Mastery Tests–Revised , 2014 .

[2]  B. Landau,et al.  Quantitative analysis of gray and white matter in Williams syndrome , 2012, Neuroreport.

[3]  Yu-Hsun Chang,et al.  Williams-Beuren syndrome , 2012 .

[4]  B. Landau,et al.  Representation of object orientation in children: Evidence from mirror-image confusions , 2011, Visual cognition.

[5]  J. Burack The Oxford handbook of intellectual disability and development , 2011 .

[6]  Robert B Terwillinger,et al.  Object recognition in Williams syndrome: uneven ventral stream activation. , 2011, Developmental science.

[7]  Benjamin R. Dering,et al.  Behavioral and ERP evidence for amodal sluggish attentional shifting in developmental dyslexia , 2010, Neuropsychologia.

[8]  M. Seghier,et al.  The Neural Substrates and Timing of Top–Down Processes during Coarse-to-Fine Categorization of Visual Scenes: A Combined fMRI and ERP Study , 2010, Journal of Cognitive Neuroscience.

[9]  Michael McCloskey,et al.  Mirror-image confusions: Implications for representation and processing of object orientation , 2010, Cognition.

[10]  V. Volterra,et al.  Lexical-semantic reading in a shallow orthography: evidence from a girl with Williams Syndrome , 2010 .

[11]  Keith T. Greaney,et al.  Defining Dyslexia , 2010, Journal of Learning Disabilities.

[12]  C. Fowler,et al.  Williams-Beuren syndrome. , 2010, The New England journal of medicine.

[13]  B. Landau,et al.  When Theories Don't Compete: Response to Thomas, Karaminis, and Knowland's Commentary on Musolino, Chunyo, and Landau , 2010 .

[14]  Gary J. Robertson,et al.  Wide‐Range Achievement Test , 2010 .

[15]  B. Pober Williams-Beuren syndrome. , 2010, The New England journal of medicine.

[16]  S. Donnadieu,et al.  Auditory and visual stream segregation in children and adults: An assessment of the amodality assumption of the ‘sluggish attentional shifting’ theory of dyslexia , 2009, Brain Research.

[17]  Vijay Kannan,et al.  Neural Substrates of Visuospatial Processing in Distinct Reference Frames: Evidence from Unilateral Spatial Neglect , 2009, Journal of Cognitive Neuroscience.

[18]  M. McCloskey Visual Reflections : A Perceptual Deficit and Its Implications , 2009 .

[19]  C. Mervis,et al.  Retinotopically defined primary visual cortex in Williams syndrome. , 2009, Brain : a journal of neurology.

[20]  B. Landau,et al.  Normal Susceptibility to Visual Illusions in Abnormal Development: Evidence from Williams Syndrome , 2009, Perception.

[21]  J. Maisog,et al.  A Meta‐analysis of Functional Neuroimaging Studies of Dyslexia , 2008, Annals of the New York Academy of Sciences.

[22]  L. Chelazzi,et al.  Sluggish engagement and disengagement of non-spatial attention in dyslexic children , 2008, Cortex.

[23]  Barbara Landau,et al.  Vision for perception and vision for action: normal and unusual development. , 2008, Developmental science.

[24]  Carlo Miniussi,et al.  Effects of Right Parietal Transcranial Magnetic Stimulation on Object Identification and Orientation Judgments , 2008, Journal of Cognitive Neuroscience.

[25]  Barbara Landau,et al.  More Than Meets the Eye , 2008, Psychological science.

[26]  F. Ramus,et al.  What Phonological Deficit? , 2008, Quarterly journal of experimental psychology.

[27]  René M. Müri,et al.  When left becomes right and vice versa: Mirrored vision after cerebral hypoxia , 2007, Neuropsychologia.

[28]  Carlo Pierpaoli,et al.  Genetic contributions to white matter architecture revealed by diffusion tensor imaging in Williams syndrome , 2007, Proceedings of the National Academy of Sciences.

[29]  S. Valdois,et al.  Developmental dyslexia: The visual attention span deficit hypothesis , 2007, Cognition.

[30]  J. Gabrieli,et al.  Functional and morphometric brain dissociation between dyslexia and reading ability , 2007, Proceedings of the National Academy of Sciences.

[31]  Barbara Landau,et al.  Object recognition with severe spatial deficits in Williams syndrome: sparing and breakdown , 2006, Cognition.

