Features of the speech processing network in post- and prelingually deaf cochlear implant users.

The onset of hearing loss can lead to altered brain structure and functions. However, hearing restoration may also result in distinct cortical reorganization. A differential pattern of functional remodeling was observed between post- and prelingual cochlear implant users, but it remains unclear how these speech processing networks are reorganized after cochlear implantation. To explore the impact of language acquisition and hearing restoration on speech perception in cochlear implant users, we conducted assessments of brain activation, functional connectivity, and graph theory-based analysis using functional near-infrared spectroscopy. We examined the effects of speech-in-noise stimuli on three groups: postlingual cochlear implant users (n = 12), prelingual cochlear implant users (n = 10), and age-matched individuals with hearing controls (HC) (n = 22). The activation of auditory-related areas in cochlear implant users showed a lower response compared with the HC group. Wernicke's area and Broca's area demonstrated differences network attributes in speech processing networks in post- and prelingual cochlear implant users. In addition, cochlear implant users maintain a high efficiency of the speech processing network to process speech information. Taken together, our results characterize the speech processing networks, in varying noise environments, in post- and prelingual cochlear implant users and provide new insights for theories of how implantation modes impact remodeling of the speech processing functional networks.

Abnormal white and gray matter functional connectivity is associated with cognitive dysfunction in presbycusis.

Presbycusis is characterized by high-frequency hearing loss and is closely associated with cognitive decline. Previous studies have observed functional reorganization of gray matter in presbycusis, but the information transmission between gray matter and white matter remains ill-defined. Using resting-state functional magnetic resonance imaging, we investigated differences in functional connectivity (GM-GM, WM-WM, and GM-WM) between 60 patients with presbycusis and 57 healthy controls. Subsequently, we examined the correlation between these connectivity differences with high-frequency hearing loss as well as cognitive impairment. Our results revealed significant alterations in functional connectivity involving the body of the corpus callosum, posterior limbs of the internal capsule, retrolenticular region of the internal capsule, and the gray matter regions in presbycusis. Notably, disrupted functional connectivity was observed between the body of the corpus callosum and ventral anterior cingulate cortex in presbycusis, which was associated with impaired attention. Additionally, enhanced functional connectivity was found in presbycusis between the internal capsule and the ventral auditory processing stream, which was related to impaired cognition in multiple domains. These two patterns of altered functional connectivity between gray matter and white matter may involve both bottom-up and top-down regulation of cognitive function. These findings provide novel insights into understanding cognitive compensation and resource redistribution mechanisms in presbycusis.

Aging effects on dual-route speech processing networks during speech perception in noise.

Healthy aging leads to complex changes in the functional network of speech processing in a noisy environment. The dual-route neural architecture has been applied to the study of speech processing. Although evidence suggests that senescent increases activity in the brain regions across the dorsal and ventral stream regions to offset reduced periphery, the regulatory mechanism of dual-route functional networks underlying such compensation remains largely unknown. Here, by utilizing functional near-infrared spectroscopy (fNIRS), we investigated the compensatory mechanism of the dual-route functional connectivity, and its relationship with healthy aging by using a speech perception task at varying signal-to-noise ratios (SNR) in healthy individuals (young adults, middle-aged adults, and older adults). Results showed that the speech perception scores showed a significant age-related decrease with the reduction of the SNR. The analysis results of dual-route speech processing networks showed that the functional connection of Wernicke's area and homolog Wernicke's area were age-related increases. Further to clarify the age-related characteristics of the dual-route speech processing networks, graph-theoretical network analysis revealed an age-related increase in the efficiency of the networks, and the age-related differences in nodal characteristics were found both in Wernicke's area and homolog Wernicke's area under noise environment. Thus, Wernicke's area might be a key network hub to maintain efficient information transfer across the speech process network with healthy aging. Moreover, older adults would recruit more resources from the homologous Wernicke's area in a noisy environment. The recruitment of the homolog of Wernicke's area might provide a means of compensation for older adults for decoding speech in an adverse listening environment. Together, our results characterized dual-route speech processing networks at varying noise environments and provided new insight for the compensatory theories of how aging modulates the dual-route speech processing functional networks.

Effects of Age on the Auditory Cortex During Speech Perception in Noise: Evidence From Functional Near-Infrared Spectroscopy.

