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Psychological and speech studies in Rubinstein-Taybi syndrome.

Forty individuals with Rubinstein-Taybi syndrome were tested using an extensive test battery to obtain more insight about their intelligence level, social competency, temperament, and behavior as well as articulation and receptive and expressive language level. Examination of individuals in the Netherlands who have this rare syndrome has been extensive, allowing some generalizations to be made. The intelligence level of affected individuals is usually low, although some persons have much higher scores than do others. The tested individuals were remarkably consistent in their social competency, temperament, and behavior. They were able to make good use of their limited verbal abilities. Comparable studies of other groups of persons with a specific syndrome are needed to determine whether the present findings are specific for Rubinstein-Taybi syndrome or may be found among persons with other syndromes.

Adolescent

Very-low-birthweight children and speech and language development.

Very low birthweight (VLBW) is often considered to be a risk factor for speech and language disorders, yet data are equivocal. The present study compared speech and language comprehension and production between 249 very-low-birthweight (VLBW: less than 1.5 kg) and 363 normal-birthweight 8-year-olds, randomly sampled in a geographic area. Mean performance for the entire group of VLBW children and for the group when 24 VLBW children with major neurologic abnormalities were excluded, was significantly lower than for controls on the majority of speech and language measures. Further analyses addressed the clinical significance of these statistically significant differences. Test scores were converted to standard scores and grouped according to standard deviation intervals, thus portraying each child's performance in terms of the magnitude of discrepancy from each test's mean. When the 24 children with major neurological abnormalities were excluded, no significant differences between the VLBW and control children were observed. Using discrepancy between WISC-R performance IQ and language to define specific language impairment (SLI), a higher percentage of control than VLBW children were identified as having SLI. Neonatal risk factors did not differentiate between VLBW children with or without SLI. A higher proportion of VLBW than control children did present subnormal language associated with IQ less than 85, hearing deficits, and/or major neurological impairments. Thus, SLI is not more common among VLBW than control children. Language deficits accompanied by more general developmental problems, however, are more frequent.

Chi-Square Distribution

Sensitivity and specificity of the neonatal brain-stem auditory evoked potential for hearing and language deficits in survivors of extracorporeal membrane oxygenation.

OBJECTIVE: We determined the sensitivity and specificity of neonatal brain-stem auditory evoked potentials (BAEP) as markers for subsequent hearing impairment and for developmental problems found later in infancy and childhood. METHODS: BAEP studies were performed before discharge in infants treated with extracorporeal membrane oxygenation (ECMO), and two specific abnormalities were analyzed: elevated threshold and delayed central auditory conduction. Behavioral audiometry was repeated during periodic follow-up until reliable responses were obtained for all frequencies, and standardized developmental testing was also conducted. The sensitivity and specificity of an elevated threshold on the neonatal BAEP for detecting subsequent hearing loss, and the relationship of any neonatal BAEP abnormality to language or developmental disorders in infancy, were calculated. RESULTS: Test results for 46 ECMO-treated infants (57.5%) were normal, and those for 34 infants (42.5%) were abnormal, with either elevated wave V threshold, prolonged wave I-V interval, or both on neonatal BAEP recordings. Most significantly, 7 (58%) of the 12 children with subsequent sensorineural hearing loss had left the hospital after showing normal results on threshold tests. There was no significant difference in the frequency of hearing loss between subjects with abnormal (5/21, or 24%) and those with normal BAEP thresholds (7/59, or 12%; Fisher Exact Test, p = 0.28). Therefore the sensitivity of neonatal BAEP testing for predicting subsequent hearing loss was only 42%. Neonatal BAEP specificity for excluding subsequent hearing loss was 76%. In contrast, on language development testing, 19 children demonstrated receptive language delay. Of these children, 12 (63%) had abnormal neonatal BAEP recordings and 7 (37%) had a normal BAEP threshold, normal central auditory conduction test results, or both (p = 0.04). CONCLUSIONS: Neonatal BAEP threshold recordings were of limited value for predicting subsequent hearing loss common in ECMO-treated survivors. However, an abnormal neonatal BAEP significantly increased the probability of finding a receptive language delay during early childhood, even in those with subsequently normal audiometry findings. Because neonatal ECMO is associated with a high risk of hearing and receptive language disorders, parents should be counseled that audiologic and developmental follow-up evaluations in surviving children are essential regardless of the results of neonatal BAEP testing.

Acoustic Impedance Tests

[Retarded development of speech and language because minimal cerebral dysfunction (author's transl)].

Retarded development of speech and language may represent a facultative symptom of minimal cerebral dysfunction. The current neurologic and psychologic or psychiatric considerations in children are reviewed, and follow the author's experiences in speech and language. The treatment for this in patients with minimal brain dysfunction is based on four principles: a) Consultation with patients; b) exercise proceedings in patients involved; c) psychological or pedigogical methods; d) drug therapy. The prognosis for effective therapy in these patients is considered to be quite good.

Attention Deficit Disorder with Hyperactivity

Otitis media and language performance in a cohort of Apache Indian children.

