BACKGROUND: Universal newborn hearing screening (UNHS) targets moderate or greater hearing loss. However, UNHS also frequently detects children with mild loss that results in many receiving early treatment. The benefits of this approach are not yet established. We aimed to (i) compare language and psychosocial outcomes between four hearing loss detection systems for children aged 5-8 years with congenital mild-moderate hearing loss; (ii) determine whether age of detection predicts outcomes; and (iii) compare outcomes between children identified via well-established UNHS and the general population. METHODS: Linear regression adjusted for potential confounding factors was used throughout. Via a quasi-experimental design, language and psychosocial outcomes were compared across four population-based Australian systems of hearing loss detection: opportunistic detection, born 1991-1993, n = 50; universal risk factor referral, born 2003-2005, n = 34; newly established UNHS, born 2003-2005, n = 41; and well-established UNHS, born 2007-2010, n = 21. In pooled analyses, we examined whether age of detection predicted outcomes. Outcomes were similarly compared between the current well-established UNHS system and typically developing children in the Early Language in Victoria Study, born 2003, n = 1217. RESULTS: Age at diagnosis and hearing aid fitting fell steadily across the four systems. For moderate losses, mean expressive language (P for trend .05) and receptive vocabulary (P for trend .06) improved across the four systems, but benefit was not obvious for mild losses. In pooled analyses, diagnosis before age six months predicted better language outcomes for moderate losses. Children with mild-moderate losses exposed to well-established UNHS continue to experience expressive language scores well below children in the general population (adjusted mean difference -8.9 points, 95% CI -14.7 to -3.1). CONCLUSIONS: Treatment arising from UNHS appears to be clearly benefitting children with moderate hearing losses. However, rigorous trials are needed to quantify benefits, versus costs and potential harms, of early aiding of children with mild losses.

BACKGROUND: Evidence suggests that language and social, emotional and behavioural (SEB) difficulties are associated in children and adolescents. When these associations emerge and whether they differ by language or SEB difficulty profile is unclear. This knowledge is crucial to guide prevention and intervention programmes for children with language and SEB difficulties. AIMS: To determine whether receptive and expressive language skills are associated with internalizing and externalizing behaviours in slow-to-talk toddlers. METHODS & PROCEDURES: In a community-based prospective study of 200 slow-to-talk children, language was measured at 24 and 36 months using Preschool Language Scale 4th Edition and at 48 months using Clinical Evaluation of Language Fundamentals-Preschool 2nd Edition. Internalizing and externalizing behaviours were measured by parent report at each age. Longitudinal data were analysed using repeated-measures regression, with up to three observations per child. Robust standard errors were used to account for non-independence of measures within participants. The shape of the associations were examined by fitting quadratic and cubic terms. The effects of confounders on the associations were examined. OUTCOMES & RESULTS: Receptive language had a negative linear association with internalizing behaviours after adjusting for confounders (β = -0.16, 95% [CI = -0.26, -0.07], p = .001); and a negative curved association with externalizing behaviours after adjusting for biological confounders (βquadratic = 0.08 [0.01, 0.15], p = .03, βcubic = -0.04 [-0.07, -0.02], p = .001), attenuating after adjusting for environmental confounders (βquadratic = 0.06 [-0.01, 0.13], p = .09, βcubic = -0.03 [-0.06, -0.003], p = .03). The curvature suggests that the negative association with externalizing behaviours only existed for children with either very low or very high receptive language scores. After controlling for confounders, there was no evidence that expressive language scores were associated with internalizing (β = -0.08, 95% [CI = -0.17, 0.01], p = .10) or externalizing behaviours (β = 0.03, 95% [CI = -0.09, 0.18], p = .61). Tests of interaction revealed no evidence of a differential association by age. CONCLUSIONS & IMPLICATIONS: In 24-48-month-old slow-to-talk children, lower receptive language scores were associated with higher internalizing behaviours. The magnitude of the association was small. For children with very poor receptive language scores, lower receptive language skills were associated with higher externalizing behaviours. Young children with low receptive language abilities may be at risk of internalizing difficulties; those with very low receptive language skills may be at particular risk of externalizing difficulties. This has clinical implications for interventions for young children with receptive language difficulties.

