Search Results (1,812)

Background: Meniere’s disease (MD) is a rare inner ear disorder characterized by endolymphatic hydrops and symptoms such as vertigo and hearing loss, with no curative treatment currently available. XuanYunNing tablets (XYN) have been clinically used to treat MD, but their molecular mechanisms remain unclear. Objective: This study aimed to systematically evaluate the pharmacological effects of XYN in a guinea pig model of MD and to elucidate the underlying molecular mechanisms of both MD pathogenesis and XYN intervention through integrated multi-omics analyses, including transcriptomics, proteomics, and bioinformatics. Methods: A guinea pig model of endolymphatic hydrops was induced by intraperitoneal injection of desmopressin acetate (dDAVP). Pharmacodynamic efficacy was evaluated via behavioral scoring and histopathological analysis. The differentially expressed genes (DEGs) and differentially expressed proteins (DEPs) modulated by XYN treatment were identified using high-throughput transcriptomic and proteomic sequencing. These data were integrated through multi-omics bioinformatic analysis. Key molecular targets and signaling pathways were further validated using RT-qPCR and Western blotting. Results: Pharmacological evaluations showed that guinea pigs in the model group exhibited a 26% increase in endolymphatic hydrops area, while high-dose XYN treatment reduced this area by 19% and significantly improved functional parameters, including overall physiological condition (e.g., weight and general appearance), auricular reflexes to low-, medium-, and high-frequency sound stimuli, nystagmus, and the righting reflex. High-throughput sequencing combined with integrative omics analysis identified 513 potential molecular targets of XYN. Subsequent network and module analyses pinpointed the JAK-STAT signaling pathway as the central axis. Mendelian randomization (MR) analysis further supported a causal relationship between MD and metabolic, immune, and inflammatory traits, reinforcing the central role of JAK-STAT signaling in both MD progression and XYN-mediated intervention. Mechanistic studies confirmed that XYN downregulated IFNG, IFNGR1, JAK1, p-STAT3/STAT3, and AOX at both mRNA and protein levels, thereby inhibiting aberrant JAK-STAT pathway activation in MD model animals. In addition, a total of 125 chemical constituents were identified in XYN by UHPLC-MS analysis. ZBTB20 and other molecules were identified as potential blood-based biomarkers for MD. Conclusions: This study reveals that XYN alleviates MD symptoms by disrupting a pathological cycle driven by JAK-STAT signaling, inflammation, and metabolic dysfunction. These findings support the clinical potential of XYN in the treatment of Meniere’s disease and may inform the development of novel therapeutic strategies. Full article

Background and Objectives: Otitis media with effusion (OME), frequently associated with obstructive adenoid hypertrophy (OAH), is a leading cause of paediatric hearing loss. Clinically distinguishing effusion types (serous vs. mucoid) and predicting postoperative hearing recovery are unresolved challenges. This study evaluated the utility of preoperative blood inflammatory markers in predicting effusion characteristics and short-term hearing outcomes following adenoidectomy with tympanostomy tube (TT) insertion. Materials and Methods: In this retrospective cohort study, 232 children under 12 years old in 2024 and undergoing adenoidectomy (with or without TT insertion) were categorised into serous OME (n = 42), mucoid OME (n = 78), and non-effusion (n = 112) groups. Preoperative blood sample analyses assessed neutrophil, lymphocyte, eosinophil, basophil, and platelet counts, along with derived indices, including neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), eosinophil-to-basophil ratio (EBR), mean platelet volume (MPV), and systemic immune–inflammation index (SII). Hearing was evaluated at 2 weeks and 1 month postoperatively. Statistical analyses used SPSS v.28, with significance set at p < 0.05. Result: Mucoid OME patients exhibited significantly elevated neutrophil counts, platelet counts, eosinophils, NLR, and SII compared to those in serous OME and non-effusion groups (p < 0.05). All serous OME children achieved normal hearing by the first follow-up, whereas 15.4% of mucoid OME cases had transient mild hearing loss persisting after 2 weeks (p = 0.008; OR=15.97) but resolving by 1 month. Preoperative neutrophil count independently predicted delayed hearing recovery (p = 0.021). Conclusions: Systemic inflammatory markers, particularly neutrophil count, NLR, and SII, effectively differentiate mucoid OME from other effusion types and correlate with short-term hearing recovery. Neutrophil count may serve as a prognostic tool for surgical planning and patient counselling. Prospective studies are warranted to validate these findings in broader paediatric populations. Full article