Language performance was evaluated in 167 healthy Apache Indian children aged 6 to 8 years, who had contrasting histories of otitis media and who had been followed since birth. All children received pneumatic otoscopy, tympanometry, pure tone audiometry, and a battery of language measures. Groups were similar with respect to age, sex, school experience, family income, housing, primary language spoken, and nonverbal intelligence. No statistically or clinically significant differences in language performance were observed between groups of children with frequent episodes of otitis media and those from the same cohort with infrequent otitis. While frequent otitis media was associated with abnormalities on tympanometry and otoscopy, bilateral hearing loss (greater than 25 dB) was observed in only 1% of the children.

Acoustic Impedance Tests

Generality in the verbal tacting of autistic children as a function of "naturalness" in antecedent control.

Two autistic children were taught to emit verbal tacts under two conditions--one employing verbal antecedent stimuli in the training process and the other employing so-called naturalistic antecedent stimuli. Although acquisition was more rapid when verbal antecedents were used, the generality of learning was substantially better for language taught under naturalistic stimulus control. Implications of these findings for the instruction of autistic children are considered in the context of current information on stimulus control.

Autistic Disorder

Stuttering, language, and cognition: a review and a model of stuttering as suprasegmental sentence plan alignment (SPA).

Extant models of stuttering do not account for the emergence of stuttering at the onset of productive language use; the greater incidence of stuttering during spontaneous speech, on complex sentences, and at sentence-initial positions; the greater incidence of stuttering in bilinguals' 2nd language; the apparent deficiency of stutterers in expressive and receptive language skills; the prevalence of spontaneous recovery from stuttering; and the lack of chronic physiological or articulatory deficits in stuttering children's fluent speech. The author presents a model of stuttering as points of suprasegmental sentence plan alignment (SPA). Such alignment processes occur when, due to on-line sentence production processes, SPAs adopted prior to utterance initiation need to be aligned with revised SPAs. This model parsimoniously accounts for the findings reviewed in the article.

Adult

Hypothesis-testing and nonlinguistic symbolic abilities in language-impaired children.

This study sought to clarify further the cognitive abilities of language-impaired children by examining their hypothesis-testing and nonlinguistic symbolic abilities. A discrimination learning task and a concept formation task were used to measure hypothesis-testing abilities, and a haptic (touch) recognition task was used to assess nonlinguistic symbolic abilities. Subjects were 10 language-impaired and 10 language-normal children matched for performance Mental Age. Measures of expressive and receptive language were also obtained from each child. The language-impaired children were found to perform significantly poorer than their MA controls on the haptic recognition task and on a portion of the discrimination learning task. No differences were found between the two groups' concept formation abilities. Correlational analyses revealed a particularly strong positive relationship between performance on the Peabody Picture Vocabulary Test and the haptic recognition tasks. It was speculated that this relationship was motivated by the symbolic demands of these tasks. One implication of this speculation is that a symbolic representational deficit might better explain the receptive language deficit than the expressive one.

Age Factors

Diagnosing specific language impairment in adults for the purpose of pedigree analysis.

Two sets of diagnostic measures were administered to a group of 35 adults with well-documented histories of specific language impairment and to a control group of 35 normal language users. These measures involved the comprehension and production of words and sentences in formal and spontaneous speaking activities as well as measures of verbal memory and auditory temporal perceptual ability. One set of tasks was administered in a standard face-to-face setting and the other set was given over the telephone. Multivariate and univariate tests indicated that the adults with a history of specific language impairment performed more poorly on all tasks administered. A discriminant analysis of the two sets of measures indicated that four measures in each set identified language-impaired individuals with 97% accuracy for the face-to-face battery and 95% accuracy for the telephone battery. These results suggest that it should be possible to diagnose specific language impairment in the adult family members of children with specific language impairment and therefore permit accurate construction of pedigrees for specific language impairment.

Adult

Preschool speech and language screening: further validation of the sentence repetition screening test.

A recent review indicated that only one speech and language screening test (the Sentence Repetition Screening Test; SRST) designed for preschoolers has been successfully validated in a representative population. This study sought to replicate the SRST validation study using a somewhat younger age group and to compare predictive indices to typical measures of parent concern and teacher judgment. A sample of (N = 343) prekindergarten children (ages 54 to 66 months) attending school registration in the spring before kindergarten entry was tested with the SRST. A stratified sample of 76 returned for criteria testing within 2 months. Teacher ratings were obtained 5 months later. Outcome measures were standard language (Bankson and Illinois Test of Psycholinguistic Abilities) and speech articulation (Arizona) tests. With a prevalence of 11%, SRST prediction of language outcomes resulted in the following indices: sensitivity, .62; specificity, .91; predictive validity, .44; overreferral, 8.4%; and underreferral, 4%. Prediction of the articulation outcome with a prevalence of 11% resulted in indices as follows: sensitivity, .57; specificity, .95; predictive validity, .75; overreferral, 3.7%; and underreferral, 8.3%. Teacher ratings and a parent questionnaire (Speech and Language Screening Questionnaire) approached the same rate of prediction of articulation but with a higher overreferral rate. The predictive indices from the SRST exceeded the comparison measures for language outcomes.

Articulation Disorders