Attentional differences in audiovisual face perception between full- and preterm very low birthweight toddlers.

AIM: To investigate whether rightward attention to the mouth during audiovisual speech perception may be a behavioural marker for early brain development, we studied very preterm and low birthweight (VLBW) and typically developing (TD) toddlers. METHODS: We tested the distribution of gaze points in Japanese-learning TD and VLBW toddlers when exposed to talking, silent and mouth moving faces at 12, 18 and 24 months (corrected age). Each participant was categorised based upon the area they gazed at most (Eye-Right, Eye-Left, Mouth-Right, Mouth-Left) per stimulus per age. A log-linear model was applied to three-dimensional contingency tables (region, side and group). RESULTS: VLBW toddlers showed fewer gaze points than TD toddlers. At 12 months, more VLBW toddlers than TD toddlers showed left attentional bias toward any one face; however, this difference in attention asymmetry receded somewhat by 24 months. In talking condition, TD toddlers showed right attentional bias from 12 to 24 months, whereas VLBW toddlers showed such bias upon reaching 24 months. Additionally, more TD toddlers than VLBW toddlers attended to the mouth. CONCLUSION: Delays in exhibiting the attentional bias for an audiovisual face or general faces displayed by typically developing children might suggest differential developmental timing for hemispheric specialisation or dominance.

Attentional asymmetry in orienting and alertness and its relationship to temperament: A longitudinal study of infants aged 6-24 months.

Previous research suggests that difficulties in orienting to visual stimuli directed by the right hemisphere are related to parental reports of distress and may be an early sign of autism spectrum disorder (ASD). One possible reason for this difficulty is infants' ability to achieve high levels of alertness, which also depends on the right hemisphere. This study examines the relationship between phasic alertness and asymmetry in visual orienting in typically-developing 6-24-month-old infants using the overlap paradigm. Participants were given a spatially non-directive warning signal shortly before the onset of the peripheral target. The signal reduced latencies in both overlap and no-overlap conditions. No main effects of age and visual field or interaction were found. However, results confirm the negative association between temperamental soothability and disengagement difficulty toward the left visual field at 6 months. This is discussed from the viewpoint of early left visual field attentional bias in relation to social cognition.

Not sleeping soundly in early infancy is not bad.

AIMS: This study sought to determine whether not sleeping soundly in early infancy predicted poor development. This complemented earlier studies of children aged 12 months or older. METHODS: Sleep/wake patterns of 35 Japanese infants (23 males) with a gestational age of 37< weeks were recorded at home using actigraphy. Families were registered with a laboratory-based database for infant research. Follow-up recordings were conducted at 3, 4, 6, 12, and 24 months. Crawling was rated by mothers at 12 months and used to create regular and irregular crawling groups. Temperament was scaled using the Japanese Infant Behaviour Questionnaire-Revised Questionnaire and the Japanese Early Childhood Behaviour Questionnaire. RESULTS: At 4 months, infants with regular crawling style had shorter night-time sleep than infants with irregular crawling style. However, at 12 months, the former had longer motionless sleep at night compared to the latter. Before 6 months, infants with regular crawling style showed lower sleep efficiency, especially during the day, compared to those with irregular crawling style. In addition, the amount of night-time active sleep at 3 and 4 months were positively correlated with day-time activity, but not at 6 months. CONCLUSION: Short fragmental sleep in early infancy did not always predict poor development.

Early temperament as a predictor of language skills at 40 months.

BACKGROUND: Mastering language involves the development of expressive and receptive skills among children. While it has been speculated that early temperament plays a role in the acquisition of language, the actual mechanism has not yet been explored. We investigated whether temperament at 18 months predicted expressive or receptive language skills at 40 months. METHODS: A representative sample of 901 children and their mothers who were enrolled and followed-up longitudinally in the Hamamatsu Birth Cohort for Mothers and Children study was included in the analysis. Child temperament was measured at 18 months using the Japanese version of the Early Childhood Behavior Questionnaire. Expressive and receptive language skills were measured at 40 months using the Mullen Scales of Early Learning. RESULTS: The multiple regression analysis, adjusting for potential confounders, suggested that higher motor activation (fidgeting) at 18 months was associated with lower expressive and receptive language skills at 40 months. Higher perceptual sensitivity was associated with higher expressive and receptive language skills at 40 months. CONCLUSIONS: Specific temperament at 18 months of age predicted the development of the child's expressive and receptive language skills at 40 months.