Background and Objectives: Cochlear implantation (CI) is a surgical procedure that offers significant benefits to individuals with sensorineural hearing loss, particularly in pediatric patients, as it can prevent long-term cognitive impairment. Despite the devices being designed for lifelong use, complications may necessitate explantation and subsequent reimplantation. Materials and Methods: Our retrospective study analyzes the incidence and causes of such procedures in pediatric CI patients over a period of 15 years, from May 2009 to June 2025. The study included patients aged between 8 months and 17 years, recording their age, the manufacturers of their first and second implants, the reasons for explantation and reimplantation, and the type of electrode array used during the second surgery. Results: During the study period, a total of 440 cochlear implantations were performed in our department. The primary causes of explantation in our study group were device hardware failures in 2.27% of cases, seromas over the implant body or antenna in 0.68% of cases, spontaneous extrusion in 0.22% of cases, and local trauma with electrode displacement in 0.22% of cases. The study confirmed that hardware failures were the most common reason for reimplantation, with an incidence influenced by the device manufacturer and the extent of trauma to the device. Surgical observations highlight the challenges regarding electrode reimplantation and available electrode choices for the surgeon. Conclusions: The use of superior materials and advanced research in manufacturing can enhance implant reliability and reduce the number of surgical procedures required in the long term for pediatric patients. Any type of electrode array can be utilized in reimplantations if meticulous surgical techniques are applied. Full article

Hearing loss is often caused by genetic and environmental factors, with inherited mutations responsible for 50–60% of cases. The GJB2 gene, encoding connexin 26, is a major contributor to nonsyndromic sensorineural hearing loss (NSHL) due to its role in cellular communication critical for auditory function. In Taiwan, common deafness-associated genes include GJB2, SLC26A4, OTOF, MYO15A, and MTRNR1, which were similar to those found in other populations. The most common pathogenic genes is GJB2 mutations and the hearing level in children with GJB2 p.V37I/p.V37I or p.V37I/c.235delC was estimated to deteriorate at approximately 1 decibel hearing level (dB HL)/year. We found another common mutation in Taiwan Biobank, GJB2 p.I203T, which were identified in our data and individuals carrying this mutation experienced more severe hearing loss, suggesting a synergistic effect of these mutations on auditory impairment. We suggest GJB2 whole genetic screening is recommended for clinical management and prevention strategies in Taiwan. This study used data from the Taiwan Biobank to analyze allele frequencies of GJB2 gene variants. Predictive software (PolyPhen-2 version 2.2, SIFT for missense variants 6.2.1, MutationTaster Ensembl 112 and Alphamissense CC BY-NC-SA 4.0) assessed the pathogenicity of specific mutations. Additionally, 82 unrelated NSHL patients were screened for mutations in these genes using PCR and DNA sequencing. The study explored the correlation between genetic mutations and the severity of hearing loss in patients. Several common GJB2 mutation sites were identified from the Taiwan Biobank, including GJB2 p.V37I (7.7%), GJB2 p.I203T (6%), GJB2 p.V27I (31%), and GJB2 p.E114G (22%). Bioinformatics analysis classified GJB2 p.I203T as pathogenic, while GJB2 p.V27I and GJB2 p.E114G were considered polymorphisms. Patients with GJB2 p.I203T mutation experienced more severe hearing loss, emphasizing the potential interaction between the gene in auditory impairment. The mutation patterns of GJB2 in the Taiwanese population are similar to other East Asian regions. Although GJB2 mutations represent the predominant genetic cause of hereditary hearing loss, the corresponding mutant proteins exhibit detectable aggregation, particularly at cell–cell junctions, suggesting at least partial trafficking to the plasma membrane. Genetic screening for these mutations—especially GJB2 p.I203T (6%), GJB2 p.V27I (31%), and GJB2 p.E114G (22%)—is essential for the effective diagnosis and management of non-syndromic hearing loss (NSHL) in Taiwan. We found GJB2 p.I203T which were identified in our data and individuals carrying this mutation experienced more severe hearing loss, suggesting a synergistic effect of these mutations on auditory impairment. We suggest whole GJB2 gene sequencing in genetic screening is recommended for clinical management and prevention strategies in Taiwan. These findings have significant clinical and public health implications for the development of preventive and therapeutic strategies. Full article

