Reading and reading-related Reading and reading-related skills in skills in

Williams and Down syndromesWilliams and Down syndromes

Yonata LevyYonata LevyPsychology DepartmentPsychology Department

and Hebrew University-Hadassah Medical School, and Hebrew University-Hadassah Medical School, JerusalemJerusalem

This work was supported by a grant from This work was supported by a grant from

the Israel Science Foundationthe Israel Science Foundation

Work on Hebrew was done in collaboration with my students:Work on Hebrew was done in collaboration with my students:

Vered AntebiVered Antebi

Shani BeiserShani Beiser

Work with the American Williams syndrome group was done in Work with the American Williams syndrome group was done in collaboration with Jason Smith and Helen Tager-Flusbergcollaboration with Jason Smith and Helen Tager-Flusberg

We are grateful to the WS Associations in the US and We are grateful to the WS Associations in the US and

in Israel and to the Down syndrome Association in in Israel and to the Down syndrome Association in

Israel, Israel, but most of allbut most of all

we are grateful to the children and their we are grateful to the children and their

familiesfamilies

The research question:The research question:

Individuals with neurodevelopmental disorders that Individuals with neurodevelopmental disorders that result in cognitive disabilities are often reading result in cognitive disabilities are often reading impairedimpaired

But are they dyslexic??But are they dyslexic??

The answer:The answer:

If reading disorders in these populations present If reading disorders in these populations present themselves along with the cluster of strengths and themselves along with the cluster of strengths and weaknesses that are characteristic of dyslexic children weaknesses that are characteristic of dyslexic children

then the answer is then the answer is YESYES

If the profile is different then the answer is If the profile is different then the answer is NONO

What is dyslexia and what is it notWhat is dyslexia and what is it not

Until the mid 90’ies dyslexia was considered a condition Until the mid 90’ies dyslexia was considered a condition affecting typically developing children who, contrary affecting typically developing children who, contrary to expectations, failed to acquire reading at the to expectations, failed to acquire reading at the expected age, i.e. during the first years of elementary expected age, i.e. during the first years of elementary schoolschool

Crucially, dyslexic children scored average or above Crucially, dyslexic children scored average or above average on IQ tests and were reading less well than average on IQ tests and were reading less well than predicted by their IQ predicted by their IQ

Hence, there was a poorly understood Hence, there was a poorly understood discrepancydiscrepancy

According to the discrepancy definitionAccording to the discrepancy definition

Dyslexia is – (quoting)Dyslexia is – (quoting)

. A disorder manifested by the difficulty in learning to . A disorder manifested by the difficulty in learning to

read, despite conventional instruction, adequate read, despite conventional instruction, adequate

intelligence and sociocultural opportunities. It is intelligence and sociocultural opportunities. It is

dependent upon fundamental cognitive disabilities dependent upon fundamental cognitive disabilities

which are frequently of constitutional naturewhich are frequently of constitutional nature

The condition we have when there is a clear The condition we have when there is a clear

discrepancy between the person’s reading ability and discrepancy between the person’s reading ability and

other intellectual abilities other intellectual abilities

Apart from ‘Apart from ‘true dyslexicstrue dyslexics’, there are children who ’, there are children who

score below average on intelligence tests who do score below average on intelligence tests who do not read wellnot read well

The literature has referred to these children asThe literature has referred to these children as

‘‘Garden variety poor readersGarden variety poor readers’’

In these children reading is one domain among many In these children reading is one domain among many in which they perform at the lower tail of their age in which they perform at the lower tail of their age level hence, they are level hence, they are not dyslexicnot dyslexic

‘‘Poor readers’Poor readers’ score low, but are still within one SD score low, but are still within one SD

of the normal range IQ (>85)of the normal range IQ (>85)

What was the theoretical appeal of the What was the theoretical appeal of the discrepancy definition?discrepancy definition?

