velocardiofacial syndrome: learning difficulties and

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61 Med Genet 1995;32:612-618 Velocardiofacial syndrome: learning difficulties and intervention Lee Ling Kok, Robert T Solman Abstract Velocardiofacial syndrome, delineated in 1978 by Shprintzen, is a multiple genetic disorder, characterised primarily by cleft palate, cardiovascular anomalies, typical facies, and learning disorders. This syn- drome with its recurrent pattern of an- omalies has significant implications for education as it is linked to a variety of educationally relevant problems including learning difficulties, behaviour disorders, and speech and language deficits. While clinical research advances and new find- ings in cytogenetics enable early diagnosis and genetic counselling, ramifications of the syndrome in the field of education are still unknown. Learning difficulties should not be viewed solely as a pathological con- dition and with data on success of in- tervention, parents and teachers can be apprised of the information. This paper discusses academic characteristics and the effects of intervention with emphasis on interactive computer based instruction on the development of reading, language, spelling, and numeracy skills. Positive effects of computer based instruction on students' self-esteem, motivation, and competence in computer operational skills were also observed and noted. We are en- couraged by the enthusiasm ofthe students for the system, the development of interest in reading, and the transfer of remedial instruction to classroom performance. Comparison of pretest and post-test res- ults indicated significant improvement in reading ability as measured using Neale Analysis. This improvement is in ac- cordance with results obtained using com- puter testing of specific subskills in reading/language and mathematics. (J7Med Genet 1995;32:612-618) Department of Education Studies, University of New South Wales, PO Box 1, Kensington, NSW 2033, Australia L L Kok R T Solman Correspondence to: Professor Solman. Received 6 September 1994 Revised version accepted for publication 20 February 1995 The velocardiofacial syndrome described in a series of reports'` is a syndrome of multiple anomalies with different manifestations of vari- ous degree but characterised by the following features: velopharyngeal insufficiency (video- fluoroscopic examination shows poor motion in the lateral pharyngeal walls), overt and sub- mucous cleft of the secondary palate causing hypernasal speech, cardiac abnormalities (vent- ricular septal defect, right aortic arch, tetralogy of Fallot, aberrant origin of the subclavian artery), and typical phenotype (the face features a prominent nose with a broad nasal root and a narrow alar base, a long face with narrow, almond shaped palpebral fissures, flattened malar eminences, a small mouth and re- trognathia, hypotonia, and short stature). Other anomalies include otitis media, scoliosis, and Robin sequence. Recently, cytogenetic studies have added significantly to the search for aetiologies. Using molecular analysis Dri- scoll6 detected DNA deletions in chromosome 22 within band 22q1 1, a band which is common to the DiGeorge critical region. Almost all VCF students have learning difficulties with specific weakness in cognitive skills involving ab- stractions and mathematical ability. Researchers in the fields of medicine, psy- chology, and education have studied children and adults with learning disabilities since 1800. The search for effective intervention methods continues into the 1990s. Students with VCF syndrome characterised by learning difficulties experience developmental delay and im- maturity of cognitive and social skills. A pre- vious study using cross sectional data by Golding-Kushner et af/ indicated that at risk behaviour was evident in young children 6 years and under. As the children progressed from kindergarten to secondary schooling cognitive development did not progress beyond the con- crete stage. Despite remediation in speech, language, and academic skills using traditional methods, all students 11 years of age and over required resource room help or were in special self-contained room placement. Integration of the learning disabled children and youths in mainstream classrooms has been generally encouraged and the creation of op- timal classroom conditions to accommodate this has been constantly discussed in special education. Johnson and Pugach8 investigated teachers' views of intervention strategies for learning and behaviour problems. Their results cited attitude of classroom teachers and pro- filed factors inhibiting teachers' selection and implementation of intervention strategies to address mild learning and behaviour problems in regular classrooms. One of the most cited reasons for not implementing intervention strategies, particularly those that have support in published reports, was that intervention strategies were too time consuming and in- volved organisational rules. Many of the VCF students in Australia are receiving mainstream education and despite remediation using either traditional one to one tuition or intervention strategies within the classroom, student performance level is gen- erally two or more years behind their peers. Hence, unless effective remediation pro- grammes are initiated at an early stage, stu- dents' performance level may fall below 612 on November 6, 2021 by guest. Protected by copyright. http://jmg.bmj.com/ J Med Genet: first published as 10.1136/jmg.32.8.612 on 1 August 1995. Downloaded from

