Search Results (484)

Objective: This paper describes the development of a speech intelligibility test in Swiss German dialects, designed for children aged four to nine who are not yet familiar with standard German. Method: Suitable monosyllabic words and trochees in different Swiss German dialects were compiled, illustrated, and evaluated. Picture-pointing test procedures appropriate for children were developed. The selected test words and the pictures representing them were evaluated in a preliminary trial with forty-six normal-hearing children between two and nine years of age. Results: A set of 60 monosyllabic words and 40 trochees was recorded in four different Swiss German dialects as well as in standard German, resulting in a total of 500 recordings. Drawings were created to illustrate each word and found to be appropriate for children aged four years old or older. A non-adaptive and an adaptive test procedure using a weighted up–down method to measure speech reception thresholds in quiet and in noise were developed. Conclusions: A novel test to determine speech intelligibility in children in four different Swiss dialects was developed and evaluated in a pilot study. A validation study with more participants was designed to evaluate the test material and procedures. Full article

Background and Clinical Significance: Prader–Willi syndrome (PWS) is an imprinting disorder not typically associated with severe congenital sensorineural hearing loss (SNHL). When profound SNHL is present in an infant with a known syndrome, an independent monogenic etiology should be considered. We report the first molecularly confirmed case of PWS co-occurring with biallelic pathogenic TMC1 variants causing congenital SNHL, outlining diagnostic challenges, cochlear implant (CI) outcomes, and implications for blended phenotypes. Case Presentation: A male infant with PWS due to a paternal 15q11.2–q13 deletion failed newborn hearing screening. Diagnostic auditory brainstem response and auditory steady-state response confirmed bilateral severe-to-profound SNHL. Temporal bone CT/MRI were normal. Comprehensive genetic testing identified compound heterozygous TMC1 variants consistent with autosomal recessive DFNB7/11 hearing loss, plus two variants of uncertain significance in SERPINB6 and EPS8L2. Sequential bilateral cochlear implantation was performed (left ear at 14 months, right at 20 months), followed by auditory–verbal therapy. Over four years, the child showed steady improvements in hearing and early-speech development. Conclusions: Early genomic evaluation is essential when clinical features appear atypical for a known syndrome. Identifying TMC1-related deafness enabled timely cochlear implantation and measurable gains. This case highlights that severe congenital SNHL in a syndromic infant may reflect a distinct monogenic disorder rather than phenotypic expansion of the primary syndrome, emphasizing the importance of recognizing blended phenotypes to guide precision-care strategies in rare disorders. Full article

Objectives: To systematically analyze the expression profiles of long non-coding RNAs (lncRNAs) in serum-derived exosomes from patients with age-related hearing loss (ARHL), and to further identify key regulatory lncRNAs involved in the pathogenesis and progression of ARHL. Methods: Peripheral blood samples were collected from patients with ARHL and age-matched normal-hearing controls. Serum was separated and exosomes were extracted. The exosomes were identified by nanoparticle tracking analysis (NTA), transmission electron microscopy (TEM), and Western blot. Subsequently, total RNA was extracted from the purified exosomes for lncRNA transcriptome sequencing. Based on the sequencing results, we identified differentially expressed lncRNAs and mRNAs and conducted multi-dimensional functional analysis, including Gene Ontology (GO), Kyoto Encyclopedia of Genes and Genomes (KEGG), Reactome pathway database (Reactome), and Disease Ontology (DO). Finally, four key mRNAs (THAP2, ZNF225, MED12, and RNF141) and four differentially expressed lncRNAs (DE-lncRNAs), namely MSTRG.150961.7, ENSG00000273015, MSTRG.336598.1, and ENSG00000273493, were experimentally verified by quantitative real-time polymerase chain reaction (RT-qPCR) technology. Results: Exosomes were successfully isolated from serum and confirmed by particle size, morphological examination, and the expression of exosome-labeled proteins. A total of 2874 DE-lncRNAs were identified, among which 988 were downregulated and 1886 were upregulated. Similarly, 2132 DE-mRNAs were detected, among which 882 were downregulated and 1250 were upregulated. GO analysis revealed significant enrichment in biological processes such as “phospholipid binding”, “phosphatidylinositol binding”, “phosphatase binding”, “phosphatidylinositol bisphosphate binding”, “phosphatidylinositol-4,5-bisphosphate binding”, “phosphatidylinositol-3,5-bisphosphate phosphatase activity”. KEGG is significantly enriched in signaling pathways including “Wnt signaling pathway”, “Hippo signaling pathway”, “Cushing syndrome”, and “Nucleocytoplasmic transport”. The functional annotations of Reactome were significantly enriched in biomolecular pathways including “tRNA processing”, “Cellular response to heat stress”, “Extra-nuclear estrogen signaling”, “Metabolism of non-coding RNA”, and “CTNNB1 T41 mutants aren’t phosphorylated”. DO is significantly enriched in diseases or pathological conditions such as “hepatitis”, “bacterial infectious disease”, “cystic fibrosis”, and “vasculitis”. Conclusions:THAP2, ZNF225, MED12, and RNF141 may serve as potential candidate biomarker for ARHL. Additionally, lncRNA MSTRG.150961.7, lncRNA MSTRG.336598.1, and lncRNA ENSG00000273493 may play significant roles in the pathogenesis of this condition. Full article

