Construction and Evaluation of a High-Frequency Hearing Loss Screening Tool for Community Residents.
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ABSTRACT:
SUBMITTER: Wang Y
PROVIDER: S-EPMC8657277 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:The OTOA gene (Locus: DFNB22) is reported to be one of the causative genes for non-syndromic autosomal recessive hearing loss. The copy number variations (CNVs) identified in this gene are also known to cause hearing loss, but have not been identified in Japanese patients with hearing loss. Furthermore, the clinical features of OTOA-associated hearing loss have not yet been clarified. In this study, we performed CNV analyses of a large Japanese hearing loss cohort, and identified CNVs in 234 of 2262 (10.3%, 234/2262) patients with autosomal recessive hearing loss. Among the identified CNVs, OTOA gene-related CNVs were the second most frequent (0.6%, 14/2262). Among the 14 cases, 2 individuals carried OTOA homozygous deletions, 4 carried heterozygous deletions with single nucleotide variants (SNVs) in another allele. Additionally, 1 individual with homozygous SNVs in the OTOA gene was also identified. Finally, we identified 7 probands with OTOA-associated hearing loss, so that its prevalence in Japanese patients with autosomal recessive hearing loss was calculated to be 0.3% (7/2262). As novel clinical features identified in this study, the audiometric configurations of patients with OTOA-associated hearing loss were found to be mid-frequency. This is the first study focused on the detailed clinical features of hearing loss caused by this gene mutation and/or gene deletion.
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Project description:As the number of genes identified for linkage to hearing loss has been increasing and more public databases have become available, we aimed to systematically evaluate all variants reported for nonsyndromic hearing loss (NSHL) based on their allele frequencies (AFs) in the general population. Among the 3,549 variants in 97 NSHL genes reported as pathogenic/likely pathogenic in ClinVar and HGMD, 1,618 were found in public databases (gnomAD, ExAC, EVS, and 1000G). To evaluate the pathogenicity of these variants, we employed AF thresholds and NSHL-optimized ACMG guidelines. AF thresholds were determined using a high-resolution variant frequency framework and Hardy-Weinberg equilibrium calculation: 0.6% and 0.1% for recessive and dominant genes, respectively. Filtering AFs of variants linked to NSHL were obtained based on AFs reported in gnomAD and ExAC. We found that 48 variants in 23 genes had filtering AFs above the suggested thresholds and assumed that these variants might be benign based on their filtering AFs. 47 variants, except for one notorious high-frequency GJB2 mutation (c.109G > A; p.Val37Ile), were confirmed to be benign/likely benign by the NSHL-optimized ACMG guidelines. The proposed systematic approach will aid in precise evaluation of NSHL variant pathogenicity in the context of filtering AFs, AF thresholds, and NSHL-specific ACMG guidelines, thus improving NSHL diagnostics.
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Project description:BACKGROUND:Hearing loss (HL) affects 466 million people of all ages worldwide, with a rapidly increasing prevalence, and therefore requires appropriate public health policies. Multi-disciplinary approaches that make use of eHealth services can build the evidence to influence public policy. The European Union-funded project EVOTION developed a platform that is fed with real-time data from hearing aids, a smartphone, and additional clinical data and makes public health policy recommendations based on hypothetical public health policy-making models, a big data engine and decision support system. The present study aimed to evaluate this platform as a new tool to support policy-making for HL. METHODS:A total of 23 key stakeholders in the United Kingdom, Croatia, Bulgaria and Poland evaluated the platform according to the Strengths, Weaknesses, Opportunities and Threats methodology. RESULTS:There was consensus that the platform, with its advanced technology as well as the amount and variety of data that it can collect, has huge potential to inform commissioning decisions, public health regulations and affect healthcare as a whole. To achieve this, several limitations and external risks need to be addressed and mitigated. Differences between countries highlighted that the EVOTION tool should be used and managed according to local constraints to maximise success. CONCLUSION:Overall, the EVOTION platform can equip HL policy-makers with a novel data-driven tool that can support public health policy-making for HL in the future.
