[Specific disorders of mental development].
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The Sonority Sequencing Principle is a presumed universal that governs the permissible sequences of consonants within syllables. In two single-subject experiments, we evaluated this principle as applied to the acquisition of onset clusters and adjuncts by children exhibiting functional phonological delays (age in years;months: 3;2 to 7;8). Experiment 1 tested the hypothesis that children abide by the Sonority Sequencing Principle in development, such that the occurrence and use of marked true clusters implies unmarked clusters, but not vice versa. This claim was validated, in part, by the gradient generalization learning patterns of children who were taught marked clusters. Others who were taught unmarked clusters exhibited limited learning characteristic of within-class generalization, with apparent gaps in sonority sequencing. Experiment 2 examined the role of adjunct sequences /sp, st, sk/, whose markedness status is questionable given their violation of the Sonority Sequencing Principle. Results indicated that children learned adjuncts consistent with patterns of within-class generalization, thereby supporting the view that these sequences are unmarked in structure. Experimental findings are integrated in discussion of the representation of onset clusters and their course of emergence in phonological acquisition relative to the Sonority Sequencing Principle.
For several decades, the aetiology of specific language impairment (SLI) has been associated with a central auditory processing deficit disrupting the normal language development of affected children. One important aspect for language acquisition is the discrimination of different acoustic features, such as frequency information. Concerning SLI, studies to date that examined frequency discrimination abilities have been contradictory. We hypothesized that an auditory processing deficit in children with SLI depends on the frequency range and the difference between the tones used. Using a passive mismatch negativity (MMN)-design, 13 boys with SLI and 13 age- and IQ-matched controls (7-11 years) were tested with two sine tones of different frequency (700Hz versus 750Hz). Reversed hemispheric activity between groups indicated abnormal processing in SLI. In a second time window, MMN2 was absent for the children with SLI. It can therefore be assumed that a frequency discrimination deficit in children with SLI becomes particularly apparent for tones below 750Hz and for a frequency difference of 50Hz. This finding may have important implications for future research and integration of various research approaches.
This study examines the developmental history of 32 Williams syndrome patients, positive to the fluorescence in situ hybridization (FISH) test. The information is intended to provide help for early diagnosis and appropriate stimulation of these patients. In the sample reported here, only about half of the patients referred with presumptive diagnosis were in fact FISH+, indicating that facial dysmorphism may not be the most reliable sign for diagnosis. Initial pediatric signs are developmental delay and nocturnal irritability. In consultation, facial dysmorphies and heart murmur are detected. There is also low birth weight, failure to thrive, unsuccessful breastfeeding, and gastroesophageal reflux. All these symptoms are strongly suggestive of Williams syndrome. Subsequent steps consist of cardiologic studies. Our results indicate that the triad of symptoms consisting of infantile hypercalcemia, dysmorphic facies, and supravalvular aortic stenosis, which until recently was considered fundamental for Williams syndrome diagnosis, is not usually present and does not lead to an early diagnosis. Cognitively, these children are characterized by hypersociability, hyperacusia, deficient visuoconstructive abilities, attentional deficit and hyperactivity, and in some cases, spontaneous musical interests. There are no special verbal skills. The results of this study indicate that the concept of Williams syndrome patients as language- and musically-gifted is not fully accurate.
Underidentification of developmental delays among young children involved with child welfare/child protective services (CW) is problematic. Caregivers of young children involved with CW may help increase identification of young children with developmental delays, but the accuracy of caregiver identification in this population and whether this varies by caregiver type is not known. This study uses data from the National Survey of Child and Adolescent Well-Being to determine if (1) caregivers of young children involved with CW accurately identify children with developmental delays and (2) foster caregivers are better able to identify developmental delays compared with other caregivers. Close to half the children had a delay in language, cognitive, and/or adaptive behavior (45%). Overall sensitivity for caregiver identification was 35% (95% confidence interval [CI]: 29%, 41%); specificity was 84% (95% CI: 80%, 87%). After controlling for certain child and caregiver characteristics, in-home caregivers had 0.15 times the odds of identifying a child with a developmental delay compared with foster caregivers (95% CI 0.1, 0.4). Results suggest that caregiver identification of developmental delays is specific but not sensitive, and that foster caregivers were more likely to identify a child with a developmental delay compared with in-home caregivers. Policy implications include improving educational programs regarding child development and developmental services for foster, kinship, as well as in-home caregivers in the hopes of increasing sensitivity of caregiver identification of developmental delays for the population of young children involved with CW.
