Hearing impairment: a population study of age at diagnosis, severity, and language outcomes at 7-8 years.
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BACKGROUND: Better language outcomes are reported for preschool children with hearing impairment (HI) diagnosed very early, irrespective of severity. However, population studies of older children are required to substantiate longer term benefits of early detection. AIMS: To study impact of age of diagnosis and severity of HI in a population cohort of 7-8 year old children. METHODS: Eighty eight 7-8 year old children born in Victoria, who were (a) fitted with hearing aids for congenital HI by 4.5 years and (b) did not have intellectual or major physical disability were studied. Main outcome measures were Clinical Evaluation of Language Fundamentals (CELF) and Peabody Picture Vocabulary Test (PPVT). Predictors were pure tone average (0.5, 1, 2 kHz) in better ear at diagnosis and age at diagnosis. Marginal (adjusted) means were estimated with general linear models. RESULTS: Response rate was 67% (n = 89; 53 boys). Mean age at diagnosis was 21.6 months (SD 14.4); 21% had mild, 34% moderate, 21% severe, and 24% profound HI; mean non-verbal IQ was 104.6 (SD 16.7). Mean total CELF score was 76.7 (SD 21.4) and mean PPVT score 78.1 (SD 18.1). Age of diagnosis, adjusted for severity and IQ, did not contribute to language scores. In contrast, adjusted mean CELF and PPVT language scores fell sequentially with increasing severity of HI. CONCLUSIONS: More severe HI, but not later diagnosis, was strongly related to poorer language outcomes at 7-8 years. Further systematic study is needed to understand why children with hearing impairment have good or poor outcomes. (+info)
Referral patterns for syndromes associated with frontotemporal lobar degeneration.
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We compared demographics of subjects diagnosed with frontotemporal degeneration (FTD) at a group of 5 clinics specializing in this non-Alzheimer dementia against those subjects diagnosed at standard Alzheimer disease centers, to determine any differences in referral patterns between such clinics. Of the two major phenotypes of FTD, behavior and language, the latter more frequently presented to the specialty clinics (46% of FTD diagnoses versus 19%, P < 0.001). Mean age at onset for the behavioral presentation phenotype was one year younger at the specialty clinics (P < 0.01). Mean age at onset for the language phenotype was 3 years older (P < 0.001) than for the behavioral phenotype at standard centers but did not differ between the two evaluating groups. Cases with FTD referred to all of the dementia evaluation sites in this study did not differ significantly from those previously reported in the literature. Clinics specializing in FTD recruit more language presentation cases. There were statistical but not clinically significant differences in ages at onset. (+info)
Persistent neurocognitive impairments associated with severe falciparum malaria in Kenyan children.
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OBJECTIVES: There is little information on the characteristics of persisting impairments associated with severe forms of falciparum malaria. Previous work has suggested the existence of a group of children with particularly poor performance on neurocognitive assessments in the context of average group performance. The aim of this study was to provide a detailed characterisation of impairments in this subgroup. METHODS: Three groups of children were recruited: children admitted up to nine years earlier with cerebral malaria (CM) (n = 152), malaria and complicated seizures (M/S) (n = 156), or those unexposed to either condition (n = 179). Each child underwent a series of developmental assessments. Standard definitions were used to classify impairment. RESULTS: Twenty-four percent of the CM and M/S groups had at least one impairment in the major domains assessed in the study, compared with 10% of the unexposed group. CM was associated with a higher proportion of multiple impairments and an increased risk of mortality in the first year after recovery in those identified with impairments on discharge. CONCLUSIONS: After severe malaria, some children have neurocognitive impairments that are evident as long as nine years later. Impairments may become more evident as children progress and face more complex cognitive and linguistic demands, socially and educationally. The child's neurological status at discharge was not a good predictor of later neurocognitive impairment. This highlights the importance of follow up for children with severe malaria and the involvement of therapists and educators in the provision of services for this population. (+info)
Language cannot be reduced to biology: perspectives from neuro-developmental disorders affecting language learning.
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The study of language knowledge guided by a purely biological perspective prioritizes the study of syntax. The essential process of syntax is recursion--the ability to generate an infinite array of expressions from a limited set of elements. Researchers working within the biological perspective argue that this ability is possible only because of an innately specified genetic makeup that is specific to human beings. Such a view of language knowledge may be fully justified in discussions on biolinguistics, and in evolutionary biology. However, it is grossly inadequate in understanding language-learning problems, particularly those experienced by children with neurodevelopmental disorders such as developmental dyslexia, Williams syndrome, specific language impairment and autism spectrum disorders. Specifically, syntax-centered definitions of language knowledge completely ignore certain crucial aspects of language learning and use, namely, that language is embedded in a social context; that the role of envrironmental triggering as a learning mechanism is grossly underestimated; that a considerable extent of visuo-spatial information accompanies speech in day-to-day communication; that the developmental process itself lies at the heart of knowledge acquisition; and that there is a tremendous variation in the orthographic systems associated with different languages. All these (socio-cultural) factors can influence the rate and quality of spoken and written language acquisition resulting in much variation in phenotypes associated with disorders known to have a genetic component. Delineation of such phenotypic variability requires inputs from varied disciplines such as neurobiology, neuropsychology, linguistics and communication disorders. In this paper, I discuss published research that questions cognitive modularity and emphasises the role of the environment for understanding linguistic capabilities of children with neuro-developmental disorders. The discussion pertains to two specific disorders, developmental dyslexia and Williams syndrome. (+info)
Executive dysfunction and its relation to language ability in verbal school-age children with autism.
