Search Results (970)

Early patterning skills, particularly those involving linear repeating patterns, are well-established predictors of mathematical development. This relationship has not yet been investigated in visually oriented deaf and hard-of-hearing (DHH) children. It also remains unclear whether a two-dimensional pattern structure contributes to predicting numerical skills in children at the time of school entry. The present study investigates the relationship between repeating patterning skills in two formats (linear and circular) and numerical skills in a total of 38 DHH and typically hearing children. Language competence was additionally assessed in the DHH group to account for its linguistic heterogeneity. In the DHH and hearing groups, repeating patterning skills in each format strongly predicted numerical skills. Among DHH children, prior language experience played a more decisive role in mathematical development. The circular format emerged as a particularly strong predictor for typically hearing children. DHH children, especially those with sign language experience, perform equally well with both formats, and it is argued that this is due to their enhanced visuospatial skills. Full article

Hereditary sensorineural hearing loss (SNHL) represents a significant global public health burden. DFNX2, an X-linked form of non-syndromic SNHL, is caused by pathogenic variants in the POU3F4 gene. This study aimed to identify a novel POU3F4 mutation and characterize its functional consequences to elucidate the molecular pathogenesis of DFNX2. A three-generation Chinese family with X-linked deafness was recruited. Targeted next-generation sequencing was used to screen candidate variants, which were validated by Sanger sequencing for co-segregation analysis. Functional assays, including subcellular localization, dual-luciferase reporter assay, Western blotting, and homology modeling, were performed to assess the mutation’s effects. A novel frameshift mutation, c.670_673dupGGTA (p.(Asn225Argfs*2)), was identified and showed complete co-segregation with the deafness phenotype. The mutant protein exhibited cytoplasmic mislocalization, and dual-luciferase assays revealed a severe reduction in transcriptional activation capacity, whereas Western blot confirmed stable expression of the truncated protein. Structural modeling predicted the loss of both the POUS and POUH DNA-binding subdomains. Collectively, this study expands the mutational spectrum of POU3F4 and supports previously reported mechanisms underlying DFNX2 pathogenesis. Full article

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

Objectives: To characterize heterozygous pathogenic COCH variants in a French cohort with non-syndromic sensorineural hearing loss (NSHL) and assess genotype–phenotype correlations in autosomal dominant NSHL (DFNA9). Setting: National Reference Center for Genetic Hearing Loss, Necker–Enfants Malades Hospital, Paris, France. Methods: This retrospective observational study included 69 individuals from 20 unrelated families diagnosed with DFNA9 (2005–2025). All individuals underwent clinical and audiological evaluations and genetic testing via targeted COCH Sanger sequencing or next-generation sequencing (NGS) panels. Variants were interpreted according to ACMG guidelines. Audiometric profiles and vestibular data were collected. Results: Seven known pathogenic COCH variants were found in ten families, and ten novel likely pathogenic variants in the others. Variants in vWFA domains were associated with early or late onset, progressive, bilateral and symmetrical hearing loss. Three variants (p.Gln410Arg, p.Ile450Val, p.Cys542Arg) were associated with congenital or prelingual onset, an atypical DFNA9 presentation. Variants in the LCCL domain were associated with later-onset hearing loss and more frequent vestibular dysfunction. Vestibular abnormalities were observed in about half of early-onset cases. Conclusions:COCH-related hearing loss is a rare cause of autosomal dominant NSHL, with only 20 families identified over two decades within the French network. This study expands the mutational spectrum of COCH by reporting ten novel variants and supports a domain-specific genotype–phenotype correlation. These findings improve the understanding of DFNA9 variability and have direct implications for clinical diagnosis, prognosis, and genetic counseling. Full article

Background/Objectives: Individuals with listening difficulties (LiD) report problems understanding speech despite normal hearing sensitivity. This study investigated how speech characteristics (clarity, speaking rate, and loudness) influence subjective speech understanding in adults with LiD under quiet and noisy conditions, compared with individuals with normal hearing (NH) and those who are hard of hearing (HH). Methods: A questionnaire-based survey assessed perceived speech intelligibility across 12 listening conditions created by combining two environments (quiet and noisy) and six speaking styles. Participants included 77 adults with LiD (50 LiD without developmental disorders [DD] and 27 LiD and DD), 30 adults with NH, and 26 individuals with HH. Ratings were analyzed using analysis of variance and correlation analyses. Results: Both the LiD and HH groups demonstrated significantly reduced speech understanding in noisy environments when the speaking style was suboptimal, compared with the NH group. In contrast, the LiD group uniquely reported difficulties even under quiet conditions with clear, slow, and loud speech. Loud speech in quiet conditions was not consistently beneficial, particularly in the LiD and DD group, possibly reflecting auditory hypersensitivity. Conclusions: Listening difficulties in the LiD group are influenced by environmental and speaker-related factors and cannot be addressed solely by conventional strategies used for hearing loss. Although individualized communication approaches may be required, further investigation, including experimental studies, is necessary to validate this interpretation. Full article