Background: Bortezomib, a proteasome inhibitor used in multiple myeloma (MM), is associated with several adverse effects, most notably peripheral neuropathy. Ototoxicity, however, remains a rare and underrecognized complication. Case presentation: We report the case of a 74-year-old man with MM who developed sudden unilateral sensorineural hearing loss following subcutaneous bortezomib administration. Audiometry confirmed severe right-sided hearing loss. MRI of the internal auditory canal was normal. Given the absence of other ototoxic agents, bortezomib was identified as the likely causative drug. The patient was treated with intratympanic dexamethasone injections, achieving partial hearing recovery. Subsequent chemotherapy re-exposure triggered another hearing decline, which again improved after repeated intratympanic treatment. Conclusions: Bortezomib-related ototoxicity is a rare but potentially reversible adverse event. This case suggests that early intratympanic corticosteroid therapy may mitigate cochlear injury, allowing continuation of chemotherapy for patients responding well to bortezomib. Full article

Dicer is crucial for the generation of microRNAs (miRNAs), which are essential for regulating gene expression and keeping neuronal health. Dicer’s conditional deletion cuts all spiral ganglion neurons but spares a small fraction of vestibular ganglion neurons, innervating the utricle and part of the saccule. Hair cells develop in the utricle, saccule, posterior crista, and the cochlea in Pax2^Cre; Dicer^f/f. Cochlear hair cells develop at the base and expand the OHC and IHC in the middle, or split into a base/middle and the apex. In contrast, Foxg1^Cre; Dicer^f/f cuts all canal cristae and cochlea hair cells, leaving a reduced utricle and an exceedingly small saccule. Likewise, Foxg1^Cre; Gata3^f/f shows no cochlear hair cells and is absent in the horizontal and reduced in the posterior crista. In contrast, the utricle, saccule, and anterior crista are nearly normal, underscoring the intricate regulatory networks involved in hair cell and neuronal development. The central projections have been described as the topology of various null deletions. Still, without spiral ganglion neurons, fibers from Dicer null mice navigate to the cochlear nuclei and expand into the vestibular nuclei to innervate the caudal brainstem. Beyond a ramification around the CN, no fibers expand to reach the cerebellum, likely due to Pax2 and Foxg1 that cut these neurons. Genetic alterations, such as Dicer deletion, can lead to hearing loss and impairments in auditory signal processing, illustrating the critical role of microRNAs in the development and function of auditory and vestibular neurons. Further studies on this topic could help in understanding potential therapeutic targets for hearing loss associated with neuronal degradation of miRNA. Full article

