Search Results (617)

This paper describes the use of an electric drive as an acoustic actuator for active noise cancellation (ANC). In the presented application, the idea is to improve the noise, vibration, harshness (NVH) characteristics of passenger cars without using additional active or passive damper systems. Many of the already existing electric drives in cars are equipped with the required hardware components to generate noise and vibration, which can be used as compensation signals in an ANC application. To demonstrate the applicability of the idea, the electrical power steering (EPS) motor is stimulated with a control signal, generated by an adaptive feedforward controller, to reduce harmonic disturbances at the driver’s ears. As it turns out, the EPS system generates higher harmonics of the harmonic compensation signal due to nonlinearities in the acoustic transfer path using a harmonic excitation signal. The higher harmonics impair an improvement in the subjective hearing experience, although the airborne noise level of the harmonic disturbance signal can be clearly reduced at the driver’s ears. Therefore, two methods are presented to reduce the amplitude of the higher harmonics. The first method is to limit the filter weights of the algorithm to reduce the amplitude of the harmonic compensation signal. The filter amplitude limitation also leads to a lower amplitude of the higher harmonics, generated by the permanent magnet synchronous machine (PMSM). The second method uses a parallel structure of adaptive filters to actively reduce the amplitude of the higher harmonics. Finally, the effectiveness of the proposed ANC system is demonstrated in two real driving situations, where in one case a synthetic noise/vibration induced by a shaker on the front axle carrier is considered to be the disturbance, and in the other case, the disturbance is a harmonic vibration generated by the combustion engine. In both cases, the subjective hearing experience of the driver could be clearly improved using the EPS motor as ANC actuator. Full article

The conversion from mild cognitive impairment (MCI) to dementia is influenced by several factors, including comorbid conditions such as metabolic and vascular diseases. Understanding the impact of these comorbidities can help in the disease management of patients with a higher risk of progressing to dementia, improving outcomes. In the current study, we aimed to analyze data from a large cohort of MCI (n = 188) by principal component analysis (PCA) and cluster analysis (CA) to classify patients into distinct groups based on their comorbidity profile and to predict the risk of conversion to dementia. From our analysis, four clusters emerged. CA showed a significantly higher rate of disease progression for Cluster 1, which was predominantly characterized by extremely high obesity and diabetes compared to other clusters. In contrast, Cluster 3, which was defined by a lower prevalence of all comorbidities, had a lower conversion rate. Cluster 2, mainly including subjects with traumatic brain injuries, showed the lowest rate of conversion. Lastly, Cluster 4, including a high load of hearing loss and depression, showed an intermediate risk of conversion. This study underscores the significant impact of specific comorbidity profiles on the progression from MCI to dementia, highlighting the need for targeted interventions and management strategies for individuals with these comorbidity profiles to potentially delay or prevent the onset of dementia. Full article

Biallelic variants in USH2A are associated with retinitis pigmentosa (RP) and Type 2 Usher Syndrome (USH2), leading to impaired vision and, additionally, hearing loss in the latter. Although the introduction of next-generation sequencing into clinical diagnostics has led to a significant uplift in molecular diagnostic rates, many patients remain molecularly unsolved. It is thought that non-coding variants or variants of uncertain significance contribute significantly to this diagnostic gap. This study aims to demonstrate the clinical utility of the reverse transcription–polymerase chain reaction (RT-PCR)–Oxford Nanopore Technology (ONT) sequencing of USH2A mRNA transcripts from nasal epithelial cells to determine the splice-altering effect of candidate variants. Five affected individuals with USH2 or non-syndromic RP who had undergone whole genome sequencing were recruited for further investigation. All individuals had uncertain genotypes in USH2A, including deep intronic rare variants, c.8682-654C>G, c.9055+389G>A, and c.9959-2971C>T; a synonymous variant of uncertain significance, c.2139C>T; p.(Gly713=); and a predicted loss of function duplication spanning an intron/exon boundary, c.3812-3_3837dup p.(Met1280Ter). In silico assessment using SpliceAI provided splice-altering predictions for all candidate variants which were investigated using ONT sequencing. All predictions were found to be accurate; however, in the case of c.3812-3_3837dup, the outcome was a complex cryptic splicing pattern with predominant in-frame exon 18 skipping and a low level of exon 18 inclusion leading to the predicted stop gain. This study detected and functionally characterised simple and complex mis-splicing patterns in USH2A arising from previously unknown deep intronic variants and previously reported variants of uncertain significance, confirming the pathogenicity of the variants. Full article

