Hearing loss in beta-thalassaemia: An Italian multicentre case-control study.

BACKGROUND: Despite numerous studies, the true scenario of hearing loss in beta-thalassaemia remains rather nebulous. MATERIALS AND METHODS: Pure tone audiometry, chelation therapy, demographics and laboratory data of 376 patients (mean age 38.5 ± 16.6 years, 204 females, 66 non-transfusion-dependent) and 139 healthy controls (mean age 37.6 ± 17.7 years, 81 females) were collected. RESULTS: Patient and control groups did not differ for age (p = 0.59) or sex (p = 0.44). Hypoacusis rate was higher in patients (26.6% vs. 7.2%; p < 0.00001), correlated with male sex (32.6% in males vs. 21.8% in females; p = 0.01) and it was sensorineural in 79/100. Hypoacusis rate correlated with increasing age (p = 0.0006) but not with phenotype (13/66 non-transfusion-dependent vs. 87/310 transfusion-dependent patients; p = 0.16). Sensorineural-notch prevalence rate did not differ between patients (11.4%) and controls (12.2%); it correlated with age (p = 0.01) but not with patients' sex or phenotype. Among adult patients without chelation therapy, the sensorineural hypoacusis rate was non-significantly lower compared to chelation-treated patients while it was significantly higher compared to controls (p = 0.003). CONCLUSIONS: Sensorineural hypoacusis rate is high in beta-thalassaemia (about 21%) and it increases with age and in males while disease severity or chelation treatment seems to be less relevant. The meaning of sensorineural-notch in beta-thalassaemia appears questionable.

Correlation Analysis and Comparison of Adult CE-Chirp ABR Response Threshold and Pure Tone Hearing Threshold.

OBJECTIVES: To study the application of CE-Chirp in the evaluation of hearing impairment in forensic medicine by testing the auditory brainstem response (ABR) in adults using CE-Chirp to analyze the relationship between the V-wave response threshold of CE-Chirp ABR test and the pure tone hearing threshold. METHODS: Subjects (aged 20-77 with a total of 100 ears) who underwent CE-Chirp ABR test in Changzhou De'an Hospital from January 2018 to June 2019 were selected to obtain the V-wave response threshold, and pure tone air conduction hearing threshold tests were conducted at 0.5, 1.0, 2.0 and 4.0 kHz, respectively, to obtain pure tone listening threshold. The differences and statistical differences between the average pure tone hearing threshold and V-wave response threshold were compared in different hearing levels and different age groups. The correlation, differences and statistical differences between the two tests at each frequency were analyzed for all subjects. The linear regression equation for estimating pure tone hearing threshold for all subjects CE-Chirp ABR V-wave response threshold was established, and the feasibility of the equation was tested. RESULTS: There was no statistical significance in the CE-Chirp ABR response threshold and pure tone hearing threshold difference between different hearing level groups and different age groups (P>0.05). There was a good correlation between adult CE-Chirp ABR V-wave response threshold and pure tone hearing threshold with statistical significance (P<0.05), and linear regression analysis showed a significant linear correlation between the two (P<0.05). CONCLUSIONS: The use of CE-Chirp ABR V-wave response threshold can be used to evaluate subjects' pure tone hearing threshold under certain conditions, and can be used as an audiological test method for forensic hearing impairment assessment.

Clinical characteristics of the "Gap" between the prevalence and incidence of hearing loss using National Health Insurance Service data.

