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[Hypoacusis after the neonatal period. Situations requiring hearing assessment].

INTRODUCTION: Because hearing plays a major role in language development, pediatric hypoacusis is especially damaging. The high frequency of hearing impairment in newborns and the need for an early diagnosis have led to the establishment of neonatal screening. Nevertheless, there are other situations which may compromise hearing quality in later stages and it is essential to identify them in order to be able to provide early and effective treatment. OBJECTIVES: To describe the most frequent reasons for referring patients for hearing evaluation to a third level hospital and to identify common situations that require hearing assessment among the pediatric population. PATIENTS AND METHODS: The clinical histories of 197 non-neonates evaluated for hypoacusis were reviewed. Clinical parameters and diagnosis were compared in patients with impaired and normal hearing. RESULTS: One hundred sixty-one patients had no previous known hypoacusis. The main reason for evaluation was suspicion by the family or child minder (53.4 %), followed by language underdevelopment. In the first examinations 78 children had hypoacusis (48.4 %), which was more frequently bilateral than unilateral. In 29.5 % of hypoacusic patients, the disease was related to recurrent otitis or adenoiditis and in 25.6 % it was genetic. The most frequent antecedent was deaf relatives in hypoacusic patients and abnormal phenotypes in children with normal hearing. Three patients with previous bacterial meningitis were studied and two of these had hypoacusis. CONCLUSIONS: Hypoacusic evaluation outside the context of newborn screening is mainly motivated by clinical suspicion of hypoacusis or language underdevelopment. Other situations such as recurrent otitis with effusion, syndromic phenotypic characteristics or bacterial meningitis are related to hearing problems and therefore require detailed evaluation.

Child↗

[Clinical analysis of 91 cases with articulation error].

OBJECTIVE: To study classification of the articulation errors, find the related factors which affect the therapy effect, and compare the therapy effect in the different patterns of the phonemic errors and build up a clinical model of the speech therapy on the basis of more than 4 years speech therapy in the clinic. METHOD: After excluding the organic articulation disorders, 91 cases of consonant phonemic errors were classified into different patterns according to the phonemic placement errors. The 91 cases were divided into two groups as effective one and ineffective one depending on whether the symptoms disappeared or not after the speech therapy. Ten factors including sex, age, oral-motor function problem, developmental delay, history of language development delay, history of middle ear affection, lingua frenata, mental retardation, the therapy frequency and times, were analyzed. The statistics software SPSS (SPSS Inc, 1997) was applied to show the factors related to the therapy effect by logistic multiple stepwise regression analysis. The therapy effect was compared between the single pattern (phonemic error < 2 groups) and multiple pattern (phonemic errors > or = 2 groups). RESULTS: The 91 cases with consonant phonemic errors were classified into four groups. The multiple pattern of the articulation errors was the most common (37 cases, 41%) among the 4 groups, followed by velar errors (33 cases, 36%). The third one was the apico-dental errors (12 cases, 13%) and the last one was the linguo-alveolar errors (8 cases, 9%). Only one case had labiodental error (/f/). The logistic multiple stepwise regression analysis showed that the history of the positive developmental delay, language delay and the frequency of the speech therapy were related to the therapy effect. The effective rate of the speech therapy in the single pattern of the phonemic error was as high as 87% while the one in the multiple pattern group was only 2.7%. The difference was significant (P < 0.000 1) when the therapy effect in these two groups was compared. The clinical model of the speech therapy included speech assessment, designing of the goal, choice of the target sound, sound production and oral-motor functional training. CONCLUSION: The consonant phonemic errors in the clinic represent four groups. The developmental delay, the language delay and frequency of the speech therapy influence the therapy effect. The frequency of the speech therapy should be increased every week, for example not less two times a week. The therapy effect of the single pattern of the phonemic errors is much better than that of the multiple pattern. The primary model of the speech therapy has been built in the clinics.

Articulation Disorders↗

Learning a morphological system without a default: the Polish genitive.