[32]  D. V. van Essen,et al.  Symmetry of Cortical Folding Abnormalities in Williams Syndrome Revealed by Surface-Based Analyses , 2006, The Journal of Neuroscience.

[33]  Andreas Meyer-Lindenberg,et al.  Neural mechanisms in Williams syndrome: a unique window to genetic influences on cognition and behaviour , 2006, Nature Reviews Neuroscience.

[34]  Motoichiro Kato,et al.  A 3-year follow-up study of ‘orientation agnosia’ , 2005, Neuropsychologia.

[35]  Barbara Landau,et al.  Motion processing specialization in Williams syndrome , 2005, Vision Research.

[36]  J. Hoffman,et al.  Multiple Object Tracking in People With Williams Syndrome and in Normally Developing Children , 2005, Psychological science.

[37]  Andreas Meyer-Lindenberg,et al.  Genetic Contributions to Human Gyrification: Sulcal Morphometry in Williams Syndrome , 2005, The Journal of Neuroscience.

[38]  Barbara Landau,et al.  Parallels between spatial cognition and spatial language: Evidence from Williams syndrome , 2005 .

[39]  Paul Koch,et al.  Functional, structural, and metabolic abnormalities of the hippocampal formation in Williams syndrome. , 2005, The Journal of clinical investigation.

[40]  M. Coltheart,et al.  Cognitive Heterogeneity in Williams Syndrome , 2005, Developmental neuropsychology.

[41]  S. Valdois,et al.  The cognitive deficits responsible for developmental dyslexia: review of evidence for a selective visual attentional disorder. , 2004, Dyslexia.

[42]  Karen M. Jones,et al.  Neural Changes following Remediation in Adult Developmental Dyslexia , 2004, Neuron.

[43]  C. Mervis,et al.  Neural Basis of Genetically Determined Visuospatial Construction Deficit in Williams Syndrome , 2004, Neuron.

[44]  James R. Booth,et al.  Development of Brain Mechanisms for Processing Orthographic and Phonologic Representations , 2004, Journal of Cognitive Neuroscience.

[45]  M. Eckert Neuroanatomical Markers for Dyslexia: A Review of Dyslexia Structural Imaging Studies , 2004, The Neuroscientist : a review journal bringing neurobiology, neurology and psychiatry.

[46]  M. Coltheart,et al.  Is there a causal link from phonological awareness to success in learning to read? , 2004, Cognition.

[47]  C. Temple Deep dyslexia in Williams syndrome , 2003, Journal of Neurolinguistics.

[48]  G Jobard,et al.  Evaluation of the dual route theory of reading: a metanalysis of 35 neuroimaging studies , 2003, NeuroImage.

[49]  S. Majerus,et al.  An Investigation of Verbal Short-term Memory and Phonological Processing in Four Children With Williams Syndrome , 2003, Neurocase.

[50]  Thomas A Zeffiro,et al.  Development of neural mechanisms for reading , 2003, Nature Neuroscience.

[51]  Y. Levy,et al.  Word reading and reading-related skills in adolescents with Williams syndrome. , 2003, Journal of child psychology and psychiatry, and allied disciplines.

[52]  J. Hoffman,et al.  Spatial breakdown in spatial construction: Evidence from eye fixations in children with Williams syndrome , 2003, Cognitive Psychology.

[53]  Janette Atkinson,et al.  Neurobiological Models of Visuospatial Cognition in Children With Williams Syndrome: Measures of Dorsal-Stream and Frontal Function , 2003, Developmental neuropsychology.

[54]  E. Laing Investigating reading development in atypical populations: The case of Williams syndrome , 2002 .

[55]  P. Skudlarski,et al.  Disruption of posterior brain systems for reading in children with developmental dyslexia , 2002, Biological Psychiatry.

[56]  H. Renvall,et al.  Impaired processing of rapid stimulus sequences in dyslexia , 2001, Trends in Cognitive Sciences.

[57]  A. Karmiloff-Smith,et al.  Learning to read in Williams syndrome: looking beneath the surface of atypical reading development. , 2001, Journal of child psychology and psychiatry, and allied disciplines.

[58]  J. Mitchell Comprehensive Test of Phonological Processing , 2001 .

[59]  V. Menon,et al.  Preliminary evidence of widespread morphological variations of the brain in dyslexia , 2001, Neurology.

[60]  M. Molteni,et al.  Is attentional focusing an inhibitory process at distractor location? , 2000, Brain research. Cognitive brain research.