Post-traumatic auditory and vestibular complaints are frequent after traumatic brain injury (TBI) and temporal bone trauma. They create particular difficulty in medico-legal practice because the evaluator must distinguish diagnosis, functional impact, plausibility of traumatic causation, and the credibility of reported deficits and/or symptoms. This manuscript is a narrative expert review, not a systematic review or a validated forensic prediction rule. It aims to synthesize clinically relevant evidence and propose a practical cross-check framework for structured audio-vestibular assessment in post-traumatic and medico-legal contexts. Pure-tone audiometry remains the functional entry point, but it should be interpreted in conjunction with speech audiometry, tympanometry, acoustic reflexes, transient-evoked and distortion-product otoacoustic emissions, auditory brainstem responses, and auditory steady-state responses. Vestibular evaluation should combine videonystagmography, video head impulse testing, cervical and ocular vestibular evoked myogenic potentials, and computerized dynamic posturography, recognizing that each method interrogates different physiological domains and frequencies. Particular emphasis is placed on the separation between clinical diagnosis, physiological localization, functional impairment, and medico-legal attribution. The article also discusses safeguards against false-positive attribution of malingering, the time course after TBI, inter-rater variability, and the role of specialist expertise in medico-legal reporting. The proposed framework does not eliminate uncertainty; rather, it is intended to make expert reasoning transparent, cautious, internally consistent, and defensible. Full article

Aims: The aim of this study was to investigate the associations between symptomatic hearing loss (HL), neuropathy, and nephropathy in subjects with Type 2 diabetes mellitus (T2DM). Furthermore, the study evaluated whether HL was associated with chronic low-grade inflammation, assessed based on plasma levels of tumour necrosis factor-alpha (TNF-α), interleukin-6 (IL-6), and high-sensitivity C-reactive protein (hsCRP), and explored potential sex-specific differences. Materials and Methods: We included 4245 subjects with T2DM from The Danish Centre for Strategic Research in Type 2 Diabetes cohort. Symptomatic HL was defined using ICD-10 codes. In 2016, a questionnaire was sent out to evaluate neuropathy using the Michigan Neuropathy Screening Instrument (MNSI ≥ 4). Nephropathy was defined as urinary albumin-to-creatinine ratio (UACR) >30 mg/g. Plasma levels of TNF-α, IL-6, and hsCRP were measured at enrolment from 2010 to 2016. Multivariable logistic regression was used, adjusting for covariates. Results: Neuropathy was significantly associated with HL (OR = 1.83, 95%CI [1.42, 2.35], p < 0.001), and the association was stronger in women (OR = 2.74 [1.81, 4.14], p < 0.001) compared to men (OR = 1.44 [1.04, 1.99], p < 0.05) (P-interaction = 0.020). No significant association was found between nephropathy and HL. Among inflammatory markers, only the highest tertile of TNF-α levels was significantly associated with HL compared to the lowest tertile (OR = 1.40 [1.07, 1.82], p < 0.05) without any sex interaction. Conclusions: In subjects with T2DM, neuropathy was associated with symptomatic HL, and the association seemed to be stronger in females. Among chronic low-grade inflammation markers, only TNF-α was significantly associated with symptomatic HL. Additionally, no significant association was found between nephropathy and HL. Full article

