Hearing Loss Research Articles

Objective The aim of the study was to prospectively evaluate the clinical audiological outcomes as well as patient satisfaction following implantation with the Osia®2 System. Methods This is a prospective clinical study was conducted at high volume academic center of patients who underwent implantation of the Cochlear Osia®2 System. Audiological outcomes included pre- and post-operative assessments of pure-tone audiometry, speech discrimination scores, and self-reported hearing difficulties questionnaire. Results The surgical procedure and subsequent healing process proceeded without complications. We observed statistically significant improvements in audibility and speech understanding with the test device, compared to pre-operative conditions. The average gain across frequencies from 0.25 to 6 kHz was 39.17 db. Speech discrimination scores also showed an increase in scores' average from 64 ± 8 pre-operatively to 100 ± 8 post-operatively (p < .001). Additionally, the number of hearing issues assessed by the APHAB scale in various acoustic environments showed a statically significant decrease postoperative. The Global Score improved from 68.2 ± 5.53 pre-operatively to 27.3 ± 4.26 post-operatively. Conclusions The Osia®2 System proves to be a safe and effective alternative for hearing rehabilitation in patients with conductive hearing loss (CHL), mixed hearing loss (MHL), or single-sided deafness (SSD).

We search the literature on data regarding the role of whole-brain radiation therapy on neurocognitive functions, hearing, and alopecia, through the use of hippocampal-, scalp-, and cochlea-sparing radiotherapy (RT). Prospective and retrospective studies with at least five patients were included in this analysis, following PRISMA recommendations. Eighteen works were selected for hippocampal sparing, published between 2015 and 2025 with 1736 patients. Neurocognitive functions were evaluated with a heterogeneous range of tests (mainly HVLT-R, TMT-A and TMT-B, Cowa test, and MME). For scalp-sparing RT, only three papers were selected, published between 2014 and 2015, for a total of 65 patients. There was an important heterogeneity in terms of scalp definitions, CTV prescriptions, used techniques and doses, and methods and scales adopted to evaluate the clinical efficacy of scalp-sparing RT. Regarding cochlea-sparing RT, no citation was selected. A meta-analysis could only be performed for the papers focusing on hippocampal-sparing procedures. Only nine papers meet the criteria, showing a high heterogeneity (chi-square = 229.96, df 8, p < 0.001, with the I2 index (96.52%) and the H2M index (27.75)). For this reason, we opted for random effect models DerSimonian Laird, maximum likelihood, and profile likelihood, which provided widely overlapping results. Although the data show an average protective effect of hippocampal avoidance on cognitive performance, the meta-analysis, based on the available studies, is unable to demonstrate its significance. A high heterogeneity in terms of hippocampal-, cochlear-, and scalp-sparing RT was registered as well as different and difficult to compare data. Our findings indicate the need for further studies to explore this issue.

Hearing loss (HL) is common in older adults and is associated with several adverse health outcomes. Although previous research has demonstrated a link between hearing impairment and depression, most studies have been cross-sectional or relied on a single baseline measure of hearing. To investigate the association between longitudinal, time-varying audiometric measures of hearing and incident depression in older adults. A secondary aim was to assess whether hearing aid use modifies this association over time in those with moderate to severe HL. We included 1,260 participants who underwent pure-tone audiometry at baseline, 18 months, and 36 months. Depression was defined using the CES-D-10 scale, with a cut-score of ≥ 8. Cox proportional hazards analyses were used to examine the link between hearing status (normal, mild HL, moderate/severe HL) and depression, adjusted for demographic, lifestyle, and clinical variables. Over a median follow-up of 7.3 years, participants with moderate to severe HL had a higher risk of incident depression (adjusted HR [aHR] 1.24; 95% CI 1.08-1.43, p < 0.01) compared with participants with normal hearing. Hearing aid use (≥ 6 hours self-reported use per day on average) in those with moderate to severe HL was associated with significantly reduced risk of incident depression (aHR 0.65; 95% CI 0.49-0.87, p < 0.01). Moderate to severe HL is a significant risk factor for incident depression among older adults. Hearing aid use attenuated this risk. Future research should investigate mechanistic pathways linking HL and mood disturbances.

