Search Results (795)

Duropathies encompass a spectrum of disorders linked to spinal dural tears and cerebrospinal fluid (CSF) leaks, resulting in significant neurological manifestations. This review synthesizes the current literature on duropathies, focusing on their anatomical and pathophysiological aspects, including conditions such as superficial siderosis, spontaneous intracranial hypotension, and spinal cord herniation. The methodologies employed include comprehensive evaluations through neuroimaging techniques such as MRI and CT myelography, alongside clinical assessments of symptoms like ataxia, hearing loss, and cognitive impairment. Key findings highlight the prevalence of dural defects in patients with superficial siderosis and the association of persistent CSF leaks with various neurological impairments. The review emphasizes the need for a standardized diagnostic and therapeutic approach to enhance patient management and improve outcomes. By addressing the interrelated nature of these conditions, the study underscores the importance of early intervention to mitigate long-term neurological consequences. Overall, the findings advocate for further research to elucidate the mechanisms underlying duropathies and the development of effective treatment strategies, ultimately aiming to improve the quality of life for affected individuals. Full article

Background/Objectives: Mobile health (mHealth) tools, such as smartphone apps, support person-centred care for persons with hearing loss engaging in the hearing aid management process. Hearing-aid-connected apps are increasingly common in audiological care, making it important to evaluate their availability and quality for clinicians, developers, and end-users. This scoping review aimed to identify, summarize, and synthesize information on clinically available hearing-aid-connected apps and evaluate their quality. Methods: A search of the Apple App Store (Canada) was conducted in August 2024 to identify current hearing-aid-connected apps that support hearing aid management. Metadata and features were extracted, and app quality was assessed using the Mobile Application Rating Scale (MARS). Quality was assessed across four objective domains (engagement, functionality, aesthetics, and information) and one subjective domain. Results: Apps had varying levels of metadata detail, including updates, compatibility, and target populations. All apps included common hearing aid controls (e.g., volume adjustment, microphone directionality), while more specialized features (tinnitus management, health tracking, remote clinician support) varied. High-performing apps scored significantly higher in engagement, functionality, aesthetics, and subjective quality, and all apps scored low in information quality, particularly for evidence and credibility. Conclusions: Findings highlight the need for transparent and informative metadata reporting and patient-centred design to improve clinical awareness, usability, and uptake of hearing-aid-connected apps. Full article

Von Hippel–Lindau disease-associated endolymphatic sac tumors (VHL-associated ELSTs) present diagnostic challenges due to their rarity and nonspecific symptoms. This study describes clinical, pathological and genotypic features to guide treatment. We retrospectively analyzed seven patients with VHL-associated ELSTs. The mean age of otologic symptom [hearing loss (100%) and facial nerve paralysis (85.71%)] onset was 22.43 ± 8.68 years (range: 10–33). Surgical management included trans-labyrinthine and subtotal temporal bone resection approaches. Among three patients with severe preoperative facial nerve dysfunction, two underwent great auricular nerve grafting improved to House–Brackmann grade IV, while one receiving hypoglossal–facial nerve anastomosis reached grade V. Genetic testing identified pathogenic VHL gene missense mutations in three patients. Two female patients demonstrated disease progression during pregnancy. Literature analysis revealed exon-specific patterns: Exon 1 mutations correlated with cerebellar/spinal hemangioblastomas in female patients, while Exon 3 mutations were associated with multisystem tumors. These findings support that VHL-associated ELSTs manifest early with otologic symptoms and demonstrate exon-specific phenotypic patterns. Optimal management requires complete surgical resection, genetic diagnosis, and a multidisciplinary approach to address these complex tumors and achieve favorable outcomes. Full article

Deaf and hearing-impaired individuals rely on sign language, a visual communication system using hand shapes, facial expressions, and body gestures. Sign languages vary by region. For example, Arabic Sign Language (ArSL) is notably different from American Sign Language (ASL). This project focuses on creating an Arabic Sign Language Recognition (ArSLR) System tailored for healthcare, aiming to bridge communication gaps resulting from a lack of sign-proficient professionals and limited region-specific technological solutions. Our research addresses limitations in sign language recognition systems by introducing a novel framework centered on ResNet50ViT, a hybrid architecture that synergistically combines ResNet50’s robust local feature extraction with the global contextual modeling of Vision Transformers (ViT). We also explored a tailored Vision Transformer variant (SignViT) for Arabic Sign Language as a comparative model. Our main contribution is the ResNet50ViT model, which significantly outperforms existing approaches, specifically targeting the challenges of capturing sequential hand movements, which traditional CNN-based methods struggle with. We utilized an extensive dataset incorporating both static (36 signs) and dynamic (92 signs) medical signs. Through targeted preprocessing techniques and optimization strategies, we achieved significant performance improvements over conventional approaches. In our experiments, the proposed ResNet50-ViT achieved a remarkable 99.86% accuracy on the ArSL dataset, setting a new state-of-the-art, demonstrating the effectiveness of integrating ResNet50’s hierarchical local feature extraction with Vision Transformer’s global contextual modeling. For comparison, a fine-tuned Vision Transformer (SignViT) attained 98.03% accuracy, confirming the strength of transformer-based approaches but underscoring the clear performance gain enabled by our hybrid architecture. We expect that RAFID will help deaf patients communicate better with healthcare providers without needing human interpreters. Full article

