Search Results (195)

Background: Genetic testing is increasingly part of the diagnostic pathway of congenital hearing loss (CHL), clarifying etiology and supporting clinical management. However, its psychosocial impact, especially differences between syndromic and non-syndromic conditions, remains underexplored. Objectives: This study evaluated the differential psychological impact of genetic diagnosis in syndromic versus non-syndromic pediatric patients, its relationship with clinical and rehabilitative variables, and the role of post-diagnostic psychological assessment. Methods: A cross-sectional post-diagnosis survey was conducted in families of children with genetically confirmed syndromic (Usher syndrome, n = 21) and non-syndromic (GJB2-related, n = 21) CHL; a total of 37 families responded. Parental empowerment was assessed using an Italian translated version of the Genetic Counseling Outcome Scale (GCOS-24). In an exploratory analysis, GCOS-24 items were grouped into three author-derived domains (understanding/awareness, emotional experience, and informational support) based on semantic content, not validated psychometrically. Results: No significant differences in GCOS-24 scores emerged between groups, nor in relation to clinical variables like hearing loss severity, auditory outcomes, or rehabilitative interventions. Genetic diagnosis occurred later in the syndromic group. Qualitative observations suggested parental empowerment varied with timing of diagnosis, clarity of information, and therapeutic alliance quality. Conclusions: Overall, these results highlight the importance of integrating psychological support and structured communication into clinical pathways to support families and patients in understanding and adapting to the diagnosis over time. Further longitudinal studies are needed to clarify the evolving psychosocial impact of genetic diagnosis in CHL. Full article

Structural inefficiencies and labor shortages within the global tomato agrifood chain pose significant threats to its economic sustainability. While vision-dominated systems, encompassing structural and spectral dimensions, have pioneered intelligent management, their limitations in environmental robustness and computational overhead necessitate a new approach. Furthermore, dimensional incompleteness remains a challenge in decoding internal states. Multisensory perception, integrating physical (tactile and auditory) and chemical (olfactory and gustatory) modalities, enables the quantitative characterization of tomato physiological states. Based on technological advancements at the leading edge of knowledge, a critical perspective on the mushrooming field of multisensory perception is highlighted. Grounded in the capabilities and bottlenecks of visual perception, the discussion outlines significant progress in multisensory perception, along with its challenges and prospects. Crucially, it delineates the construction pathway of digital fingerprints that couple instrumental sensing signals with human sensory experiences and envisions the landscape of multimodal fusion to address practical challenges. This perspective provides a roadmap for sensorially transparent evaluation systems in the tomato agrifood chain. Full article

Background/Objectives: Age-related hearing loss is one of the most prevalent chronic conditions in older adults and has emerged as a potentially modifiable risk factor for cognitive decline and dementia. Increasing evidence from epidemiological, neurobiological, and interventional studies has improved our understanding of the complex relationships between auditory dysfunction and cognitive aging. By highlighting findings in these areas, this review aims to aid clinicians and researchers gain a better understanding of the association between hearing loss, cognitive decline and dementia, and the importance of considering sensory decline during cognitive screening. Methods: This narrative review summarizes and integrates findings from epidemiological, neurobiological, and clinical studies examining relationships between hearing loss, cognitive decline, and dementia. Particular focus was placed on epidemiological associations, proposed mechanistic pathways, implications for screening and diagnostic assessment, and evidence regarding hearing rehabilitation interventions. Results: Accumulating evidence indicates that hearing loss is associated with accelerated cognitive decline and increased dementia risk. Proposed mechanisms include increased cognitive load, reduced sensory input, social isolation, depression, and shared neurodegenerative or vascular pathology, although causal pathways remain incompletely understood. Emerging evidence suggests that hearing rehabilitation may help preserve cognitive function in some groups, but findings remain heterogeneous. Clinical studies further support the importance of considering auditory function during cognitive assessment, as unrecognized hearing impairment may influence test performance, communication, and diagnostic accuracy. Conclusions: Current evidence supports hearing loss as an important factor in cognitive aging and dementia research and highlights the potential value of integrating hearing assessment and management into clinical and research settings. Full article