Background/Objectives: Ménière’s disease (MD) is a chronic inner-ear disorder characterized by vertigo, tinnitus, and fluctuating sensorineural hearing loss (SNHL) that impairs health-related quality of life (HRQoL) and is frequently accompanied by psychological distress. This study aims to quantify psychological distress and generic HRQoL in patients with definite MD compared with age- and sex-matched general-population controls and to examine the associations between hearing loss (HL) severity, symptom-related handicap, and these outcomes within the MD cohort. Methods: In this observational cross-sectional study, 45 adults with definite MD were recruited from an otolaryngology clinic in Portugal and individually matched (1:3) by sex and 10-year age strata to 135 participants from the EpiDoC population cohort. HRQoL was assessed using the SF-36v2 and EQ-5D-3L, psychological distress using the HADS, and tinnitus- and dizziness-related handicap using the THI and DHI. HL severity was staged using the AAO-HNS (1995) criteria. Results: Patients with MD had higher anxiety and depression scores and nearly twice the prevalence of abnormal anxiety compared with controls. SF-36v2 scores were significantly worse in several role and psychosocial domains, with less bodily pain but similar physical functioning and general health. EQ-5D-3L revealed a lower utility index and higher pain/discomfort and anxiety/depression. Within the MD cohort, HL stage was only weakly related to distress and HRQoL. In contrast, higher THI and DHI scores were consistently associated with more severe anxiety and depression and lower SF-36v2 scores. In multivariable analysis, abnormal anxiety was strongly associated with moderate-to-severe tinnitus handicap (OR = 17.5), whereas higher depressive symptoms independently predicted moderate-to-severe dizziness handicap, with abnormal anxiety showing a strong but borderline association with the latter. Conclusions: MD confers clinically meaningful HRQoL decrements and a higher psychological distress burden than in the general population. Tinnitus- and dizziness-related handicap and psychological distress, particularly anxiety, rather than HL severity, are key multidisciplinary management targets. Systematic screening for anxiety and depression, regardless of audiometric stage, should be integrated into MD care pathways. Full article

Background/Objectives: Functional near-infrared spectroscopy (fNIRS) has emerged as a non-invasive, implant-compatible imaging modality capable of capturing cortical hemodynamics during ecologically valid auditory and linguistic tasks. Its silent operation and tolerance to electrical artifacts make it particularly well suited to the study of hearing-impaired individuals, including cochlear implant (CI) users. However, evidence on the application of fNIRS to investigate speech perception, cognitive performance, and proxy of cortical activation patterns in patients with hearing loss (HL) remains fragmented. This systematic review aims to provide a structured, population-stratified description of current fNIRS literature on auditory and cognitive processing in adults with age-related hearing loss (ARHL) and CI users. Methods: A systematic search on PubMed Central, Web of Science and Scopus, based on PRISMA (2020) guidelines, was conducted to identify original studies that evaluate speech perception by means of fNIRS to assess auditory and cognitive process in hearing-impaired populations. Results: Across studies, fNIRS consistently detected activation of superior temporal and frontal cortices during speech-related tasks. In ARHL, increased dorsolateral prefrontal cortex (DLPFC) recruitment during speech-in-noise indicated compensatory yet inefficient processing. Longitudinal auditory training led to reduced prefrontal overactivation and enhanced temporal–frontal connectivity. In CI users, cortical responses to phonological and comprehension tasks show partially overlapping activation patterns with normal hearing (NH) peers, although arising within different neurobiological contexts, and are modulated by device experience and residual hearing (AV) speech, and stimulus-level effects further shape cortical responses. When interpreted in light of developmental evidence, these findings may be contextualized as reflecting distinct trajectories of cortical reorganization, rather than a common mechanism. Conclusions: fNIRS provides a tool to investigate auditory and cognitive responses in distinct hearing-impaired populations under ecologically valid conditions. It detects maladaptive frontal inefficiency in ARHL, tracks neuroplastic changes after rehabilitation, and captures population-specific cortical recruitment patterns in CI users. These findings are descriptive and context-dependent, and do not support cross-population mechanistic generalizations. Standardized protocols and longitudinal pediatric studies are needed to clarify the potential clinical relevance of fNIRS-derived cortical measures. Full article

Objectives: This study aims to evaluate burnout and job satisfaction levels among audiologists in Türkiye and the factors that contribute to them. Methods: The study included 152 female (59.80%) and 102 male (40.20%) audiologists aged 20–30 (n = 133, 52.40%), 31–40 (n = 82, 32.30%), and 41 and over (n = 39, 15.40%). Participants were required to have at least 1 year of experience and be an active audiologist. Analysis was performed using Structural Equation Modeling (SEM) and correlation statistics to assess the predictive relationship between burnout sub-dimensions and job satisfaction components. Results: The results showed that burnout and job satisfaction among audiologists differed according to demographic data. In Türkiye, audiologists reported high burnout and low job satisfaction. A significant relationship was found between burnout and job satisfaction. The exhaustion subscale of the Maslach Burnout Scale statistically significantly and negatively predicted the Job Satisfaction Scale scores (β = −0.35; p < 0.05). Furthermore, it was determined that audiologists working in implant centers experienced less burnout, while those working in rehabilitation centers had lower job satisfaction. Conclusions: In conclusion, addressing burnout levels among audiologists is expected to not only improve individual well-being but also contribute to the overall enhancement of hearing health services in Türkiye. Full article