It highlighted the specificity of dyslexia, and It highlighted the specificity of dyslexia, and being ‘specific’ was equivalent to being being ‘specific’ was equivalent to being ‘theoretically interesting’ ‘theoretically interesting’

DyslexiaDyslexia has been thought about as ahas been thought about as a specific specific

reading disorderreading disorder that is not related to the that is not related to the

child’s general cognitive functioningchild’s general cognitive functioning

The discrepancy definition crucially depends The discrepancy definition crucially depends upon upon

1.1. An effect of IQ An effect of IQ on reading in the ‘poor on reading in the ‘poor readers’ but not in dyslexicsreaders’ but not in dyslexics

2. That there be2. That there be Differences Differences between the between the

profiles characterizing dyslexic children profiles characterizing dyslexic children

and ‘garden variety poor readers’and ‘garden variety poor readers’

What is the characteristic profile of What is the characteristic profile of dyslexia?dyslexia?

When the clinical picture is considered, Dyslexia is a When the clinical picture is considered, Dyslexia is a mixed bag, but mixed bag, but focusing on word readingfocusing on word reading the following the following profile emergesprofile emerges Deficits in phonological awareness tasksDeficits in phonological awareness tasks Deficits in short term auditory memoryDeficits in short term auditory memory

Correlations with rapid object namingCorrelations with rapid object naming

Correlations with vocabulary size Correlations with vocabulary size Children with aChildren with a Correlations with syntax and morphology Correlations with syntax and morphology historyhistory of SLIof SLI

Peripheral vision problems??Peripheral vision problems?? Problems in processing temporally sequenced Problems in processing temporally sequenced

information??information??

Note – Note –

There has been relatively little research on reading in There has been relatively little research on reading in syndromic populations yet, reading difficulties in syndromic populations yet, reading difficulties in these populations can be easily accounted for within these populations can be easily accounted for within the discrepancy approach --the discrepancy approach --

Mentally handicapped people have Mentally handicapped people have difficulties in reading because of their difficulties in reading because of their mental handicapsmental handicaps

And this made research in this populations And this made research in this populations rather uninteresting and had an effect on rather uninteresting and had an effect on intervention as wellintervention as well

However, research has provided little However, research has provided little empirical support for the discrepancy empirical support for the discrepancy hypothesishypothesis

The profile that characterizes dyslexia is also The profile that characterizes dyslexia is also characteristic of ‘garden variety poor characteristic of ‘garden variety poor readers’ and IQ does not correlate with RD readers’ and IQ does not correlate with RD within this group any more than it does in within this group any more than it does in dyslexicsdyslexics

The research findings that forced a change in the The research findings that forced a change in the definition of dyslexia reflected a wide consensus definition of dyslexia reflected a wide consensus among researchers that the basic issues in RD relate among researchers that the basic issues in RD relate to language and that performance on intelligence to language and that performance on intelligence tests was not a key issuetests was not a key issue

Since 1995, the definition of dyslexia considers it to be Since 1995, the definition of dyslexia considers it to be

““a specific language-based disordera specific language-based disorder of constitutional of constitutional origin, characterized by difficulties in single word origin, characterized by difficulties in single word decoding, usually reflecting insufficient phonological decoding, usually reflecting insufficient phonological abilities” abilities”

(Orton Society, 1995)(Orton Society, 1995)

In the light of this definition, the research In the light of this definition, the research question that the current study addresses is question that the current study addresses is the following:the following:

Are children with neuro-developmental Are children with neuro-developmental syndromes, specifically children with syndromes, specifically children with Williams syndrome and children with Williams syndrome and children with

Down syndrome, who experience Down syndrome, who experience reading difficulties, dyslexic? reading difficulties, dyslexic?

Are they dyslexic – yes or noAre they dyslexic – yes or no??

In order for the answer to be In order for the answer to be YESYES, the reading , the reading difficulties seen in children with Williams difficulties seen in children with Williams syndrome or with Down syndrome syndrome or with Down syndrome

should should correlatecorrelate with the familiar language with the familiar language

measures and measures and not co-varynot co-vary according to IQaccording to IQ

This work refers exclusively to decoding This work refers exclusively to decoding and will only look at word readingand will only look at word reading

Williams syndrome - WSWilliams syndrome - WS

Williams syndrome (WS)Williams syndrome (WS) → →A rare genetic disorder (1:20,000) which is typically not familialA rare genetic disorder (1:20,000) which is typically not familial