Background: The click-evoked Auditory Brainstem Response (ABR) is the gold standard electrophysiological tool for assessing auditory pathway integrity in infants and young children. As normative data are inherently equipment-specific, the absence of pediatric reference values for the NeuroAudio system (Neurosoft, Ivanovo, Russia) represents a significant gap in clinical practice, given that existing normative datasets for this system are restricted to adult populations. Objective: To establish normative data for click ABR recorded with the NeuroAudio system in children under three years of age, stratified by age group according to auditory maturation patterns. Methods: A prospective, cross-sectional study was conducted at the Electrophysiology Laboratory of the Department of Speech Therapy, Paulista School of Medicine, Federal University of São Paulo (UNIFESP/EPM), under the approval of the Research Ethics Committee (protocol 7.939.564). A total of 203 children (121 males, 82 females; age range: 2 weeks to 36 months) with confirmed normal peripheral auditory function were included. Click stimuli (0.1 ms, rarefaction polarity) were delivered monaurally via ER-3A insert earphones at 80 dB nHL and a repetition rate of 19.3/s. Two average runs of 2000 artifact-free sweeps were recorded per ear. Absolute latencies of waves I, III, and V, interpeak intervals I–III, III–V, and I–V, and amplitudes of waves I and V were analyzed. Results: Statistical modeling supported the consolidation of 12 initial age bins into three clinically and statistically validated categories: 0–3, 4–12, and 13–36 months. Wave I latency remained stable across age groups, whereas waves III and V and all interpeak intervals showed progressive shortening with increasing age. Wave V amplitude increased progressively with age, while wave I amplitude remained unchanged. Females presented shorter latencies than males for waves III and V and for all interpeak intervals. The right ear exhibited a shorter III–V interpeak interval than the left ear, with a significant ear × age interaction indicating that this asymmetry is modulated during early maturation. Age, sex, and ear-stratified normative values (two SD and three SD reference limits) are reported. Conclusion: This study provides the first pediatric normative dataset for click-evoked ABR acquired with the NeuroAudio system in children under three years of age. The proposed three age stratifications, together with sex- and ear-specific reference values for the III–V interpeak interval, offer a clinically actionable framework for the accurate interpretation of pediatric ABR recordings and for the early identification of auditory pathway abnormalities. Full article

This study examines how auditory contexts, or soundscapes, shape chocolate taste perception, affective response, hedonic liking, and the extent to which emotion mediates these effects. Using a within-subjects design with 120 participants aged 18–25 years, four auditory conditions were compared: silence, natural soundscape, relatively low-pitched soundscape, and relatively high-pitched soundscape. Participants evaluated perceived bitterness, sweetness, acidity, emotional valence, arousal, and overall liking after tasting the same 65% dark chocolate under each auditory condition. The results showed that auditory context significantly modulated taste perception, affective response, and liking. The natural soundscape produced the most favorable profile, increasing liking and emotional valence while reducing arousal. In contrast, the relatively high-pitched condition increased arousal and enhanced perceived acidity (Δ ≈ 6.77 VAS points). Effect sizes indicated stronger effects on arousal (partial η² ≈ 0.46), liking (partial η² ≈ 0.29), acidity (partial η² ≈ 0.28), and valence (partial η² ≈ 0.26) than on sweetness perception (partial η² ≈ 0.05). Mediation analysis showed that emotional valence partially explained the relationship between the natural soundscape and liking, whereas arousal did not play a significant mediating role. These findings suggest that auditory environments influence chocolate evaluation through both affective and crossmodal pathways. Overall, the study provides controlled evidence that sound can function as a relevant contextual variable in multisensory chocolate-tasting experiences, with implications for sensory evaluation, gastronomy, and experience design. Full article

Background/Objectives: Hearing loss and dementia are highly prevalent conditions in older adults and represent a growing public health challenge. Over the past decade, a substantial body of epidemiological evidence has demonstrated a consistent association between age-related hearing loss and cognitive dysfunction, including incident dementia. However, the nature of this relationship remains incompletely understood. Methods: This narrative review provides a structured overview of current evidence, focusing on epidemiological findings, mechanistic pathways, and clinical implications. Hearing loss has been associated with both accelerated cognitive decline and increased dementia risk, with a clear severity–impact relationship. Results: Several interacting mechanisms have been proposed, including increased cognitive load, structural and functional brain changes, social isolation, and shared vascular and metabolic risk factors. Emerging concepts such as the “auditory brain” and central auditory dysfunction further suggest that hearing impairment may also represent an early manifestation of neurodegenerative processes. Intervention studies have yielded mixed results. While hearing rehabilitation improves communication and quality of life, randomized evidence has not consistently demonstrated a reduction in cognitive decline in the general population, but potential benefits may exist in higher-risk subgroups. Increasing attention has been directed toward the role of neuroplasticity, with evidence suggesting that delayed intervention may limit the effectiveness of rehabilitation due to long-standing auditory deprivation. Conclusions: Taken together, current evidence suggests that hearing loss may represent both a potentially modifiable risk factor and an early marker of cognitive decline. Early identification and timely management of hearing impairment may therefore play an important role in maintaining cognitive and brain health and improving quality of life in older adults. Full article