Cohort profile: Aichi regional sub-cohort of the Japan Environment and Children's Study (JECS-A).

PURPOSE: Effects of fetal, perinatal and childhood environment on the health of children at birth and during later life have become a topic of concern. The Aichi regional sub-cohort of the Japan Environment and Children's Study (JECS-A) is an ongoing birth cohort of pregnant women and their children which has been used to provide unique data, as adjunct studies of JECS, on multifaceted potential factors affecting children's health. PARTICIPANTS: The JECS-A is part of the JECS which follows a total of 100 000 pairs of children and their mothers (fathers' participation is optional) across 15 regions in Japan. In JECS-A, of the 8134 pregnant women living in Ichinomiya City and Nagoya City, Japan, a total of 5721 pregnant women and their 5554 children were included. Sociodemographic and psychological data as well as biological specimens were collected from the pregnant women and their spouses (if available) in the cohort during their pregnancy. Information on children included in the JECS-A was collected from their mothers and includes demographic, behavioural, childcare, psychological and psychiatric data. Urine extracted from disposable diapers and anthropometric data were also obtained from the children. FINDINGS TO DATE: A similar distribution trend for age at delivery was confirmed between the pregnant women enrolled in the JECS-A and the national statistics of the relevant areas. However, differences in education level and household income were observed. A total of 5502 children remained in the cohort at 18 months after delivery. Compared with the national statistics, the basic demographics of the children in the cohort represented the population in the study areas. FUTURE PLANS: The enrolled children in the JECS-A will be followed until the age of 13 years. The studies that come from JECS-A will complement JECS and bring novel results with a high level of generalisability.

Progression of hearing loss and choice of hearing aids by patients in their 60s, 70s, and 80s and older: experience in the Japanese super-aged era.

Background: Demographic data of patients with sensorineural hearing loss (SNHL) in super-aged societies are still limited.Aims/objectives: To report audiometric statistics of SNHL and hearing aid (HA) use in patients in their 60s, 70s, and 80s and older during the super-aged era.Material and methods: Medical charts and audiograms of 2064 older patients with SNHL who visited a Japanese University Hospital in 2007-2018 were retrospectively reviewed. Among 270 patients referred to the HA service unit (HASU), the percentage of final decisions to continue using HAs was calculated.Results: The average pure tone thresholds on initial visit to the clinic were 56.9, 60.6, 69.4, and 82.4 dB HL in patients in their 60s, 70s, 80s, and 90s, respectively. The rates of progression were 0.25, 0.87, 1.19, and 1.37 dB/year in patients in their 50s, 60s, 70s, and 80s, respectively. The percentage of patients in HASU who chose to use HAs did not differ among the 60s (59.3%), 70s (51.2%), and 80s and older (58.2%).Conclusions and significance: The clinical picture of patients with SNHL in their 70s and 80s differs because progression accelerates exponentially through these ages. HAs can be recommended to older adult patients in all the age groups.

Early development of attentional disengagement and phasic alertness.

Attention development is a critical foundation for cognitive abilities. This study examines the relationship between phasic aspects of alertness and disengagement in infants, using the overlap paradigm. Research shows that visual disengagement in overlap condition is modulated by auditory cues in 6-year-olds. Our participants were aged 6 months (N = 20), 12 months (N = 27), and 24 months (N = 14). Phasic alertness during overlap and no-overlap tasks was manipulated using a spatially nondirective warning signal shortly before onset of the peripheral target. Responses in overlap condition were slower and fewer than in no-overlap condition. The signal showed a tendency to reduce latencies in both overlap and no-overlap conditions. While our hypothesis that the warning signal might be more effective in younger infants was not supported, we confirmed the association reported in previous studies between temperamental soothability and disengagement latencies in infancy.

Attentional bias assessed by a facial expression cuing paradigm in infants.

To disambiguate infants' attentional bias towards fearful facial expressions, we applied a facial expression cueing paradigm to 36 6-month-old and 33 12-month-old infants, with 21 infants taking part at both ages. Infants made saccades towards a peripheral target preceded by a happy, fearful, or neutral cue directing their attention to the target location (congruent) or the wrong location (incongruent). The results show that infants were faster to respond when shown a fearful (vs. happy) face as a congruent cue, which is consistent with previous studies referring to fearful vigilance, while an incongruent fearful cue reduces attention shifts to the target on the opposite side of the monitor to a greater extent than an incongruent happy cue at 12 months, implying that a fearful facial expression prolongs attentional disengagement or is associated with a greater narrowing of attention. Additionally, the latencies of 6-month-olds were significantly faster than those of 12-month-olds in a congruent condition. The relationship between attentional bias and temperamental disposition was examined using the Infant Behavior Questionnaire-Revised. High temperamental orienting scores partly correlated with attentional bias at 12 months. The contributions of attentional brain networks to socio-cognitive and emotional development are also discussed.

