Risk Of Sensorineural Hearing Loss Research Articles

Management of Prenatal Expanded Genetic Carrier Screening Results for Autosomal Recessive Sensorineural Hearing Loss.

Expanded carrier screening (ECS) identified couples at-risk to have a baby with an autosomal recessive genetic condition. Several genes implicated in sensorineural hearing loss (SNHL) are included in prenatal or preconception genetics ECS testing. Early identification of SNHL risk may enable prognostication of hearing loss, early educational intervention, and minimization of unnecessary diagnostic testing. We sought to describe cases where ECS enabled early SNHL-risk identification. Retrospective chart review. Maternal-Fetal Care Center and Otolaryngology department at an academic tertiary hospital. Medical records of parent-infant dyads with positive ECS results for variants in autosomal recessive SNHL genes were reviewed. Data regarding genetic diagnostic testing, newborn hearing screening, time to HL diagnosis, audiological evaluation, and clinical consultations were compiled. Fifteen pregnant with positive ECS results for SNHL were referred for consultation with a pediatric otolaryngologist and genetic counselor. Generally, these couples were highly educated and adequately insured. 14 had pathogenic variants for GJB2 and 1 for USH2A. Four couples pursued prenatal genetic diagnosis via amniocentesis; 11 couples deferred genetic testing to the postnatal period or waited for initial hearing evaluation. Six babies inherited biallelic GJB2 mutations. Four were found to have SNHL on ABR by age 5 weeks and received follow-up management, 1 had a normal hearing evaluation despite being gene-positive, and 1 was lost to follow-up before hearing evaluation. Carrier screening and confirmatory prenatal or neonatal genetic testing provided considerable lead time for early audiometric testing and appropriate intervention services including hearing aid fitting.

The causal relationship between diabetes mellitus and the risk of sensorineural hearing loss: A Mendelian randomization study.

An increasing body of evidence suggests that diabetes mellitus (DM) plays a role in sensorineural hearing loss (SNHL). However, the specific causal relationship between DM and SNHL remains partially uncertain. This study aimed to investigate the causal relationship between DM and the risk of SNHL using a Mendelian randomization (MR) study. Single nucleotide polymorphisms closely related to DM were selected as instrumental variables using open genome-wide association study datasets. Three methods based on inverse variance weighted were utilized to investigate the causal relationship between DM and SNHL. Subsequently, multivariable MR (MVMR) was executed to adjust for confounding genetic associations. In addition, a range of sensitivity analyses were performed to assess the stability and reliability of the MR results. The inverse variance weighted analysis indicated a potential genetic causality between DM and SNHL (odds ratio [OR]: 2.179; 95% confidence interval [CI]: 1.123-4.231; P = .021). The sensitivity analyses showed that the included single nucleotide polymorphisms had no heterogeneity, horizontal pleiotropy, and outliers (P > .05). Moreover, the leave-one-out method further verified the robustness of the MR analysis results. Finally, the results of the MVMR study predicted that there was a genetic causal relationship between type 1 DM and SNHL (OR: 1.032; 95%CI: 1.018-1.047; P = 5.45 × 10-6), while there was no causality between type 2 DM and SNHL (OR: 1.000; 95%CI: 0.958-1.036; P = .853). Our study suggested that DM and type 1 DM may be genetically responsible for SNHL. Although our study did not detect a genetic causal relationship between type 2 DM and SNHL, this does not rule out a relationship between them at other mechanistic levels. Further studies are required to confirm the findings and look into the physiological and pathological mechanism underlying these relationships.

Optimizing Hearing Outcomes in Nasopharyngeal Cancer Survivors in the Era of Modern Radiotherapy and Systemic Therapy.