Background: Hearing loss is a widespread sensory disorder affecting over 1.5 billion people worldwide, with the number projected to exceed 700 million by 2050. It imposes social and economic burdens across all ages and regions. Approximately half of adult cases are preventable, but the underlying causes are complex, with 75–80% due to autosomal recessive genetic factors and key roles for mutations in genes such as GJB2. Advances in sequencing technologies have accelerated gene discovery, but challenges remain in interpreting variants. Epigenetic mechanisms such as DNA methylation and histone modifications are increasingly recognized as crucial in auditory biology and could offer new biomarkers and therapeutic targets. Integrating epidemiological, genetic, and epigenomic data is essential to developing targeted prevention and treatment strategies to reduce the global burden of hearing loss. Methods: This narrative review examines recent genomic and epigenomic advances in hearing loss, with particular emphasis on molecular mechanisms, emerging diagnostic applications, and translational therapeutic opportunities. A comprehensive review of current epidemiological data, genetic studies, and epigenomic research was conducted using the peer-reviewed literature from international databases. Key areas of interest include inheritance patterns, molecular pathways, and recent advances in omics technologies. Results: Epigenetic mechanisms, including DNA methylation and histone modifications, are increasingly recognized as important regulators of cochlear development and hair cell survival, although much of the current evidence remains preclinical. Studies suggest that peripheral epigenetic signatures may serve as biomarkers for early diagnosis and risk stratification. Conclusions: Integrating established screening pathways with epidemiological trends and molecular knowledge offers a promising path toward precision medicine in hearing care. Connecting these domains is essential to developing equitable and effective interventions and addressing persistent global disparities in hearing health. This review highlights the evolving landscape of auditory genetics and epigenetics and outlines future directions for translational research and personalized therapy. Full article

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. Full article

Parkinson’s disease (PD) is the second most prevalent neurodegenerative disorder worldwide, and its early diagnosis remains a major challenge due to reliance on subjective clinical assessments. This study proposes a bio-inspired computational framework for automatic PD detection that draws explicit architectural inspiration from two biological systems: the hierarchical tonotopic organization of the human auditory cortex, which motivates the design of a 1D Convolutional Neural Network (CNN) for vocal biomarker analysis, and the basal ganglia–cerebellar motor control circuit, which motivates the selection and design of features extracted from Archimedean spiral drawing tasks. Unlike previous studies that apply standard machine learning techniques without grounding architectural choices in biological mechanisms, the proposed framework establishes a direct mapping between neural processing pathways and model design decisions. A Support Vector Machine (SVM) classifier evaluated on the Kaggle vocal dataset achieved 87% test accuracy with no overfitting, outperforming AdaBoost, Random Forest, KNN, XGBoost, and Decision Trees in terms of generalization. The 1D CNN applied to UCI spiral drawing data achieved 85% test accuracy, with overfitting behavior addressed through architectural regularization strategies including early stopping. A conceptual multimodal fusion architecture integrating both modalities is proposed as a direction for future experimental validation; it was not implemented or experimentally validated within the present study. The primary novelty of the framework resides in this explicit biomimetic grounding, which distinguishes it from existing performance-driven approaches. Results confirm that biologically grounded computational models constitute promising objective decision-support tools for early PD diagnosis. Full article