The Eustachian tube (ET) is a bottleneck when it comes to middle ear (ME) health. If its function is impaired, this can lead to serious consequences for the patient, such as hearing problems or deafness. Therefore, this study investigated a tapered nitinol stent (3–5 mm × 14 mm) for the human ET as a potential new permanent treatment for chronic Eustachian tube dysfunction (ETD) and thus ME ventilation disorders. The self-expanding stent was inserted unilaterally into the ET of 24 sheep with observation periods of 3, 6, and 12 months. Local tissue effects and the safety of the stent insertion were analyzed based on regular endoscopic checks, weekly tympanometry measurements, final imaging, and histological examinations. The animals showed no stent-related health restrictions. However, the individual anatomy and stenting procedure had an influence on the results. The tissue reaction in the endoscopic examinations was mild even though no concomitant antibiotics were administered. After all three monitoring periods, stented ETs had a significantly larger ET lumen than the non-stented contralateral ETs. However, tissue growth was detected in the stent. Overall, the first long-term study on an ET stent showed that the tapered ET stent could be a promising treatment option for ETD. Full article

Background: The aim of this study was to evaluate the performance of three disposable hearing aid battery brands available in Wales. Hearing-impaired individuals who utilise hearing aids rely on the functionality of their devices, which is often contingent upon the quality and longevity of disposable batteries. Materials and Methods: A grey literature review foregrounded the battery standards. The “real-life” use of batteries was supplemented through laboratory testing. Parameters relating to performance quality were used to quantify an overall service life of five PR44- and four PR48-size batteries per manufacturer. Results: The literature review signalled a large gap in hearing aid battery consumption research. All battery brands underperformed compared to their specifications but met IEC standards. Conclusions: Revisions to battery consumption test conditions should reflect new technological features and refine expectations of real-life use. It was possible to statistically identify the best performing hearing aid battery brand. Full article

Dysfunction of some mitochondrial aminoacyl-tRNA synthetases (encoded by the KARS1, HARS2, LARS2 and NARS2 genes) results in a great variety of phenotypes ranging from non-syndromic hearing impairment (NSHI) to very complex syndromes, with a predominance of neurological signs. The diversity of roles that are played by these moonlighting enzymes and the fact that most pathogenic variants are missense and affect different domains of these proteins in diverse compound heterozygous combinations make it difficult to establish genotype–phenotype correlations. We used a targeted gene-sequencing panel to investigate the presence of pathogenic variants in those four genes in cohorts of 175 Spanish and 18 Colombian familial cases with non-DFNB1 autosomal recessive NSHI. Disease-associated variants were found in five cases. Five mutations were novel as follows: c.766C>T in KARS1, c.475C>T, c.728A>C and c.1012G>A in HARS2, and c.795A>G in LARS2. We provide audiograms from patients at different ages to document the evolution of the hearing loss, which is mostly prelingual and progresses from moderate/severe to profound, the middle frequencies being more severely affected. No additional clinical sign was observed in any affected subject. Our results confirm the involvement of KARS1 in DFNB89 NSHI, for which until now there was limited evidence. Full article