Intensity-modulated radiation therapy (IMRT) improves disease control and reduces treatment-related toxicity in patients with localized nasopharyngeal carcinoma (NPC). However, due to the proximity of the auditory apparatus to the treatment volume and the frequent incorporation of cisplatin-based chemotherapy, treatment-related sensorineural hearing loss (SNHL) remains a common debilitating complication among NPC survivors. The reported crude incidence of SNHL following IMRT for NPC varies widely at 1-46% due to differences in auditory assessment methods and thresholds, follow-up durations, chemotherapy usage, and patient compositions. International guidelines and radiation dosimetric studies have recommended constraining the cochlear mean dose to less than 44-50 Gy, but the risk of SNHL remains high despite adherence to these constraints. Potential strategies to improve hearing outcomes in NPC survivors include cautious de-escalation of radiotherapy dose and volume, individualization of cochlear constraints, optimization of radiotherapy planning techniques, and the use of substitutes or alternative schedules for cisplatin-based chemotherapy. The addition of immune checkpoint inhibitors to chemoradiotherapy did not impact ototoxicity. Prospective studies that employ both objective and patient-reported auditory outcomes are warranted to test the long-term benefits of various approaches. This article aims to provide a comprehensive review of the incidence and radiation dose-toxicity relationship of SNHL in NPC survivors and to summarize potential strategies to optimize hearing outcomes in relation to nuances in radiotherapy planning and the selection of systemic therapy.

Mendelian Randomization Study Reveals a Predicted Relationship between Sensorineural Hearing Loss and Mitochondrial Proteins.

Mitochondrial proteins assume a pivotal role in the onset and progression of diverse diseases. Nonetheless, the causal interconnections with sensorineural hearing loss (SNHL) demand meticulous exploration. Mendelian randomization analysis is a method used in observational epidemiological studies to predict the relationship between exposure factors and outcomes using genetic variants as instrumental variables. In this study, we applied this analytical approach to two distinct samples to predict the causal impact of mitochondrial proteins on SNHL. Two-sample Mendelian randomization analyses were executed to scrutinize the predicted associations between 63 mitochondrial proteins (nuclear-encoded) and SNHL, utilizing summary statistics derived from genome-wide association studies. Assessments of pleiotropy and heterogeneity were carried out to gauge the robustness of the obtained findings. Four mitochondrial proteins exhibited a suggestive causal relationship with the susceptibility to SNHL. Dihydrolipoamide dehydrogenase (DLD; OR = 0.9706, 95% CI = 0.9382-0.9953, p = 0.0230) was linked to a diminished risk of SNHL. Conversely, elevated levels of mitochondrial ribosomal protein L34 (MRPL34; OR = 1.0458, 95% CI = 1.0029-1.0906, p = 0.0362), single-pass membrane protein with aspartate-rich tail 1 (SMDT1; OR = 1.0619, 95% CI = 1.0142-1.1119, p = 0.0104), and superoxide dismutase 2 (SOD2; OR = 1.0323, 95% CI = 1.0020-1.0634, p = 0.0364) were associated with an elevated risk of SNHL. This research utilized Mendelian randomization analysis to predict the relationship between mitochondrial proteins and SNHL. It provides a potential viewpoint on the etiology and diagnosis.

A Study on the Effects of Hypothyroidism on the Senses: A Comprehensive Narrative Review.

Hypothyroidism has been found to have long-term effects on each of thesenses, but with proper treatment, many of them can be significantly minimized. This paper analyzes the research on the impact of hypothyroidism on the senses of smell, taste, hearing, vision, and thermoregulation. Data were collected from the National Library of Medicine, PubMed, and Google Scholar databases using the keywords "hypothyroidism," "taste," "smell," "vision," "hearing," and "thermoregulation." Approximately 413 articles were found when searching with these parameters, and 30 were used in this paper. Studies were excluded if they were outside this paper's scope or older than 2012. Studies were included if they specifically focused on hypothyroidism and one of the five listed senses. Patients with hypothyroidism had a significantly increased risk of sensorineural hearing loss, decreased perception of the blue-yellow color axis, decreased sense of olfaction and number of olfactory bulbs, and decreased thermogenesis. Hypothyroidism was also found to show increased length of COVID-19-induced anosmia and decreased bitter taste perception. It can be concluded that hypothyroidism has many effects on the senses, particularly an increased risk of sensorineural hearing loss. More studies need to be done on these effects.

