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Neonatal hearing screening.

Neonatal hearing screening can be performed using reliable and reproducible methods. Intervention before the age of 6 months with hearing aids and appropriate educational support services will give the infant the best possible opportunity to develop language. Potential barriers to efficient implementation of a neonatal hearing screening program include access to appropriate and timely diagnostic and support services and insurance to cover the services. Without universal neonatal hearing screening, many children with hearing loss will be missed, which will have a direct negative impact on their speech, language, educational, and social development.

Hearing Disorders↗

Evaluating the discriminant accuracy of a grammatical measure with Spanish-speaking children.

PURPOSE: The purpose of this study was to evaluate the discriminant accuracy of a grammatical measure for the identification of language impairment in Latino Spanish-speaking children. The authors hypothesized that if exposure to and use of English as a second language have an effect on the first language, bilingual children might exhibit lower rates of grammatical accuracy than their peers and be more likely to be misclassified. METHOD: Eighty children with typical language development and 80 with language impairment were sampled from 4 different geographical regions and compared using linear discriminant function analysis. RESULTS: Results indicated fair-to-good sensitivity from 4;0 to 5;1 years, good sensitivity from 5;2 to 5;11 years, and poor sensitivity above age 6 years. The discriminant functions derived from the exploratory studies were able to predict group membership in confirmatory analyses with fair-to-excellent sensitivity up to age 6 years. Children who were bilingual did not show lower scores and were not more likely to be misclassified compared with their Spanish-only peers. CONCLUSIONS: The measure seems to be appropriate for identifying language impairment in either Spanish-dominant or Spanish-only speakers between 4 and 6 years of age. However, for older children, supplemental testing is necessary.

Child Language↗

Primary degeneration of the granular layer of the cerebellum. A study of 14 patients and review of the literature.

Primary degeneration of the granular layer of the cerebellum is an autosomal recessive disorder exhibiting characteristic clinical features: hypotonia, strabismus, delayed motor development, nonprogressive ataxia, delayed language development with dysarthria and mental retardation. We studied fourteen children, seven of each gender. Neuroimaging tests including pneumoencephalography, computed tomography (CT) and magnetic resonance imaging (MRI) showed severe cerebellar atrophy in all. MRI best demonstrated the cerebellar lesion, revealing great uniformity amongst the cases. Vertebrobasilar angiography was performed in two cases and showed marked hypoplasia of the cerebellar arteries, predominantly the posterior inferior cerebellar artery (PICA) and its branches. Necropsy was performed in three cases; cerebellar atrophy with loss of granular cells and diverse abnormalities of the Purkinje cells was found in two. The third, the sister of one of the other two cases, had a similar but shorter clinical course and died at three months of age. Her sister, who died at 5 years of age, presented a severe cerebellar atrophy with typical changes in the granular cell layer and Purkinje cells. In the third patient, who lived three months, only focal cerebellar folial atrophy with no microscopic changes in the granular cell layers was present. Though this case cannot objectively be included in the cerebellar atrophy syndrome with granular cell loss, her family history and clinical picture suggest the same disease. The findings observed in our series and the study of cases described in the literature, suggest that there are several forms of this disease which differ mainly in severity and neurological evolution. The cerebellar lesion seems to be a progressive atrophic process with the most severe changes during the early years of life.

Adolescent↗

The Robin anomalad (Pierre Robin syndrome)--a follow up study.

During a 10-year period 55 patients with the Robin anomalad were admitted to the Liverpool Regional Cleft Palate Units. Fourteen (25%) children died. All deaths were within 3 months of birth. Congenital abnormalities other than mandibular retrognathia and cleft palate were present in 14 (26%) children. Peripheral limb defects were particularly common. Thirty children were recalled and reviewed to assess speech, hearing, growth, and educational achievement. There was a clear association between severe nasal escape of air in speech and atypical articulatory patterns. Almost half the children tested had abnormal articulation. Only 4 (13%) of 30 children showed delayed language development. Half the children tested audiometrically showed a binaural handicap but in only one patient was this sufficiently severe to warrant amplification. There was no trend towards abnormalities of growth and only 2 children could be firmly classified as educationally subnormal.

Child Development↗

Idiopathic external hydrocephalus: natural history and relationship to benign familial macrocephaly.

External hydrocephalus was identified in 63 infants. The 36 infants in whom external hydrocephalus was idiopathic constitute the study population. The group was homogeneous. Although not all were macrocephalic at birth, the head circumference exceeded the 95th percentile in all cases by 1 year of age, and subsequent head growth was parallel to the 95th percentile. The head computed tomographic scan showed a characteristic picture of a prominent interhemispheric fissure, a collection of subarachnoid fluid over the frontal convexities, and prominent basal cisternae. Mild ventriculomegaly was present in ten cases. These abnormalities resolved after 18 to 24 months of age. No infant required therapy for the condition. Development was normal in 32 infants, suspect in three, and clearly abnormal in only one of the 36 infants. However, of the 32 infants with normal development, 14 were found to be delayed in gross motor development and five in language development at a prior time. There was a family history of macrocephaly in 88% of cases. Several families had a mixture of members with external hydrocephalus and benign familial macrocephaly throughout several generations. These findings suggest that idiopathic external hydrocephalus is a relatively benign, self-limited condition that resolves without treatment and is closely related to benign familial macrocephaly.

Black People↗

Controlled evaluation of rural regional perinatal care: developmental and neurologic outcomes at 1 year.

The effectiveness of a rural regionalized perinatal care (RPC) program was evaluated by a controlled, population-based design. The RPC program, begun in a carefully selected study region in July 1975, evolved into a system of care which included the following major components: identification of high-risk pregnancies and high-risk newborn infants; obstetric and newborn consultation and referral services between Level I, II, and III centers; professional education for physicians, nurses, and other health professionals; and nutrition and social work consultation. Substantial resources were made available from 1975 to 1980 to implement this "total package" of RPC. A matched, control region was identified which, except for the RPC program, was as comparable as possible to the study region. It was hypothesized that the RPC intervention would have the following effects when the study region was compared with the control region: reduction in fetal and neonatal mortality, no increase in postneonatal mortality, and reduction in obstetric and newborn morbidity. These findings were reported previously. This paper presents results of hypothesized reductions in adverse developmental, neurologic, maternal-infant attachment, and selected physical health outcomes. A sample of 447 infants was assessed by an extensive battery of instruments at 1 year, adjusted for gestational age. The most notable findings were mothers' reports of receptive language development and observations of maternal attachment behaviors that significantly favored the study region. No significant differences between study and control regions were observed for Bayley Mental and Motor Scores, abnormal neurologic signs, and the physical health measures.(ABSTRACT TRUNCATED AT 250 WORDS)

Child Behavior↗