Promoting appropriate behaviors in early childhood is crucial for children’s future development. This systematic review aimed to explore the efficacy of social story (SS) intervention in teaching expected behaviors among preschool children. A structured search strategy was applied to five online electronic databases. The references were systematically screened in accordance with the PRISMA statements. Randomized or non-randomized controlled studies, as well as single-subject studies, in which SSs served as a behavioral training approach for children aged 2 to 6 years were included. Information related to study design, characteristics of the participants, target behaviors, and implementation of SS intervention was extracted. A meta-analysis was performed using the random-effects model, where similar outcomes were evaluated by similar intervention across multiple studies. Twenty-one studies were identified for qualitative analysis, while two studies formed the basis of the meta-analysis. SS interventions were employed to teach a variety of behaviors among typically developing children as well as those with various disabilities, such as autism, developmental delay, hearing impairments, attention deficit hyperactivity disorder, or other disabilities. The target behaviors included oral health practices, peer interaction, staying on-task, self-regulation, sleep habits, and controlling aggressive behavior during group activities. The SSs were used either alone or combined with other strategies, such as positive reinforcement, music therapy, role play, group discussion, video self-modeling, immediate practices, or additional audio commentary. Most studies reported improvements in appropriate behaviors and/or reductions in unfavorable behaviors. The meta-analysis indicated that children practiced more toothbrushing steps when using SS interventions compared to conventional oral health instruction (Z = 3.60, MD = 0.66, 95%CI 0.30 to 1.02, p < 0.001). SS interventions have the potential to teach target behaviors, particularly toothbrushing behaviors, among preschool children. More well-designed randomized controlled trials are warranted to determine the efficacy of SS interventions among children with various developmental profiles. Full article

The aim of this study is to develop a practical software solution for real-time recognition of sign language words using two arms. This will facilitate communication between hearing-impaired individuals and those who can hear. We are aware of several sign language recognition systems developed using different technologies, including cameras, armbands, and gloves. However, the system we propose in this study stands out for its practicality, utilizing surface electromyography (muscle activity) and inertial measurement unit (motion dynamics) data from both arms. We address the drawbacks of other methods, such as high costs, low accuracy due to ambient light and obstacles, and complex hardware requirements, which have limited their practical application. Our software can run on different operating systems using digital signal processing and machine learning methods specific to this study. For the test, we created a dataset of 80 words based on their frequency of use in daily life and performed a thorough feature extraction process. We tested the recognition performance using various classifiers and parameters and compared the results. The random forest algorithm emerged as the most successful, achieving a remarkable 99.875% accuracy, while the naïve Bayes algorithm had the lowest success rate with 87.625% accuracy. The new system promises to significantly improve communication for people with hearing disabilities and ensures seamless integration into daily life without compromising user comfort or lifestyle quality. Full article

Hearing loss is a prevalent condition affecting millions of people worldwide. Hearing loss has been linked to oxidative stress as a major factor in its onset and progression. The goal of this thorough analysis is to investigate the connection between oxidative stress and hearing loss, with an emphasis on the underlying mechanisms and possible treatments. The review addressed the many forms of hearing loss, the role of reactive oxygen species (ROS) in causing damage to the cochlea, and the auditory system’s antioxidant defensive mechanisms. The review also goes over the available data that support the use of antioxidants and other methods to lessen hearing loss brought on by oxidative stress. We found that oxidative stress is implicated in multiple types of hearing loss, including age-related, noise-induced, and ototoxic hearing impairment. The cochlea’s unique anatomical and physiological characteristics, such as high metabolic activity and limited blood supply, make it particularly susceptible to oxidative damage. Antioxidant therapies have shown promising results in both animal models and clinical studies for preventing and mitigating hearing loss. Emerging therapeutic approaches, including targeted drug delivery systems and gene therapy, offer new possibilities for addressing oxidative stress in the auditory system. The significance of this review lies in its comprehensive analysis of the intricate relationship between oxidative stress and hearing loss. By synthesizing current knowledge and identifying gaps in understanding, this review provides valuable insights for both researchers and clinicians. It highlights the potential of antioxidant-based interventions and emphasizes the need for further research into personalized treatment strategies. Our findings on oxidative stress mechanisms may also affect clinical practice and future research directions. This review serves as a foundation for developing novel therapeutic approaches and may inform evidence-based strategies for the prevention and treatment of hearing loss, ultimately contributing to improved quality of life for millions affected by this condition worldwide. Full article