Increased risk of hearing loss associated with MT-RNR1 gene mutations: a real-world investigation among Han Taiwanese Population

BackgroundPrevious studies have implicated inherited mutations in mitochondrial DNA (mtDNA) in sensorineural hearing loss (SNHL). However, the definitive association between mitochondrial 12S rRNA (MT-RNR1) variants and hearing loss in the population has not been well established, particularly in Asia. The objective of this retrospective cohort study was to assess the association between MT-RNR1 variants and the risk of SNHL in patients in Taiwan.MethodsThe cohort included 306,068 participants from Taiwan between January 2003 and December 2020. Participants were classified based on genetic variants, particularly mitochondrial mutations (rs267606618, rs267606619, rs267606617). MT-RNR1 variant cases were matched 1:10 with non-mutant patients by age, gender, and visit year, excluding those with pre-existing hearing loss. The primary endpoint was SNHL, identified using specific ICD-TM codes with a 90% positive predictive value. Medication exposure history was determined via self-report or electronic medical records in the hospital. Cox proportional hazard regression models were used to assess the association between MT-RNR1 variants and hearing loss, adjusting for various covariates. Kaplan-Meier survival curves and log-rank tests compared hearing loss incidence between groups.ResultsThe mean age of the mtDNA variants group is 32.4 years, with a standard deviation of 19.2 years. The incidence density of hearing loss for the mutation group was 36.42 per 10,000 person-years (95% Confidence Interval [CI], 27.21–47.73), which was higher than the 23.77per 10,000 person-years (95% CI, 21.32–26.42) in the wild-type group (p = 0.0036). Additionally, diabetes mellitus was associated with an increased risk of developing SNHL in individuals with MT-RNR1 variants (adjusted hazard ratio = 1.76 [95% CI, 1.00-3.09], p < 0.05).ConclusionThis study highlights the increased risk of hearing loss in patients carrying MT-RNR1 variants, particularly those with diabetes mellitus. Future research that integrates genetic and clinical data is crucial for developing more precise interventions to monitor and treat hearing loss in this vulnerable population.

Lipids and Statin Medication With Sensorineural Hearing Loss: A Mendelian Randomization Study.

Recent insights suggest that lipids and statin medication play a role in the development of sensorineural hearing loss (SNHL), yet the exact role remains controversial. This research applied Mendelian randomization (MR) to assess whether lipids and statin medication are associated with an increased risk of SNHL. A two-sample MR was used in this study. Genetic instruments were constructed from variants associated with risk factors. Data for lipids and statin medication were obtained from the IEU OpenGWAS project, and for SNHL from the Finngen research project, which comprises 32,487 individuals with SNHL and 331,736 control individuals. Genetically predicted higher levels of triglycerides were associated with an increased risk of SNHL. The use of genetically predicted atorvastatin was associated with a lower risk of SNHL. Rosuvastatin has demonstrated potential in treating SNHL, yet further investigations are warranted to elucidate its relationship with SNHL. Insufficient evidence was available to suggest that the genetically predicted level of high-density lipoprotein cholesterol or low-density lipoprotein cholesterol or the use of simvastatin were associated with SNHL. The study provides genetic evidence suggesting that increased levels of triglycerides in the blood could be a risk factor for SNHL and that the use of certain statin medications, including atorvastatin and rosuvastatin, could reduce the risk of SNHL. These results align with findings from previous observational studies that have linked hyperlipidemia with the risk of SNHL. According to the Oxford Centre for Evidence-Based Medicine 2011 levels of Evidence, the study has a third level of Evidence Laryngoscope, 134:4366-4373, 2024.

Dosimetric analysis of hearing loss after cranial radiation therapy in children: A single-institution study from the French national registry PediaRT

PurposeTo identify dosimetric predictive factors of sensorineural hearing loss (SNHL) in children after cranial radiation therapy (RT) in a single institution using dosimetric data from the French National Registry PediaRT. Methods and MaterialsComplete audiological follow-up data were available for 44 children treated with cranial RT between 2014 and 2021 at our institution. The median age at the time of RT initiation was 9 years (range: 2–17 years). No children presented with hearing loss prior to treatment. SNHL was defined as a Chang ototoxicity grade ≥ 1a or higher. ResultsMedian audiometric follow-up duration was 51 months. Seven children (16 %) developed SNHL with a median time to occurrence of 33 months (range, 18–46 months). The estimated SNHL cumulative rate at 2 years post-RT was 4,5% ± 3,1% and at 5 years was 21 % ± 7.2 %. Multiple Cox regression models showed that the association of the age at radiotherapy and the dosimetric values to the inner ear canal and cochlea were the most significant predictive factors of SNHL occurrence. No child who received less than 35 Gy on average to both cochleae (n = 26) suffered from SNHL, whereas the 5-year SNHL cumulative incidence for the children who received greater than or equal to 35 Gy on average to either cochlea (n = 18) was 51.8 % ± 15.1 %. ConclusionDoses received by the inner ear canal and cochlea, associated with the age at RT initiation, are the main predictive factors for radiation-induced SNHL. A median dose to either cochlea over 35 Gy significantly increases the risk of SNHL and justify close audiometric monitoring to detect and equip hearing loss at an early stage.