Background: Mitochondrial substrate switching plays an important role in aging. The substrate metabolic rate is closely related to mitochondrial activity, as mitochondria are the primary site for substrate oxidation and ATP production. Different substrates (glucose, amino acids, and fatty acids) enter the mitochondria through distinct pathways and are metabolized at different rates, depending on the energy demand and cellular conditions. However, it remains unclear how the mitochondrial metabolic rate of these substrates affects auditory cellular function. This study aimed to characterize the substrate-dependent mitochondrial respiratory responses of cochlear cells under varying energy supply conditions and metabolic stress, focusing on glucose, amino acids, and fatty acids as representative energy sources. Methods: The oxygen consumption rate (OCR) was measured after substrate addition using an Agilent Seahorse XF24 Flux Analyzer In-House Ear Institute-Organ of Corti 1 (HEI-OC1) cells, and the maximum OCR (MOCR) was determined as part of the mitochondrial stress test. Statistical analyses were performed using analysis of variance (ANOVA). Results: The OCR increased significantly after glutamine (L-Gln) or palmitate addition. The MOCR after L-Gln addition was significantly higher than that after glutamic acid, glycine, and phenylalanine addition. The MOCR after pyruvate addition was significantly higher than that after glucose addition. However, there was no significant increase in the MOCR after fatty acid addition. Conclusions: Glucose is essential for basal metabolism but cannot rapidly meet sudden energy demands. Pyruvate and L-Gln serve as effective substrates for short-term, high-intensity energy demands. Fatty acids increase OCR through mitochondrial uncoupling effects, though their role may be limited in inner ear cells. These findings provide a foundation for exploring metabolic interventions to support cochlear function and hearing health. Full article

Background/Objectives: Pediatric episodic vestibular syndrome (EVS) is increasingly recognized, with recurrent vertigo of childhood (RVC) and vestibular migraine of childhood (VMC) being the most prevalent disorders. In 2021, the Bárány Society and the International Headache Society proposed new diagnostic criteria for RVC, VMC, and probable VMC (pVMC), replacing the older term benign paroxysmal vertigo (BPV). This study aimed to evaluate the clinical applicability of these new criteria. Methods: We conducted a cross-sectional study at a pediatric neurotology clinic within a tertiary hospital, including patients under 18 years with episodic vestibular symptoms evaluated between 2018 and 2025. All patients underwent a standardized neuro-otological assessment. Diagnoses were assigned using both the 2018 ICHD-3 and the 2021 Bárány criteria. Patients who did not fulfill any of the three new diagnostic categories, nor met criteria for any other specific vestibular disorder, were grouped into an undetermined category referred to as episodic vestibular syndrome without hearing loss (EVSw/oHL). Demographic and clinical variables were compared across diagnostic groups using non-parametric and chi-squared tests. Results: Among the 202 children evaluated, 109 met the inclusion criteria and were classified as RVC (n = 55), VMC (n = 23), pVMC (n = 13), or EVSw/oHL (n = 18). All patients previously diagnosed with BPV met the new criteria for RVC. Application of the Bárány criteria significantly reduced the proportion of unclassified EVS cases (from 35.78% to 16.51%). Significant clinical differences were observed among the groups in terms of episode duration, presence of vomiting, migraine and headache, and family history of migraine. Conclusions: The new Bárány criteria provide a more inclusive and clinically meaningful framework for classifying pediatric EVS. They improve diagnostic clarity, reduce the proportion of unclassifiable cases, and support earlier and more tailored management strategies. Full article