OBJECTIVES: Hearing loss is the inability to hear speech or sounds well, owing to a number of causes. This study aimed to simultaneously determine the prevalence, incidence, and the Gap between them in hearing loss in South Korean patients at the same point in time as well as to identify patients who have not recovered from hearing loss. METHODS: We examined the prevalence and incidence of patients diagnosed with hearing loss in the National Health Insurance Service database over an 11-year period from 2010 to 2020. The difference between the prevalence and the incidence was defined in this study as the term "Gap". Gap is the number of patients converted into the number of patients per 100,000 people by subtracting the incidence from the prevalence. Clinical characteristics such as sex and age per 100,000 individuals were examined. RESULTS: As of 2020, the domestic prevalence obtained in this study was 1.84%, increasing annually, and the prevalence increased with age to 4.10% among those over 60. The domestic incidence was 1.57%, increasing annually, and the incidence increased with age to 3.36% for those over 60s. The Gap was 0.27%, showing a steady increase from 2011 to 2020 with a corresponding increase in insurance benefit expenses. CONCLUSION: To fully understand the burden of hearing loss and develop effective prevention and treatment strategies, it is important to measure the Gap between its prevalence and incidence. This Gap means a lot because hearing loss is an irreversible disease. Gap represents patients who have already been diagnosed with hearing loss and are being diagnosed every year, indicating that the number of patients who do not recover is increasing. In other words, the increase in Gap meant that there were many patients who constantly visited the hospital for diagnosis of hearing loss.

The impacts of exercise training programs on balance in children with hearing loss: A systematic review and meta-analysis.

INTRODUCTION AND PURPOSE: According to the prevalence of balance disorders among children with hearing loss (HL), researchers used exercise programs to improve balance in children with HL. So, the present systematic review and meta-analysis briefly summarize findings regarding the impacts of exercise training programs on balance in children with HL. METHODS: Science Direct, MEDLINE/PubMed, SCOPUS, LILACS, CINAHL, CENTRAL, Web of Science, PEDro, and Google Scholar were searched from inception until November 11th, 2023. Two independent researchers analyzed and extracted the data from potential papers whose eligibility was confirmed. Then, the PEDro scale was used to obtain quality assessment scores. The total PEDro score is 11 and incorporates the presentation of statistical analysis and evaluation criteria of internal validity. Studies that scored 7-11 were considered methodologically "high", 5 to 6 were "fair", and ≤4 were considered "poor". RESULTS: 10 studies involving a total of 304 participations were included in the systematic review. Our results demonstrate that exercise training programs positively impact static balance (p = 0.001) with level 1a evidence, the postural sway (p = 0.001) with level 1a evidence, and dynamic balance (p = 0.001) with level 1a evidence in children with HL. CONCLUSION: The findings of this systematic review and meta-analysis related to studies with excellent methodological quality revealed that the intended training programs significantly impact postural sway along with static and dynamic balance in children with HL. It is recommended that future exercise training programs be paired with rehabilitation programs for children with HL.

Efficacy of Amplification for Tinnitus Relief in People With Mild Hearing Loss.

PURPOSE: The study aimed to evaluate the efficacy of amplification with hearing aids for people with chronic subjective tinnitus and mild hearing loss. METHOD: In this randomized, controlled, three-arm trial, 38 subjects with a primary complaint of tinnitus were randomly assigned to one of the three treatment groups. Twelve subjects received informational counselling (IC) only, 13 received IC with hearing aid fitting, and 13 subjects received IC with individualized music stimulation for 12 months. The primary efficacy analysis in tinnitus severity was based on the change from baseline to 12 months after the 1st day of the intervention. Secondary outcome measures included tinnitus impact, psychological and mental health effects, subjective ratings, and psychoacoustically measured tinnitus loudness. RESULTS: A statistically significant treatment difference among the three groups in the Chinese Tinnitus Functional Index (TFI-CH) total score at the predefined end point in Month 12 was observed (F = 3.34, p = .04, partial η2 = .16). Reductions in the TFI-CH scores in both the hearing aid and the customized music group were more prominent than in the IC-only group. Only the hearing aid group showed a significantly greater treatment effect than the IC-only group. CONCLUSION: Results from this study support that a combination of hearing aid use and IC can help improve tinnitus in people with mild hearing loss. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.25015979.

Disease Manifestations and Complications in Dutch X-Linked Hypophosphatemia Patients.