Background/Objectives: Patients with Idiopathic sudden sensorineural hearing loss (ISSNHL) are often devastated by the unknown etiology coupled with the unknown pathway to recovery. The aim of this study was to evaluate whether abnormalities on the video Head Impulse Test (vHIT) performed early in the course of ISSNHL are associated with poorer hearing recovery. Methods: Forty-four patients with ISSNHL were prospectively enrolled between 2019 and 2022 following exclusion of differential diagnoses on clinical and MRI evaluation. vHIT was performed within 1–14 days of symptom onset and within 48 h of hospitalization. Recovery at six months was assessed both as a dichotomous outcome and by change in pure tone average (PTA). Group differences were analyzed using Fisher’s exact and Mann–Whitney U tests. A two-predictor logistic regression model examined the association between vHIT results, dizziness, and recovery. Results: Twelve patients exhibited abnormal vHIT findings. Abnormal vHIT was strongly associated with the presence of dizziness and with markedly poorer hearing recovery at six months. Patients with normal vHIT demonstrated substantially greater improvement in PTA thresholds compared with those showing abnormal results. Logistic regression further confirmed that abnormal vHIT was an independent predictor of reduced likelihood of hearing recovery, whereas dizziness alone did not independently influence outcomes. Conclusions: Our findings suggest that abnormal vHIT results in ISSNHL patients are linked to poor hearing recovery, which can enhance patient counseling regarding expectations. Although promising as a prognostic tool, we acknowledge our limited sample size and recommend validation in larger prospective cohorts. Full article

Using efficient voice alarms to ensure safe evacuation is important during emergencies, especially for the elderly. Factors that have important influence on speech perceptions have been investigated for several years. However, relatively few studies have specifically explored the key factors influencing perceptions of voice alarms in emergency situations. This study investigated the combined effects of speech rate (SR), signal-to-noise ratio (SNR), and reverberation time (RT) on older people’s perception of voice alarms. Thirty older adults were invited to evaluate speech intelligibility, listening difficulty, and perceived urgency after hearing 48 different voice alarm conditions. For comparison, 25 young adults were also recruited in the same experiment. The results for older adults showed that: (1) When SR increased, speech intelligibility significantly decreased, and listening difficulty significantly increased. Perceived urgency reached its maximum at the normal speech rate for older adults, in contrast to young adults, for whom urgency was greatest at the fast speech rate. (2) With the rising SNR, speech intelligibility and perceived urgency significantly increased, and listening difficulty significantly decreased. In contrast, with the rising RT, speech intelligibility and perceived urgency significantly decreased, while listening difficulty significantly increased. (3) RT exerted a relatively stronger independent influence on speech intelligibility and listening difficulty among older adults compared to young adults, which tended not to be substantially moderated by SR or SNR. The interactive effect of SR and RT on perceived urgency was significant for older people, but not significant for young people. These findings provide referential strategies for designing efficient voice alarms for the elderly. Full article

Background/Objectives: Retrocochlear auditory dysfunctions (RADs), including auditory neuropathy (AN) and auditory processing disorders (APD), encompass disorders characterized by impaired auditory processing beyond the cochlea. This narrative review critically examines their distinguishing features, synthesizing recent advances in classification, pathophysiology, clinical presentation, and treatment. Methods: This narrative review involved a comprehensive literature search across major electronic databases (e.g., PubMed, Scopus) to identify and synthesize relevant studies on the classification, diagnosis, and management of AN and APD. The goal was to update the view on etiologies (genetic/non-genetic) and individualized rehabilitative strategies. Diagnosis relies on a comprehensive assessment, including behavioral, electrophysiological, and imaging tests. Rehabilitation is categorized into bottom-up and top-down approaches. Results: ANSD is defined by neural desynchronization with preserved outer hair cell function, resulting in abnormal auditory brainstem responses and poor speech discrimination. The etiologies (distal/proximal) influence the prognosis for interventions, particularly cochlear implants (CI). APD involves central processing deficits, often with normal peripheral hearing and heterogeneous symptoms affecting speech perception and localization. Rehabilitation is multidisciplinary, utilizing bottom-up strategies (e.g., auditory training, CI) and compensatory top-down approaches. Remote microphone systems are highly effective in improving the signal-to-noise ratio. Conclusions: Accurate diagnosis and personalized, multidisciplinary management are crucial for optimizing communication and quality of life. Evidence suggests that combined bottom-up and top-down interventions may yield superior outcomes. However, methodological heterogeneity limits the generalizability of protocols, highlighting the need for further targeted research. Full article