Mitochondrial dysfunction, particularly involving energy metabolism, oxidative stress, and structural integrity, is recognized as a critical factor in the pathogenesis of hearing loss. However, evidence regarding a potential association between mitochondrial function and sudden sensorineural hearing loss (SSNHL) remains limited. This study aims to explore the potential causal relationship between mitochondrial biological function and SSNHL using Mendelian randomization (MR) analysis. We utilized genome-wide association study data on SSNHL from the FinnGen database, along with mitochondria-related genome-wide association study summary statistics from previously published studies. Inverse variance weighting served as the primary MR method, using genetic instruments associated with mitochondrial biological function to estimate their effects on SSNHL risk. Sensitivity analyses-including MR-Egger regression, MR-PRESSO, and leave-one-out analysis-were conducted to assess the robustness of the findings and to exclude potential biases. Three mitochondrial proteins were found to exhibit potential causal effects on SSNHL. MUL1 was identified as a potential risk factor, whereas HINT2 and GRPEL1 appeared to serve as potential protective factors. This study provides evidence supporting a potential causal role of mitochondrial biological function in SSNHL. The findings offer novel insights into the mechanistic underpinnings of SSNHL and emphasize the importance of mitochondrial pathways in its pathophysiology. These results may encourage otolaryngologists to consider targeting mitochondrial energy metabolism in the development of more effective clinical prevention and treatment strategies for SSNHL.

Estimates of speech intelligibility depend on the audibility of spectral bands in speech and the importance weight of each spectral band for speech recognition. In children, the transfer function that links estimates of speech intelligibility to recognition accuracy changes with age, indicating the need to incorporate developmental changes into clinical use of speech intelligibility metrics for children. Previous research has shown developmental shifts in how children use spectral cues to support speech recognition, but individual differences in band importance that form the basis for models of speech recognition have not been directly validated in children. The goal of the present study was to determine if children differ in band importance weights with age and, if such differences exist, determine how they relate to developmental changes in vocabulary and cognitive ability. Children (ages 6 to 13 years) with normal hearing (N = 42) completed a filtered word recognition task and a battery of vocabulary and cognitive assessments. Monosyllabic words and nonword auditory stimuli were filtered into six frequency bands that were centered on octave frequencies between 250 and 8000 Hz, each one octave wide. Each stimulus contained a pseudorandom combination of three out of six frequency bands, which children listened to and verbally repeated. Recognition accuracy was regressed against the presence or absence of each band across trials in a generalized linear mixed effects model to measure the contribution of each frequency band to speech recognition on an individual basis. Children also completed the Peabody Picture Vocabulary Test, the Wechsler Abbreviated Scale of Intelligence vocabulary task, the Psychology Experiment Building Language flanker and switcher tasks, and the Wechsler Abbreviated Scale of Intelligence matrix reasoning task. Changes in frequency importance weights with age were observed for bands centered on 2000 and 4000 Hz, with increasing weights for these bands in older participants. Frequency bands centered on 500, 1000, and 2000 Hz had higher weights for words than for nonwords. Interactions between age and stimulus type were not evident. None of the vocabulary and cognitive tasks predicted frequency importance weights after controlling for age. Flanker task performance was a significant predictor of recognition accuracy. Frequency band importance weights undergo developmental changes in children. Models used to predict speech recognition in children may need to be updated to account for these developmental changes; otherwise, speech intelligibility estimates will likely overestimate speech recognition accuracy. Additional work is needed to determine whether the developmental trajectory of frequency importance weights is affected by hearing loss in children who are hard of hearing.