Background and Clinical Significance: Intralabyrinthine schwannoma (ILS) is a rare benign tumor of the inner ear, often presenting with nonspecific symptoms such as hearing loss, tinnitus and vertigo. Vestibular function in ILS patients remains underexplored. This study aims to evaluate vestibulo-ocular reflex (VOR) function and inner ear magnetic resonance imaging (MRI) signal changes in ILS, and to provide insights into potential mechanisms underlying vestibular dysfunction. Case Presentation: We report four cases of MRI confirmed ILS, including two intravestibular and two intravestibulocochlear schwannomas. All patients exhibited unilateral canal paresis on caloric testing, and two of three showed abnormal video head impulse test (vHIT) with decreased VOR gain and corrective saccades. Decreased signal intensity was observed in the semicircular canals in three cases, in the vestibule in one case, and in the cochlea in one case. A systematic literature review including 10 studies (n = 171) showed a 73.3% rate of abnormal caloric responses. Five studies conducted vHIT, reporting reduced mean VOR gain and corrective saccades, though quantitative analysis was limited. Cervical and ocular vestibular evoked myogenic potential abnormalities were found in 68.4% and 65.7% of reported cases, respectively. Conclusions: Impaired VOR function in patients with ILS may result not only from anatomical disruption but also from underlying biochemical or metabolic alterations within the inner ear. Full article

Background: Hearing loss is known to cause structural and functional abnormalities in the central auditory pathways. Interventions with hearing aids that amplify acoustic signals have been developed to combat hearing loss. However, little is known about how such devices may affect the brain and mitigate the progression of hearing loss. We hypothesized that timely intervention that amplifies acoustic signals would delay further progression of hearing loss by maintaining central auditory activity and neural structure. Method: To that end, we provided eight weeks of acoustic stimulation tailored to compensate for subject-specific patterns of frequency loss in two mouse models of progressive hearing loss. We evaluated the effects of sound amplification on endbulb of Held anatomy at different ages of intervention in mice with early-onset (DBA/2) and late-onset (C57Bl/6) hearing loss. Results: We observed in both strains that endbulbs undergo rapid and progressive atrophy in untreated control subjects exposed to a baseline, unamplified, sound environment. In contrast, endbulb atrophy was significantly slowed in treated mice (p < 0.05). Conclusions: These data provide a possible explanation for how the brain benefits from sound amplification via hearing aid devices. Full article

Background/Objectives: Waardenburg syndrome (WS) is a genetic disorder characterized by sensorineural hearing loss (SNHL) and pigmentation anomalies. While hearing impairment is a well-established feature of WS, vestibular dysfunction is also reported. This study aimed to investigate vestibular deficits in pediatric WS patients with SNHL, correlating these findings with molecular, audiometric, and radiological data to establish distinct phenotypic profiles for each WS subtype and associated pathogenic variants. Methods: This retrospective study included children with a genetically confirmed diagnosis of WS who underwent vestibular, auditory, and inner ear radiological assessments as part of their routine medical care between July 2000 and May 2022. Data were collected from medical records, including medical history, clinical findings, and assessment results. Results: Vestibular dysfunction was found to be highly prevalent, affecting 64% of the cohort, often impacting the canal sensory organ (89%) and occasionally the otolithic function (33%). Patients with SOX10 pathogenic variations exhibited a markedly higher risk of vestibular dysfunction, highlighting the unique role of SOX10 in inner ear development. Notably, inner ear malformations were identified in all SOX10-mutated subjects, whereas such anomalies were rare among individuals with other WS gene variants, occurring in only two additional cases with minor malformations. Conclusions: This study reveals a significant prevalence of vestibular deficits in pediatric WS patients with SNHL, emphasizing the need for routine vestibular assessments. The higher prevalence and severity of vestibular impairments in SOX10-mutated patients underscore the importance of molecular analysis in clinical diagnosis and management. Full article