X-linked hypophosphatemia (XLH) is the most common monogenetic cause of chronic hypophosphatemia, characterized by rickets and osteomalacia. Disease manifestations and treatment of XLH patients in the Netherlands are currently unknown. Characteristics of XLH patients participating in the Dutch observational registry for genetic hypophosphatemia and acquired renal phosphate wasting were analyzed. Eighty XLH patients, including 29 children, were included. Genetic testing, performed in 78.8% of patients, showed a PHEX mutation in 96.8%. Median (range) Z-score for height was - 2.5 (- 5.5; 1.0) in adults and - 1.4 (- 3.7; 1.0) in children. Many patients were overweight or obese: 64.3% of adults and 37.0% of children. All children received XLH-related medication e.g., active vitamin D, phosphate supplementation or burosumab, while 8 adults used no medication. Lower age at start of XLH-related treatment was associated with higher height at inclusion. Hearing loss was reported in 6.9% of children and 31.4% of adults. Knee deformities were observed in 75.0% of all patients and osteoarthritis in 51.0% of adult patients. Nephrocalcinosis was observed in 62.1% of children and 33.3% of adults. Earlier start of XLH-related treatment was associated with higher risk of nephrocalcinosis and detection at younger age. Hyperparathyroidism longer than six months was reported in 37.9% of children and 35.3% of adults. This nationwide study confirms the high prevalence of adiposity, hearing loss, bone deformities, osteoarthritis, nephrocalcinosis and hyperparathyroidism in Dutch XLH patients. Early start of XLH-related treatment appears to be beneficial for longitudinal growth but may increase development of nephrocalcinosis.

Clarin-2 gene supplementation durably preserves hearing in a model of progressive hearing loss.

Hearing loss is a major health concern affecting millions of people worldwide with currently limited treatment options. In clarin-2-deficient Clrn2-/- mice, used here as a model of progressive hearing loss, we report synaptic auditory abnormalities in addition to the previously demonstrated defects of hair bundle structure and mechanoelectrical transduction. We sought an in-depth evaluation of viral-mediated gene delivery as a therapy for these hearing-impaired mice. Supplementation with either the murine Clrn2 or human CLRN2 genes preserved normal hearing in treated Clrn2-/- mice. Conversely, mutated forms of CLRN2, identified in patients with post-lingual moderate to severe hearing loss, failed to prevent hearing loss. The ectopic expression of clarin-2 successfully prevented the loss of stereocilia, maintained normal mechanoelectrical transduction, preserved inner hair cell synaptic function, and ensured near-normal hearing thresholds over time. Maximal hearing preservation was observed when Clrn2 was delivered prior to the loss of transducing stereocilia. Our findings demonstrate that gene therapy is effective for the treatment of post-lingual hearing impairment and age-related deafness associated with CLRN2 patient mutations.

Gaussian Processes for Hearing Threshold Estimation Using Auditory Brainstem Responses.

The Auditory Brainstem Response (ABR) plays an important role in diagnosing and managing hearing loss, but can be challenging and time-consuming to measure. Test times are especially long when multiple ABR measurements are needed, e.g., when estimating hearing threshold at a range of frequencies. While many detection methods have been developed to reduce ABR test times, the majority were designed to detect the ABR at a single stimulus level and do not consider correlations in ABR waveforms across levels. These correlations hold valuable information, and can be exploited for more efficient hearing threshold estimation. This was achieved in the current work using a Gaussian Process (GP), i.e., a Bayesian approach for non-linear regression. The function to estimate with the GP was the ABR's amplitude across stimulus levels, from which hearing threshold was ultimately inferred. Active learning rules were also designed to automatically adjust the stimulus level and efficiently locate hearing threshold. Simulation results show test time reductions of up to  âˆ¼ 50% for the GP compared to a sequentially applied Hotelling's T2 test, which does not consider correlations across ABR waveforms. A case study was also included to briefly assess the GP approach in ABR data from an adult volunteer.

The effect of photobiomodulation on hearing loss: A systematic review.

