The neurological core features of the infantile-onset multisystem neurologic, endocrine, and pancreatic disease: A novel nonsense mutation in an Italian family.

AIM: Biallelic mutations in the PTRH2 gene have been associated with infantile multisystem neurological, endocrine, and pancreatic disease (IMNEPD), a rare autosomal recessive disorder of variable expressivity characterized by global developmental delay, intellectual disability or borderline IQ level, sensorineural hearing loss, ataxia, and pancreatic insufficiency. Various additional features may be included, such as peripheral neuropathy, facial dysmorphism, hypothyroidism, hepatic fibrosis, postnatal microcephaly, cerebellar atrophy, and epilepsy. Here, we report the first Italian family presenting only predominant neurological features. METHODS: Extensive neurological and neurophysiological evaluations have been conducted on the two affected brothers and their healthy mother since 1996. The diagnosis of peripheral neuropathy of probable hereditary origin was confirmed through a sural nerve biopsy. Exome sequencing was performed after the analysis of major neuropathy-associated genes yielded negative results. RESULTS: Whole-exome sequencing analysis identified the homozygous substitution c.256C>T (p.Gln86Ter) in the PTRH2 gene in the two siblings. According to American College of Medical Genetics and Genomics (ACMG) guidelines, the variant has been classified as pathogenic. At 48 years old, the proband's reevaluation confirmed a demyelinating sensorimotor polyneuropathy with bilateral sensorineural hearing loss that had been noted since he was 13. Additionally, drug-resistant epileptic seizures occurred when he was 32 years old. No hepatic or endocrinological signs developed. The younger affected brother, 47 years old, has an overlapping clinical presentation without epilepsy. INTERPRETATION: Our findings expand the clinical phenotype and further demonstrate the clinical heterogeneity related to PTRH2 variants. We thereby hope to better define IMNEPD and facilitate the identification and diagnosis of this novel disease entity.

Association of sudden sensorineural hearing loss with meteorological factors: a time series study in Hefei, China, and a literature review.

Air pollution can cause disease and has become a major global environmental problem. It is currently believed that air pollution may be related to the progression of SSNHL. As a rapidly developing city in recent years, Hefei has serious air pollution. In order to explore the correlation between meteorological variables and SSNHL admissions, we conducted this study. This study investigated the short-term associations between SSNHL patients admitted to the hospital and Hefei climatic variables. The daily data on SSNHL-related hospital admissions and meteorological variables containing mean temperature (T-mean; °C), diurnal temperature range (DTR; °C), atmospheric pressure (AP; Hp), and relative humidity (RH; %), from 2014 to 2021 (2558 days), were collected. A time-series analysis integrating distributed lag non-linear models and generalized linear models was used. PubMed, Embase, Cochrane Library, and Web of Science databases were searched. Literature published up to August 2023 was reviewed to explore the potential impact mechanisms of meteorological factors on SSNHL. The mechanisms were determined in detail, focusing on wind speed, air pressure, temperature, humidity, and air pollutants. Using a median of 50.00% as a baseline, the effect of exceedingly low T-mean in the single-day hysteresis effect model began at a lag of 8 days (RR = 1.032, 95% CI: 1.001 ~ 1.064). High DTR affected the admission rate for SSNHL on lag 0 day. The significance of the effect was the greatest on that day (RR = 1.054, 95% CI: 1.007 ~ 1.104) and then gradually decreased. High and exceedingly high RH affected the admission rate SSNHL on lag 0 day, and these effects lasted for 8 and 7 days, respectively. There were significant associations between all grades of AP and SSNHL. This is the first study to assess the effect of meteorological variables on SSNHL-related admissions in China using a time-series approach. Long-term exposures to high DTR, RH values, low T-mean values, and all AP grades enhance the incidence of SSNHL in residents. Limiting exposure to extremes of ambient temperature and humidity may reduce the number of SSNHL-related hospital visits in the region. It is advisable to maintain a suitable living environment temperature and avoid extreme temperature fluctuations and high humidity. During periods of high air pollution, it is recommended to stay indoors and refrain from outdoor exercise.

Diagnosis and Management of Unexplained Conductive Hearing Loss With Intact Tympanic Membrane: A Systematic Review.