PURPOSE: Genomic newborn screening raises practical and ethical issues. Evidence is required to build a framework to introduce this technology safely and effectively. We investigated the choices made by a diverse group of parents with newborns when offered tiered genomic information from exome sequencing. METHODS: This population-derived cohort comprised infants with congenital deafness. Parents were offered exome sequencing and choice regarding the scope of analysis. Options were choice A, diagnostic analysis only; choice B, diagnostic analysis plus childhood-onset diseases with medical actionability; or choice C, diagnostic analysis plus childhood-onset diseases with or without medical actionability. RESULTS: Of the 106 participants, 72 (68%) consented to receive additional findings with 29 (27.4%) selecting choice B and 43 (40.6%) opting for choice C. Family size, ethnicity, and age of infant at time of recruitment were the significant predictors of choice. Parents who opted to have additional findings analysis demonstrated less anxiety and decisional conflict. CONCLUSIONS: These data provide evidence from a culturally diverse population that choice around additional findings is important and the age of the infant when this choice is offered impacts on their decision. We found no evidence that offering different levels of genomic information to parents of newborns has a negative psychological impact.

BACKGROUND: Conversational breakdowns are a persistent concern for older adults with hearing impairment (HI). Previous studies in experimental settings have investigated potential causes of breakdowns in conversations with a person with HI, and effective strategies for repairing these breakdowns. However, little research has explored the causes of hearing-related communication breakdowns, and their repairs, in extended, naturally occurring conversations in a healthcare setting. AIMS: To analyse systematically instances of clients' initiations of repair within video-recorded initial audiology appointments, and to examine the interactional environment in which they occurred. METHODS & PROCEDURES: Participants included 26 audiologists and their older adult clients (aged 55+ years). Companions were present in 17 of the 63 appointments. Conversation analysis (CA) was used to examine the video-recorded audiology appointments with older adults with HI. The corpus was systematically analysed for all instances of 'other-initiated repair' by clients (initiation of repair targeting the prior speakers' turn). A collection of 51 instances of other-initiated repair were identified. These instances were analysed in detail for: (1) the interactional environment in which they occurred; (2) the strategy by which the client initiated repair; and (3) the strategies used by the audiologist to repair the communication breakdown. OUTCOMES & RESULTS: In 76% (n = 39) of the 51 cases of other-initiated repair from the client, there was a lack of mutual gaze between participants (i.e., either the audiologist or the client were looking away or facing in another direction during the prior turn). More specifically, many of these instances occurred when the audiologist was speaking to the client while multitasking. Audiologists used multiple-repair strategies in their responsive turn in an attempt to repair the communication breakdown efficiently. CONCLUSIONS & IMPLICATIONS: These findings, from extended, naturally occurring conversations with older adults with HI in clinic settings, highlight the importance of face-to-face communication even in quiet one-to-one settings. Clinicians should remain aware of their movements and gaze when speaking to clients during appointments. The findings also provide further support for the importance of communication programs in hearing rehabilitation.

AIM: Hearing loss (as reflected by abnormal sound detection) is a frequently reported consequence of Type 1 diabetes mellitus. We sought to evaluate sound detection, auditory neural function and binaural processing ability in a group of school-aged participants with Type 1 diabetes and to assess their functional hearing and general communication ability. METHODS: A range of electroacoustic, electrophysiological and behavioural test techniques were used to evaluate both cochlear and auditory neural function in 19 affected children. A cohort of matched controls was also assessed. RESULTS: Although all of the participants with Type 1 diabetes enjoyed normal sound detection, 9 of the 19 (47%) showed evidence of auditory pathway abnormality with evoked potential latencies and/or amplitudes beyond age-related norms. Auditory brainstem response interpeak latencies (wave I-V) were longer than in matched controls [ 95% confidence interval (95% CI); 0.10, 0.28 ms: P < 0.001] and wave V amplitudes were reduced (95% CI; -0.21, 0.00 μV: P = 0.02). Binaural speech perception in noise was also impaired (95% CI; 0.82, 3.17 dB: P = 0.002) and perceptual ability was correlated with degree of neural disruption in the auditory brainstem (r = 0.662, P = 0.003). CONCLUSIONS: Hearing deficits severe enough to restrict communication and threaten academic progress were common on our group of school-aged children with Type 1 diabetes. Evaluation of both cochlear and auditory neural function may form an important part of the standard management regime for children with diabetes.