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Page 1: Velocardiofacial syndrome: learning difficulties and

61Med Genet 1995;32:612-618

Velocardiofacial syndrome: learning difficultiesand intervention

Lee Ling Kok, Robert T Solman

AbstractVelocardiofacial syndrome, delineated in1978 by Shprintzen, is a multiple geneticdisorder, characterised primarily by cleftpalate, cardiovascular anomalies, typicalfacies, and learning disorders. This syn-drome with its recurrent pattern of an-omalies has significant implications foreducation as it is linked to a variety ofeducationally relevant problems includinglearning difficulties, behaviour disorders,and speech and language deficits. Whileclinical research advances and new find-ings in cytogenetics enable early diagnosisand genetic counselling, ramifications ofthe syndrome in the field of education arestill unknown. Learning difficulties shouldnot be viewed solely as a pathological con-dition and with data on success of in-tervention, parents and teachers can beapprised of the information. This paperdiscusses academic characteristics and theeffects of intervention with emphasis oninteractive computer based instruction onthe development of reading, language,spelling, and numeracy skills. Positiveeffects of computer based instruction onstudents' self-esteem, motivation, andcompetence in computer operational skillswere also observed and noted. We are en-couraged by the enthusiasm ofthe studentsfor the system, the development ofinterestin reading, and the transfer of remedialinstruction to classroom performance.Comparison of pretest and post-test res-ults indicated significant improvement inreading ability as measured using NealeAnalysis. This improvement is in ac-cordance with results obtained using com-puter testing of specific subskills inreading/language and mathematics.

(J7Med Genet 1995;32:612-618)

Department ofEducation Studies,University ofNewSouth Wales,PO Box 1,Kensington,NSW 2033, AustraliaL L KokR T Solman

Correspondence to:Professor Solman.

Received 6 September 1994Revised version accepted forpublication20 February 1995

The velocardiofacial syndrome described in a

series of reports'` is a syndrome of multipleanomalies with different manifestations of vari-ous degree but characterised by the followingfeatures: velopharyngeal insufficiency (video-fluoroscopic examination shows poor motionin the lateral pharyngeal walls), overt and sub-mucous cleft of the secondary palate causinghypernasal speech, cardiac abnormalities (vent-ricular septal defect, right aortic arch, tetralogyof Fallot, aberrant origin of the subclavianartery), and typical phenotype (the face featuresa prominent nose with a broad nasal root anda narrow alar base, a long face with narrow,

almond shaped palpebral fissures, flattenedmalar eminences, a small mouth and re-trognathia, hypotonia, and short stature).Other anomalies include otitis media, scoliosis,and Robin sequence. Recently, cytogeneticstudies have added significantly to the searchfor aetiologies. Using molecular analysis Dri-scoll6 detected DNA deletions in chromosome22 within band 22q1 1, a band which is commonto the DiGeorge critical region. Almost all VCFstudents have learning difficulties with specificweakness in cognitive skills involving ab-stractions and mathematical ability.

Researchers in the fields of medicine, psy-chology, and education have studied childrenand adults with learning disabilities since 1800.The search for effective intervention methodscontinues into the 1990s. Students with VCFsyndrome characterised by learning difficultiesexperience developmental delay and im-maturity of cognitive and social skills. A pre-vious study using cross sectional data byGolding-Kushner et af/ indicated that at riskbehaviour was evident in young children 6 yearsand under. As the children progressed fromkindergarten to secondary schooling cognitivedevelopment did not progress beyond the con-crete stage. Despite remediation in speech,language, and academic skills using traditionalmethods, all students 11 years of age and overrequired resource room help or were in specialself-contained room placement.