Background/Objectives: Cisplatin-exposed pediatric cancer patients are at increased risk of ototoxicity, particularly those under 5 years of age. In this group, audiological monitoring is challenging, as interpretation of otoacoustic emissions (OAEs) and tympanometry, used to supplement behavioral audiometry, is subject to interindividual variability. This study aimed to identify key challenges and establish an international consensus on optimal testing procedures and interpretation criteria for assessing early cochlear damage. Methods: Audiological data from 11 children (<5 years) exposed to cisplatin in utero were evaluated. An international panel of 10 pediatric oncology audiology experts reviewed tympanometry, transient evoked OAEs (TEOAEs), and distortion product OAEs (DPOAEs). Areas of disagreement were analyzed, and consensus was sought regarding testing conditions, interpretation, and clinical application. Results: Agreement on cochlear status was good in 10/22 ears, moderate in 3/22 ears, and poor in 9/22 ears, highlighting substantial variability in interpretation. Consensus was achieved on minimal technical and interpretative requirements for OAE testing in this population. The panel proposes a classification framework integrating OAEs and tympanometry to guide clinical follow-up. Importantly, normal OAE results were not considered sufficient to exclude ototoxic damage or mild hearing loss. Conclusions: OAEs, particularly DPOAEs, are valuable as a screening tool in ototoxicity monitoring programs for young children, provided that testing conditions and interpretation are standardized. However, OAEs alone are insufficient for definitive assessment. Longitudinal monitoring and confirmatory testing with behavioral audiometry or ABR/ASSR remain essential. Further validation of the proposed classification system is warranted. Full article

Background: Sensorineural hearing loss represents a significant global health burden affecting over 1.5 billion individuals worldwide. Modern hearing aids, equipped with digital signal processing and smart connectivity features, constitute a cornerstone of neuro-sensory rehabilitation. However, the psychosocial impact of these assistive smart technologies on patient self-esteem remains incompletely characterized. Methods: A cross-sectional multivariate study was conducted with 245 participants, divided into three groups: normal-hearing controls (NH, n = 73), hearing-impaired patients using smart hearing aid technology (HA users, n = 86), and hearing-impaired patients not using hearing aid technology (HA non-users, n = 86). Self-esteem was measured using the Rosenberg Self-Esteem Scale (SES). Hearing disability and tinnitus severity were assessed with the Hearing Handicap Inventory for Adults (HHIA) and Tinnitus Handicap Inventory (THI), respectively. Data analysis included one-way ANOVA, Tukey’s HSD post hoc tests, Pearson correlations, and multivariate regression. Results: Hearing aid users showed significantly higher SES scores (35.41 ± 5.32) compared to non-users (22.99 ± 4.53; p < 0.001, Cohen’s d = 2.515). One-way ANOVA indicated highly significant differences among groups (F = 299.00, p < 0.001, η² = 0.712). SES was negatively correlated with HHIA (r = −0.573, p < 0.001) and THI (r = −0.443, p < 0.001), while HHIA and THI were strongly positively correlated (r = 0.729, p < 0.001). In multivariate analysis, HA use remained a strong independent predictor of self-esteem (β ≈ 11.9, p < 0.001), even after adjustment for age, sex, HHIA, and THI. Perceived hearing handicap was independently associated with lower self-esteem, whereas tinnitus severity was not a significant predictor in the fully adjusted model. The model explained approximately 65% of the variance in self-esteem scores. Conclusions: Smart hearing-aid use is strongly and independently associated with higher self-esteem in patients with sensorineural hearing loss. These results support the inclusion of modern audiological rehabilitation devices in comprehensive management strategies for long-term conditions and highlight psychosocial benefits that extend beyond hearing restoration. Full article