BACKGROUND: Trauma is the leading cause of death in children >1 year of age, with haemorrhage as the most common cause of medically preventable deaths. While massive transfusion protocols (MTPs) have been investigated and used in adults to reduce death from haemorrhage, there are a paucity of published data on MTP practices and outcomes in children. This study aimed to survey current MTP policies and the frequency of activation at paediatric care centres. STUDY DESIGN AND METHODS: We conducted a survey of MTPs at hospitals in the United States and Canada, including children's general hospitals, children's specialty hospitals and children's units in general hospitals. We collected information on how the MTP is activated, what therapeutics are given, frequency of its use, and how it is audited for compliance. RESULTS: Forty-six survey responses were analysed. Physician discretion was the most common activation criteria (89%). A majority of sites (78%) targeted a 'high' (≥1 : 2) ratio of plasma to red blood cells (RBC). Fifteen percent of sites use antifibrinolytics in their MTPs. Eighty nine percent of sites have type-O RBC units and 48% of sites had thawed plasma units stored in an immediately available location. CONCLUSION: There is a wide variation in MTPs among paediatric hospitals with regard to both activation criteria and products administered. This underscores the need for future prospective studies to determine the most effective resuscitation methods for paediatric populations to improve outcomes and therapeutic safety for massive bleeding.

Purpose There is a well-established relationship between hearing loss and psychological symptoms. To ensure audiological rehabilitation is provided appropriately for older adults with comorbid psychological symptoms, a greater understanding of their preferences and experiences is needed. This study sought to understand experiences of hearing loss and audiological rehabilitation from the perspective of older adults with comorbid psychological symptoms (e.g., depression, anxiety, psychosis). Design A qualitative study using in-depth semistructured interviews was conducted with older adults who had attended audiological rehabilitation within the last year and scored above established cutoffs on measures of depression, anxiety, and psychosis. A thematic analysis generated themes that related to participants' experiences of hearing loss and audiological rehabilitation. Results Participants included 14 older adults (eight men and six women) with an average age of 70.5 years (SD = 4.45, range: 64-80) who received hearing aids or a cochlear implant. Three major themes emerged from the analysis of participant interviews. "The cumulative impact of hearing loss and psychological symptoms" theme describes the two-way, additive relationship between hearing ability and psychological symptoms. "The experience of loss throughout hearing loss and audiological rehabilitation" captures subjective losses, the impact they have, and how participants cope with them. In contrast, "The experience of gain throughout hearing loss and audiological rehabilitation" describes the participants' reported gains, their related impacts, and coping strategies. Conclusions The experiences of participants revealed that the presence of comorbid psychological symptoms can influence the experience of hearing loss and audiological rehabilitation. These findings have implications for how audiological rehabilitation is provided to ensure optimal outcomes for adults with hearing loss and comorbid psychological symptoms. Supplemental Material https://doi.org/10.23641/asha.12985955.