The available research suggests that the rate of involvement of people with disabilities and their families in the life of the Church is significantly lower than that of people without disabilities. The engagement of people with disabilities is largely dependent on (a) the level of religiosity; (b) intrinsic motivation; (c) the level of trust in the institutions of the Church; and (d) broadly understood accessibility factors. Barriers experienced by people with disabilities are complex in nature, and make these people dependent on the help of others. Overcoming them requires significant investment, commitment, and change in the Church institution. These issues are relatively rarely addressed in the literature. The few, usually partial studies tend to concentrate on specific disabilities, discussed with no reference to the family context. However, it is usually the case that the religiosity and church activity of a person with a disability are firmly rooted in their family reality, shaped by the level of religiosity of their parents, and sometimes dependent on their presence and involvement. The aim of this article, which is both theoretical and empirical in nature, is to answer the question of which barriers form an obstacle to participation in religious life for people with disabilities and their families in Poland. This study uses the results of a nationwide qualitative research (focus group interview method) conducted among people with physical and intellectual disabilities, the hard-of-hearing and the deaf, the visually impaired, and their carers. Data analysis enabled the identification of the following barriers: infrastructural, personal and organizational (family-related and extra-familial). These research results can provide guidance in pastoral work with people with disabilities and their families, improving not only the quality of their religious experience, but also the number of the faithful in the Church community. Full article

Subjective cognitive complaints (SCCs) are a crucial modifiable risk factor for dementia. There is increasing interest in the association between SCC and sleep disturbance; however, the effects of sleep disturbance on SCC development among community-dwelling elderly individuals in Japan remain unclear. We aimed to cross-sectionally investigate the association between SCC and sleep disturbance, with adjustment for multiple factors related to cognitive decline, among 241 community-dwelling elderly persons without cognitive impairment. The measures were SCCs (Kihon Checklist-Cognitive Function, KCL-CF), sleep disturbance (Japanese version of the Athens Insomnia Scale, AIS-J), general cognitive function (Mini-Mental State Examination), and depressive symptoms (five-item version of the Geriatric Depression Scale [GDS-5]). The following data were collected: sex, age, educational history, whether the participants had visited a medical institution for diseases (hypertension, diabetes, hyperlipidemia, heart disease), and the presence/absence of established risk factors (hearing loss, history of head injury, drinking habits, smoking habits, social isolation, and physical inactivity and activity). Based on the KCL-CF, 96 and 145 participants were considered to have and lack SCCs, respectively. On logistic regression analysis, the AIS-J score and smoking history were significantly associated with SCCs. Our findings suggest that sleep disturbance is associated with SCC development among community-dwelling elderly people in Japan. Evaluating and managing sleep disturbances can be important in preventing SCCs and dementia. Full article

Sign language recognition technology can help people with hearing impairments to communicate with non-hearing-impaired people. At present, with the rapid development of society, deep learning also provides certain technical support for sign language recognition work. In sign language recognition tasks, traditional convolutional neural networks used to extract spatio-temporal features from sign language videos suffer from insufficient feature extraction, resulting in low recognition rates. Nevertheless, a large number of video-based sign language datasets require a significant amount of computing resources for training while ensuring the generalization of the network, which poses a challenge for recognition. In this paper, we present a video-based sign language recognition method based on Residual Network (ResNet) and Long Short-Term Memory (LSTM). As the number of network layers increases, the ResNet network can effectively solve the granularity explosion problem and obtain better time series features. We use the ResNet convolutional network as the backbone model. LSTM utilizes the concept of gates to control unit states and update the output feature values of sequences. ResNet extracts the sign language features. Then, the learned feature space is used as the input of the LSTM network to obtain long sequence features. It can effectively extract the spatio-temporal features in sign language videos and improve the recognition rate of sign language actions. An extensive experimental evaluation demonstrates the effectiveness and superior performance of the proposed method, with an accuracy of 85.26%, F1-score of 84.98%, and precision of 87.77% on Argentine Sign Language (LSA64). Full article

