Background High-risk pregnancies increase the likelihood of neonatal hearing impairment, yet early signs are often subtle or absent, delaying diagnosis. Early identification through newborn hearing screening is critical to prevent long-term auditory and developmental deficits. Objective To evaluate neonatal hearing screening outcomes using Automated Auditory Brainstem Response (AABR) in neonates born through high-risk pregnancies at Dhulikhel Hospital. Method In this prospective study, neonates born between 15th March 2023 and 15th September 2024 through high-risk pregnancies were screened using Automated auditory brainstem response within 24 hours of birth. Neonates failing the initial screening were re-tested at six weeks. Maternal risk factors including hypothyroidism, diabetes, hypertension, pre-eclampsia, renal disorders, STDs, psychiatric disorders, heart disease, smoking, and extreme maternal age were recorded. Neonates failing the second screening were referred and tested with diagnostic automated auditory brainstem response . Result Ninety neonates (41 males, 49 females) were included. Thirty-two neonates failed the initial automated auditory brainstem response , and nine failed the second screening, yielding a referral rate of 10%. Maternal hypothyroidism was the most common risk factor among referred cases. Other maternal factors associated with referrals included diabetes, maternal age ≥ 35 years, pre-eclampsia, smoking, and STDs. The overall diagnostic automated auditory brainstem response failure rate was 3.3%, highlighting the low but significant prevalence of hearing impairment among high-risk pregnancies. Conclusion Maternal health conditions, particularly hypothyroidism, diabetes, and advanced maternal age, are associated with increased risk of neonatal hearing impairment. automated auditory brainstem response is an effective screening tool in high-risk populations, and early detection with timely intervention is essential to optimize auditory and developmental outcomes.

Background/Objectives: Auditory neuropathy is a form of hearing loss marked by preserved outer hair cell function and abnormal or absent auditory brainstem responses. Monogenic causes play a significant role in its aetiology. This systematic review aims to identify the genetic causes of auditory neuropathy reported in the literature and to determine the diagnostic yield of genetic testing in affected individuals. Methods: A systematic search of MEDLINE, Embase, and PubMed was conducted. Studies were included if participants had a diagnosis of auditory neuropathy and if genetic testing results were reported with variant interpretation based on American College of Medical Genetics and Genomics criteria. Results/Discussion: Twenty-nine studies involving 441 children and adults with auditory neuropathy were included. Overall, 21 different genes and 136 pathogenic and likely pathogenic variants were found to be causative of auditory neuropathy, with both syndromic and non-syndromic presentations. Variants in OTOF were the most common cause, responsible for 59% of all genetic diagnoses found. A genetic diagnosis was confirmed in 195 of 362 individuals who underwent genetic testing, resulting in a diagnostic yield of 54%. After adjusting for study bias and new gene associations with AN, the diagnostic yield was 31%. Conclusions: This review identifies gene and variant-level associations with auditory neuropathy that enhance our understanding of the condition. It highlights the high diagnostic yield of genetic testing in auditory neuropathy which supports consideration of genetic testing early in the diagnostic pathway. A genetic diagnosis may support precision-based approaches to treatment, including cochlear implants and participation in gene therapy trials.

Monitoring how attention is distributed across concurrent task demands is fundamental in daily life contexts such as mobility, education and industrial control. However, existing neurophysiological measures, typically based on univariate EEG markers, are sensitive to generic cognitive load, visual complexity, or motor activity, and therefore lack specificity for attentional splitting. Here, we introduce the Attentional Split Index (ASI), a novel EEG-based metric that employ Mutual Information (MI) theory, designed to quantify the coordinated modulation of multiple neurometrics that emerges when attention is divided across tasks. Twenty-five participants completed a realistic driving protocol combining a main driving task in two different environments (Urban, Highway) with four types of attentional-split demands, i.e., Focused, Auditory Continuous Performance Test (ACPT), Matrix, Surrogate Reference Task (SURT). Traditional neurometrics (parietal alpha, inverse frontal beta, frontal theta/beta ratio) exhibited partial sensitivity to task demands but failed to selectively reflect attentional splitting. In contrast, the ASI showed a robust and systematic increase across conditions, distinguishing not only explicit multitasking segments but also subtler differences between Urban and Highway focused driving. Eye-tracking and subjective distraction rating showed a strong and significant correlation with the ASI (Urban: rET = 0.515 and rSUB = 0.744; Highway: rET = 0.357 and rSUB = 0.673; all p < 10-2), confirming high behavioural and phenomenological coherence. Surrogate analyses demonstrated that ASI effects were absent when temporal coordination across neurometrics was artificially disrupted, and that real ASI exceeded surrogate values in the vast majority of participants during multitasking but not during eyes-open baseline. Together, these findings establish the ASI as a specific, robust, and ecologically coherent neural marker of attentional splitting, with promising implications for neuroergonomics and next-generation adaptive human-machine systems.