This case report details a rare and severe presentation of refractory otomastoiditis caused by Mycobacterium abscessus in an immunocompetent pediatric patient who presented with a 1-month history of left ear pain, swelling, and fever unresponsive to antibiotics. Computed tomography imaging was suggestive of coalescent otomastoiditis, and she underwent urgent left mastoidectomy, subperiosteal abscess drainage, and myringotomy with tube insertion, followed by conventional antibiotic management. Despite these interventions, she remained symptomatic and cultures revealed M. abscessus 1 week later. Multidisciplinary management involved serial microdebridement and prolonged multidrug antimicrobial therapy with shared decision-making between otolaryngology, infectious diseases, and international experts. Pharmacological management was complicated by adverse effects including aminoglycoside-induced hearing loss, myelosuppression, and gastrointestinal intolerance requiring drug substitutions. Eighteen months after initial presentation, revision mastoidectomy, canaloplasty, mastoid obliteration, tympanoplasty, and ossiculoplasty were performed for the eradication of residual disease and reconstruction. At follow-up, there was no disease recurrence although left-sided moderate-to-severe mixed hearing loss persisted. This case highlights the importance of early recognition and coordinated medical-surgical interventions in atypical presentations of mastoiditis.

Vestibular schwannomas (VSs) treated with stereotactic radiosurgery (SRS) often show transient loss of central tumor enhancement. We hypothesized that this loss of enhancement may correlate with better tumor control on follow-up imaging. We evaluated 198 consecutive patients from a single center who met eligibility criteria. Tumor volumes on the pre-SRS and post-SRS scans were quantified along with regions of loss of enhancement on the first post-SRS study. The latter was correlated with tumor volumes at follow-up, radiation isocenter density, and loss of serviceable hearing. For the entire cohort, the median loss of enhancement on the first post-SRS scan was 14% (IQR: 6 to 25) and median rate of change in tumor volume was -4.5% per year (IQR: -10.5 to 1.5). Percent loss of central enhancement showed significant negative correlation with tumor growth not only for the entire cohort (correlation coefficient: -0.18, P=0.01), but also for the subset of patients who had available imaging until at least 2 years (n=185) and 4 years (n=179) post-SRS. Loss of enhancement also strongly correlated positively with tumor volumes and number of radiation isocenters and negatively with isocenter density. Finally, patients with greater percent loss of enhancement had an increased likelihood of progression to non-serviceable hearing, although this association was not statistically significant. These findings suggest that loss of central tumor enhancement in VSs post-SRS may be associated with improved tumor control.

The disability weight quantifies the severity of health states from diseases and injuries. It is a fundamental index to estimate the disability-adjusted life year in the Global Burden of Disease studies. Disability weight estimates have been shown to vary across different national populations, suggesting the influence of cultural differences. However, survey data of disability weights in the Global Burden of Disease study is still limited worldwide. To more accurately reflect the true health conditions of global populations, this study aims to systematically summarize the disability weight values from international authoritative surveys, and explore the influential factors of disability weight estimates. Based on the Global Burden of Disease study, surveys used paired comparison questions wherein respondents considered two hypothetical individuals with different health states and specified which person was healthier. This study comprehensively searched multiple databases, including PubMed, Web of Science, Science Direct, and Google Scholar. We identified disability weight studies that utilized the paired comparison method and were conducted in national populations, published in international peer-reviewed journals. A meta-regression analysis was conducted to estimate the overall summary effect of disability weight values for 235 unique health states. These health states were estimated for all non-fatal consequences of disease and injury, including infectious diseases, cancer, cardiovascular diseases, diabetes, chronic respiratory diseases, neurological disorders, mental, behavior, and substance use disorders, hearing and vision loss, musculoskeletal diseases, injuries and others. Heterogeneity was assessed using the I2 statistics. Univariate meta-regression analysis was conducted to explore the impact of age, sex, education, population composition, and survey regions, respectively, on the summarized effect of each health state. The total analysis sample consisted of 610,818 respondents from the Global Burden of Disease 2013 disability weight surveys, the Japanese disability weight survey, and the Chinese disability weight survey. The summarized disability weights of health states ranged from mild anaemia (summarized disability weight = 0.008, 95% uncertainty interval 0.001-0.016, I2 = 0.95) to heroin and other opioid dependence (moderate to severe) (summarized disability weight = 0.737, 0.651-0.823, I2 = 0.823). Pearson correlation analysis showed that high correlation was observed between the set of overall summary disability weights of 235 health states from this meta-analysis and those from all included disability weight studies (all Pearson's r > 0.9, P < 0.001). Univariate meta-regression analysis indicated that age, sex, education level, panel composition of survey populations, and the survey regions were associated with the summarized disability weights of some health states. The overall summary of disability weights obtained from this meta-analysis is reliable. This study indicates that respondents' sociodemographic characteristics may impact a population's preference for health states, which should be considered in future disability weight assessments.