Objective: To analyze the etiology, diagnosis, and treatment of unexplained conductive hearing loss (UCHL) with intact tympanic membrane. Methods: A systematic review was conducted based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A total of 642 articles were retrieved from databases such as PubMed, Embase, Web of Science, and Cochrane. Fifty-four research articles and 21 case reports were screened out according to the inclusion and exclusion criteria for analysis of the etiology of UCHL. Seven research articles with UCHL who underwent exploratory tympanotomy were selected for data extraction and analysis of clinical characteristics. Results: UCHL is a common manifestation of various diseases, including congenital ossicular anomalies (COA), otosclerosis (OTS), congenital middle ear cholesteatoma (CMEC), oval window atresia, superior semicircular-canal dehiscence, congenital stapedial footplate fixation, middle ear osteoma or adenoma, congenital ossification of stapedial tendon, and so on. A total of 522 patients were included in the 7 articles; among whom OTS showed a tendency to increase with age. The main symptoms were hearing loss, followed by tinnitus, dizziness, ear fullness, ear pain, facial paralysis. A total of 87.5% to 93.0% patients with COA manifested as nonprogressive deafness that occurred since childhood, with tinnitus incidence of 15.6% to 30.2%, and 86.4% to 96.4% patients with OTS presented with progressive hearing loss, with tinnitus incidence of 60.1% to 90.9%. The diagnosis positive rate of high-resolution computed tomography (HRCT) was 33.8% to 87.1%, and CMEC was higher than that of COA (83.3%-100% vs 28.6%-64%). All the articles reported good hearing recovery. The most common surgical complications included taste abnormalities, tinnitus, and dizziness. Conclusion: UCHL presents with similar clinical manifestations and poses challenges in preoperative diagnosis. Exploratory tympanotomy is the primary method for diagnosis and treatment, with good prognosis after removing the lesion and reconstructing hearing during the operation. Children can also safely undergo the surgery.

Etiology of Childhood Profound Sensorineural Hearing Loss: The Role of Hearing Loss Gene Panel Testing.

OBJECTIVE: Establishing the cause of hearing loss (HL) is important and rewarding, though not without its challenges. While our ability to identify the etiology for HL has improved with advances in scientific knowledge, a significant proportion of cases remain of unknown etiology. Recent protocol changes within the NHS Genomic Medicine Service support the utilization of the HL gene panel test, rather than individual gene tests. In light of these changes, determining the yield of these more extensive panel tests is important in informing future practice. STUDY DESIGN: Retrospective study. SETTING: The Cochlear Implant (CI) Department at Great Ormond Street Hospital (GOSH). METHODS: Four hundred seventy-six children with profound HL were identified from a database of referrals to the GOSH CI Department. Data on etiology of HL including genetic diagnosis was collected from hospital notes on an electronic patient records system and hospital genetics database. RESULTS: We identified a positive result in 163/476 (34%) cases through the gene panel test, representing an additional 19% yield to current level 1 investigations. Genetic HL, including both syndromic (including those not covered by the HL gene panel) and nonsyndromic (209/476, 44%) was the most common etiology in our cohort. Perinatal, intrauterine, ototoxicity, meningitis, and encephalitis categories altogether comprised 97/476 (20%) cases. CONCLUSION: Gene panel testing provides significant additional yield over current level 1 investigations which include GJB2 testing only. This has far-reaching implications for how we optimize investigations into HL in children and counsel families, and for future early interventions.

Hearing Loss: Self-Reported Onset and Etiology Among Older Adults in the United States.

This study investigated self-reported age of onset and etiology of hearing loss among older adults in the United States. Study cohort included older adult (≥70 years) survey respondents from the 2017 to 2020 National Health and Nutrition Examination Survey (n = 797). Overall, 51.1% [95% confidence interval [CI]: 46.1-56.1] of older adults self-reported hearing loss. Among older adults who reported hearing loss, the most reported age of onset was age 70 or older (41.7% [95% CI: 38.1%-45.3%]), followed by sequentially younger age brackets including ages 60 to 69 years (27.3% [95% CI: 23.6%-31.3%]) and ages 40 to 59 years (15.7% [95% CI: 12.9%-19.0%]). The most common etiology of hearing loss was aging (66.3% [95% CI: 60.8%-71.4%]) followed by loud long-term noise (30.3% [95% CI: 26.2%-34.9%]) and loud brief noise (13.8% [95% CI: 10.3%-18.4%]). Our study describes the most common age of onset and etiologies of hearing loss among a representative sample of United States older adults.