Integration of the learning disabled childrenand youths in mainstream classrooms has beengenerally encouraged and the creation of op-timal classroom conditions to accommodatethis has been constantly discussed in specialeducation. Johnson and Pugach8 investigatedteachers' views of intervention strategies forlearning and behaviour problems. Their resultscited attitude of classroom teachers and pro-filed factors inhibiting teachers' selection andimplementation of intervention strategies toaddress mild learning and behaviour problemsin regular classrooms. One of the most citedreasons for not implementing interventionstrategies, particularly those that have supportin published reports, was that interventionstrategies were too time consuming and in-volved organisational rules.Many of the VCF students in Australia are

receiving mainstream education and despiteremediation using either traditional one to onetuition or intervention strategies within theclassroom, student performance level is gen-erally two or more years behind their peers.Hence, unless effective remediation pro-grammes are initiated at an early stage, stu-dents' performance level may fall below

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Velocardiofacial syndrome: learning difficulties and intervention

achievement expected of their mental age. Thisleads to a search for effective interventionmethods that would be successful with thesechildren and readily implemented by teachers.The use of microcomputers in society,

school, and home has escalated exponentially.While emphasis has been placed on computerliteracy in the work place, equal importancehas been attributed to the effect of computertechnology with children and its impact asan efficient tool in education. For learningdisabled students microcomputers providethem with a new learning experience. Studentsare able to learn new skills and practise them,as the fear of displaying failure in front oftheir peers and teachers is removed. Drill andpractice programs enable students to practiseand review skills for as long as they need to.Tutorial programs are also available to teachstudents new skills. The learning environmentprovided is non-threatening and students aremotivated, empowered, and encouraged toachieve mastery in skills taught.

Research in special education to determinethe effects of computer assisted instruction as aremediation strategy has shown an exceptionalrange of possibilities and potentials. Wise et al,9in implementing a long term computerised re-medial reading program with synthetic speechfeedback, found that microcomputers equippedwith synthetic speech can provide a helpful re-medial tool for reading disabled children. Re-sponses from resource teachers and studentswere also most positive. Farmer et al,'0 in com-puter assisted reading, reported that in spite ofthe non-significant improvement in readingability over the short period of interaction time,other positive effects were noted. Enthusiasmamong students was overwhelming and avail-ability of the system enabled more readingpractice for reading disabled students. As com-puters have become more accessible and resultsfrom research into their use in special educationhave become more encouraging, the efficacy ofinteractive computer assisted instruction as aremedial tool for VCF children was examinedhere.

MethodsSUBJECTSThe subjects for this study were VCF studentsaged 7 to 16 years. They formed the school agedchildren living within the Sydney metropolitanregion, New South Wales, Australia. All thesubjects were school identified learning dis-abled students and were either receiving or havereceived remediation in academic subjects. Atthe initial time of investigation, all subjectsexcept SS6 were receiving special educationservices at school in either regular or remedialclasses (table 1).SS1 was placed in a regular classroom situ-

ation but had been identified to be performingsix months below the expected grade level.Remediation in mathematics and English hadbeen received in school during the school year.SS2 was reported to be coping at a sat-

isfactory level, performing well above the ex-pected level ofthe mildly intellectually disabled.

Table 1 Summary of school placementSubjects Age School year School placement

SS1 7 2 Regular, remedialSS2 8 2 Composite

remedialSS3 10 4 Composite

remedialSS4 11 6 Regular, remedialSS5 13 8 Regular, remedialSS6 16 11 Regular

Progress at school was being monitored closelyand the student was in a regular classroomsituation but receiving services of special edu-cation.

SS3, though 10 years of age, was placed ina composite class with students two or threeyears younger. The student had very low self-esteem, exhibited poor posture, and could nei-ther read nor write. At the onset of the pro-gramme the student announced his illiteracyand hence expected failure in all work to begiven. Constant reassurance of ability and highexpectations in tasks assigned were required.SS4 had been in a special class but with

progress was transferred to a regular classroomsituation. However, work given was two yearsbelow the expected grade level and the studentwas experiencing constant failure with the aca-demic work at school.SS5 had been mainstreamed, was receiving

six hours ofone to one tuition weekly at school,but was still experiencing constant failure espe-cially in areas of mathematics.SS6 was in regular school achieving average

results of expected year level in most subjects.The student's performance in mathematics wasabove average and was preparing for the HigherSchool Certificate, an external examinationgenerally taken by year 12 students in theirfinal year of secondary studies in Australia.