BACKGROUND: Developmental delay (DD) in infants and children is one of the chief complaints of parents. It has been established that the concerns of parents are as accurate as quality screening tests. Some kinds of concerns are particularly useful in the early detection of associated developmental problems. The purpose of this study was to investigate the relationship between the main parental concerns and the final diagnosis based on professional assessment of children who are suspected to have DD. METHODS: One-hundred and 1 infants or children were recruited into this study. The major concerns of parents were elicited and categorized by various developmental domains: speech, motor, behavioral, cognitive, global, and non-specific developmental problems. All children underwent comprehensive combined assessments by professionals in the hospital, and were classified into 6 subtypes: speech, motor, behavioral, cognitive, and global DD, and normal development. RESULTS: Our results revealed that parental concerns about speech, motor, and behavioral development yielded a high sensitivity to the final diagnosis of the same developmental domain (77-89%). However, concerns about cognitive and global DD had limited sensitivity (15-36%). On the other hand, concerns about global, speech, and motor DD had relatively higher positive predictive values (55-77%). Comparatively, cognitive or behavioral concerns had lower positive predictive values (25-33%). CONCLUSIONS: Such results indicate that parents play an important role in detecting speech, motor and behavioral DD in children. Parental concerns about cognition and behavior should be questioned in terms of their association with the real problem. This information may be useful in establishing trends in understanding the discrepancy or relationship between parental concerns and professional assessments in DD.
The literature on various aspects of learning and performance in autistic children is reviewed and interpreted as indicating very little that is specific to autism. Inadequate and inconsistent methodology precludes generalizations concerning the nature of the disorder. It is suggested that future research should be particularly concerned with controlling for developmental influences on performance, and with investigation of the higher functioning autistic children who are less governed by retardation factors. The current evidence is considered to support a hypothesis concerning abnormal hemisphere functioning in this group of children.
The Behavior Summarized Evaluation (BSE), developed for the assessment of autistic behavior, was specifically designed to evaluate the severity of behavioral problems in autistic children involved in bioclinical and therapeutic studies. The reliability studies and the factorial analysis of this scale have been previously published. The present paper examines the effectiveness of the BSE to discriminate 58 autistic from 58 nonautistic mentally retarded children. The BSE clearly separated the two samples of children. A most efficient combination of 8 items emerged from the stepwise item selection procedure. The between-group differences were highest on 4 items, indicating that the most particular pattern in autistic compared to nonautistic children could be the association of autistic withdrawal and stereotypic behaviors. Our findings suggest that the BSE could help in the detection and evaluation of autistic developmental deviance. Implications for further research are discussed.
UNLABELLED: The Floating-Harbor syndrome is a growth retardation syndrome with delayed bone age, speech development, and typical facial features. The face is triangular with deep-set eyes, long eyelashes, bulbous nose, wide columella, short philtrum, and thin lips. We present an additional patient and review 16 cases from the literature. The possible phenotype in the patient's mother suggests a dominant mode of inheritance for the syndrome. CONCLUSION: The Floating Harbor syndrome is a growth deficiency syndrome characterized by proportionate short stature, characteristic face and delayed speech development. Inheritance is possibly autosomal dominant.
Approaches to the treatment of developmental verbal dyspraxia based upon current theories about phonological development are explored. The author presents the concept of "bridging"; for example, making the transition from one sound to another or from one word to the next, as a reflection of the child's ability to generate hierarchical linguistic structures. The author suggests that children who have developmental dyspraxia must build phonological systems despite the fact that their ability to discover and use these hierarchical structures is impaired. Idiosyncratic patterns are expected, and should be used by the clinician to determine appropriate approaches to remediation for individual children. Case studies illustrate two children's shared difficulties in developing and using phonological hierarchies and the individual differences that provided a basis for appropriate remediation for each child.