The acquisition of the English past tense inflection is the paradigm example of rule learning in the child language literature and has become something of a test case for theories of language development. This is unfortunate, as the idiosyncratic properties of the English system of marking tense make it a rather unrepresentative example of morphological development. In this paper, I contrast this familiar inflection with a much more complex morphological subsystem, the Polish genitive. The genitive case has three different markers, each restricted to a different subset of nouns, in both the singular and the plural. Analysis of the spontaneous speech of three children between the ages of 1;4 and 4;11 showed that they generalized, and overgeneralized, all three singular endings. However, error rates were extremely low and there is no evidence that they treated any one ending as the 'default'. The genitive plural, on the other hand, showed a strikingly different pattern of acquisition, similar to that seen in English-speaking children learning the past tense. It is argued that in the latter two cases, the default-like character of one of the affixes is attributable to the properties of the relevant inflectional subsystems, not to the predispositions that children bring to the language-learning task.

Child Language↗

Theory of mind development in deaf children: a nonverbal test of false-belief understanding.

Our aim in this study was to investigate whether previous findings pointing to a delay in deaf children's theory of mind development are replicated when linguistic demands placed on the deaf child are minimized in a nonverbal version of standard false-belief tasks. Twenty-four prelingually deaf, orally trained children born of hearing parents were tested with both a verbal and a nonverbal version of a false-belief task. Neither the younger (range: 4 years 7 months-6 years 5 months) nor the older (range: 6 years 9 months-11 years 11 months) children of the final sample of 21 children performed above chance in the verbal task. The nonverbal task significantly facilitated performance in children of all ages. Despite this facilitation, we observed a developmental delay: only the older group performed significantly above chance in the nonverbal false-belief task, even though the younger children were at the average age when hearing children normally pass standard false-belief tests. We discuss these findings in light of the hypothesis that language development and conversational competence are crucial to the acquisition of a theory of mind.

Journal Article↗

Neurophysiological indexes of speech processing deficits in children with specific language impairment.

We used neurophysiological and behavioral measures to examine whether children with specific language impairment (SLI) have deficits in automatic processing of brief, phonetically similar vowels, and whether attention plays a role in such deficits. The neurophysiological measure mismatch negativity (MMN) was used as an index of discrimination in two tasks; one in which children ignored the auditory stimuli and watched a silent video and a second in which they attended to the auditory modality. Children with SLI showed good behavioral discrimination, but significantly poorer behavioral identification of the brief vowels than the children with typical language development (TLD). For the TLD children, two neurophysiological measures (MMN and a later negativity, LN) indexed discrimination of the vowels in both tasks. In contrast, only the LN was elicited in either task for the SLI group. We did not see a direct correspondence between the absence of MMN and poor behavioral performance in the children with SLI. This pattern of findings indicates that children with SLI have speech perception deficiencies, although the underlying cause may vary.

Acoustic Stimulation↗

Limitations in verbal fluency following heavy burdens of early childhood diarrhea in Brazilian shantytown children.

The effects of heavy burdens of diarrhea in the first 2 years of life on specific executive control function like verbal fluency are not well understood. In previous studies, we have shown associations of early childhood diarrhea (ECD) with nonverbal intelligence and school functioning. Therefore, we postulated that ECD might affect early neuropsychological development leading to long-term deficits in normal cognitive development. Based on our extensive 14-year prospective cohort studies of early childhood diarrheal illnesses in a Brazilian shantytown community, we examined ECD correlations between specific impairments of higher mental function and executive skills in shantytown children 5-10 years later (now at 6-12) years of age. Specifically we examined whether heavy diarrheal illnesses correlate with reduced performance on selected tests of executive function. Our study, for the first time, suggests a disproportional impairment in semantic but not phonetic fluency in a subset of children with heavy burdens of diarrhea in their first 2 years of life even when controlling for maternal education, breastfeeding, and child schooling. Similar semantic decrements have been associated with impaired recovery from brain injury. These exploratory studies suggest the importance of verbal fluency tests to assess executive functioning in children challenged by poor nutrition and diarrhea in early life. In addition, our unique findings show the potential influences of early childhood diarrhea on language development that is so critical to productive adulthood and potentially set a foundation for new neuropsychological approaches, which assess early burdens of enteric illnesses on childhood development.

Age Factors↗

Benign partial epilepsy of childhood: a longitudinal neuropsychological and EEG study of cognitive function.