ASSESSMENT INSTRUMENTSAssessments used for the students with VCFwere both curriculum and norm referencedbased. Neale Analysis of reading ability wasused to obtain the respective reading ages andto assess the strengths and weaknesses of thecomponent reading skills. Pretest and post-testresults obtained in each experimental periodformed an indication as to whether transfer oflearning skills from individual remedial in-struction to the reading process had takenplace. Results were used to provide normativedata on the student's current reading abilityand insight into the information processingskills, namely speed, fluency of decoding, andcomprehension of materials read. Errors ob-tained from the student's test record were alsoanalysed. Students were also assessed usingadaptive computer testing and results obtainedwere used to structure individualised cur-riculum and make instructional decisions.The World Institute of Computer Assisted

Teaching (WICAT) instructional system wasadopted and students participated in the vari-ous testing programmes. Curlette," in hisevaluation for selection of an integrated learn-ing system for schools, recommended WICAT

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(based on decisions by a committee of in-structional leaders and school personnel),partly for its comprehensive instructional soft-ware and vital information yielded. Mys andPetrie," in a special report for the MichiganEducational Research Association, describedthe effectiveness of the WICAT instructionalsystem as a teaching tool for individualisedinstruction. Results showed a significantgrowth in both reading and mathematics instudents at three schools. Low achieving math-ematics students appeared to show the greatestgrowth.VCF students have been reported to exhibit

immature language usage and poor de-velopment of numerical concepts.7 Hence, theWICAT instructional system was used in thisstudy. WICAT Test of Basic Skills (WTBS), a

battery of computerised curriculum based testsin mathematics and reading/language arts, wastaken by all students. WTBS for grades 2 to 8is designed to measure students' performancelevel in administered objectives. A list of ob-jectives were tested at each level and tests forgrades 2 and 3 include audio facility. Resultsobtained provided specific information aboutstudents' achievement, hence enabling thecharacterisation of each student's strengths andweakness. They further assisted in the planningof individualised instructional programmes forthe students and provided an evaluation ofadaptive computer assisted instruction as an

effective remediation tool for VCF students.The Waterford Learner Profile (WLP) com-

prises seven test modules with a wide range oftests in the area of attitudes, thinking, andlearning skills. It aims firstly to profile students'learning ability in five ability areas, namelyverbal, visual, quantitative, reasoning, andmemory. This module focuses on the process oflearning, providing information about students'learning potential. The other five tests assess

students' effectiveness with language, images,numbers, relationships, and inferences. Theremaining preference and attitude inventoriesofWLP were administered to the older studentsto profile their preferences, learning styles, andattitudes that affect learning.

PROCEDUREAll students started by taking pretests usingboth WICAT computer adaptive testing andNeale Analysis of reading ability. These pretestmeasures were used in later calculations ofpost-test improvements. They also providedinformation about cognitive, literacy, and nu-

meracy abilities of the students necessary forthe construction ofindividualised remedial pro-grammes for them.The experimental period of observation and

remediation was ofapproximately three monthsduration at a time. A three month non-in-tervention term interspersed between the twointervention periods and a total of about sixmonths, two hourly, weekly interaction withmost of the students was maintained to allowevaluation of the remediation system and pro-gress made by the students. All students re-

ceived instructions in an introductory computer

keyboard training course and were requiredto pass the test designed to check keyboardcompetency at the end of the training pro-gramme before working on the computer.WITBS was used and all students' placementswere determined through an adaptive computerprogram. Students were then enrolled in spe-cific courses in both mathematics and reading/language arts. Pretest/post-test comparisonswere made to determine the progress of thestudents in each academic area.

ResultsData obtained from this study provided evid-ence to support the effectiveness of computerassisted instruction in remediating studentswith VCF when implemented under the con-ditions used in the study (table 2).

Results from the Neale Analysis of readingability showed a significant improvement instudents' reading skills as indicated by theirrespective reading ages. This contrasts withthe absence of reported improvements despiteremediation at school.Computer based instruction was able to meet

the needs of the poor readers in the provisionof individualised instruction, practice, and im-mediate feedback. Computer drill and practiceprogrammes helped students learn phonics andsight word recognition and increased their de-coding ability and fluency. Hence students werehelped in developing automaticity in basic read-ing skills. Furthermore, since the computerprovided immediate interactive feedback, stu-dents did not practise their mistakes for longand were guided through problem solving andmetacognitive skills. The computer also pro-vided private tuition in a non-threatening en-vironment allowing students to make mistakeswithout fear of ridicule from their peers. Asstudents learned and experienced success, theygained in confidence and were motivated tocontinue learming.