OBJECTIVES: Age-related speech perception difficulties may be related to a decline in central auditory processing abilities, particularly in noisy or challenging environments. However, how the activation patterns related to speech stimulation in different noise situations change with normal aging has yet to be elucidated. In this study, we aimed to investigate the effects of noisy environments and aging on patterns of auditory cortical activation. DESIGN: We analyzed the functional near-infrared spectroscopy signals of 20 young adults, 21 middle-aged adults, and 21 elderly adults, and evaluated their cortical response patterns to speech stimuli under five different signal to noise ratios (SNRs). In addition, we analyzed the behavior score, activation intensity, oxyhemoglobin variability, and dominant hemisphere, to investigate the effects of aging and noisy environments on auditory cortical activation. RESULTS: Activation intensity and oxyhemoglobin variability both showed a decreasing trend with aging at an SNR of 0 dB; we also identified a strong correlation between activation intensity and age under this condition. However, we observed an inconsistent activation pattern when the SNR was 5 dB. Furthermore, our analysis revealed that the left hemisphere may be more susceptible to aging than the right hemisphere. Activation in the right hemisphere was more evident in older adults than in the left hemisphere; in contrast, younger adults showed leftward lateralization. CONCLUSIONS: Our analysis showed that with aging, auditory cortical regions gradually become inflexible in noisy environments. Furthermore, changes in cortical activation patterns with aging may be related to SNR conditions, and that understandable speech with a low SNR ratio but still understandable may induce the highest level of activation. We also found that the left hemisphere was more affected by aging than the right hemisphere in speech perception tasks; the left-sided dominance observed in younger individuals gradually shifted to the right hemisphere with aging.

A new active bone-conduction implant: surgical experiences and audiological outcomes in patients with bilateral congenital microtia.

PURPOSE: First-generation bone bridges (BBs) have demonstrated favorable safety and audiological benefits in patients with conductive hearing loss. However, studies on the effects of second-generation BBs are limited, especially among children. In this study, we aimed to explore the surgical and audiological effects of second-generation BBs in patients with bilateral congenital microtia. METHODS: This single-center prospective study included nine Mandarin-speaking patients with bilateral microtia. All the patients underwent BCI Generation 602 (BCI602; MED-EL, Innsbruck, Austria) implant surgery between September 2021 and June 2023. Audiological and sound localization tests were performed under unaided and BB-aided conditions. RESULTS: The transmastoid and retrosigmoid sinus approaches were implemented in three and six patients, respectively. No patient underwent preoperative planning, lifts were unnecessary, and no sigmoid sinus or dural compression occurred. The mean function gain at 0.5-4.0 kHz was 28.06 ± 4.55-dB HL. The word recognition scores improved significantly in quiet under the BB aided condition. Signal-to-noise ratio reduction by 10.56 ± 2.30 dB improved the speech reception threshold in noise. Patients fitted with a unilateral BB demonstrated inferior sound source localization after the initial activation. CONCLUSIONS: Second-generation BBs are safe and effective for patients with bilateral congenital microtia and may be suitable for children with mastoid hypoplasia without preoperative three-dimensional reconstruction.

Learning a Second Language in Adulthood Changes Subcortical Neural Encoding.

Second language learning has been shown to impact and reshape the central nervous system, anatomically and functionally. Most of the studies on second language learning and neuroplasticity have been focused on cortical areas, whereas the subcortical neural encoding mechanism and its relationship with L2 learning have not been examined extensively. The purpose of this study was to utilize frequency-following response (FFR) to examine if and how learning a tonal language in adulthood changes the subcortical neural encoding in hearing adults. Three groups of subjects were recruited: native speakers of Mandarin Chinese (native speakers (NS)), learners of the language (L2 learners), and those with no experience (native speakers of foreign languages (NSFL)). It is hypothesized that differences would exist in FFRs obtained from the three language experience groups. Results revealed that FFRs obtained from L2 learners were found to be more robust than the NSFL group, yet not on a par with the NS group. Such results may suggest that in human adulthood, subcortical neural encoding ability may be trainable with the acquisition of a new language and that neuroplasticity at the brainstem level can indeed be influenced by L2 learning.

Audiological effectiveness of Bonebridge implantation for bilateral congenital malformation of the external and middle ear.