The study combined prospective neuropsychological and EEG results of 22 children presenting with typical benign partial epilepsy with rolandic spikes (n=19) and occipital spikes (n=3). The aims were to assess the types of cognitive problems which may be encountered in this population, to evaluate the course of cognitive and learning capacities during the active phase of epilepsy, and to see if there was a correlation with paroxysmal activity on the EEG. Average age at entry in the study was 8.4 years and each child was seen two to four times over a period of 1 to 3 years. EEGs showed persistent spike foci in most cases that worsened in three cases, but there were no continuous spike-waves during sleep. No child had persistent stagnation, marked fluctuations, or a regression in cognitive abilities. Of 22 children, 21 had average IQ (>80). Eight children had school difficulties requiring special adjustment. No single cognitive profile was identified. Four children had delayed language development and eight children had transient weak scores in one isolated domain (verbal, visuospatial, memory) which improved or normalized during the course of the study with concomitant EEG improvement or normalization. In two of the three children with aggravation of the paroxysmal EEG activity, clinical changes were documented. A proportion of children with typical benign partial epilepsy with rolandic spikes showed mild, varied, and transient cognitive difficulties during the course of their epilepsy, and in most cases this probably had a direct relation with the paroxysmal EEG activity.

Child↗

Beyond newborn screening: early diagnosis and management of hearing loss in infants.

As a result of the 1993 National Institutes of Health Consensus Statement recommending hearing screening for all infants before discharge from the newborn nursery, there are an increasing number of newborns identified with hearing impairment requiring follow-up services. Research has shown that hearing-impaired infants who receive intervention by the time they are 6 months adjusted gestational age are likely to develop language that is equal to their normal-hearing peers. Newborn hearing screening will not attain its goal of early intervention for hearing loss unless a comprehensive follow-up program is in place. The neonatal nurse plays an important role in the follow-up program by providing information to parents and stressing the importance of follow-up. Follow-up for infants identified through the screening process includes rescreening within 4 to 6 weeks of discharge and a full diagnostic evaluation for infants who fail the rescreening. The diagnostic evaluation includes Auditory Brainstem Response (ABR), Otoacoustic Emissions (OAE), and middle ear assessment. The course of treatment or therapy and the type of amplification chosen for hearing-impaired infants depends on the type and degree of hearing loss as well as the communication mode chosen by the family. The ultimate goal of early audiological diagnosis of hearing loss is to begin treatment as soon as possible to minimize delays in speech/language and academic development. This article focuses on what needs to happen after newborn hearing screening to ensure early and optimal intervention for hearing-impaired infants.

Hearing Loss↗

Sequencing instructional tasks. A comparison of contingent and noncontingent interspersal of preferred academic tasks.

This study compared two strategies for increasing accurate responding on a low-preference academic task by interspersing presentations of a preferred academic task. Five children attending a preschool program for children with delayed language development participated in this study. Preferred and nonpreferred tasks were identified through a multiple-stimulus, free-operant preference assessment. Contingent access to a preferred academic task was associated with improved response accuracy when compared to noncontingent access to that activity for 3 students. For 1 student, noncontingent access to the preferred activity led to improved response accuracy, and 1 student's analysis suggested the importance of procedural variety. The implications of these findings for use of preference assessments to devise instructional sequences that improve student responding are discussed.

Child, Preschool↗

Pneumographic findings in the infantile autism syndrome. A correlation with temporal lobe disease.

Pneumoencephalographic findings are described in a group of 18 children who presented to us with a history of retarded language development and autistic behaviour disturbances. None had specific diagnosable neurological diseases nor gross motor disorders. PEG findings included, most prominently, pathological enlargement of the left temporal horn in 15 cases; some cases showed enlargement of both temporal horns or mild variable enlargement of the lateral ventricles, especially the left. Comparisons between infantile autism and recognized patterns of temporal lobe disease (especially Korsakoff syndrome and Kluver-Bucy syndrome) are drawn. We have suggested that medial temporal lobe dysfunction may be a major factor in the pathogenesis of the syndrome of infantile autism.

Autistic Disorder↗

[An evaluation and comparison of the methodology of 2 otophoniatric screenings performed in the 1989-1990 biennium on a sample of 4-year-old children attending nursery schools].