Table 2A Results from Neale Analysis and reading agesusing accuracy

Students Pretest Post-test Pretest Post-test(Y) (Y) (Y) (Y)

SS1 <6 6-4 6.4 6-9SS2 6-1 7-4 7-4 8-6SS3 <6 <6 <6 6-0SS4 7-8 8-2 8-2 8-8SS5 10 2 12-5 12-5 13-4

Table 2B Results from Neale Analysis and reading agesusing comprehension

Students Pretest Post-test Pretest Post-test(Y) (Y) (Y) (Y)

SS1 6-2 6-11 6-11 7-1SS2 6-1 7.4 7.4 8-3SS3 <6 <6 <6 6SS4 8-3 8-11 8-11 8-4SS5 8-11 9-11 9-11 10-2

Table 2C Results from Neale Analysis and reading agesusing rate

Students Pretest Post-test Pretest Post-test(Y) (Y) (Y) (Y)

SS1 6-4 7-5 7-5 7-6SS2 6-8 7-3 7-3 8-5SS3 <6 <6 <6 6SS4 6-9 8-4 8-4 12-5SS5 12-5 13-4 13-4 13-9

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Table 3 Profile of learning ability of SS4, SS5, and SS6(mean= 100, SD= 15)

SS4 SS5 SS6

Key learning skills(1) Vocabulary 81-5 80-4 87-9(2) Paper folding 47-5 95-7 80-6(3) Number sentences 89-9 79-3 107-5(4) Analogies 82-7 87-5 95-4(5) Word memory 94-9 97-3 104-9Deviation IQ scores 82-4 88 95-3Verbal ability(1) The test of vocabulary 81-5 80-4 87-9(4) The test of sentence verification 74-3 90 4 102-6(6) The test of phonetic decoding 101-8 87-2 87-8Vtsuallspatial ability(1) The test of paper folding 47-5 957 80-6(3) The test of hidden figures 63-1 69-5 99-8Quantitative ability(1) The test of number sentences 69-6 79-3 107-5(2) The test of story problems 80-6 80-4 106-6Reasoning ability(1) The test of everyday critical 80-3 775 105-5thinking(2) The test of everyday problem 86 80-3 90-2solving(3) The test of series completion 81-4 82-2 105-3Memory and attention ability(1) The test of word memory 94 9 97-3 104-9(2) The test of attention 76% - 85%

Observation of SS3 during the experimentalintervention period showed a progression froman insecure, unmotivated, passive learner tosomeone who became actively involved in hisown learning when his confidence was restored.Through encouragement and success in at-tempted work, SS3 progressed at an un-

expected rate which surprised the classroomteacher who had at this stage accepted the lowacademic performance as expected, whilethe student, through constant failure, hadaccepted his helplessness in a learning situ-ation. Torgesen'3 indicated that learning dis-abled students through years of constantfailure doubt their intellectual ability andconsider their efforts to achieve as futile.They are not motivated and are not persistentin their efforts.The Waterford Learner Profile (WLP) was

used to gain insight in each student's learningabilities in the five key learning areas. Strategies,attitudes, and styles associated with the learningof these skills were also investigated. In-formation so obtained from the student's profileallowed the detection of specific strength andweakness. WLP is a wide range series of abilitytests designed to measure learning potentialand learning skills. It uses seven specific testingmodules to provide information on studentabilities. In the area ofgeneral ability this profileuses a battery of tests in five key learning skillsto give a general assessment of the five abilityareas. Results have been tabulated and thismodule is akin to a standard intelligence testbattery (table 3).From table 3, the result obtained by SS4 in

the test of paper folding was unusually lowcompared to those in other key learning skills.Hence for the calculation of deviation IQ, thetest for hidden figures was used as a measureof spatial ability. The deviation IQ scores forSS4, SS5, and SS6 were 82-4, 88-0, and 95-3respectively. Visual/spatial ability was the weak-est area for SS4 and SS6 followed by verbal

ability in all the students. Students performedconsistently within their ability level and nounique weakness was observed.To investigate students' strengths and

weaknesses, extended work from the WICATreading and mathematics programs wereadministered. Results from WICAT test ofbasic skills provided an educational profile ofthese students in various subskills of math-ematics and reading/language arts (table 4).A test of subskills in mathematics showed a

general weakness in manipulation of numbersin all students except SS6. Concepts of nu-merical computation were easily understood byall students when taught and were executedproficiently. All students had difficulties withproblem solving at their own performance leveland required instructions in problem solvingstrategies. Students found conversion ofwrittenlanguage to number sentences difficult. Tellingof time, time calculations, and problems in-volving money presented great difficulties asindicated by the results. Decimals, fractions,and mensuration were also areas of weakness.Older students had difficulties applying con-cepts to new situations and hence require prac-tice in a variety of questions using the sameconcepts.