Morquio A syndrome (mucopolysaccharidosis IVA) is a lysosomal storage disorder characterized by systemic skeletal dysplasia and multisystem involvement. Hearing loss is frequently reported but remains insufficiently characterized, with few studies incorporating comprehensive behavioral and electrophysiologic assessments. In this prospective study, the largest Morquio A auditory cohort to date, participants underwent detailed audiologic and imaging testing at Nemours Children's Health as part of the National Institutes of Health-sponsored natural history program. Assessments included pure-tone thresholds, speech-recognition thresholds, speech perception in quiet and noise, tympanometry, distortion-product otoacoustic emissions, auditory brainstem responses, and cortical auditory evoked potentials. Outcomes included the prevalence, type, and severity of hearing loss, as well as associations with age and clinical disease severity. Hearing loss was identified in 50.9% of 56 patients, with sensorineural loss more frequent than conductive or mixed types. Prevalence of hearing loss was significantly correlated with clinical severity (height percentile), whereas no significant correlation was observed between hearing loss severity and diagnostic age. Reduced or absent otoacoustic emissions with preserved brainstem and cortical responses indicate predominant cochlear involvement with potential secondary central contributions. Comprehensive imaging of the middle and inner ear showed no significant structural differences between patients with and without hearing loss. Hearing loss is common in Morquio A and is primarily sensorineural. The absence of associations with age or disease severity underscores the heterogeneity of auditory involvement. These findings emphasize the need for routine, comprehensive audiologic monitoring to support early detection and mitigate long-term impacts on communication and quality of life.

Developmental language disorder (DLD) is a persistent difficulty in the acquisition and use of expressive and/or receptive language, which negatively impacts academic and social development. The present study evaluated the validity of the statistical learning model proposed to account for language difficulties in children with DLD. To this end, two auditory paradigms of varying complexity, framed within predictive coding theory, were passively presented to children diagnosed with DLD and to typically developing children without neurological impairments. The paradigms consisted of stimulus sequences with decreasing or increasing frequencies, interspersed with the sporadic occurrence of unexpected tone endings. The psychophysiological response was recorded using EEG, focusing on the P1, mismatch negativity (MMN), postimperative negative variation (PINV), and contingent negative variation (CNV) components. Results showed an absent MMN and a higher P1 response to deviant tones in children with DLD, suggesting an impaired development of frontal MMN generators, potentially compensated by activity in the primary auditory cortex. DLD participants also showed increased PINV and CNV responses during the most complex paradigm, which could imply greater cognitive effort and resource allocation for reassessment of stimulus patterns. Finally, incomplete maturation of frontal areas in children within this age range (3-11 years) was proposed as a possible explanation for the absence of differences between groups in P1 and N1/MMN responses elicited by simple and complex conditions. These findings support statistical learning as a valid model for understanding the possible neural basis of DLD and highlight this predictive EEG design as a potential protocol for early detection.

Predictive coding conceptualizes attention as a weighting of prediction error signals. However, empirical findings on how attention influences common markers of prediction error have been inconsistent, likely because these markers are typically derived from stimulus-evoked responses. To avoid stimulus-related confounds and isolate effects related purely to prediction, we investigated how attention modulates brain responses to unexpected stimulus omissions. Using visual-auditory couplings where the auditory stimulus was occasionally omitted, we recorded EEG responses that revealed a multistage omission response-from early sensory to later higher-level prediction error activity. Voluntary attention was manipulated along two dimensions: (1) toward the visual or auditory modality, and (2) toward the moment of stimulus presentation or sustained over time. Early sensory prediction error, reflected by the omission N1, was unaffected by any manipulation of attention. In contrast, later high-level prediction error processing, reflected by omission P3 responses, was strongly affected by directing attention: robust responses were elicited when attention was directed to the auditory modality-where the prediction had been violated-but these were markedly reduced or absent when attention was directed to the visual modality. These results suggest an attentional system that does not affect low-level sensory prediction error but is capable of influencing distinct stages in the processing hierarchy in service of task performance. This first investigation of how attention affects different stages of omission activity suggests that voluntary attention may modulate prediction error processing via specific neurotransmitter systems and demonstrates this approach's potential for reliably studying precision-weighting in the brain.

The presence of another human in our immediate or distant environment is a crucial factor for survival and social behavior. Our actions vary depending on the other person's relative proximity, involving integration of brain functions subserved by neural oscillations, to generate the most appropriate behavior. This study highlights the alpha EEG role and its neural generators in interpersonal space sensitivity (IPSS). To investigate this, participants stood face-to-face, gazing into each other's eyes, while wearing a mask preventing them from staring at each other. An auditory order instructed them to alternately close and open their eyes. It was observed that alpha power of the occipito-parietal region increased as the distance decreased, transitioning from extrapersonal space (4 m) to peripersonal space (< 40 cm) when the eyes were closed. Conversely, alpha power decreased for the same distances when the eyes were open. These significant event-related synchronization (ERS) and desynchronization (ERD) effects were absent when participants' heads were masked. Beta oscillations were not significantly involved in IPSS. The number of spontaneous blinks was not significantly influenced by IPSS. Source modeling identified alpha ERS and ERD in cortical and subcortical generators significantly involved in IPSS with independent components localized in: the parietal cortex bilaterally (BA7), the left limbic gyrus (BA23), the anterior cingulate cortex (ACC) (right BA31, left and right BA24, right BA32), the right premotor cortex (BA6, including the supplementary motor area [SMA]), and the right frontal eye fields (BA8). These cortical areas were accompanied by the recruitment of the thalamus and cerebellum.