Comparative analysis of hearing loss caused by steady-state noise and impulse noise.

BACKGROUND: Varied noise environments, such as impulse noise and steady-state noise, may induce distinct patterns of hearing impairment among personnel exposed to prolonged noise. However, comparative studies on these effects remain limited. OBJECTIVE: This study aims to delineate the different characteristics of hearing loss in workers exposed to steady-state noise and impulse noise. METHODS: As of December 2020, 96 workers exposed to steady-state noise and 177 workers exposed to impulse noise were assessed. Hearing loss across various frequencies was measured using pure tone audiometry and distortion product otoacoustic emission (DPOAE) audiometry. RESULTS: Both groups of workers exposed to steady-state noise and impulse noise exhibited high frequencies hearing loss. The steady-state noise group displayed significantly greater hearing loss at lower frequencies in the early stages, spanning 1- 5 years of work (P < 0.05). Among individuals exposed to impulse noise for extended periods (over 10 years), the observed hearing loss surpassed that of the steady-state noise group, displaying a statistically significant difference (P < 0.05). CONCLUSION: Hearing loss resulting from both steady-state noise and impulse noise predominantly occurs at high frequencies. Early exposure to steady-state noise induces more pronounced hearing loss at speech frequencies compared to impulse noise.

Advances in the Study of Etiology and Molecular Mechanisms of Sensorineural Hearing Loss.

Sensorineural hearing loss (SNHL), a multifactorial progressive disorder, results from a complex interplay of genetic and environmental factors, with its underlying mechanisms remaining unclear. Several pathological factors are believed to contribute to SNHL, including genetic factors, ion homeostasis, cell apoptosis, immune inflammatory responses, oxidative stress, hormones, metabolic syndrome, human cytomegalovirus infection, mitochondrial damage, and impaired autophagy. These factors collectively interact and play significant roles in the onset and progression of SNHL. The present review offers a comprehensive overview of the various factors that contribute to SNHL, emphasizes recent developments in understanding its etiology, and explores relevant preventive and intervention measures.

Impact of cochlear implants use on voice production and quality.

Cochlear implant users experience difficulties controlling their vocalizations compared to normal hearing peers. However, less is known about their voice quality. The primary aim of the present study was to determine if cochlear implant users' voice quality would be categorized as dysphonic by the Acoustic Voice Quality Index (AVQI) and smoothed cepstral peak prominence (CPPS). A secondary aim was to determine if vocal quality is further impacted when using bilateral implants compared to using only one implant. The final aim was to determine how residual hearing impacts voice quality. Twenty-seven cochlear implant users participated in the present study and were recorded while sustaining a vowel and while reading a standardized passage. These recordings were analyzed to calculate the AVQI and CPPS. The results indicate that CI users' voice quality was detrimentally affected by using their CI, raising to the level of a dysphonic voice. Specifically, when using their CI, mean AVQI scores were 4.0 and mean CPPS values were 11.4 dB, which indicates dysphonia. There were no significant differences in voice quality when comparing participants with bilateral implants to those with one implant. Finally, for participants with residual hearing, as hearing thresholds worsened, the likelihood of a dysphonic voice decreased.

Causal association between type 2 diabetes mellitus and acute suppurative otitis media: insights from a univariate and multivariate Mendelian randomization study.

Background: Type 2 diabetes mellitus (T2DM) and hearing loss (HL) constitute significant public health challenges worldwide. Recently, the association between T2DM and HL has aroused attention. However, possible residual confounding factors and other biases inherent to observational study designs make this association undetermined. In this study, we performed univariate and multivariable Mendelian Randomization (MR) analysis to elucidate the causal association between T2DM and common hearing disorders that lead to HL. Methods: Our study employed univariate and multivariable MR analyses, with the Inverse Variance Weighted method as the primary approach to assessing the potential causal association between T2DM and hearing disorders. We selected 164 and 9 genetic variants representing T2DM from the NHGRI-EBI and DIAGRAM consortium, respectively. Summary-level data for 10 hearing disorders were obtained from over 500,000 participants in the FinnGen consortium and MRC-IEU. Sensitivity analysis revealed no significant heterogeneity of instrumental variables or pleiotropy was detected. Results: In univariate MR analysis, genetically predicted T2DM from both sources was associated with an increased risk of acute suppurative otitis media (ASOM) (In NHGRI-EBI: OR = 1.07, 95% CI: 1.02-1.13, P = 0.012; In DIAGRAM: OR = 1.14, 95% CI: 1.02-1.26, P = 0.016). Multivariable MR analysis, adjusting for genetically predicted sleep duration, alcohol consumption, body mass index, and smoking, either individually or collectively, maintained these associations. Sensitivity analyses confirmed the robustness of the results. Conclusion: T2DM was associated with an increased risk of ASOM. Strict glycemic control is essential for the minimization of the effects of T2DM on ASOM.