Causal relationship between psychiatric disorders and sensorineural hearing loss: A bidirectional two-sample mendelian randomization analysis

ObjectiveThis study employed bidirectional two-sample Mendelian randomization (MR) to investigate the causal links between psychiatric disorders and sensorineural hearing loss (SNHL). MethodsInstrumental variables were chosen from genome-wide association studies of schizophrenia (SCH, N = 127,906), bipolar disorder (BD, N = 51,710), major depressive disorder (MDD, N = 500,199), and SNHL (N = 212,544). In the univariable MR analysis, the inverse-variance weighted method (IVW) was conducted as the primary analysis, complemented by various sensitivity analyses to ensure result robustness. ResultsSCH exhibited a decreased the risk of SNHL (OR = 0.949, P = 0.005), whereas BD showed an increased incidence of SNHL (OR = 1.145, P = 0.005). No causal association was found for MDD on SNHL (OR = 1.088, P = 0.246). Multivariable MR validated these results. In the reverse direction, genetically predicted SNHL was linked to a decreased risk of SCH with suggestive significance (OR = 0.912, P = 0.023). No reverse causal relationships were observed for SNHL influencing BD or MDD. These findings remained consistent across various MR methods and sensitivity analyses. ConclusionThis study demonstrated that the causal relationships between diverse psychiatric disorders with SNHL were heterogeneous. Specifically, SCH was inversely associated with SNHL susceptibility, and similarly, a reduced risk of SNHL was observed in schizophrenia patients. In contrast, BD exhibited an increased incidence of SNHL, although SNHL did not influence the prevalence of BD. No causal association between MDD and SNHL was found.

L-shaped association of triglyceride glucose index and sensorineural hearing loss: results from a cross-sectional study and Mendelian randomization analysis.

The association between the sensorineural hearing loss (SNHL) and triglyceride-glucose (TyG) index remains inadequately understood. This investigation seeks to elucidate the connection between the TyG index and SNHL. In this cross-sectional study, we utilized datasets sourced from the National Health and Nutrition Examination Survey (NHANES). A comprehensive analysis was conducted on 1,851 participants aged 20 to 69, utilizing complete audiometry data from the NHANES database spanning from 2007 to 2018. All enrolled participants had accessible hearing data, and the average thresholds were measured and calculated as both low-frequency pure-tone average and high-frequency pure-tone average. Sensorineural hearing loss (SNHL) was defined as an average pure tone of 20 dB or higher in at least one better ear. Our analysis involved the application of multivariate linear regression models to examine the linear relationship between the TyG index and SNHL. To delineate any non-linear associations, we utilized fitted smoothing curves and conducted threshold effect analysis. Furthermore, we conducted a two-sample Mendelian randomization (MR) study, leveraging genetic data from genome-wide association studies (GWAS) on circulating lipids, blood glucose, and SNHL. The primary analytical method for the MR study was the application of the inverse-variance-weighted (IVW) approach. In our multivariate linear regression analysis, a substantial positive correlation emerged between the TyG index and SNHL [2.10 (1.80-2.44), p < 0.0001]. Furthermore, using a two-segment linear regression model, we found an L-shaped relationship between TyG index, fasting blood glucose and SNHL with an inflection point of 9.07 and 94 mg/dL, respectively. Specifically, TyG index [3.60, (1.42-9.14)] and blood glucose [1.01, (1.00-1.01)] concentration higher than the threshold values was positively associated with SNHL risk. Genetically determined triglyceride levels demonstrated a causal impact on SNHL (OR = 1.092, p = 8.006 × 10-4). In addition, blood glucose was found to have a protective effect on SNHL (OR = 0.886, p = 1.012 × 10-2). An L-shaped association was identified among the TyG index, fasting blood glucose, and SNHL in the American population. TyG index of more than 9.07 and blood glucose of more than 94 mg/dL were significantly and positively associated with SNHL risk, respectively.