OBJECTIVES: To assess outcomes associated with photobiomodulation therapy (PBMT) for hearing loss in human and animal studies. DESIGN: Systematic review and narrative synthesis in accordance with PRISMA guidelines. SETTING: Data bases searched: MEDLINE, EMBASE, CENTRAL, ClinicalTrials.gov and Web of Science. No limits were placed on language or year of publication. Review conducted in accordance with the PRISMA 2020 statement. PARTICIPANTS: All human and animal subjects treated with PBMT for hearing loss. MAIN OUTCOME MEASURES: Pre- and post-PBMT audio metric outcomes. RESULTS: Searches identified 122 abstracts and 49 full text articles. Of these, 17 studies met the inclusion criteria, reporting outcomes in 327 animals (11 studies), 30 humans (1 study), and 40 animal specimens (5 studies). PBMT parameters included 6 different wavelengths: 908 nm (1 study), 810 nm (1 study), 532 & 635 nm (1 study), 830 nm (3 studies), 808 nm (11 studies). The duration ranged from 4 to 60 minutes in a session, and the follow-up ranged from 5-28 days. Outcomes improved significantly when wavelengths within the range of 800-830 nm were used, and with greater duration of PBMT exposure. Included studies predominantly consisted of non-randomized controlled trials (10 studies). CONCLUSIONS: Hearing outcomes following PBMT appear to be superior to no PBMT for subjects with hearing loss, although higher level evidence is required to verify this. PBMT enables concentrated, focused delivery of light therapy to the inner ear through a non-invasive manner with minimal side effects. As a result of heterogeneity in reporting PBMT parameters and outcomes across the included studies, direct comparison is challenging.

Puerarin inhibits cisplatin-induced ototoxicity in mice through regulation of TRPV1-dependent calcium overload.

Puerarin (PUE), a flavonoid derivative with vasodilatory effects found in the traditional Chinese medicine kudzu, has anti-sensorineural hearing loss properties. However, the mechanism of its protective effect against ototoxicity is not well understood. In this study, we used in vitro and in vivo methods to investigate the protective mechanism of puerarin against cisplatin (CDDP)-induced ototoxicity. We established an ototoxicity model of CDDP in BALB/c mice and assessed the degree of hearing loss and cochlear cell damage. We used bioinformatics analysis, molecular docking, histological analysis, and biochemical and molecular biology to detect the expression of relevant factors. Our results show that puerarin improved CDDP-induced hearing loss and reduced hair cell loss. It also blocked CDDP-induced activation of TRPV1 and inhibited activation of IP3R1 to prevent intracellular calcium overload. Additionally, puerarin blocked CDDP-stimulated p65 activation, reduced excessive ROS production, and alleviated cochlear cell apoptosis. Our study provides new evidence and potential targets for the protective effect of puerarin against drug-induced hearing loss. Puerarin ameliorates cisplatin-induced ototoxicity and blocks cellular apoptosis by inhibiting CDDP activated TRPV1/IP3R1/p65 pathway, blocking induction of calcium overload and excessive ROS expression.

Drug screening identifies aldose reductase as a novel target for treating cisplatin-induced hearing loss.

Cisplatin is a frequently used chemotherapeutic medicine for cancer treatment. Permanent hearing loss is one of the most serious side effects of cisplatin, but there are few FDA-approved medicines to prevent it. We applied high-through screening and target fishing and identified aldose reductase, a key enzyme of the polyol pathway, as a novel target for treating cisplatin ototoxicity. Cisplatin treatment significantly increased the expression level and enzyme activity of aldose reductase in the cochlear sensory epithelium. Genetic knockdown or pharmacological inhibition of aldose reductase showed a significant protective effect on cochlear hair cells. Cisplatin-induced overactivation of aldose reductase led to the decrease of NADPH/NADP+ and GSH/GSSG ratios, as well as the increase of oxidative stress, and contributed to hair cell death. Results of target prediction, molecular docking, and enzyme activity detection further identified that Tiliroside was an effective inhibitor of aldose reductase. Tiliroside was proven to inhibit the enzymatic activity of aldose reductase via competitively interfering with the substrate-binding region. Both Tiliroside and another clinically approved aldose reductase inhibitor, Epalrestat, inhibited cisplatin-induced oxidative stress and subsequent cell death and thus protected hearing function. These findings discovered the role of aldose reductase in the pathogenesis of cisplatin-induced deafness and identified aldose reductase as a new target for the prevention and treatment of hearing loss.