→ →The clinical phenotype includes 50-70 IQ on average, short stature, The clinical phenotype includes 50-70 IQ on average, short stature, unique facial features, high frequency of SVAS, hyper-acusis, unique facial features, high frequency of SVAS, hyper-acusis, hyper-calcemia, orthopedic problems and a typical gaithyper-calcemia, orthopedic problems and a typical gait

→ →A FISH test reveals a micro-deletion on 7q11.23 with around 24 A FISH test reveals a micro-deletion on 7q11.23 with around 24 genes missing, including the ELN genegenes missing, including the ELN gene

→ →Individuals with WS are very friendly, talkative, with no fear of Individuals with WS are very friendly, talkative, with no fear of strangers and with great love for musicstrangers and with great love for music

→ →WS cognitive profile is often described as unique – their expressive WS cognitive profile is often described as unique – their expressive language is fluent and grammatical, their vocabulary is rich, their language is fluent and grammatical, their vocabulary is rich, their auditory short term memory is very good, their face processing is auditory short term memory is very good, their face processing is good but their visual-motor and motor integration skills are way good but their visual-motor and motor integration skills are way below what is expected of their mental agebelow what is expected of their mental age

A recent MRI study by Galaburda and his team (2004) suggests A recent MRI study by Galaburda and his team (2004) suggests that participants with WS have reduced thalamic and occipital that participants with WS have reduced thalamic and occipital lobe gray matter volumes and reduced gray matter density in lobe gray matter volumes and reduced gray matter density in subcortical and cortical regions comprising the human visual-subcortical and cortical regions comprising the human visual-spatial system, compared with controlsspatial system, compared with controls

The WS group also showed disproportionate increases in volume The WS group also showed disproportionate increases in volume and gray matter density in several areas known to participate and gray matter density in several areas known to participate in emotion and face processing, including the amygdala, in emotion and face processing, including the amygdala, orbital and medial prefrontal cortices, anterior cingulate, orbital and medial prefrontal cortices, anterior cingulate, insular cortex, and superior temporal gyrusinsular cortex, and superior temporal gyrus

Down syndrome -Down syndrome - DSDS

Down syndrome (DS)Down syndrome (DS)

→ →A common neurogenetic disorder, 1:600 with equal frequency A common neurogenetic disorder, 1:600 with equal frequency between males and femalesbetween males and females

→ →DS occurs when there is duplication of chromosome 21 DS occurs when there is duplication of chromosome 21 (trisomy 21) or part of it (mosaic)(trisomy 21) or part of it (mosaic)

→ →The phenotype includes IQ 50-70 on average, unique facial The phenotype includes IQ 50-70 on average, unique facial features, cardiac problems, frequent otitis media, early signs of features, cardiac problems, frequent otitis media, early signs of aging and high frequency of Alzheimer’s diseaseaging and high frequency of Alzheimer’s disease

→ →The cognitive profile of individuals with DS is described as The cognitive profile of individuals with DS is described as depressed across tasks, with specific deficits in language skillsdepressed across tasks, with specific deficits in language skills

→ →Individuals with DS have poor auditory short term memory Individuals with DS have poor auditory short term memory for words, poor vocabulary and even poorer syntaxfor words, poor vocabulary and even poorer syntax

Brain imaging studies of individuals with DS (Pinter et, 2001) Brain imaging studies of individuals with DS (Pinter et, 2001)

report of smaller overall brain volumes, with report of smaller overall brain volumes, with disproportionately smaller cerebellar volumes and relatively disproportionately smaller cerebellar volumes and relatively larger subcortical gray matter volumes larger subcortical gray matter volumes

Also noted is relative preservation of parietal lobe gray and Also noted is relative preservation of parietal lobe gray and temporal lobe white matter relative to control subjects. No temporal lobe white matter relative to control subjects. No abnormalities in patterns of brain asymmetry have been noted abnormalities in patterns of brain asymmetry have been noted in Down's syndrome subjectsin Down's syndrome subjects

Recall what the characteristic dyslexia profile is likeRecall what the characteristic dyslexia profile is like::

Deficits in phonological awareness tasksDeficits in phonological awareness tasks Deficits in short term auditory memoryDeficits in short term auditory memory

Correlations with rapid object namingCorrelations with rapid object naming

Correlations with vocabulary size Correlations with vocabulary size Children with aChildren with a Correlations with syntax and morphology Correlations with syntax and morphology historyhistory of SLIof SLI

Peripheral vision problems??Peripheral vision problems?? Problems in processing temporally sequenced Problems in processing temporally sequenced

information??information??