Disabling hearing loss affects more than 5% of the global population, with numbers expected to double by 2050. The burden is especially high in low- and middle-income countries, where access to cochlear implant (CI) technology and the required follow-up care is limited. While CIs are a proven treatment for certain types of hearing loss, their adoption in these countries is hindered by high costs, the need for specialised rehabilitation, and the financial and time commitment required for long-term device maintenance. Although remote programming has improved accessibility to standard care, specialised interventions for complications remain restricted mainly to areas with clinical centres. Computational modelling offers a promising solution to this access-to-care dilemma. The models may be used to simulate complications, such as non-auditory stimulation (NAS), to investigate and plan personalised interventions, and ultimately predict device parameters, without requiring the recipient’s physical presence. Both phenomenological and biophysical models have already demonstrated useful application in CIs: the former streamlines clinical workflows and aims to establish consistency in device fitting, and the latter provides insights into patient-specific auditory biophysiology. Despite decades of research, clinical translation of biophysical models has been limited by data constraints, parameter uncertainty, and validation challenges. In this perspective piece, it is argued that biophysical models have now reached sufficient maturity to be integrated into routine CI care. Apart from the advantages that this approach will bring to the overall advancement of person-centred CI care, it is envisioned to improve accessibility, personalisation, and long-term outcomes for CI recipients in low- and middle-income countries. Full article

Background/Objectives: Cochlear implants (CIs) are a common treatment of severe-to-profound hearing loss and provide reasonable speech understanding, at least in quiet situations. However, their limited spectro-temporal resolution restricts sound quality, which is especially crucial for music appraisal. Many CI recipients wear a hearing aid (HA) on the non-implanted ear (bimodal users), which may enhance music perception by adding acoustic fine structure cues. Since it is unclear how the HA should be fitted in conjunction with the CI to achieve optimal benefit, this study aimed to systematically vary HA fitting parameters and assess their impact on music sound quality in bimodal users. Methods: Thirteen bimodal CI recipients participated in a listening experiment using a master hearing aid that allowed controlled manipulation of HA settings. Participants evaluated three music excerpts (pop with vocals, pop without vocals, classical) using the multiple-stimulus with hidden reference and anchor (MUSHRA) test. To assess the reliability of individual judgments, each participant repeated the test, and responses were analyzed with the eGauge method. Results: Most participants provided reliable and consistent sound quality ratings. Compared to a standard DSL v5.0 prescriptive fitting, modifications in compression settings and low-frequency gain significantly influenced perceived music quality. The effect of low-frequency gain adjustments was especially pronounced for pop music with vocals, indicating stimulus-dependent benefits. Conclusions: The study demonstrates that HA fitting for bimodal CI users can be optimized beyond standard prescriptive rules to enhance music sound quality by increasing low-frequency gain, particularly for vocal-rich pieces. Additionally, the testing method shows promise for clinical application, enabling individualized HA adjustments based on patient-specific listening preferences, hence fostering personalized audiology care. Full article