Comparison of ASSR and frequency specificity ABR induced by NB CE-Chirp for prediction of behavioral hearing thresholds in children with conductive hearing loss.

BACKGROUND: Electrophysiological tests are often used to evaluate hearing loss in infants and young children with conductive hearing loss, no matter to quantify or characterize. However, there are advantages and disadvantages associated with the various electrophysiological tests that are currently available. Therefore, there is no gold standard test. This study aimed to compare the value of narrow-band (NB) CE-Chirp-induced auditory steady-state response (ASSR) and auditory brainstem response (ABR) for assessing hearing thresholds in children with conductive hearing loss. We hope to identify an effective electrophysiological testing method to evaluate conductive hearing loss and provide a reference for clinical hearing assessment of infants with conductive hearing loss. SUBJECTS: and Methods: We selected 27 children (41 ears) aged 3-6 years with otitis media with effusion (OME). Within 1 day, they underwent behavioral audiometry and NB CE-Chirp-induced ASSR and ABR tests in sequence. Pearson's correlation analysis was performed to compare behavioral audiometry thresholds and ASSR and ABR response thresholds at 500, 1000, 2000, and 4000 Hz. RESULTS: The behavioral audiometry thresholds of all children were strongly correlated with the response thresholds of the two electrophysiological tests, with correlation coefficients of 0.659, 0.605, 0.723, and 0.857 for ASSR, and 0.587, 0.684, 0.753, and 0.802 for ABR. The proportion of children with a difference of ≤10 dB between ASSR and behavioral audiometry thresholds or between ABR and behavioral audiometry thresholds was not high, especially in the low frequencies. ABR results were superior to ASSR results in terms of predicting actual hearing levels. At 0.5, 1, 2, and 4 kHz, the average differences between the behavioral hearing thresholds and ASSR thresholds in the 41 ears were 5.6, 5.7, 2, and 5.6 dB, respectively. The average differences between behavioral hearing thresholds and ABR thresholds was -5.6, -1.4, -6.8, and 3.2 dB, respectively. The hearing loss configuration of the ASSR exhibited a peaked pattern, similar to behavioral audiometry, whereas the ABR exhibited an ascending pattern. The time to perform the single-ear ASSR test was 5.9 min, whereas the ABR test took 17.0 min. CONCLUSION: ASSR and ABR induced by the NB CE-Chirp correlated well with behavioral audiometry in children with conductive hearing loss. The NB CE-Chirp ASSR has advantages in terms of testing time and hearing configuration evaluation, whereas ABR has better reliability than ASSR. However, the stability of ASSR and ABR induced by the NB CE-Chirp is poor, and the thresholds obtained cannot replace behavioral audiometry in evaluating the true hearing of children with conductive hearing loss. However, ASSR and ABR can be used as auxiliary tests for cross-validation.

Does Hearing Impairment Impact Spatial Orientation, Navigation, and Rotation Abilities?