Gram-negative Bacteria are Associated With Sensorineural Hearing Loss in Chronic Otitis Media.

Chronic otitis media (COM) is an inflammatory disease that commonly presents with otorrhea and hearing loss. Bacteria-induced inflammation can cause inner ear damage, leading to sensorineural hearing loss (SNHL). This study aimed to compare the prevalence and severity of SNHL in patients with gram-negative versus gram-positive cultures and examine associations between the concentrations of circulating monocytes and neutrophils with bacteria species and SNHL. This was a retrospective study. Cholesteatoma or chronic suppurative otitis media patients with otorrhea were enrolled. Middle ear secretions were collected using sterile swabs under an otoscope, and sent for bacterial detection within 30 min. Pure tone audiometry and circulating leukocyte counts were recorded and analyzed in patients infected with different pathogens. Logistic regression analysis was used to identify the risk factors associated with SNHL. A total of 137 patients were enrolled, including 45 patients infected with gram-negative bacteria, 41 with gram-positive bacteria, 20 with polymicrobial infection, and 31 with no bacterial growth. Logistic regression analysis showed that bacterial culture positive infections (OR = 7.265, 95% CI 2.219-23.786, p = 0.001) were an independent risk factor for SNHL. Patients with gram-negative bacteria had higher risks of SNHL (p < 0.0001) and more severe hearing loss (p = 0.005) than those with gram-positive bacteria. COM patients infected with gram-negative bacteria showed an increase in circulating monocytes, which correlated with the occurrence of SNHL (p = 0.0343). Gram-negative bacteria are associated with elevated circulating monocyte counts and have a higher risk of severe SNHL. 4 Laryngoscope, 134:3335-3341, 2024.

The multifaceted links between hearing loss and chronic kidney disease.

Hearing loss affects nearly 1.6 billion people and is the third-leading cause of disability worldwide. Chronic kidney disease (CKD) is also a common condition that is associated with adverse clinical outcomes and high health-care costs. From a developmental perspective, the structures responsible for hearing have a common morphogenetic origin with the kidney, and genetic abnormalities that cause familial forms of hearing loss can also lead to kidney disease. On a cellular level, normal kidney and cochlea function both depend on cilial activities at the apical surface, and kidney tubular cells and sensory epithelial cells of the inner ear use similar transport mechanisms to modify luminal fluid. The two organs also share the same collagen IV basement membrane network. Thus, strong developmental and physiological links exist between hearing and kidney function. These theoretical considerations are supported by epidemiological data demonstrating that CKD is associated with a graded and independent excess risk of sensorineural hearing loss. In addition to developmental and physiological links between kidney and cochlear function, hearing loss in patients with CKD may be driven by specific medications or treatments, including haemodialysis. The associations between these two common conditions are not commonly appreciated, yet have important implications for research and clinical practice.

Inflammatory diet, gut microbiota and sensorineural hearing loss: a cross-sectional and Mendelian randomization study.

Inflammatory diets can trigger chronic inflammation and affect gut microbiota. However, the relationship between dietary preferences and sensorineural hearing loss (SNHL) remains unclear. This study aims to elucidate the relationship between different dietary preferences and sensorineural deafness. The Dietary Inflammation Index (DII) and SNHL were defined by data from the National Health and Nutrition Examination Survey (NHANES), and exploring their relationship. Using Mendelian randomization (MR) to analyze the relationship between 34 dietary preferences, 211 gut microbiota, and SNHL. Smooth curve fitting indicated that the risk of SNHL increased with increasing DII score when the DII score was greater than 5.15. MR results suggest that a diet including both oily and non-oily fish can substantially reduce the risk of SNHL. Additionally, six specific gut microbiota were found to have significant causal relationship with SNHL. An inflammatory diet may increase the risk of developing SNHL. The observed relationship between fish consumption, gut microbiota, and SNHL suggests the existence of a gut-inner ear axis.

Hearing Loss and Risk Factors in Very Low Birth Weight Infants.