If reading achievements and reading difficulties If reading achievements and reading difficulties in these populations draw upon similar in these populations draw upon similar language skills as does reading in the normal language skills as does reading in the normal population, we should expect –population, we should expect –

1. Children with WS to acquire reading relatively 1. Children with WS to acquire reading relatively easily since their language skills are preserved easily since their language skills are preserved and their auditory short term memory for words and their auditory short term memory for words is at age levelis at age level

2. Children with DS to acquire reading with great 2. Children with DS to acquire reading with great difficulty, if at all, since their language skills are difficulty, if at all, since their language skills are poor as is their auditory short term memory for poor as is their auditory short term memory for verbal materialverbal material

3. IQ levels within the groups should not correlate 3. IQ levels within the groups should not correlate with word readingwith word reading

We studied word reading and reading related We studied word reading and reading related skills in :skills in :

1. 1. English speaking adolescents and young adults English speaking adolescents and young adults in the US, n=20 (Levy, Smith & Tager-Flusberg, in the US, n=20 (Levy, Smith & Tager-Flusberg, 2003) 2003)

2. Hebrew speaking adolescents and young adults 2. Hebrew speaking adolescents and young adults with WS in Israel, n=17 (Levy & Antebi, 2004)with WS in Israel, n=17 (Levy & Antebi, 2004)

3. Hebrew speaking adolescents and young adults 3. Hebrew speaking adolescents and young adults with DS, in Israel, n=17 (Levy & Bieser, in with DS, in Israel, n=17 (Levy & Bieser, in preparation)preparation)

Participants were beyond the learning phase and Participants were beyond the learning phase and were considered by their educators as having were considered by their educators as having reached the highest reading level that they were reached the highest reading level that they were capable ofcapable of

Individuals who did not recognize the letters of the Individuals who did not recognize the letters of the alphabet were not included in the studyalphabet were not included in the study

Individuals older than 22 years old were not Individuals older than 22 years old were not included in the studies in order to maintain some included in the studies in order to maintain some uniformity in the educational practices uniformity in the educational practices

Since our interest was on word decoding - the focus of Since our interest was on word decoding - the focus of

the study was on reading the study was on reading novel wordsnovel words

Participants were tested on familiar word reading, Participants were tested on familiar word reading, rhyme, phonological awareness tasks, rapid object rhyme, phonological awareness tasks, rapid object naming, vocabulary and syntaxnaming, vocabulary and syntax

We used normative tests whenever possible –We used normative tests whenever possible –

The K-BIT, CTOPP, Woodcock-Johnson and the PPVT for The K-BIT, CTOPP, Woodcock-Johnson and the PPVT for English, and the Wechsler, Nizan and BNT for Hebrew English, and the Wechsler, Nizan and BNT for Hebrew

Experimental tasks were used for rhyme detection and Experimental tasks were used for rhyme detection and for syntaxfor syntax

Summary of findings in study 1Summary of findings in study 1(Levy, Smith, Tager-Flusberg, 2003(Levy, Smith, Tager-Flusberg, 2003((

English speaking adolescents with WSEnglish speaking adolescents with WS n=20; age=16.4 (12.8-20.4)n=20; age=16.4 (12.8-20.4)

Three participants (15%) could not read even a Three participants (15%) could not read even a single novel word, i.e. they were “non-readers” single novel word, i.e. they were “non-readers”

Non-readers failed the rhyming task, whereas the Non-readers failed the rhyming task, whereas the 17 readers were at ceiling on rhyme detection17 readers were at ceiling on rhyme detection

Rapid naming was poor in the group as a wholeRapid naming was poor in the group as a whole The best predictor of word decoding was The best predictor of word decoding was

performance on the non verbal part of K-BITperformance on the non verbal part of K-BIT

Correlations between word decoding Correlations between word decoding (WordAtt), IQ and reading-related skills in (WordAtt), IQ and reading-related skills in English speaking readers with WS (n=17)English speaking readers with WS (n=17)

Correlations between word decoding Correlations between word decoding (WordAtt), IQ and reading-related skills in (WordAtt), IQ and reading-related skills in