Expressive communication impairment is associated with haploinsufficiency of SETBP1, as reported in small case series. Heterozygous pathogenic loss-of-function (LoF) variants in SETBP1 have also been identified in independent cohorts ascertained for childhood apraxia of speech (CAS), warranting further investigation of the roles of this gene in speech development. Thirty-one participants (12 males, aged 0; 8-23; 2 years, 28 with pathogenic SETBP1 LoF variants, 3 with 18q12.3 deletions) were assessed for speech, language and literacy abilities. Broader development was examined with standardised motor, social and daily life skills assessments. Gross and fine motor deficits (94%) and intellectual impairments (68%) were common. Protracted and aberrant speech development was consistently seen, regardless of motor or intellectual ability. We expand the linguistic phenotype associated with SETBP1 LoF syndrome (SETBP1 haploinsufficiency disorder), revealing a striking speech presentation that implicates both motor (CAS, dysarthria) and language (phonological errors) systems, with CAS (80%) being the most common diagnosis. In contrast to past reports, the understanding of language was rarely better preserved than language expression (29%). Language was typically low, to moderately impaired, with commensurate expression and comprehension ability. Children were sociable with a strong desire to communicate. Minimally verbal children (32%) augmented speech with sign language, gestures or digital devices. Overall, relative to general development, spoken language and literacy were poorer than social, daily living, motor and adaptive behaviour skills. Our findings show that poor communication is a central feature of SETBP1 haploinsufficiency disorder, confirming this gene as a strong candidate for speech and language disorders.

Children's speech difficulties can be motor (phone misarticulation) or linguistic (impaired knowledge of phonological contrasts and constraints). These two difficulties sometimes co-occur. This paper reports longitudinal data from the Early Language in Victoria Study (ELVS) at 4 and 7 years of age. Of 1494 participants, 93 made non-age appropriate speech errors on standardised assessments at 4 years, and were able to be reassessed at 7 years. At 4 years, 85% of these children only made phonological errors, 14% made both articulation and phonological errors and one child only made articulation errors (a lateral lisp). In total, 8 of 13 children making both articulation and phonological errors at 4 years had resolved by 7 years. Unexpectedly, eight children who had demonstrated articulation of fricatives at 4 years, acquired distorted production of ≥ 50% of occurrences of/s, z/ by 7 years. In total, then, 22 children (24% of children with speech difficulties) made articulatory errors at one or both assessments. Case data for all children are presented. Theoretical and clinical implications are considered.

Speech sound disorder (SSD) is common, yet its neurobiology is poorly understood. Recent studies indicate atypical structural and functional anomalies either in one hemisphere or both hemispheres, which might be accompanied by alterations in inter-hemispheric connectivity. Indeed, abnormalities of the corpus callosum - the main fiber tract connecting the two hemispheres - have been linked to speech and language deficits in associated disorders, such as stuttering, dyslexia, aphasia, etc. However, there is a dearth of studies examining the corpus callosum in SSD. Here, we investigated whether a sample of 18 children with SSD differed in callosal morphology from 18 typically developing children carefully matched for age. Significantly reduced dimensions of the corpus callosum, particularly in the callosal anterior third, were observed in children with SSD. These findings indicating pronounced callosal aberrations in SSD make an important contribution to an understudied field of research and may suggest that SSD is accompanied by atypical lateralization of speech and language function.

Diffusion MRI has provided the neuroimaging community with a powerful tool to acquire in-vivo data sensitive to microstructural features of white matter, up to 3 orders of magnitude smaller than typical voxel sizes. The key to extracting such valuable information lies in complex modelling techniques, which form the link between the rich diffusion MRI data and various metrics related to the microstructural organization. Over time, increasingly advanced techniques have been developed, up to the point where some diffusion MRI models can now provide access to properties specific to individual fibre populations in each voxel in the presence of multiple "crossing" fibre pathways. While highly valuable, such fibre-specific information poses unique challenges for typical image processing pipelines and statistical analysis. In this work, we review the "Fixel-Based Analysis" (FBA) framework, which implements bespoke solutions to this end. It has recently seen a stark increase in adoption for studies of both typical (healthy) populations as well as a wide range of clinical populations. We describe the main concepts related to Fixel-Based Analyses, as well as the methods and specific steps involved in a state-of-the-art FBA pipeline, with a focus on providing researchers with practical advice on how to interpret results. We also include an overview of the scope of all current FBA studies, categorized across a broad range of neuro-scientific domains, listing key design choices and summarizing their main results and conclusions. Finally, we critically discuss several aspects and challenges involved with the FBA framework, and outline some directions and future opportunities.