In reading/language arts, students haddifficulties with basic instruction of punc-tuation and grammar. Phonemic awareness waslow in most students and students who wereaware of letter sounds were not able to applythem to recognition ofnew words. It was foundthat, with some students, formal phonetic in-struction had not been received and emphasishad been on learning of sight words. One mightconsider here the difficulty in the acquisitionofan adequate visual vocabulary without phon-ological support. Liberman and Shankweiler"4stated that many of the children who en-countered reading difficulties had poor phon-ological awareness and had not attained theability to recognise phonemes or syllables. Bra-dley and Bryant,'5 who found a linkage betweena lack of phonological awareness and poorreading skills, also reported a higher successrate in reading and spelling for students whoreceived training in phonological awareness.Skills of phonological awareness should playan important role in literacy education, par-ticularly for VCF students, and a blending ofaspects of the meaning from context approachcan be beneficial.

In the area of comprehension, all older stu-dents, while scoring well in literal com-prehension questions, experienced difficultieswith inferential comprehension of prosematerials. This was expected as inferentialitems demand a higher level of cognition thatis beyond word recognition and recall of facts.Consequently weakness in other subskills in-cluding obtaining main idea or purpose ofwriter and drawing conclusions was also noted.VCF students need to be taught these skillsthrough practice and drills in reading com-prehension programmes. As a characteristic ofVCF children, all basic facts and what is as-sumed as common sense knowledge to otherchildren have to be taught. Hence, com-

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Table 4 Summary of significant improvements for each student in specific readingllanguage arts and mathematicalsubskills using WICAT programsA Pretest and post-test results of SSI

Mathematics subskills Pretest (%) Post-test (%) Readingllanguage arts subskills Pretest (%) Post-test (%/)Problem solving 10 35 Mechanics 21 32Integers 10 35 Capitalisation 17 50Addition facts 0 50 Punctuation 17 33Subtraction facts 25 100 Expression 33 59Time/money 0 20 Usage 30 59Place value 20 40 Noun form 0 75Representation 33 67 Verb form 17 50Geometry 60 80 Modifiers 33 83

Sentence structure 35 53Complete sentence 24 41

B Pretest and post-test results of SS2

Mathematics subskills Pretest (%l) Post-test (%lo) Readingllanguage arts subskills Pretest (%) Post-test (%)

Problem solving 10 42 Mechanics 26 35Integers 10 50 Capitalisation 33 50Addition facts 0 50 Punctuation 17 33Subtraction facts 25 100 Expression 48 48Numeration 32 41 Usage 48 48Place value 20 20 Noun form 75 100Representation 33 67 Verb form 33 50Geometry 60 40 Modifiers 33 83Measurement 10 50 Sentence structure 48 53Time/money 0 60 Complete sentence 17 67

C Pretest and post-test results of SS3

Mathematics subskills Pretest (%lo) Post-test (%) Readingllanguage arts subskills Pretest (%) Post-test (%)

Counting 25 100 Identify lett comb 33 50Order/compare Nos 25 75 Sounds initial 17 67Subtr 1&2 digits 25 50 Rhyming 25 50Mult 1 digit Nos 0 50 Blends 20 60Measurement 25 75 Phonetic 36 55Time 50 75 Vocabulary 64 73Money 25 100 Sentences 75 100Representation 50 100 Word usage 70 80Problem solving 75 100 Noun form 60 100Concepts 67 87 Verb form 60 80Numeration 63 94 Comprehension 88 100

D Pretest and post-test results of SS4

Mathematics subskills Pretest (%) Post-test (%) Readingllanguage arts subskills Pretest (%lo) Post-test (%)

Numerals 25 50 Spelling 13 25Place values 25 50 Prefixes 0 50Add 2 digits 0 25 Punctuation 0 50Subtr 2 digits 50 75 Sentences 25 50Mult 2 digits 0 25 Antonyms 0 50Add decimals 25 50 Cathegorising 0 25Mult decimals 25 75 Plot, cause-effect 0 50Perimeter 0 50 Implied main idea 25 50Money calculation 0 50 Implied elements 0 25Word problems 0 50 Characterisation 25 75