This is a narrative review that explores the development of non-implantable vestibular devices designed to address postural instability, particularly in aging populations and patients with vestibular hypofunction. It establishes that balance relies on complex sensory integration and that the functional decline of this system creates a significant medical need. Three principal technological strategies are examined: sensory substitution devices, galvanic vestibular stimulation (GVS), and immersive visual feedback systems. Sensory substitution devices, which convert balance data into auditory, tactile, or electrotactile cues, demonstrate significant promise. Examples like vibrotactile belts provide feedback that reduces postural sway, enhancing stability and patient confidence. Parallel to this, GVS—using electrical currents applied to the mastoids—emerges as a potent non-invasive method to modulate vestibular pathways, improving balance control and even inducing neuroplastic changes, especially with stochastic “noisy” signals. The most recently developed devices include augmented and virtual reality technologies that offer innovative visual feedback, creating enriched rehabilitation environments that accelerate recovery by promoting sensory reweighting and neural adaptation. This review concludes that while implantable prostheses are advancing, non-invasive devices offer versatile, affordable, and complementary solutions for balance restoration. The future success of non-invasive alternatives hinges on developing more sophisticated stimulation protocols that account for the complexity of natural movement and individual patient contexts, expanding therapeutic options for vestibular disorders. Full article

Virtual reality heritage experiences can be understood as multisensory interaction systems, yet how auditory, haptic, and gestural cues combine at the system level to shape electronic word-of-mouth (eWOM) intention remains insufficiently understood. Addressing this problem from a configurational systems perspective, this study applies fuzzy-set qualitative comparative analysis (fsQCA) to five auditable interaction cues (acoustic clarity, rhythmic drive, vibrotactile actuation level, gesture complexity, and compound gesture frequency) across a set of widely used VR cultural heritage applications. The results identify two sufficient system-level pathways to high eWOM intention: a rhythm-driven, low-burden pathway and a coordination-driven pathway characterized by clearer audio, stronger rhythmic structure, and tighter haptic and gestural action closure. Low eWOM intention is most consistently associated with weak cue interpretability, limited temporal drive, or unbalanced stimulation patterns, suggesting that isolated enhancement of single channels does not reliably translate into downstream sharing intentions. These findings reposition VR heritage design as a problem of configuring coherent multisensory interaction systems rather than maximizing individual stimuli. The study contributes a bounded, case-comparative account of how auditable cue bundles shape eWOM intention and offers system design guidance for resource-sensitive multisensory coordination in VR heritage applications. Full article

Background: Prolonged exposure to moderate and loud noise is known to impair hearing; however, the safety of long-duration exposure to low-level sound, such as that encountered during everyday conversation, remains unclear. This study aimed to determine the effect of continuous exposure to sound at a 65 dB sound pressure level (SPL) on auditory processing. Methods: Auditory brainstem responses (ABRs) were recorded in C57BL/6 mice before and after a 1 h exposure to a continuous pure tone at 65 dB SPL. Changes in ABR thresholds, wave amplitudes, and latencies were analyzed across frequencies and time points. Correlations between amplitude and latency changes across ABR waves were also assessed. Results: Tone exposure induced a significant, frequency-specific increase in ABR thresholds, with a mean elevation of approximately 6 dB and a maximum shift of 15 dB. Significant reductions in amplitudes and prolongations of latencies were observed in Waves I–III, while Wave V amplitude remained relatively stable. A strong negative correlation between amplitude reduction and latency increase was found in Wave I, which progressively weakened from Wave II to Wave V. These functional changes persisted for up to three hours following exposure before gradually returning to baseline. Conclusions: Prolonged exposure to low-level sound at intensities typical of conversational speech can transiently impair auditory function and alter early neural processing in the auditory pathway. These findings suggest that sound levels commonly considered safe may still pose a risk when exposure is sustained, with implications for understanding hidden hearing loss and improving early diagnostic approaches. Full article

Background: Neural stability, defined as trial-by-trial fluctuations in neural responses to the repetitive sensory input, is an indicator of neural processing stability. The auditory brainstem response (ABR) can provide an electrophysiological measure of neural stability. Findings on neural stability differences between autistic and neurotypical individuals are inconsistent, potentially due to methodological differences and sample heterogeneity. This study aimed to investigate the relationship between neural stability in the brainstem and autistic traits in a group of children with and without a diagnosis of autism. We examined whether the degree of neural stability differs based on the evoking stimulus and response component analyzed, and whether neural stability relates to parent-reported autistic traits, as measured by the Autism Spectrum Quotient (AQ) and social responsiveness scale-2 (SRS-2). Methods: In total, 41 participants had usable click ABRs and 34 had usable sABRs. Neural stability was quantified using Pearson correlation analyses between binaurally evoked subaverage ABR waveforms. Parent-reported measures of autistic traits were collected. Results: Neural stability differed across ABR components, with the click ABR being significantly more stable than sABR components. Decreased neural stability is significantly related to autistic traits measured by the AQ but not the SRS-2. There was no significant response component by AQ interaction. Conclusions: Neural stability in the auditory brainstem pathway is linked to individual differences in autistic traits measured by the AQ but not the SRS, implying that early sensory processing neural stability may be related to broader features of autistic traits rather than social communication alone. Full article