PURPOSE: To evaluate the audiological effectiveness of Bonebridge implantation in patients with bilateral congenital malformation of the external and middle ear. METHODS: Twenty-eight cases [17 boys and 11 girls; median age, 12 years (range 8-36 years)] had unilateral Bonebridge implantation. Audiological tests were performed preoperatively and postoperatively, and included the pure-tone audiometry test, speech discrimination score (SDS), and evaluation of the unaided and aided hearing thresholds in sound fields. For the group of patients aged > 12 years, Mandarin Speech Test Materials were used to determine the SDS. For the other cases, the Mandarin Lexical Neighborhood Test was used. The daily life efficacy was assessed using the Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire postoperatively. The t and t' tests were used in the statistical analyses. RESULTS: The hearing threshold with the Bonebridge improved by 25-35 dB HL, compared to that of the unaided condition. The SDS of patients aged > 12 years improved by about 50%; the SDS also improved by 10-20% in the three patients aged < 12 years. According to the APHAB, the mean Bonebridge scores of ease of communication, background noise, and reverberation decreased by 60.3 ± 15.7, 50.2 ± 11.1, and 59.4 ± 7.8, respectively, compared to the preoperative scores, and the Bonebridge score of aversiveness was higher (69.8 ± 10.8) than the unaided score. CONCLUSIONS: The audiological effectiveness of the Bonebridge was significant and noticeable in bilateral congenital malformation of the external and middle ear. The Bonebridge can be an alternative and effective solution for those patients to improve hearing.

Genome-wide DNA methylation analysis of human peripheral blood reveals susceptibility loci of diabetes-related hearing loss.

Diabetes-related hearing loss (DRHL) is a complication of diabetes mellitus that is drawing more attention currently. DNA methylation has a critical role in the pathogenesis of type 2 diabetes mellitus (T2DM) and its complications. Therefore, we investigated the genome-wide DNA methylation of peripheral blood of T2DM patients with/without hearing loss in order to explore the susceptibility loci of DRHL. Between DRHL group and control group, 113 gene sites were identified to be differentially methylated regions (DMRs). Among 38 DMRs with whole samples, the classification accuracy is up to 90%. With alignment to T2DM susceptibility genes and deafness genes published, KCNJ11 was found to be the only overlapped gene. The DNA methylation level of KCNJ11 was associated with stroke (t = 2.595, p < 0.05), but not with diabetic nephropathy and diabetic retinopathy. The detective rate of distortion product otoacoustic emissions (DPOAE) from low to high frequencies (0.7-6 kHz) on the right ear was significantly correlated with the methylation level of KCNJ11. The auditory brainstem response (ABR) threshold on the right ear was also correlated (r = 0.678, p < 0.05). This DNA methylation profile indicates the susceptibility loci of DRHL. The potassium metabolism may have a critical role in the hearing loss caused by hyperglycemia.

Genome-wide identification and comparative analysis of grafting-responsive mRNA in watermelon grafted onto bottle gourd and squash rootstocks by high-throughput sequencing.

Grafting is an important agricultural technique widely used to improve plant growth, yield, and adaptation to either biotic or abiotic stresses. However, the molecular mechanisms underlying grafting-induced physiological processes remain unclear. Watermelon (Citrullus lanatus L.) is an important horticultural crop worldwide. Grafting technique is commonly used in watermelon production for improving its tolerance to stresses, especially to the soil-borne fusarium wilt disease. In the present study, we used high-throughput sequencing to perform a genome-wide transcript analysis of scions from watermelon grafted onto bottle gourd and squash rootstocks. Our transcriptome and digital gene expression (DGE) profiling data provided insights into the molecular aspects of gene regulation in grafted watermelon. Compared with self-grafted watermelon, there were 787 and 3485 genes differentially expressed in watermelon grafted onto bottle gourd and squash rootstocks, respectively. These genes were associated with primary and secondary metabolism, hormone signaling, transcription factors, transporters, and response to stimuli. Grafting led to changes in expression of these genes, suggesting that they may play important roles in mediating the physiological processes of grafted seedlings. The potential roles of the grafting-responsive mRNAs in diverse biological and metabolic processes were discussed. Obviously, the data obtained in this study provide an excellent resource for unraveling the mechanisms of candidate genes function in diverse biological processes and in environmental adaptation in a graft system.

A novel mutation in the OTOF gene in a Chinese family with auditory neuropathy.