The incidence of sensorineural hearing loss (SNHL) is still high in very low birth weight (VLBW) infants. The purpose of our study was to provide the prevalence rates of SNHL and to analyze the risk factors of hearing impairment and changes in hearing thresholds in a cohort of VLBW infants. A retrospective observational study was conducted in our neonatal intensive care unit (NICU) from 2012 to 2016. All VLBW infants included were screened by transient evoked otoacoustic emissions (TEOAEs) and diagnostic auditory brainstem response (ABR). In total, we enrolled 316 infants and SNHL was diagnosed in 68, leading to an early incidence of 21.5% as 36 infants out of 68 improved. Finally, SNHL was confirmed in 20 patients (6.3%) who needed hearing aids. They were significantly smaller, sicker, had longer hospitalizations, and received more ototoxic therapies. Logistic regression analysis showed that gestational age (GA) influenced the association between drugs and SNHL. The results underlined how the total exposure to antibiotics is significantly associated with SNHL, even after GA correction. In conclusion, GA, birth weight and, above all, the length and complexity of NICU stay quantify the risk of SNHL and should be considered at the individual level for parent counseling.

19 Auditory and Cognitive Function in Adults Living With and Without HIV

Objective:Hearing consists of peripheral components (outer and middle ear, cochlea) and the central auditory system (cochlear nuclei to the auditory cortex). Speech perception relies on peripheral hearing abilities (i.e., pure-tone thresholds) and central auditory processing (CAP) and cognitive functioning. Specifically, working memory, executive function, attention, and verbal functioning allow for speech understanding. As a result, CAP deficits are also influenced by peripheral hearing sensitivity and cognitive functioning. Assessing CAP deficits can be difficult because of these complex interactions. Prior work has shown persons living with HIV (PWH) are at higher risk for sensorineural hearing loss compared to persons living without HIV (PWOH) after adjusting for age, sex, and noise exposure. Further, HIV is a risk factor for cognitive impairment, one example being Alzheimer's disease (AD) and its precursor, Mild Cognitive Impairment (MCI), with auditory dysfunction occurring in earlier stages of AD. Therefore, the purpose of this study was to evaluate: 1) the peripheral hearing sensitivity and CAP in PWH and PWOH; and 2) the association between cognitive function measures and CAP in PWH and PWOH.Participants and Methods:Participants included 59 PWH (39 men and 20 women, mean age=66.7 years [SD=4.4 years]) and 27 PWOH (13 men and 14 women, mean age=71.9 years [SD=7.1 years]). Participants completed a standard neuropsychological battery assessing the domains of learning, recall, executive function, working memory, verbal fluency, processing speed and motor. Raw scores were transformed to demographically corrected, domain T-scores. Cognitive function was normal for 39 (66.1%) PWH and 16 (59.3%) PWOH while 43 (72.9%) PWH and 17 (63.0%) PWOH were determined to have MCI. Participants with dementia were excluded. Participants also completed a hearing assessment, a portion of which consisted of pure-tone thresholds, peripheral hearing measure, and dichotic digits testing (DDT), a CAP measure. Pure-tone air-conduction thresholds were obtained at octave frequencies from 0.25 through 8 kHz, including 3 and 6 kHz. A pure-tone average (PTA) was calculated from 0.5, 1, 2, and 4 kHz thresholds for each ear. The DDT involves the presentation of numbers from 1 to 10, excluding 7, in which two different digits are presented to one ear while two other digits are simultaneously presented to the opposite ear. The outcome of DDT is percent correct.Results:PWH had slightly lower (i.e., better) mean PTAs in both ears compared to PWOH, but this was not statistically significant. Conversely, PWH had lower percent correct DDT results compared to PWOH, but this difference was also not statistically significant. Participants with impairment in verbal fluency, executive functioning, and working memory had significantly worse DDT results by approximately 10%, but only for right ear data.Conclusions:PWH in our sample had better hearing than PWOH, which can be explained by PWH having a lower mean age. PWH had poorer DDT results, however, indicative of CAP deficits rather than peripheral hearing problems. Poor right ear DDT was associated with impairments specifically in frontal-based cognitive processes with an executive component.