Maternally inherited diabetes and deafness with a variable presentation across three generations within a pedigree, South Africa.

Introduction: Maternally inherited diabetes and deafness (MIDD) is caused by the m.3243A>G pathogenic variant in maternally inherited mitochondrial DNA. Diabetes is prevalent in our setting; however, MIDD is rarely diagnosed. This study, undertaken in Pretoria, South Africa, highlights the variable presentation of MIDD in different patients within the same family. Case presentation: A 45-year-old man (proband) with hearing impairment was referred to the endocrine unit in July 2015 due to poor glycaemic control (HbA1c = 13%). His clinical and biochemical features were in keeping with MIDD. A genetic study of accessible maternal relatives was pursued. His mother had difficulty hearing and reportedly died from an unspecified cardiovascular cause. Two sisters with diabetes and deafness died of cardiac-related conditions. One nephew had diabetes (HbA1c = 7.7%), hearing loss and tested positive for m.3243A>G. A third sister tested positive for m3243A>G, but aside from bilateral mild hearing loss in higher frequencies, showed no other signs of target organ damage. Her daughter developed end-stage kidney failure necessitating a transplant, while her son had no biochemical abnormalities and was negative for m.3243A>G. Management and outcome: A multidisciplinary team managed and screened for complications of the patient and his maternal relatives. Proband died prior to genetic testing. Conclusion: Most MIDD patients initially present with symptoms of diabetes only, and it is probable that many cases remain undiagnosed. A high index of suspicion is necessary when encountering a family history of both diabetes and impaired hearing, and screening should be offered to the patient's maternal relatives. What the study adds: This study demonstrates the importance of proper assessment when evaluating a patient with diabetes and a family history of hearing loss.

Genetic analysis of 106 sporadic cases with hearing loss in the UAE population.

BACKGROUND: Hereditary hearing loss is a rare hereditary condition that has a significant presence in consanguineous populations. Despite its prevalence, hearing loss is marked by substantial genetic diversity, which poses challenges for diagnosis and screening, particularly in cases with no clear family history or when the impact of the genetic variant requires functional analysis, such as in the case of missense mutations and UTR variants. The advent of next-generation sequencing (NGS) has transformed the identification of genes and variants linked to various conditions, including hearing loss. However, there remains a high proportion of undiagnosed patients, attributable to various factors, including limitations in sequencing coverage and gaps in our knowledge of the entire genome, among other factors. In this study, our objective was to comprehensively identify the spectrum of genes and variants associated with hearing loss in a cohort of 106 affected individuals from the UAE. RESULTS: In this study, we investigated 106 sporadic cases of hearing impairment and performed genetic analyses to identify causative mutations. Screening of the GJB2 gene in these cases revealed its involvement in 24 affected individuals, with specific mutations identified. For individuals without GJB2 mutations, whole exome sequencing (WES) was conducted. WES revealed 33 genetic variants, including 6 homozygous and 27 heterozygous DNA changes, two of which were previously implicated in hearing loss, while 25 variants were novel. We also observed multiple potential pathogenic heterozygous variants across different genes in some cases. Notably, a significant proportion of cases remained without potential pathogenic variants. CONCLUSIONS: Our findings confirm the complex genetic landscape of hearing loss and the limitations of WES in achieving a 100% diagnostic rate, especially in conditions characterized by genetic heterogeneity. These results contribute to our understanding of the genetic basis of hearing loss and emphasize the need for further research and comprehensive genetic analyses to elucidate the underlying causes of this condition.

Clinical profile, otologic, and auditory outcomes in patients with Down syndrome.