Relationship between pure-tone audiogram findings and speech perception among older Japanese persons.

OBJECTIVE: To clarify how the pure-tone threshold (PTT) on the PTA predicts speech perception (SP) in elderly Japanese persons. METHODS: Data on PTT and SP were cross-sectionally analyzed in Japanese persons (656 ears in 353 patients, aged ≥65 years). Correlations of SP and average PTT in all tested frequencies were evaluated by Pearson's correlation coefficient and simple linear regression. After adjusting for sex, laterality of ears, and age, the relationship of average and frequency-specific PTT with impaired SP ≤50% was estimated by logistic regression models. RESULTS: SP correlated well (r = -0.699) with the average PTT of all tested frequencies. On the other hand, the correlation between patient age and SP was weak, especially among ≤85-year-old persons (r = -0.092). Linear regression showed that the average PTT corresponding to SP of 50% was 76.4 dB nHL. Odds ratios for impaired SP were highest for PTT at 2000 Hz. Odds ratios were higher for middle (500, 1000, 2000 Hz) and high frequencies (4000, 8000 Hz) than low frequencies (125, 250 Hz). CONCLUSION: The PTT on the pure-tone audiogram (PTA) is a good predictor of SP by speech audiometry among older persons, which could provide clinically important information for hearing aid fitting and cochlear implantation.

Subjective hearing-related quality-of-life is a major factor in the decision to continue using hearing aids among older persons.

CONCLUSION: Audiological parameters alone do not determine the choice to use hearing aids (HA). Subjective hearing-related QoL is a major factor that determines whether or not an older person will continue to wear HA. OBJECTIVE: This study aimed to identify which audiological parameters and quality-of-life (QoL) measures determine whether or not older persons will continue wearing HA. METHODS: Charts of 157 patients aged ≥65 years who attended the HA service unit at the Otolaryngology Department were retrospectively reviewed. After HA fitting and a trial, the patients were divided into groups, depending upon whether or not they wanted to continue wearing the HA (users, 58.2%; non-users, 41.8%) and then audiological parameters were compared between them. At least 4 months after the HA fitting, the self-reported QoL questionnaire, Hearing Handicap Inventory for the Elderly (HHIE), was mailed to all 157 patients and HHIE scores were compared between HA users and non-users. RESULT: Speech discrimination score and dynamic range did not significantly differ between HA users and non-users. A difference in the average hearing threshold was marginally significant. The response rate to the HHIE was 65.2%. Total HHIE and emotional scores were higher (more impaired) among HA users than non-users.

Relations between Temperament, Sensory Processing, and Motor Coordination in 3-Year-Old Children.

Poor motor skills and differences in sensory processing have been noted as behavioral markers of common neurodevelopmental disorders. A total of 171 healthy children (81 girls, 90 boys) were investigated at age 3 to examine relations between temperament, sensory processing, and motor coordination. Using the Japanese versions of the Children's Behavior Questionnaire (CBQ), the Sensory Profile (SP-J), and the Little Developmental Coordination Disorder Questionnaire (LDCDQ), this study examines an expanded model based on Rothbart's three-factor temperamental theory (surgency, negative affect, effortful control) through covariance structure analysis. The results indicate that effortful control affects both sensory processing and motor coordination. The subscale of the LDCDQ, control during movement, is also influenced by surgency, while temperamental negative affect and surgency each have an effect on subscales of the SP-J.

Pediatric central auditory processing disorder showing elevated threshold on pure tone audiogram.