Background and Objectives: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection during pregnancy has raised concerns regarding possible fetal consequences, including potential effects on auditory system development. Although the current literature suggests that overt congenital hearing loss is uncommon among newborns exposed in utero, subtle cochlear functional alterations may not be detectable through conventional threshold-based screening alone. The objective of this study is to investigate whether in utero exposure to maternal COVID-19 is associated with early cochlear functional changes in newborns, as assessed by frequency-specific transient evoked otoacoustic emission (TEOAE) amplitude ratios, and to determine whether such alterations are accompanied by differences in click-evoked auditory brainstem response (ABR) thresholds. Materials and Methods: This prospective cohort study was conducted between October 2021 and September 2022 and included 61 pregnant women: 30 with laboratory-confirmed SARS-CoV-2 infection during pregnancy (study group) and 31 without documented infection (control group). All newborns underwent standardized audiological evaluation shortly after birth, including otoscopy, TEOAE, click-evoked ABR, and tympanometry. Frequency-specific TEOAE amplitude ratios at 500, 1000, 1500, 2000, and 4000 Hz were compared between groups. A logistic regression analysis was performed to identify audiological predictors of newborn exposure to SARS-CoV-2 in utero. Results: No significant differences were observed in ABR thresholds or in TEOAE “pass/refer” outcomes between the control and study groups, indicating the absence of clinically overt HL. However, newborns exposed to SARS-CoV-2 in utero showed significantly reduced TEOAE amplitude ratios at 2000 Hz (p = 0.0077) and 4000 Hz (p = 0.020). Logistic regression identified the 4000 Hz amplitude ratio as an independent negative predictor of in utero exposure (OR = 0.75; p = 0.0352). No significant differences were detected at lower frequencies. Conclusions: Maternal COVID-19 during pregnancy was not associated with immediate neonatal HL but was linked to subtle high-frequency cochlear functional modulation. Longitudinal audiological follow-up is needed to clarify the clinical significance of these findings. Full article

Vestibular schwannomas (VSs) are benign tumors of the vestibulocochlear nerve that often cause significant neurological and functional impairment, affecting patients’ overall quality of life (QoL). While clinical assessments have traditionally focused on hearing preservation and tumor control, patients often emphasize other critical symptoms such as dizziness, pain, cognitive difficulties and satisfaction with care. Therefore, patient-centered care that addresses the full range of patient experiences is essential. Despite its importance, patient-centered care in VS remains underexplored. This study will address this gap by psychometrically validating the German version of the Vestibular Schwannoma Quality of Life (VSQOL) Index, a newly developed QoL tool that includes key patient-centered domains. The primary objective is to validate the reliability and validity of the German VSQOL Index. The secondary aim is to assess VS patients’ experience of patient-centered care and its impact on their well-being. This multicenter, cross-sectional study will involve German-speaking VS patients from several clinical centers in Germany and Switzerland as well as an online cohort. Psychometric testing of the German VSQOL will include reliability assessments (e.g., Cronbach’s alpha, test–retest reliability), confirmatory factor analysis and convergent validity. In parallel, the study will assess patient-centered experiences of care using the EPAT questionnaire. Ethical approval has been obtained and all participants will be asked to provide written informed consent. The results will be shared through scientific publications and conferences, as well as with patient groups, in order to support improvements in clinical care. Full article

Introduction: The present study aimed to compare cortical auditory maturation, as reflected by P1 latency of cortical auditory evoked potentials (CAEPs), in children with congenital severe-to-profound sensorineural hearing loss rehabilitated with unilateral cochlear implants (CIs) or bilateral conventional hearing aids (HAs). Materials and Methods: Eighty-five children with congenital severe-to-profound sensorineural hearing loss were included in this retrospective comparative study. Participants were divided into two groups: unilateral CI users (n = 42) and bilateral HA users (n = 43). All children were fitted with their devices before 48 months of age and achieved aided free-field thresholds between 30 and 50 dB HL. CAEPs were recorded using the Fonix^® HEARLab System with speech stimuli (/m/, /g/, /t/) presented at 55 dB SPL. P1 latency values were measured and compared between groups using independent samples t-tests. Correlation analyses were performed to assess the relationship between duration of device use and P1 latency. Results: Eighty-five children were included (CI: n = 42; HA: n = 43). Mean P1 latency values did not differ significantly between groups for the /m/ stimulus (126.4 ± 29.13 ms vs. 126.4 ± 29.28 ms, p = 1.00), /g/ stimulus (106.5 ± 26.46 ms vs. 110.1 ± 29.49 ms, p = 0.55), or /t/ stimulus (114.7 ± 22.93 ms vs. 118.5 ± 27.19 ms, p = 0.48). Age at device fitting was comparable between groups (27.95 ± 9.10 vs. 26.88 ± 14.15 months, p = 0.68). The duration of device use was significantly longer in the HA group (48.02 ± 28.39 months) compared to the CI group (26.00 ± 15.92 months) (p < 0.001). Correlation analysis revealed no significant association between duration of device use and P1 latency for any stimulus (/m/: p = 0.28; /g/: p = 0.17; /t/: p = 0.09). Conclusions: When devices were fitted before 48 months of age and aided thresholds were optimized, unilateral cochlear implantation and bilateral conventional hearing aids showed comparable P1 latency values as an index of cortical auditory maturation. These findings suggest that early and adequate auditory stimulation may play an important role in supporting cortical auditory development in children with congenital hearing loss, although results should be interpreted within the context of individualized clinical management. Full article