Background/Objectives: Tinnitus is a complex auditory phenomenon with multifactorial origins, often involving both peripheral and central auditory pathways. Given the multifactorial nature of tinnitus, this review specifically focuses on the auditory brainstem as it represents the first central relay for auditory input and a key site of abnormal synchrony and central gain, which may generate or modulate tinnitus even when peripheral hearing appears normal. Several studies suggest a potential role of brainstem dysfunction in its pathogenesis, even among patients with normal hearing thresholds. Although the physiopathological data provide evidence for the role of brainstem in the generation and magnification of tinnitus, the diagnostic tools are still unclear. This systematic review aimed to investigate the diagnostic relevance of brainstem-level abnormalities in individuals with tinnitus. Methods: Following PRISMA guidelines, a literature search was conducted using PubMed, Scopus, and Web of Science from January 2000 to June 2025. Studies were included if they addressed the diagnostic relationship between tinnitus and brainstem involvement. Data on auditory brainstem response (ABR), otoacoustic emissions (used to differentiate peripheral from central auditory abnormalities), neuroimaging, and electrophysiological markers were extracted. Results: Twenty studies were included. Most used ABR as a diagnostic tool, revealing significant amplitude and latency alterations in tinnitus patients compared to controls, particularly in wave V and V/I amplitude ratios. Imaging studies supported altered brainstem–cortical connectivity and localized changes in inferior colliculus (IC) activity. Additional techniques, such as middle-latency evoked potentials and gap-in-noise detection, showed potential but lacked consistent clinical utility. Conclusions: Evidence suggests that brainstem dysfunction may contribute to tinnitus generation or persistence. ABR and advanced imaging represent specific diagnostic tools, though standardization and high-quality studies are still needed to improve clinical applicability. Full article

Background: Cochlear implants (CIs) are transformative neuroprosthetics that restore speech perception for individuals with severe-to-profound hearing loss. However, temporal envelope cues are well-represented within the signal processing, while spectral envelope cues are poorly accessed by CI users, resulting in substantial deficits compared to normal-hearing individuals. This profoundly impairs the perception of complex auditory stimuli like music and vocal prosody, significantly impacting users’ quality of life, social engagement, and artistic expression. Methods: This narrative review synthesizes research on CI signal-processing limitations, perceptual and production challenges in music and singing, the role of the auditory–motor feedback loop, and strategies for improvement, including rehabilitation, technology, and the influence of neuroplasticity and sensitive developmental periods. Results: The degraded signal causes marked deficits in pitch, timbre, and vocal emotion perception. Critically, this impoverished input functionally breaks the high-fidelity auditory–motor feedback loop essential for vocal control, transforming it from a precise fine-tuner into a gross error detector sensitive only to massive pitch shifts (~6 semitones). This neurophysiological breakdown directly causes pervasive pitch inaccuracies and melodic distortion in singing. Despite these challenges, improvements are possible through advanced sound-processing strategies, targeted auditory–motor training that leverages neuroplasticity, and capitalizing on sensitive periods for auditory development. Conclusions: The standard CI signal creates a fundamental neurophysiological barrier to singing. Overcoming this requires a paradigm shift toward holistic, patient-centered care that moves beyond speech-centric goals. Integrating personalized, music-based rehabilitation with advanced CI programming is essential for improving vocal production, fostering musical engagement, and ultimately enhancing the overall quality of life for CI users. Full article

In bone conduction (BC) hearing, sound is transmitted directly to the cochlea via skull vibrations, bypassing the outer and middle ear. This provides a therapeutic option for patients with conductive or mixed hearing loss and single-sided deafness. Although finite-element models have advanced understanding of the mechanisms underlying BC, progress toward personalized treatment strategies remains limited by a lack of standardized, experimentally validated, subject-specific models. This study proposes a hierarchical validation framework to support the development and validation of individualized computational models of the human head under BC stimulation. The framework spans four anatomical levels: system, subsystems, structures, and tissues. This approach enables systematic acquisition of data from intact cadaver heads down to isolated material domains. To demonstrate the applications of the framework, an experimental study was conducted on a single cadaver head, targeting three levels: the intact head (system), extracted bone pieces (structures), and isolated cortical layers (tissues). Subsystems were not addressed. High-resolution photon-counting computed tomography (CT) and energy-integrating cone-beam CT were used to acquire anatomical data. One-dimensional laser Doppler vibrometry was used to capture vibrational responses of bone pieces and cortical layers under wet and dry conditions. Representative results were analyzed to assess the impact of preparation state on resonance behavior. Comparative analysis showed that photon-counting CT provided superior structural resolution compared with energy-integrating cone-beam CT, particularly at the full-head (system) level. Vibrational measurements at the structure and tissue levels from the same anatomical region revealed broadly consistent resonance vibration patterns, enabling comparison of resonance frequencies. The influence of hydration state and thickness reduction on vibrational behavior was highlighted. The proposed framework provides a scalable methodology for validation of subject-specific BC models with the potential for more accurate BC simulations based on the hypothesis of functional variability rooted in anatomical variability. Obvious use cases would include the development of improved hearing aid designs and personalized treatments. In parallel, a successful correlation of anatomical and functional variability can serve as inspiration for design principles of metamaterials. Full article