Central auditory processing disorder (CAPD) is a condition in which dysfunction in the central auditory system causes difficulty in listening to conversations, particularly under noisy conditions, despite normal peripheral auditory function. Central auditory testing is generally performed in patients with normal hearing on the pure tone audiogram (PTA). This report shows that diagnosis of CAPD is possible even in the presence of an elevated threshold on the PTA, provided that the normal function of the peripheral auditory pathway was verified by distortion product otoacoustic emission (DPOAE), auditory brainstem response (ABR), and auditory steady state response (ASSR). Three pediatric cases (9- and 10-year-old girls and an 8-year-old boy) of CAPD with elevated thresholds on PTAs are presented. The chief complaint was difficulty in listening to conversations. PTA showed elevated thresholds, but the responses and thresholds for DPOAE, ABR, and ASSR were normal, showing that peripheral auditory function was normal. Significant findings of central auditory testing such as dichotic speech tests, time compression of speech signals, and binaural interaction tests confirmed the diagnosis of CAPD. These threshold shifts in PTA may provide a new concept of a clinical symptom due to central auditory dysfunction in CAPD.

Characteristics of audiogram configuration in multiple-system atrophy C and cortical cerebellar atrophy.

CONCLUSION: The prevalence of low-tone hearing loss (LTHL) is significantly high in spinocerebellar degeneration (SCD) with cerebellar predominance, including multiple-system atrophy C (MSA-C) and cortical cerebellar atrophy (CCA). OBJECTIVE: This study aimed to test the hypothesis that SCD with cerebellar predominance, MSA-C and CCA may cause auditory symptoms. METHODS: The shape and threshold of pure-tone audiograms were evaluated for MSA-C (n = 47; mean (± SD) age, 61.6 ± 8.9 years), CCA (n = 16; 62.8 ± 9.5 years), and age-matched controls (n = 169; 62.5 ± 10.7 years). To differentiate specific hearing loss for MSA-C and CCA from presbycusis, the shape of audiograms was examined based on previously established audiological criteria. RESULTS: When audiogram shape was defined according to audiological criteria, the odds ratio for LTHL in SCD compared to controls was 2.492 (95% confidence interval (CI) = 1.208-5.139; p < 0.05, Pearson's Chi-square test) in MSA-C and 2.194 (95% CI = 0.709-6.795) in CCA. When the selection of audiogram shape according to these criteria was verified by three certified audiologists, odds ratios for LTHL in MSA-C and CCA were 3.243 (95% CI = 1.320-7.969) and 3.692 (95% CI = 1.052-12.957), respectively, significantly higher than in controls.

Individual differences in disengagement of fixation and temperament: longitudinal research on toddlers.

It has been suggested that a shift occurs in the brain's control system from the orienting network in infancy to the executive network by the age of 3-4 years; however, there has been little empirical evidence of this shift during toddlerhood. Therefore, the present study examined how the orienting system in infancy is related to an effortful control system at a later age. Children were assessed longitudinally at 12, 18, 24, and 36 months of age, using a gap-overlap task in which dynamic geometrical-shape stimuli were presented. Parents completed temperament questionnaires about the children at each age. A delayed-gratification task was also given to 36-month-olds. Overall, saccadic latencies in the gap-overlap task were significantly faster at 36 months. At all ages, responses were slower during overlap trials than during gap or no-overlap trials. Longer latencies in the overlap condition were associated with low temperamental orienting/regulation scores at 12 months but with high effortful control scores at 18 and 24 months. The associations at 18 and 24 months are thought to represent a genuine positive association between effortful control and sustained and focused attention.

Variable coordination of eye and head movements during the early development of attention: a longitudinal study of infants aged 12-36 months.

This longitudinal study investigated the effects of attentional development on peripheral stimulus localization by analyzing the eye and head movements of toddlers as they matured from 12 to 36 months. On each trial of an experiment, a central fixation point and a 30° peripheral stimulus were presented, such that in the gap condition the fixation disappeared 300 ms before the peripheral stimulus, whereas in the no-overlap condition it disappeared simultaneously as the peripheral stimulus, and in the overlap condition the fixation remained present when the peripheral target occurred. Results showed that eye and head movement latencies were highly correlated in all conditions and ages. However, at 12 months, head movements were as fast as eye movements, whereas during the subsequent development, eye movements became increasingly faster than head movements. These findings are indicative of a transition between 12 and 36 months due either to a change in attentional control, or to changes in the size of the visual field in which only eye movements occur.

Dependence receptor UNC5D mediates nerve growth factor depletion-induced neuroblastoma regression.