Age-related hearing loss (HL) is an increasing contributor to the global burden of disease in aging populations. Its interactions with major chronic diseases remain insufficiently characterized. We analyzed data from the Global Burden of Disease 2021 study for adults aged ≥ 45 years. YLDs for HL, diabetes, cardiovascular diseases (CVD), stroke, and depressive disorders were examined from 1990 to 2021. Data were stratified by age, sex, and region. Multivariate regression models and Pearson correlations were applied to evaluate associations, and a linear forecast projected HL burden to 2030. Visualizations included temporal trends, boxplots, and correlation heatmaps. Between 1990 and 2021, HL YLDs increased from 28.7million to 67.9million (+ 136.7%). Diabetes (+ 311.9%), CVD (+ 123.8%), and stroke (+ 112.6%) also rose substantially. Regression analysis indicated significant associations between HL and stroke (β = 1.798, p = 0.0010) and depression (β = 0.218, p < 0.001), whereas diabetes showed no independent effect and CVD displayed a negative relationship (β = -1.374, p = 0.0016). Correlation analyses revealed regional clustering, with HL aligning more closely with stroke than with diabetes or CVD. Forecasting projected HL YLDs to reach 75.6million by 2030, underscoring its growing impact. HL has become a major global health challenge in aging populations, strongly linked to multimorbidity, particularly stroke and depression. Integrating auditory health into chronic disease frameworks and healthy aging strategies is essential to reduce future disease burden.

BackgroundNo vaccine is currently available for Lassa fever, a viral hemorrhagic disease that is estimated to cause thousands of deaths each year in western Africa. A replication-competent recombinant vesicular stomatitis virus–vectored vaccine encoding a Lassa virus (LASV) glycoprotein complex, rVSVΔG-LASV-GPC, has been developed, but data on its safety and immunogenicity are limited.MethodsIn this phase 1, double-blind trial conducted in the United States and Liberia, we randomly assigned healthy adults (18 to 50 years of age) to receive rVSVΔG-LASV-GPC or placebo intramuscularly. Participants received a single vaccine dose of 2×104 plaque-forming units (PFU), 2×105 PFU, 2×106 PFU, or 2×107 PFU or placebo or received two vaccine doses of 2×107 PFU or placebo, within a window of 6 to 20 weeks. The side-effect profile was assessed according to the incidence of solicited and unsolicited adverse events (primary end point). Because Lassa fever can cause sensorineural hearing loss, hearing acuity was measured before and after the injection. Secondary end points were levels of binding antibodies against LASV glycoprotein, neutralizing antibodies, and vaccine vector–derived viral RNA and PFU in plasma, urine, and saliva.ResultsA total of 114 adults were enrolled. No serious vaccine-related adverse events were reported. The vaccine caused minimal local reactions and dose-dependent, mild-to-severe early-onset systemic reactogenicity events that were transient. No hearing loss was detected. All doses induced robust long-lasting cellular and humoral (binding and neutralizing) responses that cross-reacted against common LASV lineages. No infectious vaccine virus particles were found in plasma, urine, or saliva.ConclusionsThe rVSVΔG-LASV-GPC vaccine resulted in transient local and systemic reactogenicity events but no hearing loss or serious adverse events. The vaccine had immunogenicity over a wide dose range in healthy adults in the United States and Liberia. (Funded by the Coalition for Epidemic Preparedness Innovations and the National Institute of Allergy and Infectious Diseases; ClinicalTrials.gov number, NCT04794218; Pan African Clinical Trials Registry number, PACTR2021106625781067.)