English speaking readers with WS with K-BIT English speaking readers with WS with K-BIT def. held constant (n=17)def. held constant (n=17)

Reading and related skills in English speaking Reading and related skills in English speaking adolescents with WS by IQ range (n=20)adolescents with WS by IQ range (n=20)

IQ Range

0

10

20

30

40

Perc

en

tile

R

an

k o

f

Mean

Sta

nd

ard

Sco

re

40--49 (n = 8)

50--69 (n = 9)

69--84 (n = 3)

Summary of findings in study 2:Summary of findings in study 2:(Levy and Antebi, 2004)(Levy and Antebi, 2004)

Hebrew speaking adolescents with WS Hebrew speaking adolescents with WS N=17; mean age 14;4 (11;3-22)N=17; mean age 14;4 (11;3-22)

Non-readers were twice as many as in the English Non-readers were twice as many as in the English

speaking group – 6 individuals - ~35%speaking group – 6 individuals - ~35% Performance on rhyming among the readers was at Performance on rhyming among the readers was at

ceiling ceiling Performance on rapid naming was poor across the Performance on rapid naming was poor across the

boardboard The best predictor of word decoding was The best predictor of word decoding was

performance on the Wechslerperformance on the Wechsler

Some relevant facts about Hebrew Some relevant facts about Hebrew orthographyorthography

Hebrew marks most of its vowels as diacritics over Hebrew marks most of its vowels as diacritics over and below the line. For example:and below the line. For example:

ד ֶל� ד ֶי� ֶל� yeledyeledֶי�ֶל ד� ְג� ֶלִמ� ד� ְג� migdalmigdal ִמ�

When diacritics are marked Hebrew has a shallow When diacritics are marked Hebrew has a shallow orthography, i.e. there is a good mapping orthography, i.e. there is a good mapping between letters + diacritics and soundsbetween letters + diacritics and sounds

Hebrew orthography without diacritics is opaqueHebrew orthography without diacritics is opaque

yldyld ֶיֶלד ֶיֶלד mgdlmgdl ִמְגדֶל ִמְגדֶל

Children are taught to read with diacritics Children are taught to read with diacritics

Word reading is mastered relatively earlyWord reading is mastered relatively early

However, diacritics are gradually dropped from However, diacritics are gradually dropped from written texts from grade 3 and on and most adult written texts from grade 3 and on and most adult written Hebrew is without diacritics written Hebrew is without diacritics

Our task presented novel words with diacriticsOur task presented novel words with diacritics

It is possible that for individuals with WS who It is possible that for individuals with WS who experience specific difficulties in visual motor and experience specific difficulties in visual motor and visual integration tasks, integrating two visual integration tasks, integrating two perceptually disparate systems created a unique perceptually disparate systems created a unique difficultydifficulty

Age, IQ and level of word reading in Hebrew Age, IQ and level of word reading in Hebrew

speaking participants with WSspeaking participants with WS

Correlations between word reading, rapid Correlations between word reading, rapid naming, vocabulary, phonological awareness naming, vocabulary, phonological awareness and IQ in Hebrew speaking readers with WS and IQ in Hebrew speaking readers with WS

(n=11) (n=11)

Word reading and language skills by IQ, Word reading and language skills by IQ, in Hebrew speaking adolescents with in Hebrew speaking adolescents with

WSWS

-1.5

-1

-0.5

0

0.5

1

1.5

2IQ=41-49

IQ=50-69

IQ>70

Previous studies of reading in individuals Previous studies of reading in individuals with WSwith WS Several studies have looked at reading in WS – Udwin et al Several studies have looked at reading in WS – Udwin et al

(1987), Howlin et al (1998), Laing et al (2001), Vicari et al (1987), Howlin et al (1998), Laing et al (2001), Vicari et al (2004)(2004)

Similar percentages of non-readers to our English sample were Similar percentages of non-readers to our English sample were reportedreported

The studies did not focus on word decoding but looked at The studies did not focus on word decoding but looked at comprehension as wellcomprehension as well

There has not been a systematic attempt to correlate word There has not been a systematic attempt to correlate word reading with scores on IQ testreading with scores on IQ test