INTRODUCTION AND OBJECTIVES: Even though the incidence has decreased in recent years, Down syndrome (DS) remains the most common chromosomal disorder today. Despite being a condition with multisystemic involvement, it often tends to affect the head and neck area, making it a frequent reason for consultation with pediatric otolaryngologists or otologists. The purpose of this work is to be one of the first in Spain to characterize and describe the pathology and therapeutic approach typically provided to these patients, analyzing the evolution from a clinical and auditory perspective. MATERIAL AND METHODS: We aim to analyze a sample of 16 pediatric patients recruited over the past 24 years, diagnosed with Down syndrome, and experiencing a wide range of diseases affecting the ear and its auditory function. RESULTS: 62.50% of the patients were women, whose main reason for seeking specialist care was acute and serous otitis media, accounting for 31.25%. These patients have an indication for treatment for various entities within the otological sphere that usually do not differ from those of a healthy child. However, the evolution and response to treatments can take on a torpid character due to the anatomical characteristics of the ears of these patients. CONCLUSIONS: Although the frequency of children with DS in the pediatric otolaryngologist's clinic is decreasing, these patients have a predisposition to ear diseases with auditory repercussions, with variable evolution depending on the disease and the child's intrinsic characteristics.

Modeling the Intelligibility Benefit of Active Noise Cancelation in Hearing Devices That Improve Signal-to-Noise Ratio.

The extent to which active noise cancelation (ANC), when combined with hearing assistance, can improve speech intelligibility in noise is not well understood. One possible source of benefit is ANC's ability to reduce the sound level of the direct (i.e., vent-transmitted) path. This reduction lowers the "floor" imposed by the direct path, thereby allowing any increases to the signal-to-noise ratio (SNR) created in the amplified path to be "realized" at the eardrum. Here we used a modeling approach to estimate this benefit. We compared pairs of simulated hearing aids that differ only in terms of their ability to provide ANC and computed intelligibility metrics on their outputs. The difference in metric scores between simulated devices is termed the "ANC Benefit." These simulations show that ANC Benefit increases as (1) the environmental sound level increases, (2) the ability of the hearing aid to improve SNR increases, (3) the strength of the ANC increases, and (4) the hearing loss severity decreases. The predicted size of the ANC Benefit can be substantial. For a moderate hearing loss, the model predicts improvement in intelligibility metrics of >30% when environments are moderately loud (>70 dB SPL) and devices are moderately capable of increasing SNR (by >4 dB). It appears that ANC can be a critical ingredient in hearing devices that attempt to improve SNR in loud environments. ANC will become more and more important as advanced SNR-improving algorithms (e.g., artificial intelligence speech enhancement) are included in hearing devices.

Higher exposure to 1,3-butadiene is associated with more severe hearing loss.

While volatile organic compounds (VOCs) impair various organs, their influence on hearing loss (HL) has not been extensively researched. We aimed to identify the association between VOCs and HL or high-frequency hearing loss (HFHL). We extracted data on age, sex, pure tone audiometry, hypertension, occupational noise exposure, and creatinine-corrected urine VOC metabolite concentrations from the eighth Korea National Health and Nutrition Survey. Among the VOC metabolites, N-acetyl-S-(benzyl)-L-cysteine (BMA, P = 0.004), N-acetyl-S-(phenyl)-L-cysteine (SPMA, P = 0.027), and N-acetyl-S-(3,4-dihydroxybutyl)-L-cysteine (DHBMA, P < 0.001) showed associations with HL. Additionally, HFHL exhibited significant associations with BMA (P = 0.005), 3- and 4-methylhippuric acid (3, 4 MHA, P = 0.049), mandelic acid (MA, P = 0.015), SPMA (P < 0.001), N-acetyl-S-(3-hydroxypropyl)-L-cysteine (3-HPMA, P < 0.001), and DHBMA (P < 0.001). After controlling other factors, DHBMA were associated with HL (P = 0.021) and HFHL (P = 0.014) and exhibited a linear association with the mean hearing level (ß = 0.054, P = 0.024) and high-frequency hearing level (ß = 0.045, P = 0.037). Since 1,3-butadiene may act as an ototoxic material, early screening for workers exposed to 1,3-butadiene and reducing exposure to 1,3-butadiene in everyday life may be helpful to prevent further HL.

Do Surgical Smoke Evacuators Increase the Risk of Hearing Loss for Operative Personnel During Routine Adenotonsillectomy Surgery?