1 Ototoxicity and Cognitive Outcomes among Children Treated for Brain Tumors in Infancy

Objective:Treatment of childhood central nervous system (CNS) tumors can lead to sensorineural hearing loss (SNHL), with prior research indicating associations between SNHL and cognitive difficulties. Infants (0-3 years) treated for CNS tumors are at particular risk for neurocognitive deficits due to increased vulnerability of the developing brain and missed developmental opportunities secondary to prolonged treatment. This study expands upon existing research by examining the association between treatment-related SNHL and later neurocognitive outcomes among infants.Participants and Methods:Serial audiology and neurocognitive assessments were conducted as part of a prospective, multisite, longitudinal trial (SJYC07). Children with newly diagnosed CNS tumors were treated with chemotherapy, with or without focal proton or photon radiation therapy (RT). SNHL was dichotomized based on hearing in the better ear as present versus not present (Chang grade ≥1a vs. &lt;1a). Neurocognitive assessments included intellectual functioning (IQ), and parent ratings of executive functioning and behavioral functioning. Demographic and clinical variables investigated included: sex, age at diagnosis (years), treatment type (chemotherapy only vs. chemotherapy + RT), risk group (low vs. intermediate vs. high), and socioeconomic status (SES, continuous). Logistic regression models were used to identify factors associated with SNHL. Change point longitudinal models were used to examine the effect of each covariate individually and the potential impact of SNHL on trajectories of neurocognitive outcomes.Results:Of 135 patients (median age at diagnosis= 1.5 years), 67% had mild-to-severe SNHL as defined by Chang grade ≥1a at last follow-up. SNHL occurred early after treatment with a 1-year cumulative incidence 63.0% ±4.3%. SNHL was associated with age at diagnosis (p &lt;.001) but not sex, treatment exposure or study risk arm (p &gt;.10). At pretreatment baseline, IQ was associated with age at diagnosis (older age= higher IQ) and SES (higher SES= higher IQ) with a change in the trajectory of IQ after SNHL (stable prior to SNHL and declined 1.46 points/year after SNHL), which was impacted by tumor location (patients with supratentorial tumors stable prior to SNHL and declined 2.84 points/year after SNHL; whereas, patients with infratentorial tumors increased 1.93 points/year prior to SNHL and were stable after SNHL). At pre-treatment baseline, adaptive functioning was associated with age at diagnosis (older age= higher skills) with a change in adaptive functioning after SNHL that varied by age. There was a change in trajectory of attention problems (stable before SNHL and worsening 1.39 points/year after SNHL). SNHL was not associated with parent report of emerging executive functioning.Conclusions:Children with brain tumors experience SNHL and cognitive difficulties early in treatment that can worsen over time. Younger age at diagnosis is associated with greater risk for SNHL and cognitive difficulties. Analyses of the time course between the emergence of SNHL and cognitive late effects suggests even mild SNHL is associated with a clinically signficant decline in IQ and attention problems. These findings have notable implications with respect to refining monitoring guidelines, informing modifications to treatment, advocating for interventions, and helping educate parents, teachers, and providers about the significant impact of mild SNHL.

Association between gut microbiota and sensorineural hearing loss: a Mendelian randomization study.

Several recent studies speculated that the gut microbiota is associated with sensorineural hearing loss (SNHL) and proposed the concept of the gut-inner ear axis. However, the causal effect of gut microbiota on SNHL is still unknown. In this study, we performed a two-sample Mendelian randomization (MR) analysis to estimate the causal effect of gut microbiota on SNHL. Gut microbiota data were obtained from the largest available genome-wide association study (n = 18,340) conducted by the MiBioGen consortium. The summary statistics of SNHL were obtained from the FinnGen consortium R8 release data (28,310 cases and 302,750 controls). The causal effects were estimated with inverse-variance weighted, MR-Egger, and weighted median. Reverse Mendelian randomization analysis was performed on the bacteria that were found to be associated with SNHL in forward Mendelian randomization analysis. We then performed sensitivity analyses, including Cochran's Q-test, MR-Egger intercept test, MR-PRESSO, cML-MA-BIC, and leave-one-out analysis, to detect heterogeneity and pleiotropy. The inverse-variance weighted results suggested that Lachnospiraceae (UCG001) had a significant protective effect against SNHL (odds ratio = 0.85, 95% confidence interval: 0.78-0.93, P = 6.99 × 10-4). In addition, Intestinimonas (odds ratio = 0.89, 95% confidence interval: 0.82-0.97, P = 8.53 × 10-3) presented a suggestively protective effect on SNHL. Rikenellaceae (RC9gutgroup) (odds ratio = 1.08, 95% confidence interval: 1.02-1.15, P = 0.01) and Eubacterium (hallii group) (odds ratio = 1.12, 95% confidence interval: 1.00-1.24, P = 0.048) suggestively increase the risk of SNHL. The results of the reverse MR analysis showed that there is no significant causal effect of SNHL on the gut microbiota. No significant heterogeneity of instrumental variables or pleiotropy was detected. The evidence that the four genera mentioned above are associated with SNHL supports the hypothesis of a gut-inner ear axis. Our study provides microbial markers for the prevention and treatment of SNHL, and further studies are needed to explore the mechanisms of the gut microbiome-inner ear axis in health and diseases.