OBJECTIVES: Spatial cognition is a perceptual-motor function that pertains to the comprehension and processing of two-dimensional and three-dimensional space. The impairment of any sensory system can have adverse effects on cognitive functioning. The objective of this study is to examine spatial cognition in adults with hearing impairments. METHODS: There were a total of 61 individuals in this study: thirty-six with hearing loss and 25 with normal hearing. The Spatial Orientation Test (SOT), the Mental Rotation test (MR), and the Money's Road Map Test (RMT) were administered to assess participants' spatial learning-orientation, mental imagery-rotation, and spatial navigation abilities. A high number of errors in RMT, high angle difference in SOT and a low score in MR suggest poor spatial abilities. RESULTS: Participants with hearing loss had a greater number of RMT errors and SOT angle difference, but lower MR scores than those with normal hearing (P < .001). Hearing impairment negatively impacted all 3 spatial cognitive assessments. Hearing loss was associated with a 6.9 increase in the number of RMT errors (95% Confidence Interval (CI): 4.8, 9), a 23.6 increase in the SOT angle difference (95% CI: 16, 31.2), and an 8.5 decrease in the MR score (95% CI: -10.8, -6.2). CONCLUSIONS: The study found that individuals with hearing loss exhibited lower performance in various cognitive tasks related to spatial orientation, navigation, spatial learning, mental imagery, and rotation abilities when compared to an age and sex matched control group. In future study, it is imperative to place greater emphasis on hearing loss as a potential detrimental factor in the prediction of spatial cognition impairment.

Trend of incidence rate of age-related diseases: results from the National Health Insurance Service-National Sample Cohort (NHIS-NSC) database in Korea: a cross- sectional study.

BACKGROUND: This study aimed to identify and select age-related diseases (ARDs) in Korea, which is about to have a super-aged society, and to elucidate patterns in their incidence rates. METHODS: The National Health Insurance Service-National Sample Cohort, comprising 1 million health insurance and medical benefit beneficiaries in Korea from 2002 to 2019, was utilized. We selected 14 diseases with high disease burden and prevalence among Koreans from the 92 diseases defined in the Global Burden of Diseases, Injuries, and Risk Factors Study as ARDs. The annual incidence rate represented the number of patients newly diagnosed with an ARD each year from 2006 to 2019, excluding those with a history of ARD diagnosis from 2002 to 2005. The incidence rate by age was categorized into 10-year units based on age as of 2019. The number of patients with ARDs in each age group was used as the numerator, and the incidence rate for each age group was calculated with the age group as the denominator. RESULTS: Regarding the annual incidence rates of ARDs from 2006 to 2019, chronic obstructive pulmonary disease, congestive heart failure, and ischemic heart disease decreased annually, whereas dyslipidemia, chronic kidney disease, cataracts, hearing loss, and Parkinson's disease showed a significant increase. Hypertension, diabetes, cerebrovascular disease, osteoporosis, osteoarthritis, and age-related macular degeneration initially displayed a gradual decrease in incidence but exhibited a tendency to increase after 2015. Concerning age-specific incidence rates of ARDs, two types of curves emerged. The first type, characterized by an exponential increase with age, was exemplified by congestive heart failure. The second type, marked by an exponential increase peaking between ages 60 and 80, followed by stability or decrease, was observed in 13 ARDs, excluding congestive heart failure. However, hypertension, ischemic heart disease, cerebrovascular disease, chronic obstructive pulmonary disease, and hearing loss in men belonged to the first type. CONCLUSIONS: From an epidemiological perspective, there are similar characteristics in age-specific ARDs that increase with age, reaching a peak followed by a plateau or decrease in Koreans.

Reliability and Accuracy of Chirp Based Multiple Auditory Steady State Response (MSSR) and Auditory Brainstem Response (ABR) in Children.

OBJECTIVE: The purpose of this study was to compare the reliability and accuracy of chirp-based Multiple Auditory Steady State Response (MSSR) and Auditory Brainstem Response (ABR) in children. METHODS: The prospective clinical study was conducted at Selayang Hospital (SH) and Hospital Canselor Tuanku Muhriz (HCTM) within one year. A total of 38 children ranging from 3 to 18 years old underwent hearing evaluation using ABR tests and MSSR under sedation. The duration of both tests were then compared. RESULTS: The estimated hearing threshold of frequency specific chirp MSSR showed good correlation with ABR especially in higher frequencies such as 2000 Hz and 4000Hz with the value of cronbach alpha of 0.890, 0.933, 0.970 and 0.969 on 500Hz, 1000Hz, 2000Hz and 4000Hz. The sensitivity of MSSR is 0.786, 0.75, 0.957 and 0.889 and specificity is 0.85, 0.882, 0.979 and 0.966 over 500Hz, 1000Hz, 2000Hz and 4000Hz. The duration of MSSR tests were shorter than ABR tests in normal hearing children with an average of 35.3 minutes for MSSR tests and 46.4 minutes for ABR tests. This can also be seen in children with hearing loss where the average duration for MSSR tests is 40.0 minutes and 52.0 minutes for ABR tests. CONCLUSION: MSSR showed good correlation and reliability in comparison with ABR especially on higher frequencies. Hence, MSSR is a good clinical test to diagnose children with hearing loss.