TSPEAR (chr. 21q22.3) encodes a protein involved in tooth development and is predominantly expressed in the enamel knot. Biallelic loss of function variants in TSPEAR cause ectodermal dysplasia, tooth agenesis and sensorineural hearing loss. However, the role of TSPEAR in auditory processes is unclear. This study aimed at better delineating the allelic and clinical spectrum of TSPEAR-associated disorders. We identified homozygous and compound heterozygous causative variants in TSPEAR [NM_144991.3] in 11 patients from seven families. Abnormalities in tooth number and shape (conical teeth and tooth agenesis with a variable number of missing teeth) were found in all affected individuals. Maxillary retrusion was present in 6/11. Manifestations in other ectodermal-derived organs were seen in a minority of patients. None of the individuals had hearing loss. We identified a total of 10 variants, of which seven have not been previously published, and analyzed the effect of missense variants to support their pathogenicity. Our results demonstrate that individuals with biallelic variants in TSPEAR show complete penetrance for dental manifestations, but not for other ectodermal abnormalities. TSPEAR-related disorder is more common than previously thought, while hearing loss is not a feature of the disease.

Live music events expose attendees to unsafe noise levels associated with noise-induced hearing loss. High-fidelity earplugs (HFEs), a convenient alternative to other hearing protection modalities (foam, moldable earplugs, etc.), provide greater protection without impacting sound quality. This study examines the otologic benefits and factors associated with HFE use, including hearing-related beliefs, exposure history, and symptoms, compared with other hearing protection (OHP) users. Cross-sectional survey. Participants in online music communities (n=2352) were queried. Data were characterized through descriptive statistics, paired t tests, Pearson χ2, and multivariable logistic regression. HFEs were the most used modality among hearing protection users (57%). Relative to OHP users, HFE users were more concerned about the impact of music venues on their hearing and more often cited previous post-event hearing-related symptoms and maintained music quality as justifications for using HFEs (all P<0.001). HFE users less often reported difficulty hearing others, difficulty hearing high-pitched sounds, and dizziness or loss of balance (all P<0.05). In a multivariable logistic regression controlling for demographic and attendance characteristics, HFE users were more likely to have a history of diagnosed hearing problems (OR=2.71, 95% CI: 1.83-4.00) and experience symptom resolution [2.38 (1.67-3.40)]. Increased annual event attendance was positively associated with HFE usage [1.04 (1.02-1.07)]. Relative to EDM events, country [0.51 (0.27-0.96)], hip-hop/rap [0.58 (0.39-0.86)], and pop [0.40 (0.27-0.60)] event attendance decreased use likelihood. HFE users more often reported improved post-event otologic symptomology while maintaining music enjoyment. Increased awareness of HFEs may encourage healthier hearing habits among music venue attendees. Level 4.