Draw conclusion 25 50

E Pretest and post-test results of SS5

Mathematics subskills Pretest (%l) Post-test (%) Readingllanguage arts subskills Pretest (%lo) Post-test (%)

Add/sub 4 digits 0 75 Syllables 25 50Mult 2&3 digits 25 50 Spelling 25 50Add/sub decimals 25 75 Parts of speech 25 50Univalent fract 25 50 Style and form 25 50Factors HCF 0 25 Verb form 25 50Add/sub fract 0 25 Possessive forms 25 75Reciprocals 50 100 Plot, cause-effect 25 75Mult/div fract 0 50 Implied main idea 25 50Protractor use 75 100 Implied relationship 0 25Units of measure 0 25 Author's purpose 25 50Geom terms, shape 50 100 Fact, persuasion 0 25Areas 0 50 Directions, forms 25 50Coordinates 75 100 Dictionary use 25 75Problems, dec/frac 0 50 Proofread mech 0 75Problems, 2 steps 0 50

F Pretest and post-test results of SS6

Mathematics subskills Pretest (%) Post-test (%) Readingllanguage arts subskills Pretest (%) Post-test (%)

Measurement 25 35 Overall writing 33 57Rate of motion 25 50 Subject, verb 43 71Prob time, money 25 50 Verb tense 40 80Neg exponents 50 75 Mechanics 44 69Algebra 42 50 Spelling 33 83Geometry 58 75 Popular literature 50 59Pythagoras' theorem 75 100 Popular fiction 83 100Basic maths set up 50 100 Popular non-fiction 63 75

Popular poetry 0 25Popular drama 0 25Classical literature 33 44Classical non-fict 40 60Inf comprehension 40 45Analysis 36 55

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prehension of an inferential nature would beparticularly difficult for them.During the experimental intervention period,

comments by students and parents regardingattitude, self-esteem, response to CAI, andschool performance were recorded. The gen-eral preferences inventories section of WLPassesses students' learning styles and attitudesusing five subtests. It should be emphasisedthat this individual student profile providesonly a general guide to students' learning styles.It was interesting to note that social styles ofthese students appeared to be generally non-

assertive in nature and the compliance score

obtained was high. This indicates the import-ance placed on group acceptance and studentswould go with the group rather than assertthemselves and risk social acceptance. Studentswere also unimaginative but possessed an ana-

lytical, orderly learning style. They preferredlogical explanations and specific instructionsand generally focused their attention on

one thing at a time. Learning styles showedimpulsiveness in two of the students, indicatinga lack of efficient learning strategies for studies.All students preferred to be taught as opposedto learning from reading, and enjoyed workingwith computers.

All results obtained lend support to theeffectiveness of computer assisted instructionas a remediation tool for these VCF students.VCF children often have a bland expressionand this in many situations can be interpretedas non-comprehension resulting in incorrectplacement and low expectation and per-

formance level at school. This problem be-comes insignificant when the student works inan individualised adaptive computer pro-

gramme and is allowed to progress according tohis achievement. Though students were havingremediation using traditional techniques atschool there was no reported improvement inperformance level.

Progress made by SS3 was almost im-mediate, as within a few lessons he was writinghis own name, spelling simple words, andpleading to be taught reading and challengingwork. SS3 remained enthusiastic through theyear and was able to embark on a daily readingprogramme at school towards the end of theexperimental period. Basic skills learned bystudents were transferred to classroom learningand there were reported improvements fromschool. With the progress made, SS3 has sincebeen removed from his composite infants' re-

medial class to be with students of the ap-

propriate age and grade level.Older VCF students exhibited poor learning

strategies. SS4 and SS5 were not independentlearners as they had not acquired learning strat-

egies or metacognitive skills to help themselvesin the learning process. However, with in-teractive individualised computer based in-struction, students began to develop learningstrategies and applied them particularly to thelearning of spelling, reading comprehension,and mathematics. Again, basic skills learnt weretransferred to classroom learning. SS4 receivedher first merit certificate for a perfect score inspelling awarded by the school at a school

assembly and SS5 passed her first mathematicsgrade level test. SS5 has since developed goodreading habits and her parents reported herfirst incident of voluntary silent reading.