The Pediatric Otophoniatric Department of U.S.L.75II, the Multicenter of Preventive Medicine in Via Cherasco, 5, Milan, carried out two otophoniatric screenings on about 70 four-year-old nursery school children. While this screening was considered experimental in four schools, the ultimate goal is to extend it to all the nursery schools in the same area. The primary aim of the screening was to individualize several pathologies of the upper respiratory tract in this age range. The second aim was to identify language pathologies in children under the age of six in order to treat them as soon as possible with adequate and preventive therapy and appropriate language re-education before their entrance into primary school. The materials and methods employed were as follows: O.R.L. examination: otoscopy, rhinoscopy, oropharyngoscopy, indirect laryngoscopy (after individualization of dysphonia); Audiometric test: peep show in free field with a 250 Hz-4000 Hz frequency range at 30 dB; Language evaluation through examination and interpretation of questionnaires formulated in order to evaluate language development filled in by the children's teachers. Two modifications in the method used in the 1989 study were made in the 1990 screening: The preventive use of an auditory duct cleaner and the distribution of the above-mentioned questionnaires at the very beginning of the study. These two changes improved screening planning as well as development. The results of the two screenings were compared and an increase in the percentage of children examined and subjected to the audiometric test was noted. In fact, in the 1989 study, only 77% of the children underwent the audiometric test (23% had ear wax), while in the 1990 study 100% underwent the test (ear cleaner had been used). With regard to the questionnaires, the data were gathered four months after screening in the 1989 study, whereas in the 1990 study this phase was carried out on the same day of the screening. The new methodology saved time in the screening.

Audiometry↗

Experience of transferring an integrated hospital-administration system from a CODASYL data-base to a standard MUMPS file structure.

University College Hospital, a teaching hospital situated in Central London, has been using computers since 1964. Development of an integrated on-line hospital administration computer system was started in 1974. By mid 1977, the components of the system which had been implemented included a patient Master Index, Registration of new patients, Waiting Lists, Bed State, Out-patient Appointments, X-ray, Microbiology and Disease Index. The system was implemented using the Rank Xerox Data Systems (CODASYL based data-base management software, with the majority of applications running on a terminal network supported by transaction processing programs. The experience gained during three years of using such a system is reviewed, with particular emphasis on the ways in which the system matched up in practice to the expected benefits of data-base management facilities. In early 1977, it was decided to replace the Rank Xerox mainframe with a minicomputer which was considered to be a more cost-effective solution to the hospital's computing requirements. The machine selected a PDP 11-70, is running the Digital Equipment Corporation's implementation of Standard MUMPS, an interpretive language developed in a hospital environment, with its own file management software. The decisions which were made in redesigning the existing computer systems for the new machine are discussed. The progress made to date in the transfer of applications from the mainframe to the mini is reviewed, and some of the features of the software available on each machine and its suitability for the implementation of on-line data management and retrieval systems are compared.

Computers↗

Communicative interaction between teachers and children with severe handicaps.

The content and structure of teacher input language to children with severe handicaps during early stages of language development were examined. Teacher-child dyads (N = 21) were videotaped during interactions in a semi-structured play setting. Each child was ranked as to level of communicative abilities on a scale of early communicative behaviors. Results showed that teachers adjusted the complexity of their linguistic requests based on the child's level of communication and modulated their requests based on children's responses from one moment to the next. The structure of the teacher's utterances was not as closely attuned to the communicative levels of the children. Issues and problems relating to adults' linguistic interaction with children with handicaps were discussed.

Child↗

Absence of growth hormone effects on cognitive function in girls with Turner syndrome.

Turner syndrome (TS) is a genetic disorder characterized by short stature, gonadal dysgenesis, and a particular neurocognitive profile of normally developed language abilities (particularly verbal IQ) and impaired visual-spatial and/or visual-perceptual abilities. We have followed a large sample of girls with Turner syndrome who were enrolled in a long-term, double-blind, placebo-controlled trial of the effects of growth hormone (GH) treatment on final adult height. This study provides a unique opportunity to prospectively evaluate the effects of GH treatment on neurocognitive function in this population of girls with Turner syndrome. The GH- and placebo-treated Turner syndrome subjects were well matched for age, treatment duration, race, karyotype, and socioeconomic status. Treatment (GH or placebo) durations ranged from 1-7 yr. Whether GH deficiency and/or treatment in childhood and adolescence influences cognitive outcome in short children or GH-children is controversial. The major result of this study was the absence of GH treatment effects on cognitive function in girls with Turner syndrome. Our findings are in agreement with most of the previous studies that found no apparent growth hormone treatment effects on cognitive function in growth-hormone deficient children. We conclude that this study does not support a role for growth hormone in influencing childhood brain development in girls with Turner syndrome. Their characteristic nonverbal neurocognitive deficits were not altered with GH treatment into early adolescence.