Influence of hearing loss on sibling relationships: Perspectives of the normal hearing sibling.

BACKGROUND: Sibling relationships, one of the most extended relationships in life, contribute to an individual's social and emotional development. However, this relationship may be influenced if one sibling has a hearing loss. OBJECTIVES: This study explored the influence of a hearing loss on the sibling relationship by gaining the perspectives of the sibling with normal hearing. METHOD: Adopting a phenomenological research design, semi-structured online interviews were conducted using Zoom and WhatsApp with nine participants who have siblings with hearing loss. RESULTS: Hearing loss may significantly impact the normal hearing sibling's quality of life, affecting their psychological and social well-being, familial and peer relationships and overall experiences. Personal development, independence, maturity and closer sibling bonds were some of the positive influences reported by the participants. Attendance to aural rehabilitation therapy sessions, knowledge of hearing loss, personalities and household living arrangements positively influenced the relationship between siblings. The lack of attendance to aural rehabilitation sessions resulted in communication difficulties between siblings. CONCLUSION: There is a need to include siblings in aural rehabilitation and family engagements related to the child with hearing loss to provide a holistic and more effective rehabilitation and adjustment process.Contribution: This study aimed to improve family-centred intervention as it is focussing on the siblings' perspectives of the hearing loss. Furthermore, previous studies have generally focussed on adults and not much research has been conducted surrounding the sibling relationship.

Investigating the optimal stimulus to evoke the binaural interaction component of the auditory brainstem response.

Objective assessment of spatial and binaural hearing deficits remains a major clinical challenge. The binaural interaction component (BIC) of the auditory brainstem response (ABR) holds promise as a non-invasive biomarker for diagnosing such deficits. However, while comparative studies have reported robust BIC in animal models, BIC in humans can sometimes be unreliably evoked even in subjects with normal hearing. Here we explore the hypothesis that the standard methodology for collecting monaural ABRs may not be ideal for electrophysiological assessment of binaural hearing. This study aims to improve ABR BIC measurements by determining more optimal stimuli to evoke it. Building on previous methodology demonstrated to enhance peak amplitude of monaural ABRs, we constructed a series of level-dependent chirp stimuli based on empirically derived latencies of monaural-evoked ABR waves I, IV and the binaural-evoked BIC DN1, the most prominent BIC peak, in a cohort of ten chinchillas. We hypothesized that chirps designed based on BIC DN1 latency would specifically enhance across-frequency temporal synchrony in the afferent inputs leading to the binaural circuits that produce the BIC and would thus produce a larger DN1 than either traditional clicks or chirps designed to optimize monaural ABRs. Compared to clicks, we found that level-specific chirp stimuli evoked significantly greater BIC DN1 amplitudes, and that this effect persisted across all stimulation levels tested. However, we found no significant differences between BICs resulting from chirps created using binaural-evoked BIC DN1 latencies and those using monaural-evoked ABR waves I or IV. These data indicate that existing level-specific, monaural-based chirp stimuli may improve BIC detectability and reduce variability in human BIC measurements.

The effect of age on the hearing-related quality of life in normal-hearing adults.