Since very young children – 6 yr. olds – were among the Since very young children – 6 yr. olds – were among the participants studied such correlations would not be meaningfulparticipants studied such correlations would not be meaningful

Summary of study 3Summary of study 3(Levy & Bieser, in press)(Levy & Bieser, in press)

Hebrew speaking adolescents with DS Hebrew speaking adolescents with DS N=17; mean age 17;06 (13;2 – 20;1)N=17; mean age 17;06 (13;2 – 20;1)

Four participants ~ 27%, were non-readers, i.e. they Four participants ~ 27%, were non-readers, i.e. they

could not read a single novel word could not read a single novel word Rhyme and rapid object naming did not correlate with Rhyme and rapid object naming did not correlate with

word readingword reading Syntax comprehension and production did not correlate Syntax comprehension and production did not correlate

with word readingwith word reading Scores on the Wechsler as well as performance on Scores on the Wechsler as well as performance on

phonological awareness tasks correlated with word phonological awareness tasks correlated with word

readingreading Grammaticality judgments correlated with word readingGrammaticality judgments correlated with word reading

IQ and word reading in Hebrew speaking children IQ and word reading in Hebrew speaking children with DSwith DS

NWords (N=24)

RWords (N=59)

IQ Participant

3 22 48 1 2 24 51 2 0 27 51 3 0 6 54 4 0 10 54 5 9 20 54 6 2 29 54 7 6 40 54 8 0 6 56 9 6 41 59 10 9 36 62 11 11 45 62 12 7 33 68 13 13 34 71 14 13 42 71 15 21 48 80 16 15 46 85 17

Correlations between word reading, rhyme, Correlations between word reading, rhyme, phonological awareness tasks and IQ in phonological awareness tasks and IQ in

Hebrew speaking participants with Down Hebrew speaking participants with Down syndromesyndrome

Correlation between word decoding, syntax Correlation between word decoding, syntax

comprehension and production and comprehension and production and

grammaticality judgments in Hebrew grammaticality judgments in Hebrew

speaking adolescents with Down syndromespeaking adolescents with Down syndrome

high IQ (>70) med IQ (52-69)

low IQ (<52)

RON BNT

NonWords RealWords

PA1 Alliteration

BlockDesign

Variables

-1.5

-1.0

-0.5

0.0

0.5

1.0

1.5

Word reading and reading-related skills by IQ, in Hebrew speaking adolescents with DS

Previous studies of reading in individuals Previous studies of reading in individuals with DSwith DS

Not many studied have looked at word reading in this populationNot many studied have looked at word reading in this population

There have been reports of hyper-lexic children with Down There have been reports of hyper-lexic children with Down syndromesyndrome

A recent study (Laws & Gunn, 2002) reports of a higher percent A recent study (Laws & Gunn, 2002) reports of a higher percent of non-readers than we found. However, age and IQ have not of non-readers than we found. However, age and IQ have not been controlled forbeen controlled for

Their follow-up study suggests that the higher the IQ, the more Their follow-up study suggests that the higher the IQ, the more likely the children are to acquire word decodinglikely the children are to acquire word decoding

Summary of the findingsSummary of the findings

Contrary to expectations, reading level in Contrary to expectations, reading level in individuals with WS was similar to reading level of individuals with WS was similar to reading level of IQ matched individuals with DSIQ matched individuals with DS

Rapid naming and vocabulary did not predict Rapid naming and vocabulary did not predict word decoding in either groupword decoding in either group

Syntax did not predict word decoding in Syntax did not predict word decoding in individuals with DSindividuals with DS

Phonological awareness and grammaticality Phonological awareness and grammaticality judgments were correlated with word decodingjudgments were correlated with word decoding

Performance on IQ tests correlated with word Performance on IQ tests correlated with word

decodingdecoding

Hebrew speaking individuals withHebrew speaking individuals with

WS DS WS DS

high IQ (>70) med IQ (52-69) low IQ (<52)

RON BNT

NonWords RealWords

PA1 Alliteration

BlockDesign

Variables

-1.5

-1.0

-0.5

0.0

0.5

1.0

1.5

Mea

n (

Z S

core

) -1.5

-1

-0.5

0

0.5

1

1.5

2IQ=41-49

IQ=50-69

IQ>70

high IQ (>70) med IQ (52-69) low IQ (<52)