Background: Older adults often struggle to comprehend speech in noisy environments, a challenge influenced by declines in both auditory processing and cognitive functions. This study aimed to investigate how differences in speech-in-noise perception among individual with clinically normal hearing thresholds (ranging from normal to mild hearing loss in older adults) are related to neural speech tracking and cognitive function, particularly working memory. Method: Specifically, we examined delta (1–4 Hz) and theta (4–8 Hz) EEG oscillations during speech recognition tasks to determine their association with cognitive performance in older adults. EEG data were collected from 23 young adults (20–35 years) and 23 older adults (65–80 years). Cognitive assessments were administered to older adults, and both groups completed an EEG task involving speech recognition in Speech-Shaped Noise (SSN) at individualized noise levels based on their Sentence Recognition Scores (SRS). Results: The results showed that age significantly impacted hit rates and reaction times in noisy speech recognition tasks. Theta-band neural tracking was notably stronger in older adults, while delta-band tracking showed no age-related difference. Pearson’s correlations indicated significant associations between age-related cognitive decline, reduced hearing sensitivity, and Mini-Mental State Examination (MMSE) scores. Regression analyses showed that theta-band neural tracking at specific SRS levels significantly predicted word list recognition in the higher SRT group, while constructional recall was strongly predicted in the lower SRT group. Conclusions: These findings suggest that older adults may rely on theta-band neural tracking as a compensatory mechanism. However, regression results alone were not sufficient to fully explain how working memory affects neural tracking, and additional cognitive and linguistic factors should be considered in future studies. Furthermore, cognitive assessments were administered only to older adults, which limits the ability to determine whether group differences are driven by age, hearing, or cognitive status—a major limitation that should be addressed in future research. Full article

Background/Objectives: Sudden sensorineural hearing loss (SSNHL) represents a challenging clinical entity with variable prognosis. Audiometric curve configuration has been proposed as a predictor of recovery. This study aimed to evaluate the association between audiogram morphology at onset and hearing outcome in patients with idiopathic unilateral SSNHL treated with standardized therapy. Methods: We retrospectively analyzed 156 patients with idiopathic SSNHL. Hearing thresholds at key frequencies were measured at baseline and 4 weeks post-treatment. Patients were categorized into upsloping, flat, downsloping, or U-shaped audiogram subgroups. Recovery was classified into four levels. Comparisons were made across subgroups for audiometric and laboratory data using ANOVA and chi-square tests. Results: Baseline PTA values were comparable across audiogram subgroups (p = 0.12). Hearing recovery differed significantly according to audiogram configuration (chi-square, p < 0.001), with upsloping and U-shaped patterns showing the best outcomes. Flat and downsloping curves were associated with poorer recovery, lower HDL, and elevated NLR values. Conclusions: Audiogram configuration is a relevant prognostic marker in SSNHL. Patterns linked to adverse metabolic and inflammatory profiles may benefit from tailored treatment strategies in a personalized medicine framework. Full article

Sign language serves as a vital way to communicate with individuals with hearing loss, deafness, or a speech disorder, yet accessibility remains limited, requiring technological advances to bridge the gap. This study presents the first real-time Greek Sign Language recognition system utilizing deep learning and embedded computers. The recognition system is implemented using You Only Look Once (YOLO11X-seg), an advanced object detection model, which is embedded in a Python-based framework. The model is trained to recognize Greek Sign Language letters and an expandable set of specific words, i.e., the model is capable of distinguishing between static hand shapes (letters) and dynamic gestures (words). The most important advantage of the proposed system is its mobility and scalable processing power. The data are recorded using a mobile IP camera (based on Raspberry Pi 4) via a Motion-Joint Photographic Experts Group (MJPEG) Stream. The image is transmitted over a private ZeroTier network to a remote powerful computer capable of quickly processing large sign language models, employing Moonlight streaming technology. Smaller models can run on an embedded computer. The experimental evaluation shows excellent 99.07% recognition accuracy, while real-time operation is supported, with the image frames processed in 42.7 ms (23.4 frames/s), offering remote accessibility without requiring a direct connection to the processing unit. Full article