All VCF students who participated in thisstudy expressed enthusiasm working with com-puters. If this enthusiasm would motivate stu-dents to spend more time reading or practisingother skills using computer assisted systems,then this would lead to achievement gains inthe various areas. Computer assisted in-struction was successful in the remediation ofVCF students and consequently an im-provement in reading ability and mathematicalsubskills for these students was recorded. Thesuccess experienced created higher self-esteemand confidence in these students.

DiscussionPrevious research has indicated learningdifficulties or disabilities as characteristic ofchildren affected by VCFS. Responses fromquestionnaires of 10 families have also shownevidence of developmental learning disabilitywhich included deficits in the prerequisite skillsneeded for learning ofacademic subjects. Theseinclude motor, perceptual, language, cognitive,and social skills that are generally acquired bypreschool children with no learning problems.However, for children with VCF these skillshave to be taught. Consequently interventionat an early stage and provision of an immediateintervention programme for these establishedat risk children can prove to be a success story.It is anticipated that potential learning failurescan be lessened. Though deviation IQ scoresof these students are within the low normalrange, their performance levels were ap-proximately two years behind their peers. Chil-dren with learning difficulties detected in theearly grades need continued instructional sup-port and failing to receive this would lead to awider gap between their performance and thatof their peers.

Results indicate support for the use of in-teractive computer instruction for VCF stu-dents. This technique has also been successfullyused by Van Daal and Van der Leij16 with othergroups of learning disabled children in thelearning of reading and spelling. It is en-couraging to note the improvement made byall the VCF students as they were all involvedwith other forms of intervention with no meas-urable improvement. However, with no othercomparison study many questions are left un-answered. With intervention at the infant orearly primary level, would intervention at a laterstage be reduced, intermittent, continuous, ornecessary? Would intervention provided at anearly stage raise performance level to enablestudents to be successful at school? Academiclearning difficulties detected during the periodof intervention include deficits in reading, writ-ing, spelling, and mathematics. Throughoutthe experimental intervention period, it wasnoted that for successful learning, there shouldbe no assumed knowledge and students haveto be taught basic skills for each topic area. Allstudents exhibited varying degrees of learning

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difficulties and responded positively to in-teractive computer assisted instruction withresults showing marked improvement in vari-ous subskills. Reading ages and self-esteemhave been raised in all students. This is con-sistent with results obtained by MacArthur andMalouf" in their study of teachers' response tocomputer assisted instruction. In addition tocomments on specific academic outcomes,teachers emphasised global affective outcomes,such as increased motivation and self-esteem.They strongly believe that computer skillswould allow their students to have fun whilestill getting some instruction, foster computerliteracy, gain a competitive edge over otherstudents, and help their self-esteem.

Observations of these students have led tocharacteristics described in this paragraph.Older VCF students have become inefficientand passive learners. Through constant failurethe attitude oflearned helplessness has emergedand students await specific instructions ratherthan be motivated to solve the problem them-selves. However, as they became more com-fortable requesting help from the computerby using the "help" key and were guided byproblem solving strategies and metacognitiveskills, they became more independent learners.Frustration and previous failure in learning hadcaused the loss of self-confidence, developmentof low self-concept, and consequential emo-tional problems. Most of these students hadinept social skills, were also not sensitive tosubtle hints, and had to be taught specific socialbehaviour. Students also tended to be impulsivelearners, always quick to respond withoutthinking, which is detrimental to learning.There were also motivational problems andstudents generally gave up easily as they doubttheir own intellectual ability. Hence studentsmust be taught how to learn allowing them to

be more efficient learners and be equipped withan array of learning strategies to cope withschool subjects.The treatment ofVCF children lies primarily

in education. The significance of this studylies in its attempt to use interactive computerassisted instruction to investigate outcome ofintervention for VCF students, learning habits,and characterisation of the VCF student. Theresults of this study provided strong supportfor this method as an effective remediation toolfor the students. However, the effectiveness ofcomputer assisted instruction is influenced bylimitations to this form of instruction. Barriersencountered include the current inadequatecomputer availability in many schools and the

inaccessibility to appropriate instructional soft-ware. However, as shown by the results, im-provements in some academic areas for theseVCF students are possible. These results ob-tained are contrary to the accepted view thatdespite remediation VCF students continuedto be poor learners at school with no significantimprovement in performance. The enthusiasticresponse by all students for the computer as-sisted system was most encouraging and furtherresearch should be conducted to explore itspotential to facilitate appropriate educationalintervention.

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