Gene therapy for monogenic auditory neuropathy (AN) has successfully improved hearing function in target gene-deficient mice. Accurate genetic diagnosis can not only clarify the etiology but also accurately locate the lesion site, providing a basis for gene therapy and guiding patient intervention and management strategies. In this study, we collected data from a family with a pair of sisters with prelingual deafness. According to their auditory tests, subject Ⅱ-1 was diagnosed with profound sensorineural hearing loss (SNHL), Ⅱ-2 was diagnosed with AN, Ⅰ-1 was diagnosed with high-frequency SNHL, and Ⅰ-2 had normal hearing. Using whole-exome sequencing (WES), one nonsense mutation, c.4030C>T (p.R1344X), and one missense mutation, c.5000C>A (p.A1667D), in the OTOF (NM_001287489.1) gene were identified in the two siblings. Their parents were heterozygous carriers of c.5000C>A (father) and c.4030C>T (mother). We hypothesized that c.5000C>A is a novel pathogenic mutation. Thus, subject Ⅱ-1 should also be diagnosed with AN caused by OTOF mutations. These findings not only expand the OTOF gene mutation spectrum for AN but also indicate that WES is an effective approach for accurately diagnosing AN.

Coping with alpine habitats: genomic insights into the adaptation strategies of Triplostegia glandulifera (Caprifoliaceae).

How plants find a way to thrive in alpine habitats remains largely unknown. Here we present a chromosome-level genome assembly for an alpine medicinal herb, Triplostegia glandulifera (Caprifoliaceae), and 13 transcriptomes from other species of Dipsacales. We detected a whole-genome duplication event in T. glandulifera that occurred prior to the diversification of Dipsacales. Preferential gene retention after whole-genome duplication was found to contribute to increasing cold-related genes in T. glandulifera. A series of genes putatively associated with alpine adaptation (e.g. CBFs, ERF-VIIs, and RAD51C) exhibited higher expression levels in T. glandulifera than in its low-elevation relative, Lonicera japonica. Comparative genomic analysis among five pairs of high- vs low-elevation species, including a comparison of T. glandulifera and L. japonica, indicated that the gene families related to disease resistance experienced a significantly convergent contraction in alpine plants compared with their lowland relatives. The reduction in gene repertory size was largely concentrated in clades of genes for pathogen recognition (e.g. CNLs, prRLPs, and XII RLKs), while the clades for signal transduction and development remained nearly unchanged. This finding reflects an energy-saving strategy for survival in hostile alpine areas, where there is a tradeoff with less challenge from pathogens and limited resources for growth. We also identified candidate genes for alpine adaptation (e.g. RAD1, DMC1, and MSH3) that were under convergent positive selection or that exhibited a convergent acceleration in evolutionary rate in the investigated alpine plants. Overall, our study provides novel insights into the high-elevation adaptation strategies of this and other alpine plants.

[Locally weighted least squares estimation of DPOAE evoked by continuously sweeping primaries].

Distortion product otoacoustic emission (DPOAE) signal can be used for diagnosis of hearing loss so that it has an important clinical value. Continuously using sweeping primaries to measure DPOAE provides an efficient tool to record DPOAE data rapidly when DPOAE is measured in a large frequency range. In this paper, locally weighted least squares estimation (LWLSE) of 2f1-f2 DPOAE is presented based on least-squares-fit (LSF) algorithm, in which DPOAE is evoked by continuously sweeping tones. In our study, we used a weighted error function as the loss function and the weighting matrixes in the local sense to obtain a smaller estimated variance. Firstly, ordinary least squares estimation of the DPOAE parameters was obtained. Then the error vectors were grouped and the different local weighting matrixes were calculated in each group. And finally, the parameters of the DPOAE signal were estimated based on least squares estimation principle using the local weighting matrixes. The simulation results showed that the estimate variance and fluctuation errors were reduced, so the method estimates DPOAE and stimuli more accurately and stably, which facilitates extraction of clearer DPOAE fine structure.

Comparisons of air-conduction hearing thresholds between manual and automated methods in a commercial audiometer.