Cardiocerebrovascular risk in sensorineural hearing loss: results from the National Health and Nutrition Examination Survey 2015 to 2018.

This study aims to determine whether the risks of cardiocerebrovascular disease are relevant to sensorineural hearing loss (SNHL) based on a national database. A total of 1,321 participants aged from 18 to 69 with complete data including medical history and audiometry from the NHANES database (2015-2018) were analyzed. All included participants had available hearing data and the average thresholds of the hearing data were measured and calculated as low-frequency pure-tone average (LFPTA; 500, 1,000, and 2,000 Hz) and high-frequency pure-tone average (HFPTA; 3,000, 4,000, 6,000, and 8,000 kHz). SNHL was defined as an average pure tone of more than or equal to 20 dB in at least one better ear. Multivariable models to assess the association between cardiocerebrovascular risks and SNHL were used in this study. The prevalence of stroke was 1.6% in individuals with SNHL and 0.4% in individuals without SNHL (p = 0.023). A higher cardiovascular risk score was observed in SNHL patients compared to participants without SNHL (1.58 vs. 0.90, p < 0.001). Stroke was associated with a 3.67-fold increase in the risk of SNHL (95% CI: 1.12-12.00, p = 0.032) in univariable logistic regression, and the association (OR = 4.22, 95%CI = 1.28-13.93, p = 0.020) remained significant after adjusting for several covariates. Multivariable logistic regression models indicated a positive correlation between cardiovascular risk and SNHL (OR = 1.66, 95% CI = 1.40-1.96, p < 0.001), but no significant relationship was shown with all covariates adjusted. However, significant associations were found between SNHL and both age and sex in both univariable and multivariable logistic regression models. Our findings suggested that a higher cardiocerebrovascular risk burden was associated with an increased risk of SNHL, and the relationship may be influenced by age and sex. Future longitudinal studies are needed to investigate the mechanistic and pathologic vascular hypothesis of SNHL.

Hearing Loss and Blood Coagulation Disorders: A Review.

A relationship between microvascular disorders and sensorineural hearing loss (SNHL) has been widely proposed. The vascular hypothesis, theorized for the onset of sudden SNHL (SSNHL), is among the most acknowledged: a localized acute cochlear damage, of ischemic or haemorrhagic nature, could be considered a causative factor of SSNHL. The aim of this review is to assess (i) the effect on hearing in patients affected by blood coagulation disorders (prothrombotic or haemorrhagic) and (ii) the possible etiopathogenetic mechanisms of the related hearing loss. A PRISMA-compliant review was performed. Medline, Embase, and Cinahl databases were searched from inception to 31 January 2023, and a total of 14 studies have been included in the review. The available data suggest that it is possible to consider clotting disorders as a potential condition at risk for sensorineural hearing loss; in particular, coagulation tests and eventually the assessment of genetic and acquired prothrombotic factors should be recommended in patients with SSNHL. Also, an audiological evaluation should be recommended for patients with blood coagulation disorders presenting cochlear symptoms, especially in those suffering from clotting diseases.

Auditory Deficits in Employees of Business Process Outsourcing (BPO): Need to Take Preventative Action.

The present study aimed to explore auditory deficits in full-time call center workers. A total of sixty participants participated, which was divided into two groups, viz. experimental group and control group. The complete audiological test battery was performed. On comparing the groups, significant differences were obtained for both ears while analyzing the TEOAEs, PTA1, and PTA2 (high-frequency audiometry). From the results, it can be delineated that BPO employees are at risk for sensorineural hearing loss following continuous noise exposure. We conclude that this type of hearing loss may be considered an iceberg, and to overcome all the issues related to noise exposure, all BPO employees should undergo periodic audiological, psychological, and health screening.