Background: Low-cost virtual reality exergames may help maintain and improve postural control in children with spastic hemiplegia cerebral palsy (CP). This study aimed to evaluate the comparative effectiveness of the same six-week, low-cost exergame programme delivered via telerehabilitation (TR) and face-to-face (FF) in CP children. Methods: In this randomised controlled trial, 15 CP patients completed 18 sessions over 6 weeks. The TR group received remotely delivered sessions, whereas the FF control group completed in-person sessions with a physiotherapist. Outcomes were assessed at baseline; weeks 2, 4, and 6; and follow-ups at weeks 8 and 10. Postural control (centre-of-pressure sway area; CoP_sway) was measured during eyes open (EO), eyes closed (EC), voluntary mediolateral sway to a 30 bpm auditory cue (EO/EC), and during exergames targeting mediolateral (ML-WS) and anteroposterior (AP-WS) weight-shifting. Timed Up and Go (TUG) and Modified Modified Ashworth Scale (MMAS) were also assessed. Results: At week 6, both TR and FF significantly reduced CoP_sway (TR: p = 0.001, EC; FF: p = 0.01, EO). TR also improved dynamic postural control (p < 0.05) and TUG scores (p = 0.03), with functional gains sustained until week 10. Between-group comparisons revealed that TR achieved significantly greater reductions in AP weight-shifting (SD_ML, p = 0.001; V_ML, p = 0.004) and TUG (p = 0.009) than FF, with these advantages persisting throughout follow-up as revealed by post hoc analysis. Conversely, only FF significantly reduced ankle muscle tone (MMAS, p = 0.05). TR demonstrated broader improvements in secondary CoP metrics and superior long-term retention of functional mobility gains. Conclusions: Both six-week exergame interventions led to improvements in postural control. This trial demonstrated that telerehabilitation is a viable, comparable alternative to face-to-face delivery. Long-term retention suggests both modalities are complementary, offering flexible solutions to enhance routine physiotherapy service pathways. These findings provide a basis for validating these models across larger clinical cohorts. Full article

Moth auditory systems, evolutionarily adapted and structurally diverse with ultrasonic sensitivity, underpin the development of acoustic-based pest management strategies. Here, based on hypotheses derived from previous findings, we tested whether and how audible sounds (music, bird chirp, noise; 0.25–1 kHz, 80/120 dB) affect the development, survival, behavior and fecundity, as well as the molecular responses, using both short-term and long-term exposure (three successive generations) experimental designs. Behavioral assays showed dose-specific responses: high-intensity (120 dB) bird chirp and noise suppressed larval and adult activity, while low-intensity (80 dB) counterparts promoted larval crawling. Long-term exposure revealed that bird chirp and noise significantly impaired fitness, reducing larval/pupal body weight, pupation/eclosion rates, and egg hatching rate, with 120 dB noise exerting the strongest effects; 80 dB music showed neutral or positive impacts. Transcriptomic analysis identified 71–235 differentially expressed genes (DEGs) across treatment groups, with bird chirp and noise inducing more downregulated DEGs related to metabolism, immunity, and development. Notably, all cuticle-related DEGs in the 80 dB noise group and 53.2% in the 120 dB noise group were upregulated, suggesting stress-induced cuticular remodeling. GO/KEGG enrichment indicated distinct patterns: 80 dB music, bird chirp and 120 dB noise groups only had downregulated DEGs enriched in certain terms/pathways, mainly associated with cellular components; the 80 dB noise group had upregulated DEGs enriched in sensory, cuticle, metabolism and longevity-related terms/pathways, and downregulated DEGs in metabolism and human disease-related terms/pathways. Analysis of the expression patterns of all the longevity pathway-related genes suggested that sound stress induces lifespan regulation in this insect. These findings clarify S. frugiperda’s multidimensional responses to audible sound, providing a foundation for sound-based pest management. Full article