Our study aimed to investigate the role of ferroptosis in sevoflurane-induced hearing impairment and explore the mechanism of the microRNA-182-5p (miR-182-5p)/Glutathione Peroxidase 4 (GPX4) pathway in sevoflurane-induced ototoxicity. Immunofluorescence staining was performed using myosin 7a and CtBP2. Cell viability was assessed using the CCK-8 kit. Fe²⁺ concentration was measured using FerroOrange and Mi-to-FerroGreen fluorescent probes. The lipid peroxide level was assessed using BODIPY 581/591 C11 and MitoSOX fluorescent probes. The auditory brainstem response (ABR) test was conducted to evaluate the hearing status. Bioinformatics tools and dual luciferase gene reporter analysis were used to confirm the direct targeting of miR-182-5p on GPX4 mRNA. GPX4 and miR-182-5p expression in cells was assessed by qRT-PCR and Western blot. Ferrostatin-1 (Fer-1) pretreatment significantly improved hearing impairment and damage to ribbon synapses in mice caused by sevoflurane exposure. Immunofluorescence staining revealed that Fer-1 pretreatment reduced intracellular and mitochondrial iron overload, as well as lipid peroxide accumulation. Our findings indicated that miR-182-5p was upregulated in sevoflurane-exposed HEI-OC1 cells, and miR-182-5p regulated GPX4 expression by binding to the 3′UTR of GPX4 mRNA. The inhibition of miR-182-5p attenuated sevoflurane-induced iron overload and lipid peroxide accumulation. Our study elucidated that the miR-182-5p/GPX4 pathway was implicated in sevoflurane-induced ototoxicity by promoting ferroptosis. Full article

The 5xFAD transgenic mouse model widely used in Alzheimer’s disease (AD) research recapitulates many AD-related phenotypes with a relatively early onset and aggressive age-dependent progression. Besides developing amyloid peptide deposits alongside neuroinflammation by the age of 2 months, as well as exhibiting neuronal decline by the age of 4 months that intensifies by the age of 9 months, these mice manifest a broad spectrum of behavioural impairments. In this review, we present the extensive repertoire of behavioural dysfunctions in 5xFAD mice, organised into four categories: motor skills, sensory function, learning and memory abilities, and neuropsychiatric-like symptoms. The motor problems, associated with agility and reflex movements, as well as balance and coordination, and skeletal muscle function, typically arise by the time mice reach 9 months of age. The sensory function (such as taste, smell, hearing, and vision) starts to deteriorate when amyloid peptide buildups and neuroinflammation spread into related anatomical structures. The cognitive functions, encompassing learning and memory abilities, such as visual recognition, associative, spatial working, reference learning, and memory show signs of decline from 4 to 6 months of age. Concerning neuropsychiatric-like symptoms, comprising apathy, anxiety and depression, and the willingness for exploratory behaviour, it is believed that motivational changes emerge by approximately 6 months of age. Unfortunately, numerous studies from different laboratories are often contradictory on the conclusions drawn and the identification of onset age, making preclinical studies in rodent models not easily translatable to humans. This variability is likely due to a range of factors associated with animals themselves, housing and husbandry conditions, and experimental settings. In the forthcoming studies, greater clarity in experimental details when conducting behavioural testing in 5xFAD transgenic mice could minimise the inconsistencies and could ensure the reliability and the reproducibility of the results. Full article

Background: Accurate prognostic prediction is crucial for managing Idiopathic Sudden Sensorineural Hearing Loss (ISSHL). Previous studies developing ISSHL prognosis models often overlooked individual variability in hearing damage by relying on fixed frequency domains. This study aims to develop models predicting ISSHL prognosis one month after treatment, focusing on patient-specific hearing impairments. Methods: Patient-Personalized Seigel’s Criteria (PPSC) were developed considering patient-specific hearing impairment related to ISSHL criteria. We performed a statistical test to assess the shift in the recovery assessment when applying PPSC. The utilized dataset of 581 patients comprised demographic information, health records, laboratory testing, onset and treatment, and hearing levels. To reduce the model’s reliance on hearing level features, we used only the averages of hearing levels of the impaired frequencies. Then, model development, evaluation, and interpretation proceeded. Results: The chi-square test (p-value: 0.106) indicated that the shift in recovery assessment is not statistically significant. The soft-voting ensemble model was most effective, achieving an Area Under the Receiver Operating Characteristic Curve (AUROC) of 0.864 (95% CI: 0.801–0.927), with model interpretation based on the SHapley Additive exPlanations value. Conclusions: With PPSC, providing a hearing assessment comparable to traditional Seigel’s criteria, the developed models successfully predicted ISSHL recovery one month post-treatment by considering patient-specific impairments. Full article