INTRODUCTION: Recently, a new holistic Patient Reported Outcome Measure (PROM) to assess hearing-related quality of life was developed, named the hearing-related quality of life questionnaire for Auditory-VIsual, COgnitive and Psychosocial functioning (hAVICOP). The purpose of the current study was to evaluate if the hAVICOP is sufficiently sensitive to detect an age effect in the hearing-related quality of life. METHODS: One-hundred thirteen normal-hearing participants (mean age: 42.13; range: 19 to 69 years) filled in the entire hAVICOP questionnaire online through the Research Electronic Data Capture surface. The hAVICOP consists of 27 statements, across three major subdomains (auditory-visual, cognitive, and psychosocial functioning), which have to be rated on a visual analogue scale ranging from 0 (rarely to never) to 100 (almost always). Mean scores were calculated for each subdomain separately as well as combined within a total score; the worse one's hearing-related quality of life, the lower the score. Linear regression models were run to predict the hAVICOP total as well as the three subdomain scores from age and sex. RESULTS: A significant main effect of age was observed for the total hAVICOP and all three subdomain scores, indicating a decrease in hearing-related quality of life with increasing age. For none of the analyses, a significant sex effect was found. CONCLUSION: The hAVICOP is sufficiently sensitive to detect an age effect in the hearing-related quality of life within a large group of normal-hearing adults, emphasizing its clinical utility. This age effect on the hearing-related quality of life might be related to the interplay of age-related changes in the bottom-up and top-down processes involved during speech processing.

A multi-channel EEG mini-cap can improve reliability for recording auditory brainstem responses in chinchillas.

BACKGROUND: Disabling hearing loss affects nearly 466 million people worldwide (World Health Organization). The auditory brainstem response (ABR) is the most common non-invasive clinical measure of evoked potentials, e.g., as an objective measure for universal newborn hearing screening. In research, the ABR is widely used for estimating hearing thresholds and cochlear synaptopathy in animal models of hearing loss. The ABR contains multiple waves representing neural activity across different peripheral auditory pathway stages, which arise within the first 10 ms after stimulus onset. Multi-channel (e.g., 32 or higher) caps provide robust measures for a wide variety of EEG applications for the study of human hearing. However, translational studies using preclinical animal models typically rely on only a few subdermal electrodes. NEW METHOD: We evaluated the feasibility of a 32-channel rodent EEG mini-cap for improving the reliability of ABR measures in chinchillas, a common model of human hearing. RESULTS: After confirming initial feasibility, a systematic experimental design tested five potential sources of variability inherent to the mini-cap methodology. We found each source of variance minimally affected mini-cap ABR waveform morphology, thresholds, and wave-1 amplitudes. COMPARISON WITH EXISTING METHOD: The mini-cap methodology was statistically more robust and less variable than the conventional subdermal-needle methodology, most notably when analyzing ABR thresholds. Additionally, fewer repetitions were required to produce a robust ABR response when using the mini-cap. CONCLUSIONS: These results suggest the EEG mini-cap can improve translational studies of peripheral auditory evoked responses. Future work will evaluate the potential of the mini-cap to improve the reliability of more centrally evoked (e.g., cortical) EEG responses.

Distributional learning of musical pitch despite tone deafness in individuals with congenital amusia.

Congenital amusia is an innate and lifelong deficit of music processing. This study investigated whether adult listeners with amusia were still able to learn pitch-related musical chords based on stimulus frequency of statistical distribution, i.e., via distributional learning. Following a pretest-training-posttest design, 18 amusics and 19 typical, musically intact listeners were assigned to bimodal and unimodal conditions that differed in distribution of the stimuli. Participants' task was to discriminate chord minimal pairs, which were transposed to a novel microtonal scale. Accuracy rates for each test session were collected and compared between the two groups using generalized mixed-effects models. Results showed that amusics were less accurate than typical listeners at all comparisons, thus corroborating previous findings. Importantly, amusics-like typical listeners-demonstrated perceptual gains from pretest to posttest in the bimodal condition (but not the unimodal condition). The findings reveal that amusics' distributional learning of music remains largely preserved despite their deficient music processing. Implications of the results for statistical learning and intervention programs to mitigate amusia are discussed.