INTRODUCTION: Aerosol mitigation equipment implemented due to COVID-19 has increased noise levels in the operating room (OR) during otolaryngological procedures. Intraoperative sound levels may potentially place personnel at risk for occupational hearing loss. This study hypothesized that cumulative intraoperative noise exposures with aerosol mitigation equipment exceed recommended occupational noise exposure levels. METHODS: Sound levels generated by the surgical smoke evacuator (SSE) during adenotonsillectomy were measured using a sound level meter and compared to surgery without SSE. RESULTS: Thirteen adenotonsillectomy surgeries were recorded. Mean sound levels with the SSE were greater than the control (72 ± 3 A-weighted decibels (dBA) vs. 68 ± 2 dBA; p=0.015). Maximum noise levels during surgery with SSE reached 82 ± 3 dBA. CONCLUSION: Surgeons performing adenotonsillectomy with aerosol mitigation equipment are exposed to significant noise levels. Intraoperative sound levels exceeded international standards for work requiring concentration. Innovation is needed to reduce cumulative OR noise exposures.

Four-Hour-Delayed Gadolinium 3D REAL IR and SPACE FLAIR MRI Correlated to Meniere Disease Histology.

Objectives: This study aims to showcase the complementary nature of utilizing both histopathology and magnetic resonance imaging (MRI) in understanding the otologic pathophysiology of Meniere disease. In addition, it seeks to raise awareness of the value of preserving and curating historical temporal bone collections which continue to inform our understanding of otologic diseases. Methods: The essential anatomical feature of Meniere disease-the distended membranous labyrinth-is explored through a comparison of early temporal bone studies with contemporary MRI techniques. The histopathologic photomicrographs are of inner ear specimens from deceased patients with symptoms consistent with Meniere disease. The MRI sequences from living patients exhibiting classic Meniere disease symptoms during life are captured 4 hours post-administration of gadolinium. Results: Both histopathologic examination and MRI imaging reveal consistent distention of the saccule, utricle, and scala media in patients with Meniere disease. The study shows the histologic photomicrographs of actual Meniere patients compared to the MRIs and successfully demonstrates the correlation between postmortem histological findings and MRI evidence of distension in living patients. Conclusions: A corresponding distension of the membranous labyrinth is seen in both the histologic specimens and the Meniere MRIs. This correlation suggests the potential utility of utilizing MRI to aid in diagnosing atypical Meniere disease and distinguishing it from other disease processes, such as migraine equivalent vertigo. The integration of historical temporal bone studies with modern MRI techniques offers valuable insights into the pathophysiology of otologic diseases. In addition, it emphasizes the importance of preserving and curating historical temporal bone collections for continued research and medical education purposes. Previous studies of delayed MRIs did not use Meniere disease temporal bone histopathology images.

Association between the home-to-healthcare center distance and hearing aid abandonment among older adults.

Background: Access to audiology services for older adults residing in sparsely populated regions is often limited compared to those in central urban areas. The geographic accessibility to follow-up care, particularly the influence of distance, may contribute to an increased risk of hearing aid abandonment. Objective: To assess the association between the home-to-healthcare-calibration-center distance and hearing aid abandonment among older adults fitted in the Chilean public health system. Methods: 455 patients who received hearing aids from two public hospitals in two regions were considered. Univariate and multivariate Poisson regression models with robust variance estimation were used to analyze the association between the geographical distance and hearing aid abandonment, accounting for confounding effects. Results: Approximately 18% of the sample abandoned the hearing aid, and around 50% reported using the hearing aid every day. A twofold increase in distance between home and the hearing center yielded a 35% (RR = 1.35; 95% CI: 1.04-1.74; p = 0.022) increased risk of hearing aid abandonment. Also, those in the second quintile had a 2.17 times the risk of abandoning the hearing aid compared to the first quintile (up to 2.3 km). Under the assumption that patients reside within the first quintile of distance, a potential reduction of 45% in the incidence of hearing aid abandonment would be observed. The observed risk remained consistent across different statistical models to assess sensitivity. Conclusion: A higher distance between the residence and the healthcare center increases hearing aid abandonment risk. The association may be explained by barriers in purchasing supplies required to maintain the device (batteries, cleaning elements, potential repairs, or maintenance).

How to remain in working life with hearing loss - health factors for a sustainable work situation.