Background/Objectives: Tinnitus has been increasingly recognized not only as an auditory disturbance but also as a condition that is potentially linked to cognitive decline, particularly in older adults. However, the relationship between tinnitus characteristics and cognitive impairment remains underexplored in clinical settings. This study aimed to (1) evaluate the association between chronic tinnitus and cognitive function in adults aged 60 years and above using standardized tools, and (2) determine whether tinnitus severity and duration are associated with increased risk of cognitive impairment. Methods: A cross-sectional study was conducted among 240 older adults, divided into tinnitus (n = 120) and non-tinnitus (n = 120) groups. Cognitive function was assessed using the Mini-Cog (0–5) and SPMSQ (0–10) tools. Tinnitus severity and duration were evaluated using the Tinnitus Handicap Inventory (THI), Visual Analogue Scale (VAS), and duration categories. Results: Participants with tinnitus were significantly older and had higher rates of hearing loss (58.33% vs. 33.33%, p = 0.001), depression (37.50% vs. 18.33%, p = 0.002), and poor sleep quality (51.67% vs. 31.67%, p = 0.003). Mini-Cog and SPMSQ scores were significantly lower in the tinnitus group (2.87 ± 1.14 vs. 3.52 ± 1.06; 6.95 ± 1.42 vs. 8.02 ± 1.18; both p < 0.001). Tinnitus presence, longer duration, and higher severity were independently associated with cognitive impairment. Each 10-point increase in THI score increased the odds of impairment by 45% (OR = 1.45, p < 0.001). Conclusions: Tinnitus burden, particularly when severe and prolonged, is significantly associated with cognitive impairment in older adults. These findings highlight the need for cognitive screening and integrated management in this population. Full article

Background: Orofacial dysfunctions are a source of discontent and impair daily living activities. Patients with hearing impairments exhibit an elevated risk of stomatognathic system changes. Hence, this pilot study aims to evaluate changes in stomatognathic system functions in patients with hearing impairments and cochlear implants. Methods: During the examination, the Nordic Orofacial Test-Screening was used to assess orofacial functions. The range of motion within the temporomandibular joints was measured using a vernier caliper. A socio-medical study was conducted to collect data regarding the patients, the cochlear implant sides, and the methods of communication. Results: The statistical analysis showed a significant difference in the total NOT-S scores (p < 0.001) and examination scores (p < 0.001) between patients with hearing impairments and the control group. These significant differences of the total NOT-S score and examination score have large effect sizes (r > 0.5). In particular, significant differences were observed in the results of maximum mouth opening (p = 0.006) and right laterotrusion (p = 0.020). Differences were also observed in the answers regarding the method of communication and the examination score of the NOT-S questionnaire (p = 0.040). The 6A of the NOT-S significantly affected the examination score (p = 0.015) and the total NOT-S score (p = 0.037), while the result of section 6B only significantly affected the NOT-S examination score (p = 0.032). Conclusions: Patients with hearing impairments presented orofacial dysfunctions significantly more often than the control group. The side of cochlear implant implantation is important for stomatognathic system changes. Full article

Cytomegalovirus (CMV) represents the most prevalent cause of congenital viral infection in newborns and the leading non-genetic etiology of sensorineural hearing loss (SNHL) in children. Notably, only 10–15% of congenitally infected infants possibly present with classic clinical symptoms at birth, including Small for gestational age, Microcephaly, Petechiae or purpura, Blueberry muffin rash, Jaundice, Hepatomegaly, Splenomegaly and abnormal neurologic signs. In contrast, approximately 90% of infected neonates exhibit no apparent symptoms initially. Current research predominantly focuses on symptomatic cases due to their severe acute presentations and high rates of long-term sequelae (40–60%), including SNHL and neurodevelopmental impairments. However, significant controversy persists regarding the management of asymptomatic infants. Emerging evidence reveals that 8–15% of asymptomatic carriers develop Late-onset Hearing Loss (LOHL) beyond the neonatal period. Additionally, 5–10% may manifest neurodevelopmental abnormalities including mild intellectual disability, learning difficulties, or motor coordination disorders. Crucially, given the substantial population of asymptomatic cCMV cases, these delayed complications account for 30–40% of all cCMV-related long-term morbidity, underscoring their considerable public health impact. This review synthesizes current evidence and controversies regarding cCMV-related SNHL in asymptomatic or mildly symptomatic children, with a focus on screening, diagnostic classification, and antiviral management gaps, to heighten clinical awareness of this underrecognized cause of hearing loss. Full article