Spontaneous regression of neuroblastoma (NB) resembles the developmentally regulated programmed cell death (PCD) of sympathetic neurons. Regressing tumor cells express high levels of the nerve growth factor (NGF) receptors TRKA and p75NTR and are dependent on NGF for survival; however, the underlying molecular mechanism remains elusive. Here, we show that UNC5D, a dependence receptor that is directly targeted by p53 family members, is highly expressed in favorable NBs. NGF withdrawal strongly upregulated UNC5D, E2F1, and p53 in human primary favorable NBs. The induced UNC5D was cleaved by caspases 2/3, and the released intracellular fragment translocated into the nucleus and interacted with E2F1 to selectively transactivate the proapoptotic target gene. The cleavage of UNC5D and its induction of apoptosis were strongly inhibited by addition of netrin-1. Unc5d(-/-) mice consistently exhibited a significant increase in dorsal root ganglia neurons and resistance to NGF depletion-induced apoptosis in sympathetic neurons compared with wild-type cells. Our data suggest that UNC5D forms a positive feedback loop with p53 and E2F1 to promote NGF dependence-mediated PCD during NB regression.

Difficulty in disengaging from threat and temperamental negative affectivity in early life: a longitudinal study of infants aged 12-36 months.

BACKGROUND: Attention disengagement is reportedly influenced by perceiving a fearful facial expression even in the first year of life. In the present study, we examined whether individual differences in disengaging from fearful expressions predict temperamental negative affectivity. METHOD: Twenty-six infants were studied longitudinally at 12, 18, 24, and 36 months, using an overlap paradigm and two temperament questionnaires: the Japanese versions of the revised Infant Behavior Questionnaire and Early Childhood Behavior Questionnaire. RESULTS: The infants fixated significantly more frequently to fearful than to happy or neutral faces. The attentional bias to threat (i.e., the number of fixed responses on fearful faces divided by the total number of fixed responses on faces) at 12 months was significantly positively correlated with negative affect at 12 months, and its relations with negative affect measured later in development was in the expected positive direction at each age. In addition, a moderation analysis indicates that the orienting network and not the executive network marginally moderated the relation between early attentional bias and later fear. CONCLUSIONS: The results suggest that at 12 months, infants with more negative affectivity exhibit greater difficulty in disengaging their attention from fearful faces. We also found evidence that the association between parent-reported fear and disengagement might be modulated in the second year, perhaps because of the differences in temperamental control networks.

Prognostic impact of morphologic and phenotypic features of childhood ALK-positive anaplastic large-cell lymphoma: results of the ALCL99 study.

PURPOSE: The prognostic value of pathologic characteristics of childhood ALK-positive anaplastic large-cell lymphomas (ALCL), such as histologic subtypes, immunophenotype, and presence of the t(2;5) translocation or its variants, was assessed. PATIENTS AND METHODS: All 375 patients with systemic ALK-positive ALCL included in an international trial launched by the European Intergroup for Childhood Non-Hodgkin's Lymphoma were reviewed by an international panel of pathologists based on conventional hematoxylin and eosin-stained and immunostained sections and classified according to the 2001 WHO classification. RESULTS: A small-cell (SC) or lymphohistiocytic (LH) component was observed in 114 (32%) of 361 patients, whereas ALCL of common type was diagnosed in 235 (65%) of 361 patients. Regarding the histologic subtyping of patients within the two categories of ALCL (with v without SC/LH component), the concordance between the national and international reviews was quite good, with a κ index equal to 0.67 (95% CI, 0.57 to 0.75). The presence of an SC/LH component was significantly associated with a high risk of failure (hazard ratio [HR], 2.0; 95% CI, 1.3 to 3.0; P = .002) in the multivariate analysis controlling for clinical characteristics, as well as the perivascular pattern (HR, 1.7; 95% CI, 1.1 to 2.7; P = .01), whereas CD3 positivity was significantly associated with a high risk of failure only in univariate analysis. CONCLUSION: Our study, which to our knowledge includes the largest series of childhood systemic ALK-positive ALCL so far, demonstrates the adverse prognostic value of SC and/or LH morphologic features. Combining these histologic characteristics with other biologic or clinical factors might have a high potential for future risk stratification and treatment.

Living donor liver transplantation for multiple intrahepatic portosystemic shunts after involution of infantile hepatic hemangiomas.

We describe a 6-year-old girl presenting with multiple intrahepatic portosystemic shunts after the involution of infantile hepatic hemangiomas (IHHs), who successfully underwent living donor liver transplantation. The chronological changes of radiologic findings indicated that remnant portovenous shunts at the time of IHHs involution developed gradually on the background of atrophic intrahepatic portal veins. This suggests that patients should be carefully followed up for the late onset of intrahepatic portosystemic shunts after the involution of IHHs.