This study compared 20 prelingually profoundly deaf adolescents (age 11-16 years) and 20 matched, hearing adolescents on a picture-sequencing task and a social judgment test. Results did not support the hypothesis that deaf adolescents possess significantly poorer knowledge about social reasoning than hearing peers but it did support the…

Analysis of data from 126 children with autism found macrocephaly (head circumstance microcephaly (head circumference <3rd centile) was found in 15.1%. Microcephaly was significantly associated with the presence of medical disorders. (Author/DB)

This study examined whether the variability in development in fragile X syndrome is related to FMR protein deficiency in 53 young males with various degrees of the syndrome. FMR protein expression accounted for a small but significant amount of variance in level, but not rate, of development. Findings support the hypothesized relationship but…

This paper describes the developmental and behavioral patterns of 13 prelinguistic children (ages 11 to 47 months) with Cri-du-chat syndrome (CDCS). Parents provided demographic and genetic information, descriptions of their child's typical behavior, and completed a developmental checklist. Developmental, behavioral, and genetic profiles are…

Seven individuals with Prader-Willi Syndrome and seven obese individuals without the syndrome were compared on their choice of either a larger quantity of food delivered after a delay or a small quantity of food delivered immediately. Individuals with Prader-Willi selected the larger food quantity whereas choices by the obese comparison group was…

The study investigated the communicative development of 18 children (ages 8-17) with Prader-Willi syndrome. A variety of communicative deficiencies were found in the children's speech, language, voice, and fluency. (Author/DB)

The performance of 17 prelingually severely and profoundly hearing-impaired subjects on the Speech Pattern Contrast Test (Version II) was evaluated. Although performance on most of the contrast subtests was significantly correlated with phoneme and word recognition, intercorrelations among most subtests were also significant. (Author/DB)

Comparison of the production of repetitive speech during conversations in 30 people with either fragile X syndrome, autistic disorder, or mental retardation not caused by fragile X found repetitive speech more prevalent among those with fragile X. Results support the hypothesis that such speech dysfluency reflects the effects of physiological…

This study evaluated figure drawings of 28 persons with Williams syndrome, 28 with mixed etiologies, and 28 with Down syndrome. Human figures from participants with Williams syndrome were no more deviant than their counterparts, nor did they show "local-global" differences. Findings support a developmental, rather than deviant,…

Two experiments, one with congenitally deaf and one with hearing individuals, investigated memory for serial order via Cued Speech (CS). Deaf individuals, but not hearing individuals experienced with CS, appeared to use the phonology of CS to support their recall. The recency effect was greater for hearing individuals provided with sound than for…

Analysis of the incidence of autistic disorder (AD) among 5,271 children in a neonatal intensive care unit in Japan found that 18 children were later diagnosed with AD, an incidence more than twice as high as previously reported. Children with AD had a significantly higher history of the meconium aspiration syndrome than the controls. (Author/DB)

This article presents a case report of a male child with Fragile X syndrome, typical autistic behavior, and cerebellar hypoplasia. Questions are raised concerning the possible role and importance of cerebellar abnormalities in relation to autistic symptoms. (DB)

This article notes a current de-emphasis on genetic syndromes in published articles concerning mental retardation despite major deoxyribonucleic acid (DNA) advances in determining mental retardation causes. The article calls for incorporation of these genetic advances into behavioral research of mental retardation, especially as reflected in the…

Analysis of questionnaires completed by caregivers of 77 individuals with Cornelia de Lange syndrome in the United Kingdom found a significant association between self-injurious behaviors and self-restraint, and those displaying both behaviors displayed significantly more compulsions than did those not exhibiting them. Findings extend the…

Three models of behavioral effects of genetic disorders characterized by mental retardation were compared: (1) no-specific effect model (all genetic disorders have identical behavioral outcomes); (2) totally specific model (each genetic disorder has unique outcomes); and (3) partially specific model (two or more genetic disorders share outcomes…