[61]  M. McCloskey,et al.  A Visually Based Developmental Reading Deficit , 2000 .

[62]  M. Coltheart,et al.  The relationship between language-processing and visual-processing deficits in developmental dyslexia , 1999, Cognition.

[63]  A. Olson,et al.  Developmental Surface Dysgraphia: What is the Underlying Cognitive Impairment? , 1999, The Quarterly journal of experimental psychology. A, Human experimental psychology.

[64]  Guinevere F. Eden,et al.  Neural Systems Affected in Developmental Dyslexia Revealed by Functional Neuroimaging , 1998, Neuron.

[65]  B. Horwitz,et al.  Functional connectivity of the angular gyrus in normal reading and dyslexia. , 1998, Proceedings of the National Academy of Sciences of the United States of America.

[66]  P. Howlin,et al.  Cognitive functioning in adults with Williams syndrome. , 1998, Journal of child psychology and psychiatry, and allied disciplines.

[67]  J. Stein,et al.  To see but not to read; the magnocellular theory of dyslexia , 1997, Trends in Neurosciences.

[68]  Sergio Della Sala,et al.  Agnosia for object orientation: Implications for theories of object recognition , 1997, Neuropsychologia.

[69]  R. Woods,et al.  Abnormal processing of visual motion in dyslexia revealed by functional brain imaging , 1996, Nature.

[70]  Max Coltheart,et al.  Psycholinguistic assessments of language processing in aphasia (PALPA) , 1996 .

[71]  U. Bellugi,et al.  Unique Profile of Visuo-Perceptual Skills in a Genetic Syndrome , 1995, Brain and Cognition.

[72]  Keith R. Laws,et al.  Object Recognition without Knowledge of Object Orientation , 1995, Cortex.

[73]  Gislin Dagnelie,et al.  A Developmental Deficit in Localizing Objects from Vision , 1995 .

[74]  L. Hildman,et al.  Kaufman Brief Intelligence Test , 1993 .

[75]  J. Hanley,et al.  Developmental Surface Dyslexia and Dysgraphia: An Orthographic Processing Impairment , 1992, The Quarterly journal of experimental psychology. A, Human experimental psychology.

[76]  M. Snowling,et al.  Visual Memory Deficits: A Plausible Cause of Developmental Dyslexia? Evidence from a Single Case Study , 1991 .

[77]  R. Pagon,et al.  Williams syndrome: features in late childhood and adolescence. , 1987, Pediatrics.

[78]  F. Martin,et al.  A theoretical and experimental case for a visual deficit in specific reading disability , 1986 .

[79]  G. N. Getman,et al.  A Commentary on Vision Training , 1985, Journal of learning disabilities.

[80]  P. Tallal Auditory temporal perception, phonics, and reading disabilities in children , 1980, Brain and Language.

[81]  D. Shankweiler,et al.  Explicit Syllable and Phoneme Segmentation in the Young Child , 1974 .

[82]  Pawel Stankiewicz,et al.  Genomic disorders : the genomic basis of disease , 2006 .

[83]  S. Vicari,et al.  Reading and phonological awareness in Williams syndrome. , 2004, Neuropsychology.

[84]  J. Fletcher,et al.  Specific reading disability (dyslexia): what have we learned in the past four decades? , 2004, Journal of child psychology and psychiatry, and allied disciplines.

[85]  M Coltheart,et al.  DRC: a dual route cascaded model of visual word recognition and reading aloud. , 2001, Psychological review.

[86]  L. Katz,et al.  Functional neuroimaging studies of reading and reading disability (developmental dyslexia). , 2000, Mental retardation and developmental disabilities research reviews.

[87]  Carolyn B. Mervis,et al.  Williams syndrome: Findings from an integrated program of research , 1999 .

[88]  R. Wagner,et al.  Comprehensive Test of Phonological Processing , 1997 .

[89]  S. Bain Differential Ability Scales , 1991 .

[90]  B. Breitmeyer A Visually Based Deficit in Specific Reading Disability , 1989 .

[91]  Ursula Bellugi,et al.  Dissociation between language and cognitive functions in Williams syndrome , 1988 .

[92]  D. Bishop,et al.  Language development in exceptional circumstances , 1988 .

[93]  R. Woodcock Woodcock Reading Mastery Tests-Revised , 1987 .

[94]  P. Bryant,et al.  Categorizing sounds and learning to read—a causal connection , 1983, Nature.