Background/Objectives: An exploratory study was conducted to investigate the effect of Ginkgo biloba extract EGb 761^® in the management of chronic tinnitus, and whether comorbidities have an impact on the treatment outcome. Methods: The exploratory, uncontrolled, open-label study enrolled 170 patients (Full Analysis Set) with chronic tinnitus who took 120 mg EGb 761^® twice daily for 24 weeks. Outcomes were assessed using the Tinnitus Questionnaire, Tinnitus Handicap Inventory, and 11-Point Box Scales for loudness and annoyance. Comorbidities were recorded with audiometry, the Hospital Anxiety and Depression Scale, and the Perceived Stress Questionnaire. The effectiveness was further examined in responder analyses (at least 30% score reduction in 3 out of 4 outcomes) and in subgroups defined by baseline anxiety, hearing impairment, stress and depression. Results: At week 24, significant improvements were observed in all tinnitus-related outcomes compared to baseline (all p < 0.0001). In subgroup analyses, patients with high baseline anxiety or stress as well as those with normacusis improved more, whereas baseline depression had no influence. The overall response rate was 18.8%. Conclusions: The results of this exploratory study indicate that EGb 761^® improved complaints in patients with chronic tinnitus. The therapy appears to be particularly beneficial for patients with normal hearing and/or concomitant anxiety and/or stress. Trial registration: The study was registered at ISRCTN (ISRCTN83863387, registration date 14 October 2016). Full article

This study aims to examine the acoustic characteristics of Mandarin vowels produced by children with cochlear implants and to explore the differences in their speech production compared with those of children with normal hearing. We propose a multiview model-based method for vowel feature analysis. This approach involves extracting and fusing formant features, Mel-frequency cepstral coefficients (MFCCs), and linear predictive coding coefficients (LPCCs) to comprehensively represent vowel articulation. We conducted k-means clustering on individual features and applied multiview clustering to the fused features. The results showed that children with cochlear implants formed discernible vowel clusters in the formant space, though with lower compactness than those of normal-hearing children. Furthermore, the MFCCs and LPCCs features revealed significant inter-group differences. Most importantly, the multiview model, utilizing fused features, achieved superior clustering performance compared to any single feature. These findings demonstrated that effective fusion of frequency domain features provided a more comprehensive representation of phonetic characteristics, offering potential value for clinical assessment and targeted speech intervention in children with hearing impairment. Full article

Otitis media with effusion (OME) is a common middle ear disease characterized by fluid accumulation without acute infection, leading to conductive hearing loss. Conventional diagnostic tools, such as tympanometry and otoscopy, have limited sensitivity and rely on expert interpretation. This study investigates vibro-acoustic radiation (VAR) as a novel, non-invasive, and objective method for OME detection. VAR signals were obtained from 36 OME patients (43 ears) and 15 normal ears using bone-conduction excitation and stereo microphones, and the frequency response functions were analyzed. OME increases the mechanical loading of the tympanic membrane and ossicular chain, thereby modifying sound transmission across the middle ear. Using a simplified theoretical model, we estimated acoustic parameters of the ear canal, eardrum, and middle ear, including specific acoustic impedance and resonance frequency ranges, to interpret changes in VAR. VAR analysis revealed significantly reduced signal amplitude in the 8–10 kHz range in OME ears compared with normal ears (p < 0.05). A classification algorithm based on these features achieved 86.7% accuracy, 85.0% sensitivity, and 80.0% specificity, with an area under the ROC curve of 0.986. These findings suggest that VAR has strong potential as a non-invasive diagnostic tool for OME, warranting validation in larger clinical studies. Full article

Connexins, as key players in intercellular communication in the inner ear, are vital for maintaining normal hearing function. While numerous studies have explored their role in congenital hereditary hearing loss, the underlying mechanisms and therapeutic potential of connexins in acquired hearing loss remain to be fully elucidated. This review summarizes recent advances in connexin research in the context of acquired hearing loss, with a focus on presbycusis, noise-induced, and drug-induced hearing loss, and delves into their pathophysiological roles. Through the analysis and organization of these research findings, the article aims to provide a theoretical basis and research direction for future connexin-targeted therapies for acquired hearing loss. Full article

