6 resultados para Congenital Deafness


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The spectral content of the myoelectric signals from the muscles of the remnant forearms of three persons with congenital absences (CA) of their forearms was compared with signals from their intact contra-lateral limbs, similar muscles in three persons with acquired losses (AL) and seven persons without absences [no loss (NL)]. The observed bandwidth for the CA subjects was broader with peak energy between 200 and 300 Hz. While the signals from the contra-lateral limbs and the AL and NL subjects was in the 100-150 Hz range: The mean skew of the signals from the AL subjects was 46.3 +/- 6.7 and those with NL of 45.4 +/- 8.7, while the signals from those with CAs had a skew of 11.0 +/- 11. The structure of the muscles of one CA subject was observed ultrasonically. The muscle showed greater disruption than normally developed muscles. It is speculated that the myographic signal reflects the structure of the muscle. which has developed in a more disorganized manner as a result of the muscle not being stretched by other muscles across the missing distal joint, even in the muscles that are used regularly to control arm prostheses.

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Our research investigates the impact that hearing has on the perception of digital video clips, with and without captions, by discussing how hearing loss, captions and deafness type affects user QoP (Quality of Perception). QoP encompasses not only a user's satisfaction with the quality of a multimedia presentation, but also their ability to analyse, synthesise and assimilate informational content of multimedia . Results show that hearing has a significant effect on participants’ ability to assimilate information, independent of video type and use of captions. It is shown that captions do not necessarily provide deaf users with a ‘greater level of information’ from video, but cause a change in user QoP, depending on deafness type, which provides a ‘greater level of context of the video’. It is also shown that post-lingual mild and moderately deaf participants predict less accurately their level of information assimilation than post-lingual profoundly deaf participants, despite residual hearing. A positive correlation was identified between level of enjoyment (LOE) and self-predicted level of information assimilation (PIA), independent of hearing level or hearing type. When this is considered in a QoP quality framework, it puts into question how the user perceives certain factors, such as ‘informative’ and ‘quality’.

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RATIONALE: Children with congenital heart disease are at risk of gut barrier dysfunction and translocation of gut bacterial antigens into the bloodstream. This may contribute to inflammatory activation and organ dysfunction postoperatively. OBJECTIVES: To investigate the role of intestinal injury and endotoxemia in the pathogenesis of organ dysfunction after surgery for congenital heart disease. METHODS: We analyzed blood levels of intestinal fatty acid binding protein and endotoxin (endotoxin activity assay) alongside global transcriptomic profiling and assays of monocyte endotoxin receptor expression in children undergoing surgery for congenital heart disease. MEASUREMENTS AND MAIN RESULTS: Levels of intestinal fatty acid binding protein and endotoxin were greater in children with duct-dependent cardiac lesions. Endotoxemia was associated with severity of vital organ dysfunction and intensive care stay. We identified activation of pathogen-sensing, antigen-processing, and immune-suppressing pathways at the genomic level postoperatively and down-regulation of pathogen-sensing receptors on circulating immune cells. CONCLUSIONS: Children undergoing surgery for congenital heart disease are at increased risk of intestinal mucosal injury and endotoxemia. Endotoxin activity correlates with a number of outcome variables in this population, and may be used to guide the use of gut-protective strategies.

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Background: Word deafness is a rare condition where pathologically degraded speech perception results in impaired repetition and comprehension but otherwise intact linguistic skills. Although impaired linguistic systems in aphasias resulting from damage to the neural language system (here termed central impairments), have been consistently shown to be amenable to external influences such as linguistic or contextual information (e.g. cueing effects in naming), it is not known whether similar influences can be shown for aphasia arising from damage to a perceptual system (here termed peripheral impairments). Aims: This study aimed to investigate the extent to which pathologically degraded speech perception could be facilitated or disrupted by providing visual as well as auditory information. Methods and Procedures: In three word repetition tasks, the participant with word deafness (AB) repeated words under different conditions: words were repeated in the context of a pictorial or written target, a distractor (semantic, unrelated, rhyme or phonological neighbour) or a blank page (nothing). Accuracy and error types were analysed. Results: AB was impaired at repetition in the blank condition, confirming her degraded speech perception. Repetition was significantly facilitated when accompanied by a picture or written example of the word and significantly impaired by the presence of a written rhyme. Errors in the blank condition were primarily formal whereas errors in the rhyme condition were primarily miscues (saying the distractor word rather than the target). Conclusions: Cross-modal input can both facilitate and further disrupt repetition in word deafness. The cognitive mechanisms behind these findings are discussed. Both top-down influence from the lexical layer on perceptual processes as well as intra-lexical competition within the lexical layer may play a role.