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This study examined executive dysfunction and its relation to language ability in verbal school-age children with autism. Participants were 37 children with autism and 31 nonautistic comparison participants who were matched on age and on verbal and nonverbal IQ but not on language ability, which was lower in the autism group. Children with autism exhibited deficits compared to the comparison group across all 3 domains of executive function that were assessed including working memory (Block Span Backward; Isaacs & Vargha-Khadem, 1989), working memory and inhibitory control (NEPSY Knock-Tap; Korkman, Kirk, & Kemp, 1998), and planning (NEPSY Tower; Korkman et al., 1998). Children with autism were less developed than the comparison group in their language skills, but correlational analyses revealed no specific association between language ability and executive performance in the autism group. In contrast, executive performance was positively correlated with language ability in the comparison group. This pattern of findings suggest that executive dysfunction in autism is not directly related to language impairment per se but rather involves an executive failure to use of language for self-regulation. (+info)
The natural history of temporal variant frontotemporal dementia.
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BACKGROUND: The temporal variant of frontotemporal dementia (tvFTD) features asymmetric anterior temporal/amygdala degeneration as well as ventromedial frontal, insular, and inferoposterior temporal involvement. Left temporal atrophy has been linked to loss of semantic knowledge, whereas behavioral symptoms dominate the right temporal variant. OBJECTIVE: To investigate the first symptoms and the timing of subsequent symptoms in patients with left versus right tvFTD. METHODS: Twenty-six patients with tvFTD were identified. Six had right > left temporal atrophy (right temporal lobe variant [RTLV]) and were matched with six having comparable left > right temporal atrophy (left temporal lobe variant [LTLV]). Clinical records were reviewed to generate individualized symptom chronologies. RESULTS: In all patients, first symptoms involved semantics (4/6 LTLV, 1/6 RTLV), behavior (4/6 RTLV, 1/6 LTLV), or both (1 LTLV, 1 RTLV). Semantic loss began with anomia, word-finding difficulties, and repetitive speech, whereas the early behavioral syndrome was characterized by emotional distance, irritability, and disruption of physiologic drives (sleep, appetite, libido). After an average of 3 years, patients developed whichever of the two initial syndromes--semantic or behavioral--that they lacked at onset. A third stage, 5 to 7 years from onset, saw the emergence of disinhibition, compulsions, impaired face recognition, altered food preference, and weight gain. Compulsions in LTLV were directed toward visual, nonverbal stimuli, whereas patients with RTLV were drawn to games with words and symbols. CONCLUSIONS: The temporal variant of frontotemporal dementia follows a characteristic cognitive and behavioral progression that suggests early spread from one anterior temporal lobe to the other. Later symptoms implicate ventromedial frontal, insular, and inferoposterior temporal regions, but their precise anatomic correlates await confirmation. (+info)
Individual differences in auditory processing in specific language impairment: a follow-up study using event-related potentials and behavioural thresholds.
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It has frequently been claimed that children with specific language impairment (SLI) have impaired auditory perception, but there is much controversy about the role of such deficits in causing their language problems, and it has been difficult to establish solid, replicable findings in this area. Discrepancies in this field may arise because (a) a focus on mean results obscures the heterogeneity in the population and (b) insufficient attention has been paid to maturational aspects of auditory processing. We conducted a study of 16 young people with specific language impairment (SLI) and 16 control participants, 24 of whom had had auditory event-related potentials (ERPs) and frequency discrimination thresholds assessed 18 months previously. When originally assessed, around one third of the listeners with SLI had poor behavioural frequency discrimination thresholds, and these tended to be the younger participants. However, most of the SLI group had age-inappropriate late components of the auditory ERP, regardless of their frequency discrimination. At follow-up, the behavioural thresholds of those with poor frequency discrimination improved, though some remained outside the control range. At follow-up, ERPs for many of the individuals in the SLI group were still not age-appropriate. In several cases, waveforms of individuals in the SLI group resembled those of younger typically-developing children, though in other cases the waveform was unlike that of control cases at any age. Electrophysiological methods may reveal underlying immaturity or other abnormality of auditory processing even when behavioural thresholds look normal. This study emphasises the variability seen in SLI, and the importance of studying individual cases rather than focusing on group means. (+info)
Identification of FOXP2 truncation as a novel cause of developmental speech and language deficits.
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FOXP2, the first gene to have been implicated in a developmental communication disorder, offers a unique entry point into neuromolecular mechanisms influencing human speech and language acquisition. In multiple members of the well-studied KE family, a heterozygous missense mutation in FOXP2 causes problems in sequencing muscle movements required for articulating speech (developmental verbal dyspraxia), accompanied by wider deficits in linguistic and grammatical processing. Chromosomal rearrangements involving this locus have also been identified. Analyses of FOXP2 coding sequence in typical forms of specific language impairment (SLI), autism, and dyslexia have not uncovered any etiological variants. However, no previous study has performed mutation screening of children with a primary diagnosis of verbal dyspraxia, the most overt feature of the disorder in affected members of the KE family. Here, we report investigations of the entire coding region of FOXP2, including alternatively spliced exons, in 49 probands affected with verbal dyspraxia. We detected variants that alter FOXP2 protein sequence in three probands. One such variant is a heterozygous nonsense mutation that yields a dramatically truncated protein product and cosegregates with speech and language difficulties in the proband, his affected sibling, and their mother. Our discovery of the first nonsense mutation in FOXP2 now opens the door for detailed investigations of neurodevelopment in people carrying different etiological variants of the gene. This endeavor will be crucial for gaining insight into the role of FOXP2 in human cognition. (+info)