A 70-year-old Japanese woman with longstanding hearing loss and asthma developed floating sensations, left finger numbness, and postural instability one day after influenza vaccination, leading to hospital admission. Neurological examinations showed hearing loss, hyperreflexia, left-predominant ataxia, bilateral mild bathyanesthesia, and inability to tandem gait. Cerebrospinal fluid (CSF) analysis showed no pleocytosis or malignant cells, but revealed positive oligoclonal bands and elevated myelin basic protein. Despite no contrast agent use due to asthma, brain magnetic resonance imaging (MRI) revealed pontine hyperintensities on diffusion-weighted imaging (DWI) and T2-fluid attenuated inversion recovery (T2-FLAIR) sequences, along with hyperperfusion on arterial spin labeling (ASL) imaging. Serum anti-aquaporin-4 antibodies (AQP4-Ab) were negative by ELISA. Given the temporal proximity to vaccination and elevated demyelination markers, brainstem-type acute disseminated encephalomyelitis (ADEM) was initially suspected. Symptoms nearly resolved after two cycles of methylprednisolone pulse therapy. Notably, hyperperfusion gradually improved on ASL imaging. Post-discharge, a cell-based assay confirmed the diagnosis of neuromyelitis optica spectrum disorder (NMOSD) by detecting positive anti-AQP4-Ab. She has been relapse-free for about a year without any immunosuppressants or biologics. Although contrast-enhanced MRI remains the gold standard modality for lesion evaluation due to its high sensitivity, hyperperfusion on ASL may provide a useful alternative in patients for whom contrast agents are contraindicated, such as those with asthma or impaired renal function. Full article

Background/Objectives: Hearing loss constitutes a modifiable risk factor for dementia. Auditory rehabilitation with devices such as cochlear implants (CIs) has been reported to prevent cognitive decline in older adults. However, post-implant cognitive effects remain highly heterogeneous across studies. Thus, the aim of this review is to synthesize the evidence on cognitive outcomes and their interplay with speech perception, quality of life (QoL), and psychological status. Methods: A bibliographic search was conducted following PRISMA guidelines from January 2015 to July 2025. Studies were eligible if they included adult CI candidates who completed cognitive and audiometric assessments. In total, 43 studies, including longitudinal and cross-sectional designs, were reviewed. Several studies also assessed hearing aid (HA) users and normal-hearing (NH) controls. Principal results were identified and analyzed across cognitive domains, audiological performance, QoL, and psychological outcomes. Results: CIs significantly improved cognition across longitudinal studies, with a higher number of assessments reporting gains in memory (61%), global cognition (57%), and executive function (46%); while attention, language, and visuospatial skills were less frequently evaluated. Though findings are not fully consistent, interactions between speech intelligibility and cognitive subdomains have also been found in several studies: global cognition (25%), executive function (22%), visuospatial skills (20%), attention (21%), language (17%), and memory (12%). Improvements in QoL, social engagement, depression, and anxiety are frequently observed. Conclusions: The lack of unified and adapted neurocognitive tools may prevent the observation of consistent outcomes across studies. Further research and multimodal data are still needed to fully understand the interaction between cognition, speech intelligibility, and QoL in CI users. Full article

Hearing loss is the most common sensory disorder; It is estimated that nearly 2.5 billion people will have some degree of hearing loss by 2050. Although the causes are diverse, a significant proportion of cases have a genetic origin, which is the main focus of the models discussed in this review. Many loci corresponding to deafness genes have already been identified, and approximately 150 genes are responsible for non-syndromic deafness, which is characterized by partial or total hearing loss that is not associated with other signs or symptoms. Although hearing aids and cochlear implants are widely available today, their effectiveness is often limited, especially in noisy environments, prompting the development of advanced therapies for hearing loss. To evaluate new therapies and improve our understanding of hearing physiology, various models, including cellular, animal, and organoid models, are used to study the inner ear. In this review, we present these different models in detail, with their respective strengths and limitations. This analysis will be particularly valuable in helping researchers to identify the most appropriate model for their specific research questions and to justify their choices from an ethical perspective. Full article