BACKGROUND: Persons with hearing loss (HL) are a vulnerable group in working life. Studies have shown that they are more likely than the general population to be in part-time work, to be unemployed, receive disability pension, and to be on sick leave. Many workers with HL also experience unhealthy work conditions, such as jobs where they experience high demands combined with low control as well as safety concerns and social isolation. There is a lack of studies that focus on factors that promote a healthy, sustainable work situation for the target group. OBJECTIVE: To investigate health factors that contribute to a sustainable work situation for employees with HL. METHODS: The current study was a comparative, observational study with a cross-sectional design including a clinical population of adults with HL. Comparisons were made between workers with HL "in work" and workers with HL on "HL-related sick leave". RESULTS: Seven health factors were identified. Those "in work" experienced a healthier work environment as well as lower levels of mental strain, hearing-related work characteristics and content, cognitively demanding work content, hearing-related symptoms, energy-demanding activities, and bodily aches and pain than those on "HL-related sick leave". CONCLUSION: The results demonstrate a clear pattern regarding health factors for a sustainable working life. The type of job was not related to whether an individual was on sick leave or working. Rather, the work climate and the content of the work mattered.

Application of self-assembly palladium single-atom nanozyme over polyoxometalates in protection against neomycin-induced hearing loss by inhibiting ferroptosis.

Deafness mainly results from irreversible impairment of hair cells (HCs), which may relate to oxidative stress, yet therapeutical solutions is lacked due to limited understanding on the exact molecular mechanism. Herein, mimicking the molecular structure of natural enzymes, a palladium (Pd) single-atom nanozyme (SAN) was fabricated, exhibiting superoxide dismutase and catalase activity, transforming reactive oxygen species (ROS) into O2 and H2O. We examined the involvement of Pd in neomycin-induced HCs loss in vitro and in vivo over zebrafish. Our results revealed that neomycin treatment induced apoptosis in HCs, resulting in substantial of ROS elevation in HEI-OC1 cells, decrease in mitochondrial membrane potential, and increase in lipid peroxidation and iron accumulation, ultimately leading to iron-mediated cell death. Noteworthy, Pd SAN treatment exhibited significant protective effects against HCs damage and impaired HCs function in zebrafish by inhibiting ferroptosis. Furthermore, the application of iron death inducer RSL3 resulted in notable exacerbation of neomycin-induced harm, which was mitigated by Pd administration. Our investigation demonstrates that antioxidants is promising for inhibiting ferroptosis and repairing of mitochondrial function in HCs and the enzyme-mimic SAN provides a good strategy for designing drugs alleviating neomycin-induced ototoxicity.

L-Shaped Associations Between Composite Dietary Antioxidant Index and Hearing Loss: A Cross-Sectional Study From the National Health and Nutrition Examination Survey.

BACKGROUND: Oxidative stress has been implicated in the pathogenesis of hearing loss (HL). Dietary intake is a modifiable factor that could influence the oxidant and antioxidant capacity. We hypothesized that a higher composite dietary antioxidant index (CDAI) is associated with a reduced odds for HL. METHODS: Adult participants from the 2001-2012 & 2015-2018 National Health and Nutrition Examination Study were included in this cross-sectional study. The CDAI was calculated from vitamins A, C, E, selenium, zinc, and caretenoid through 24-h dietary recall. Outcomes were HL, speech frequency HL (SFHL), and high frequency HL (HFHL). The associations between CDAI and HL, SFHL, and HFHL were evaluated by weighted multivariable logistic regression. RESULTS: CDAI was associated with lower odds of HL (OR = 0.98, 95%CI 0.95-1.00, p = .043) and SFHL (OR = 0.97, 95%CI 0.95-1.00, p = .041), but not HFHL (OR = 0.98, 95%CI 0.96-1.00, p = .118) after adjustment for confounders. The multivariable-adjusted model showed a significant trend toward decreased risk of HL, SFHL, and HFHL with increasing CDAI quartile (all p for trend < 0.05). Restricted cubic spline analysis suggested that the associations between CDAI and HL, SFHL, and HFHL were L-shaped, with inflection points of CDAI at -0.61, 2.33, and 4.32, respectively. Subgroup analysis showed that participants with exposure to loud noise benefited from a higher CDAI for SFHL (p for interaction = 0.039). CONCLUSION: Higher CDAI is associated with reduced odds of HL and SFHL in the U.S. adult population and serves as a promising intervention target to be further explored in prospective longitudinal studies in the future.