RON BNT

NonWords RealWords

PA1 Alliteration

BlockDesign

Variables

-1.5

-1.0

-0.5

0.0

0.5

1.0

1.5

M

ea

n (

Z S

co

re)

-1.5

-1

-0.5

0

0.5

1

1.5

2IQ=41-49

IQ=50-69

IQ>70

IQ Range

0

10

20

30

40

Perc

en

tile

R

an

k o

f

Mean

Sta

nd

ard

Sco

re

40--49 (n = 8)

50--69 (n = 9)

69--84 (n = 3)

The aim of this research was to find out whether The aim of this research was to find out whether adolescents with either WS or DS who speak adolescents with either WS or DS who speak Hebrew or English are, in fact, dyslexic Hebrew or English are, in fact, dyslexic

We asked whether the pattern of strengths and We asked whether the pattern of strengths and weaknesses in word reading seen in the groups weaknesses in word reading seen in the groups studied was the same as the familiar dyslexia studied was the same as the familiar dyslexia clustercluster

Based on our findings the answer is Based on our findings the answer is

NO!NO!

We make the following claims:We make the following claims:

Poor reading in WS and in DS does not arise from poor Poor reading in WS and in DS does not arise from poor language skills. The problem lies with the need to engage language skills. The problem lies with the need to engage in explicit learning of word decodingin explicit learning of word decoding

Performance on IQ tests is a good predictor of a person’s Performance on IQ tests is a good predictor of a person’s potential performance when explicit learning is called forpotential performance when explicit learning is called for

(phonological awareness tasks, grammaticality (phonological awareness tasks, grammaticality judgments)judgments)

The prediction is that in children with cognitive disabilities The prediction is that in children with cognitive disabilities other skills requiring explicit learning will show a similar other skills requiring explicit learning will show a similar dependency on IQ dependency on IQ

We offer to restore the intuition behind the We offer to restore the intuition behind the

discrepancy definition of dyslexiadiscrepancy definition of dyslexia since we think since we think that reading disorders in syndromic populations is that reading disorders in syndromic populations is

unlike dyslexiaunlike dyslexia

The reading disorder seen in WS and in DS differ from The reading disorder seen in WS and in DS differ from dyslexia in that they are not language related and dyslexia in that they are not language related and they concern difficulties with explicit learning of they concern difficulties with explicit learning of which reading is but one domainwhich reading is but one domain

Such difficulties will be seen in people with intellectual Such difficulties will be seen in people with intellectual disabilities across syndromes and across knowledge disabilities across syndromes and across knowledge areas areas

Understanding probability in WSUnderstanding probability in WS

Step I: Step II:Step I: Step II:

Experimenter throws Which dreidel Experimenter throws Which dreidel

the dice would you like to spin? the dice would you like to spin?

The experiment was run with the Israeli group of The experiment was run with the Israeli group of WS in two different versions: WS in two different versions:

a roulette version and a dreidel versiona roulette version and a dreidel version

After making their choices and trying their luck, After making their choices and trying their luck, children were asked to explain why they picked children were asked to explain why they picked one dreidel or the otherone dreidel or the other

All the participants made the right choices well All the participants made the right choices well above chance levelabove chance level

roulette: 72% (69-78; p<.001) roulette: 72% (69-78; p<.001)

dreidel: 69.8% (66-75; p<.004)dreidel: 69.8% (66-75; p<.004)

Only four out of the 17 participants manage to Only four out of the 17 participants manage to provide some explanation for their choices that provide some explanation for their choices that was vaguely related to probabilitywas vaguely related to probability

ConclusionConclusionLearning of school type skills is primarily affected by Learning of school type skills is primarily affected by

a person’s general cognitive abilities rather than a person’s general cognitive abilities rather than

by the cognitive profile that is unique to a given by the cognitive profile that is unique to a given

syndromesyndrome

When explicit meta-cognitive learning is involved When explicit meta-cognitive learning is involved general cognitive disability is the major general cognitive disability is the major impedimentimpediment

To the extent that one can make the child To the extent that one can make the child attend to the classroom material in anattend to the classroom material in an explicit manner, learning is likely to be explicit manner, learning is likely to be enhancedenhanced