Background/Objectives: Audiometric methods for hearing-impaired patients are constantly evolving as new therapeutic interventions and improved clinical standards are established. This study aimed to explore the relationship between patient-reported outcome measures in cochlear implant users and scores from audiometric test procedures in quiet and noise. Methods: In a prospective study, 20 postlingually deafened CI users were included. Speech comprehension was measured in quiet (by Freiburg words) and in noise (by the Oldenburg sentence test), while stationary speech-simulating or temporally fluctuating noise was applied and the noise sources were varied. Subjective feedback from the patients was obtained using the HISQUI19 questionnaire. Results: Word scores in quiet showed a significant positive correlation with the user’s subjective assessment of hearing ability using the questionnaire (Spearman’s R = 0.57). A greater correlation of the subjective evaluation of comprehension against fluctuating background noise as compared with stationary background noise was evident. On the other hand, the test–retest accuracy was reduced by a substantial factor in the transition from stationary to fluctuating background noise. Conclusions: By introducing temporal fluctuations in the background noise, the ecological validity can be improved, but at the cost of a parallel decrease in the accuracy of the test procedure. Especially in the context of studies, this knowledge may help to improve the choice of the specific test method used in evaluating the relationship between ecological validity and precision audiometry. Full article

Objective: We aimed to explore the efficacy of repetitive transcranial magnetic stimulation (rTMS) for idiopathic sudden sensorineural hearing loss (ISSNHL) and evaluate the correlation between treatment courses of rTMS and hearing outcomes. Methods: A prospective observational study was conducted at West China Fourth Hospital, Sichuan University, from January 2018 to January 2025. The study enrolled 339 patients (342 affected ears) diagnosed with ISSNHL. Among them, 67 patients (group A) received standard therapy combined with rTMS, while the control group (group B) received conventional therapy only. To verify the correlation between different treatment courses of rTMS and hearing outcomes, patients in Group A were divided into Group A1 (treatment courses ≤ 10) and Group A2 (treatment courses > 10). Hearing thresholds and clinical characteristics were evaluated at admission, discharge day and 6 months post-treatment. The SDRG’s criteria were used for the grading of hearing recovery. Results: Tinnitus (79.89% vs. 75.32%, p = 0.361) and sleep disorders (33.70% vs. 41.14%, p = 0.178) were highly prevalent among patients in group A and group B. 1 Hz rTMS significantly improved these symptoms (PSQI: 52.32% vs. 44.44%, p = 0.032; THI: 16.67 ± 19.41 vs. 8.22 ± 12.77, p = 0.002). Compared to high-tone hearing loss patients, those with low-tone loss in groupA2 showed a more rapid improvement (250 Hz: 17.66 ± 16.59 vs. 14.09 ± 15.58, p = 0.041; 500 Hz: 21.20 ± 18.03 vs. 17.31 ± 16.24, p = 0.036) than grouA1, with benefits sustained at 6-month follow-up (250 Hz: 27.79 ± 18.74 vs. 22.71 ± 18.31, p = 0.012; 500 Hz: 31.89 ± 19.73 vs. 26.49 ± 20.08, p = 0.013). Conclusions: rTMS at 1 Hz, administered in courses > 10 sessions, demonstrated both short-term and long-term beneficial effects in the ISSNHL. Those with low-tone hearing loss exhibit better recovery but a higher chance of relapse than high-tone loss patients. As a non-invasive approach with minimal side effects, rTMS is suitable for routine ISSNHL treatment. Full article

Background: Stapes prostheses represent one of the earliest and most widely applied “biomedical actuators” designed to restore hearing in patients with otosclerosis. Unlike conventional actuators, which convert energy into motion, stapes prostheses function as passive or smart micro-actuators, transmitting and modulating acoustic energy through the ossicular chain. Objective: This paper provides a comprehensive analysis of stapes prostheses from an engineering and biomedical perspective, emphasizing design principles, materials science, and recent innovations in smart actuators based on shape-memory alloys combined with surgical applicability. Methods: A narrative review of the evolution of stapes prostheses was consolidated by institutional surgical experience. Comparative evaluation focused on materials (Teflon, Fluoroplastic, Titanium, Nitinol) and design solutions (manual crimping, clip-on, heat-activated prostheses). Special attention was given to endoscopic stapes surgery, which highlights the ergonomic and functional requirements of new device designs. Results: Traditional fluoroplastic and titanium pistons provide reliable sound conduction but require manual crimping, with a higher risk of incus necrosis and displacement. Innovative prostheses, particularly those manufactured from nitinol, act as self-crimping actuators activated by heat, improving coupling precision and reducing surgical trauma. Emerging designs, including bucket-handle and malleus pistons, expand applicability to complex or revision cases. Advances in additive manufacturing and middle ear cement fixation offer opportunities for customized, patient-specific actuators. Conclusions: Stapes prostheses have evolved from simple passive pistons to innovative biomedical actuators exploiting shape-memory and biocompatible materials. Future developments in stapes prosthesis design are closely linked to 3D printing technologies. These developments have the potential to enhance acoustic performance, durability, and patient outcomes, thereby bridging the gap between otologic surgery and biomedical engineering. Full article