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Project description:BackgroundHigh-frequency hearing loss is a significant occupational health concern in many countries, and early identification can be effective for preventing hearing loss. The study aims to construct and validate a risk model for HFHL, and develop a nomogram for predicting the individual risk in noise-exposed workers.MethodsThe current research used archival data from the National Key Occupational Diseases Survey-Sichuan conducted in China from 2014 to 2017. A total of 32,121 noise-exposed workers completed the survey, of whom 80% workers (n = 25,732) comprised the training cohort for risk model development and 20% workers (n = 6389) constituted the validation cohort for model validation. The risk model and nomogram were constructed using binary logistic models. The effectiveness and calibration of the model were evaluated with the receiver operating characteristic curve and calibration plots, respectively.ResultsA total of 10.06% of noise-exposed workers had HFHL. Age (OR = 1.09, 95% CI: 1.083-1.104), male sex (OR = 3.25, 95% CI: 2.85-3.702), noise exposure duration (NED) (OR = 1.15, 95% CI: 1.093-1.201), and a history of working in manufacturing (OR = 1.50, 95% CI: 1.314-1.713), construction (OR = 2.29, 95% CI: 1.531-3.421), mining (OR = 2.63, 95% CI: 2.238-3.081), or for a private-owned enterprise (POE) (OR = 1.33, 95% CI: 1.202-1.476) were associated with an increased risk of HFHL (P < 0.05).ConclusionsThe risk model and nomogram for HFHL can be used in application-oriented research on the prevention and management of HFHL in workplaces with high levels of noise exposure.
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Project description:BackgroundHigh-frequency hearing loss is one of the most common problems in the aging population and with those who have a history of exposure to loud noises. This type of hearing loss can be frustrating and disabling, making it difficult to understand speech communication and interact effectively with the world.ObjectiveThis study aimed to examine the impact of spatially unique haptic vibrations representing high-frequency phonemes on the self-perceived ability to understand conversations in everyday situations.MethodsTo address high-frequency hearing loss, a multi-motor wristband was developed that uses machine learning to listen for specific high-frequency phonemes. The wristband vibrates in spatially unique locations to represent which phoneme was present in real time. A total of 16 participants with high-frequency hearing loss were recruited and asked to wear the wristband for 6 weeks. The degree of disability associated with hearing loss was measured weekly using the Abbreviated Profile of Hearing Aid Benefit (APHAB).ResultsBy the end of the 6-week study, the average APHAB benefit score across all participants reached 12.39 points, from a baseline of 40.32 to a final score of 27.93 (SD 13.11; N=16; P=.002, 2-tailed dependent t test). Those without hearing aids showed a 10.78-point larger improvement in average APHAB benefit score at 6 weeks than those with hearing aids (t14=2.14; P=.10, 2-tailed independent t test). The average benefit score across all participants for ease of communication was 15.44 (SD 13.88; N=16; P<.001, 2-tailed dependent t test). The average benefit score across all participants for background noise was 10.88 (SD 17.54; N=16; P=.03, 2-tailed dependent t test). The average benefit score across all participants for reverberation was 10.84 (SD 16.95; N=16; P=.02, 2-tailed dependent t test).ConclusionsThese findings show that vibrotactile sensory substitution delivered by a wristband that produces spatially distinguishable vibrations in correspondence with high-frequency phonemes helps individuals with high-frequency hearing loss improve their perceived understanding of verbal communication. Vibrotactile feedback provides benefits whether or not a person wears hearing aids, albeit in slightly different ways. Finally, individuals with the greatest perceived difficulty understanding speech experienced the greatest amount of perceived benefit from vibrotactile feedback.
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Project description:BackgroundMutations in CDH23 are responsible for Usher syndrome 1D and recessive non-syndromic hearing loss. In this study, we revealed the prevalence of CDH23 mutations among patients with specific clinical characteristics.MethodsAfter excluding patients with GJB2 mutations and mitochondrial m.1555A > G and m.3243A > G mutations, subjects for CDH23 mutation analysis were selected according to the following criteria: 1) Sporadic or recessively inherited hearing loss 2) bilateral non-syndromic congenital hearing loss, 3) no cochlear malformation, 4) a poorer hearing level at high frequencies than at low frequencies, and 5) severe or profound hearing loss at higher frequencies.ResultsSeventy-two subjects were selected from 621 consecutive probands who did not have environmental causes for their hearing loss. After direct sequencing, 13 of the 72 probands (18.1%) had homozygous or compound heterozygous CDH23 mutations. In total, we identified 16 CDH23 mutations, including five novel mutations. The 16 mutations included 12 missense, two frameshift, and two splice-site mutations.ConclusionsThese results revealed that CDH23 mutations are highly prevalent in patients with congenital high-frequency sporadic or recessively inherited hearing loss and that the mutation spectrum was diverse, indicating that patients with these clinical features merit genetic analysis.