Background: Individuals with untreated hearing loss often experience cognitive decline as a result of increased cognitive load and reduced sensory stimulation. Despite the well-established link between untreated hearing loss and cognitive decline in older adults, its impact on cognition in young and middle-aged adults has not been systematically examined. Given the Lancet Commission’s identification of midlife hearing loss as the leading modifiable risk factor for dementia, early identification of cognitive decline is essential. This review explored the cognitive impact of untreated hearing loss in adults. Method: A comprehensive search was conducted in PubMed, Scopus, Web of Science, and EMBASE to include studies comparing cognitive function between adults with normal hearing and those with untreated hearing loss aged 18–65 years. The methodological quality of the included studies was examined via the Joanna Briggs Institute Critical Appraisal Checklist for Analytical Cross-Sectional Studies. Pooled mean differences and heterogeneity were analysed for each domain. Results: Seven studies included in the qualitative synthesis had “moderate” to “strong” methodological quality. The cognitive domains assessed in these studies were global cognitive function, memory, attention, and executive function. Of these, six were eligible for meta-analysis, which revealed a small but statistically significant decline in overall cognitive performance and memory and executive function among adults with untreated hearing loss. Conclusions: Cognitive vulnerabilities exist in young and middle-aged adults with untreated hearing loss. Hence, incorporating cognitive assessment into routine audiological evaluation may enable earlier intervention and delay the future burden of Alzheimer’s disease and related dementias in such a population. Full article

Objective: The aim of the study was to evaluate the validity, clinical applicability, and developmental sensitivity of the Romanian LEESPQ in children with cochlear implants (CIs), by analyzing its association with age at implantation, duration of auditory experience, and implantation laterality, and by comparing the developmental trajectory with that of normal-hearing (NH) children. Methods: The study assesses the validity, reliability, and clinical sensitivity of the LEESPQ in pediatric cochlear implant users. Furthermore, it investigates the associations between total questionnaire scores and key clinical variables, including implantation laterality (unilateral versus bilateral), age at device activation, and duration of implant use. Forty-seven children with CIs (26 boys, 21 girls) were included, with implantation ages ranging from 9 months to 5 years. Of these, 21 received unilateral implants and 26 bilateral implants. Responses were analyzed both in relation to clinical variables and in comparison with available normative data from NH children, in order to delineate potential differences in linguistic developmental trajectories. Results: Findings suggest that the LEESPQ is a reliable and clinically valuable instrument for monitoring post-implant linguistic progress. It provides relevant insights into early auditory access, the linguistic environment within the family, and the development of early verbal production. Scores were significantly influenced by age at implantation and duration of auditory experience, confirming the role of early stimulation and neural plasticity in shaping speech development after cochlear implantation. Conclusions: The LEESPQ demonstrates strong clinical utility as a sensitive tool for monitoring early preverbal and verbal development in children with CIs. By capturing score variations associated with age at implantation, auditory experience, and implantation laterality, the questionnaire provides meaningful insights into early post-implant outcomes and supports individualized rehabilitation planning. These findings highlight the value of the LEESPQ for early outcome assessment in pediatric cochlear implant users. Full article

Background/Objectives: This narrative review aims to evaluate the use of pupillometry as an objective measure of auditory perception and listening effort across the lifespan. Specifically, it synthesizes research examining pupillary responses in individuals with and without hearing impairment across pediatric, adult, and older adult populations. The review addresses methodological practices and clinical implications for integrating pupillometry into routine audiological assessment. Methods: 12 peer-reviewed studies published between 2010 and 2025 were selected through a systematic search of databases including PubMed, Scopus, Web of Science, and Google Scholar. Inclusion criteria required empirical use of pupillometry in auditory tasks involving human participants with normal hearing or hearing impairment. Studies were analyzed for population characteristics, experimental paradigms, pupillometric metrics (e.g., peak pupil dilation), level of evidence, and relevance to clinical audiology. This article uses a narrative review approach to organize and interpret findings. Results: Across age groups and hearing conditions, pupillometry consistently demonstrated sensitivity to cognitive load and listening effort, particularly in noisy environments or during complex auditory tasks. Pediatric studies revealed its potential as a non-invasive tool for preverbal children. Adult and older adult studies confirmed that pupillary responses reflect device performance (e.g., hearing aids, cochlear implants) and cognitive–linguistic demands. Methodological variability and individual differences in pupil response patterns were noted as limitations. Conclusions: The findings support the use of pupillometry as a valuable adjunct to behavioral audiometry, offering objective insight into auditory–cognitive load. Its application holds promise for pediatric diagnostics, hearing technology evaluation, and geriatric audiology. Standardization of measurement protocols and development of normative data are necessary to enhance clinical applicability and generalizability. Full article