Background D-bifunctional protein deficiency (D-BPD) is a rare fatal autosomal recessive peroxisomal disorder caused by biallelic pathogenic mutations in the hydroxysteroid 17-beta dehydrogenase 4 ( HSD17B4 ) gene; it is characterized by hypotonia, seizures, and facial dysmorphisms during the neonatal period. Case presentation In this report, we describe a female neonate from China who was diagnosed with D-BPD. The patient presented with neonatal asphyxia, hypotonia, weak reflexes, and feeding difficulty after birth. Seizures occurred on the fifth day of life and were initially treated with phenobarbital. However, the seizures reoccurred and became more difficult to control because of their increased frequency, duration, and anticonvulsive drug resistance. Whole-genome sequencing (WGS) revealed novel compound heterozygous mutations c.1145G&amp;gt;A(p.Gly382Asp)/c.1193C&amp;gt;G(p.Ser398*) in exon 13 of the HSD17B4 gene, which was confirmed by parental Sanger sequencing. Neither variant has been reported previously. Very-long-chain fatty acid (VLCFA) testing revealed markedly elevated levels of hexacosanoic acid (C26:0), tetracosanoic acid/docosanoic acid (C24:0/C22:0), and C26:0/C22:0. The patient was managed with formula nasogastric feeding and antiepileptic therapy. At 7 months of age, she demonstrated severe psychomotor retardation, inability to grasp and manipulate objects, no language development, hearing loss, and poor visual response. Conclusion We described the incidence of D-BPD in a Chinese neonate caused by novel biallelic pathogenic variants in HSD17B4 , which expands its mutational spectrum.

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.

Kelch-like protein 11 (KLHL11) encephalitis is a rare clinical condition characterized by autoimmune-mediated encephalitis associated with the presence of KLHL11 antibodies, which commonly presents with ataxia, diplopia, vertigo, hearing loss, and seizures. While the link between this high-risk paraneoplastic autoantibody and male testicular germ cell tumors is well-established, female cases are rare, and no case of ductal breast carcinoma or dual primary malignancies have been reported. The disease is generally resistant to conventional therapies. In this study, we reported a case of KLHL11 encephalitis in a female patient presenting with fever, seizures, and ataxia, alongside dual primary malignancies: ductal breast carcinoma and pulmonary adenocarcinoma. Following immunotherapy with a neonatal Fc receptor (FcRn) inhibitor (efgartigimod) and resection of tumors, the patient achieved complete symptomatic remission with no recurrence. Current studies showed that KLHL11 encephalitis contributed to pathogenesis through cytotoxic T-cell-mediated neuronal injury and loss. However, in this case, rapid clinical improvement was observed after FcRn inhibitor therapy. This is the first report of FcRn inhibitor in the treatment of KLHL11 encephalitis. This case demonstrated a rare association of KLHL11 encephalitis with breast and lung cancer, and expanded on the clinical manifestations, associated tumor types, and treatment options of KLHL11 encephalitis in women.

Low-Level Laser Therapy and Photobiomodulation for Tinnitus and Sudden Sensorineural Hearing Loss: A Systematic Review

18p deletion syndrome is a rare chromosomal disorder that can present with a wide range of phenotypic features and is occasionally associated with autoimmune diseases. We report the case of a 3-year and 8-month-old girl who presented with polydipsia and polyuria and was subsequently diagnosed with type 1 diabetes mellitus (T1DM) based on clinical and laboratory findings. The patient exhibited dysmorphic facial features and developmental delay, leading to genetic testing, which revealed a 13.7 Mb deletion on the short arm of chromosome 18 (18p11.32p11.21). Over the following years, she developed additional features, including Hashimoto's thyroiditis, epilepsy, subaortic stenosis requiring surgical resection, IgA deficiency, bilateral sensorineural hearing loss, and myopia. Genetic analysis also identified the deletion of several potentially disease-modifying genes, including PTPN2, PTPRM, LPIN2, USP14, and ADCYAP1. This case highlights the potential role of genes within the 18p region in the pathogenesis of autoimmune endocrinopathies. It supports further investigation into the immunogenetic mechanisms in 18p deletion syndrome.