Children of women who smoke cigarettes during pregnancy display cognitive deficits in the auditory-verbal domain. Clinical studies have implicated developmental exposure to nicotine, the main psychoactive ingredient of tobacco, as a probable cause of subsequent auditory deficits. To test for a causal link, we have developed an animal model to determine how neonatal nicotine exposure affects adult auditory function. In adult control rats, nicotine administered systemically (0.7 mg/kg, s.c.) enhanced the sensitivity to sound of neural responses recorded in primary auditory cortex. The effect was strongest in cortical layers 3 and 4, where there is a dense concentration of nicotinic acetylcholine receptors (nAChRs) that has been hypothesized to regulate thalamocortical inputs. In support of the hypothesis, microinjection into layer 4 of the nonspecific nAChR antagonist mecamylamine (10 microM) strongly reduced sound-evoked responses. In contrast to the effects of acute nicotine and mecamylamine in adult control animals, neither drug was as effective in adult animals that had been treated with 5 days of chronic nicotine exposure (CNE) shortly after birth. Neonatal CNE also impaired performance on an auditory-cued active avoidance task, while having little effect on basic auditory or motor functions. Thus, neonatal CNE impairs nicotinic regulation of cortical function, and auditory learning, in the adult. Our results provide evidence that developmental nicotine exposure is responsible for auditory-cognitive deficits in the offspring of women who smoke during pregnancy, and suggest a potential underlying mechanism, namely diminished function of cortical nAChRs.
The adaptive behaviors of 20 autistic, 20 Down syndrome, and 20 developmentally normal children were compared using the Vineland Adaptive Behavior Scale. Unlike previous studies, we included a comparison group of very young normally developing children and matched subjects on overall adaptive behavior as well as several pertinent demographic characteristics. Findings revealed that, relative to children with Down syndrome or normal development, autistic children displayed significant and pervasive deficits in the acquisition of adaptive social skills, and greater variability in adaptive skills. These findings underscore the need to longitudinally assess the development of socialization in autistic children and further highlight the utility of the Vineland in operationally defining the nature of social dysfunction in autistic children.
The communicative behavior of autistic and developmental language delay (DLD) children matched for nonverbal mental age and mean length of utterance (MLU) was compared to that of normally-developing (ND) 2-yr-olds. Autistic children were less able than other children to respond correctly to language or gestures used to direct their attention, used attention directing (pointing, showing) less frequently, and produced more echolalic speech when making requests. DLD and ND children differed only in number of pronouns used while requesting. Attention-directing gestures were associated with receptive and expressive language abilities for autistic, DLD and normal children.
This study consecutively examined the effects of 2 social interventions-- peer training and written text treatment--on the social communication of 5 elementary students with pervasive developmental disorder. Each child with autism was paired with 2 peers without disabilities to form 5 triads. In Intervention 1 (peer training), peers were taught to use 5 facilitative social skills over 5 days. After peer training, 4 children with autism increased or used more stable rates of initiations and contingent responses overall. However, all children continued to demonstrate deficits in specific social-communication skills. Once Intervention 2 (direct instruction using written text cues) was implemented, increased use of 3 different communication skills was observed across all 5 participants. In addition, social validity outcomes revealed improved quality of child-peer interactions, 2 teacher reports of improved social skill development, and improved acceptance and friendship ratings for the children with autism. Results support the use of written text cues to improve children's social communication with peers, and suggest that combining approaches may be necessary to improve the quality of children's relationships.
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The prevalence of 3-methylglutaconic aciduria was evaluated among children with developmental language disorders. A urine specimen was obtained from 40 children referred for developmental language delay to the Tel-Aviv Child Development Center during 12/96-6/97 and from 50 age-matched controls. Urine organic acids were analysed by gas chromatography-mass spectrometry. Urinary 3-methylglutaconic acid was quantified. A mildly increased excretion of 3-methylglutaconic acid was found in 8 children with developmental language delay. The combined excretion of 3-methylglutaconic and 3-methylglutaric acid was increased in 9 patients. There were no differences in the excretion of other organic acids. The patients with elevated 3-methylglutaconic acid did not differ from the other patients with developmental language disorders in any of the parameters evaluated. Mildly elevated urinary levels of 3-methylglutaconic acid may be a marker of a still undefined metabolic disorder presenting with developmental language delay. A further study in large groups of children with different developmental disorders is mandatory.
Setting and task conditions were assessed for their effects on the language of hyperactive children. Verbal data were recorded for 22 hyperactive children and 22 elementary-age controls during four storytelling tasks and transitions, and under two conditions of a nonverbal performance task in a counterbalanced order. Findings were that hyperactive children were more spontaneously talkative than their classmates during transitions and nonverbal tasks (nonelicited conditions) but were less talkative when they were asked to tell stories (elicited conditions). These findings and those attributable to the story comparisons were interpreted in line with the optimal stimulation theory, which suggests that minimal stimulus input (delays and nonelicited conditions) precipitate excessive verbal activity from hyperactive children. Production deficiencies, on the other hand, were specific to type of stimulus input to be processed. Stories requiring organization and planning without the external structure or salience of visual cues (a sequence of word cards or pictures) produced production deficiencies.