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Project description:Some hummingbirds produce unique high-frequency vocalizations. It remains unknown whether these hummingbirds can hear these sounds, which are produced at frequencies beyond the range at which most birds can hear. Here, we show behavioral and neural evidence of high-frequency hearing in a hummingbird, the Ecuadorian Hillstar (Oreotrochilus chimborazo). In the field, hummingbirds responded to playback of high-frequency song with changes in body posture and approaching behavior. We assessed neural activation by inducing ZENK expression in the brain auditory areas in response to the high-frequency song. We found higher ZENK expression in the auditory regions of hummingbirds exposed to the high-frequency song compared to controls, while no difference was observed in the hippocampus between groups. The behavioral and neural responses show that this hummingbird can hear sounds at high frequencies. This is the first evidence of the use of high-frequency vocalizations and high-frequency hearing in conspecific communication in a bird.
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Project description:There is increasing evidence that hearing-impaired (HI) individuals do not use the same listening strategies as normal-hearing (NH) individuals, even when wearing optimally fitted hearing aids. In this perspective, better characterization of individual perceptual strategies is an important step toward designing more effective speech-processing algorithms. Here, we describe two complementary approaches for (a) revealing the acoustic cues used by a participant in a /d/-/g/ categorization task in noise and (b) measuring the relative contributions of these cues to decision. These two approaches involve natural speech recordings altered by the addition of a “bump noise.” The bumps were narrowband bursts of noise localized on the spectrotemporal locations of the acoustic cues, allowing the experimenter to manipulate the consonant percept. The cue-weighting strategies were estimated for three groups of participants: 17 NH listeners, 18 HI listeners with high-frequency loss, and 15 HI listeners with flat loss. HI participants were provided with individual frequency-dependent amplification to compensate for their hearing loss. Although all listeners relied more heavily on the high-frequency cue than on the low-frequency cue, an important variability was observed in the individual weights, mostly explained by differences in internal noise. Individuals with high-frequency loss relied slightly less heavily on the high-frequency cue relative to the low-frequency cue, compared with NH individuals, suggesting a possible influence of supra-threshold deficits on cue-weighting strategies. Altogether, these results suggest a need for individually tailored speech-in-noise processing in hearing aids, if more effective speech discriminability in noise is to be achieved.
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Project description:We demonstrate a new method, using a universal array approach termed multiplex allele-specific PCR-based universal array (ASPUA), and applied it to the mutation detection of hereditary hearing loss. Mutations in many different genes may be the cause of hereditary hearing loss, a sensory defect disorder. Effective methods for genetic diagnosis are clearly needed to provide clinical management. Owing to the broad genetic basis of this condition, clinical assay of such a highly heterogeneous disorder is expensive and time consuming. In ASPUA, the allele discrimination reaction is carried out in solution by multiplex allele-specific PCR and a universal solid phase array with different tag probes is used to display the PCR result. The purpose of developing the ASPUA platform was to utilize the rapidity and simplicity of the amplification refractory mutation system (ARMS) with the detection power of microarray hybridization. This is the first report of the combination of these two technologies, which allow for the completion of allele-specific detection of 11 of the most frequent mutations causing hereditary hearing loss in under 5 hr. The ASPUA platform was validated by accurately analyzing 141 patient samples that had been previously genotyped for GJB2, GJB3, SLC26A4, and MTRNR1. In addition, we also developed a simplified assay by using streptavidin-coated magnetic beads instead of fluorescence for signal display that can be assessed through a conventional light microscope. We demonstrate that the ASPUA platform is rapid, cost-effective, and easily-used, and is especially appropriate for mutation detection in clinical genetic diagnostics.
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