Child↗

Attenuated auditory event-related potential (mismatch negativity) in children with developmental dysphasia.

An attention-independent negative wave-form termed 'mismatch negativity' (MMN) of the auditory event-related potentials (ERPs) was studied in ten children (3-6 years) with developmental dysphasia and in fourteen control children (3-7 years) with normal speech and language development. The MMNs were elicited with pure sine tone stimuli using the oddball paradigm. The peak latency and peak amplitude of MMN response to frequency (500/553 H2) difference was measured. The grand average amplitude of frequency MMN was significantly attenuated in dysphasic children as compared to controls, but no significant difference was observed in the latency of peak frequency MMN. The results indicate that dysphasic children have a defect in automatic auditory processing of frequency differences. Because the MMN response reflects central auditory processing and is modal specific for auditory stimuli we argue that the MMN method can serve as an objective tool to assess central auditory deficits in children.

Analysis of Variance↗

Standard EEG and dyslexia in children--new evidence for specific correlates?

Contrary to the current assumption that there are no specific correlates of dyslexia in the standard clinical EEG, we have often noted a spontaneous "intermittent left parietal alpha desynchronization" (ILPAD) when visually evaluating the standard EEGs of children with delayed speech and language development and/or dyslexia. Visual evaluations of EEGs, as well as computer-assisted frequency analysis of three pairs of matched groups (healthy and hyperkinetic children with vs. without relatively "low levels" of verbal performance, as well as children with other child psychiatric disorders with vs. without dyslexia), revealed that healthy children did not display the ILPAD phenomenon. It was seen neither in healthy children with a homogeneous performance profile ("high level" of verbal performance) nor in those with a relatively "low level" of verbal performance. However, the ILPAD phenomenon was evident mainly in children with psychiatric disorders who suffered from dyslexia. Its intermittent occurrence prevented its detection by means of the usual computer-supported analyses of EEG power spectra. Nevertheless, auditory cognitive loading was accompanied by a decrease in alpha power in both healthy and hyperkinetic children with "high" or "low" levels of verbal performance. In a further group of 8-year-old children with dyslexia, but otherwise healthy, the ILPAD phenomenon was also observed if their CNS maturation as reflected in the EEG was normal for their age. These results seemed to indicate at least a deficit-specificity of the ILPAD phenomenon, which is interpreted as an electrophysiological correlate of a "brain-electrical developmental deviation" with regard to the "functions of communication": speech, language, reading and spelling.

Alpha Rhythm↗

Mutation detection of GJB2 using IsoCode and real-time quantitative polymerase chain reaction with SYBR green I dye for newborn hearing screening.

OBJECTIVES/HYPOTHESIS: Recent developments in molecular genetics have opened a new era in genetic analysis accompanied by new concepts concerning genetic disorders. Although 30 genes responsible for nonsyndromic deafness have been discovered as of March 27, 2003, the connexin 26 gene (GJB2) is commonly found in cases of deafness of unknown origins. The GJB2 contains a predicted open reading frame of 785 base pairs, which makes it relatively easy to detect mutations. Accordingly, mutation analysis of GJB2 should be suitable for the screening of congenital deafness. STUDY DESIGN: Prospective study. METHODS: IsoCode Stix is a useful device to isolate DNA from small samples of blood, which can be delivered from remote areas. To apply the detection of common mutations of GJB2 to hearing screening, DNA was extracted from several droplets of blood applied to the IsoCode device, and an allele-specific amplification method with real-time quantitative polymerase chain reaction was performed using GeneAmp 7700 with SYBR Green I dye. RESULTS: DNA extracted from IsoCode was purified within 45 minutes, which was sufficient to detect the full sequence of GJB2. Four types of common GJB2 mutations were reliably detected within 2.5 hours. CONCLUSION: IsoCode and real-time quantitative polymerase chain reaction will be promising tools for newborn screening of deafness genes in the future in DNA-based deafness screening, allowing early diagnosis of deafness and prompt training for language development.

Benzothiazoles↗