In utero acquisition of cytomegalovirus (CMV) represents the most common infectious cause of paediatric developmental disability. With a global prevalence of approximately 0.7%, congenital CMV (cCMV) infection can produce wide-ranging injury to the developing fetal and neonatal central nervous system, leading to microcephaly, intracranial calcifications, neuronal migration defects and damage to the developing cochlea and retina. Clinical sequelae include cerebral palsy, seizure disorder, intellectual disabilities, developmental delay, autism spectrum disorders, sensorineural hearing loss (SNHL) and visual impairment. It has been generally believed that most cCMV infections are asymptomatic in nature, and are not associated with long-term neurodevelopmental impairment. This dogma, however, has been called into question in the context of several state and provincial universal cCMV screening programmes that have been implemented in recent years in the United States and Canada. Moreover, the full spectrum of neurodevelopmental sequelae amongst asymptomatic cCMV cases is just starting to be recognized. Host and/or viral factors that predict which asymptomatic infants will have sequelae, including SNHL, are unknown. This review summarizes the current state of the art with respect to the search for predictive biomarkers that can inform the prognosis of asymptomatic cCMV, and aid in decision-making about therapeutic intervention.This article is part of the discussion meeting issue 'The indirect effects of cytomegalovirus infection: mechanisms and consequences'.

Age-related hearing loss is a potentially modifiable risk factor for cognitive impairment and dementia. To investigate the association of hearing loss with brain structure changes, cognitive function, and incident dementia. This cohort study included Framingham Heart Study Offspring Study participants attending their sixth quadrennial examination (1995-1998) divided into 2 partially overlapping samples. Sample 1 participants underwent brain magnetic resonance imaging and cognitive assessment at their seventh and eighth quadrennial examinations. Sample 2 included participants aged 60 years or older at pure tone average (PTA) analysis and subsequently followed up for incident dementia. The data analysis was performed between January 12, 2024, and August 24, 2025. Hearing loss. The main outcomes were changes in brain volume on MRI, cognitive performance on neuropsychological testing, and incident all-cause dementia. Multivariable linear regressions were used to compare PTA and hearing loss categories with neuropsychological measures and total cerebral, hippocampal, and white matter hyperintensity brain volumes. Cox proportional hazards regression was used to examine longitudinal associations between PTA and hearing loss categories and incident dementia. Sample 1 included 1656 participants (mean [range] age, 58.1 [29.7-85.6] years; 903 female [54.5%]), and sample 2 included 935 participants (mean [range] age, 67.6 [60.0-85.6] years; 518 female [55.4%]). Participants with mild or greater hearing loss, compared with those with no or only slight hearing loss, had significantly smaller brain volumes (β [SE], -4.10 [1.76]) and declines in executive function (β [SE], -0.04 [0.01]). Participants with at least slight hearing loss, compared with those with no hearing loss, had significant increases in white matter hyperintensity volume (β [SE], 0.02 [0.01]) and a higher risk of developing all-cause dementia (hazard ratio, 1.71; 95% CI, 1.01-2.90) over 15 years of follow-up, particularly among those with at least 1 apolipoprotein E ε4 allele (hazard ratio, 2.86; 95% CI, 1.12-7.28). This cohort study found that midlife hearing loss was associated with smaller brain volumes, accelerated worsening in executive function, and an increased risk of dementia, suggesting that it may be an early marker of brain aging and dementia risk.

This study explored genetic and drug-induced hearing loss by focusing on the m.827A>G variation of the MT-RNR1 gene. In particular, we investigated the variant's frequency, its association with hearing loss, and its potential interaction with gentamicin-induced damage within the Taiwanese adult population. The study included 59,091 participants from the Taiwan Precision Medicine Initiative dataset. We examined the relationship between m.827A>G variant carriers, age, gentamicin exposure, and sensorineural hearing loss. Pure-tone audiometry assessed hearing thresholds and severity, while genetic analysis determined the mutation frequency. Phenome-wide association studies established connections between the variant and clinical diagnoses. Genotyped from 58,091 Taiwanese adults, the m.827A>G variant minor allele frequency was 4.49%. Analyzing data from 186 carriers included age, sex, and audiograms. The carriers of m.827A>G variant who had been exposed to gentamicin did not display significant hearing level distinction. PheWAS analysis was conducted and confirmed a significant association between the variant and hearing loss. This study confirms the association between the m.827A>G variant and hearing loss, while suggesting that its role in gentamicin-induced ototoxicity may be limited.