Objective: To investigate the correlation of air-conduction thresholds between automated audiometry in a non-isolated environment and manual audiometry in participants with normal hearing and different degrees of hearing loss. Methods: Eighty-three participants aged 11-88 years old underwent automated pure-tone audiometry in a non-acoustically isolated environment, and the results were compared with those of manual pure-tone audiometry performed in a standard acoustically isolated booth, with the order of testing randomised. Six frequencies of 250, 500, 1,000, 2000, 4,000 and 8,000 Hz were tested. Results: All 166 ears were completed and 996 valid hearing threshold data were obtained, with 28 data exceeding the 95% confidence interval in the Bland-Altman plot, accounting for 2.81% of all data. The means and standard deviations of the differences for the six frequencies from 250 to 8,000 Hz were, respectively, 0.63 ± 5.31, 0.69 ± 4.50, 0.45 ± 4.99, 0.3 ± 6.2, -0.15 ± 4.8, and 0.21 ± 4.97 dB. The correlation coefficients of the two test results for normal hearing, mild, moderate, severe and above hearing loss groups were 0.95, 0.92, 0.97, and 0.96, respectively. The correlation coefficient of the automated and manual audiometry thresholds for the age groups under 40 years, 40-60 years, and 60 years above, were 0.98, 0.97 and 0.97, respectively, with all being statistically significant (p < 0.01). The response time of the three age groups were 791 ± 181 ms, 900 ± 190 ms and 1,063 ± 332 ms, respectively, and there was a significant difference between the groups under 40 years and over 60 years. Conclusion: There was good consistency between automated pure-tone audiometry in a non-acoustically isolated environment and manual pure-tone audiometry in participants with different hearing levels and different age groups.

The longitudinal relationship between hearing loss and cognitive decline in tonal language-speaking older adults in China.

Introduction: Previous longitudinal studies indicate that hearing loss and cognitive impairment are associated in non-tonal language-speaking older adults. This study aimed to investigate whether there is a longitudinal association between hearing loss and cognitive decline in older adults who speak a tonal language. Methods: Chinese-speaking older adults aged 60 years and above were recruited for baseline and 12 month follow-up measurements. All participants completed a pure tone audiometric hearing test, Hearing Impaired-Montreal Cognitive Assessment Test (HI-MoCA), and a Computerized Neuropsychological Test Battery (CANTAB). The De Jong Gierveld Loneliness Scale was used to measure loneliness, and the 21-item Depression Anxiety Stress Scale (DASS-21) was used to measure aspects of mental health. Associations between baseline hearing loss and various cognitive, mental and psychosocial measures were evaluated using logistic regression. Results: A total of 71 (29.6%) of the participants had normal hearing, 70 (29.2%) had mild hearing loss, and 99 (41.2%) had moderate or severe hearing loss at baseline, based on mean hearing thresholds in the better ear. After adjusting for demographic and other factors, baseline moderate/severe audiometric hearing loss was associated with an increased risk of cognitive impairment at follow-up (OR: 2.20, 95% CI: 1.06, 4.50). When pure-tone average (PTA) was modeled continuously, an average difference of 0.24 in HI-MoCA scores for every 10 dB increase in BE4FA existed, and an average difference of 0.07 in the change of HI-MoCA scores in a 12 month period. Discussion: The results revealed a significant longitudinal relationship between age-related hearing loss and cognitive decline in this cohort of tonal language-speaking older adults. Steps should also be taken to incorporate hearing assessment and cognitive screening in clinical protocols for older adults 60 years and above in both hearing and memory clinics.

The Relationship of Age-Related Hearing Loss with Cognitive Decline and Dementia in a Sinitic Language-Speaking Adult Population: A Systematic Review and Meta-Analysis.

Background and Objectives: Substantial evidence supports the association between untreated hearing loss, cognitive decline, and dementia in the non-tonal language-speaking population. Whether a similar association between hearing loss and cognitive decline and dementia exists in Sinitic tonal language-speaking people is yet to be elucidated. We aimed to systematically review the current evidence on the association between hearing loss and cognitive impairment/decline, and dementia in older adults who speak a Sinitic tonal language. Research Design and Methods: This systematic review considered peer-reviewed articles that employed objective or subjective hearing measurement and cognitive function, cognitive impairment, or diagnosis of dementia. All articles written in English and Chinese and published before March 2022 were included. Databases including Embase, MEDLINE, Web of Science, PsycINFO and Google Scholar, SinoMed, and CBM were utilized using MeSH terms and keywords. Results: Thirty-five articles met our inclusion criteria. Of these, 29 unique studies with an estimated 372,154 participants were included in the meta-analyses. Among all included studies, the effect size of cognitive function with hearing loss, the regression coefficient was -0.26 (95% confidence interval [CI], -0.45 to -0.07). Among cross-sectional and cohort studies, a significant association was found between hearing loss and cognitive impairment and dementia, with odds ratios of 1.85 (95% CI, 1.59-2.17) and 1.89 (95% CI, 1.50-2.38), respectively. Discussion and Implications: Most of the studies included in this systematic review observed a significant association between hearing loss and cognitive impairment and dementia. There was no significant difference to the findings in non-tonal language populations.

Features of beta-gamma phase-amplitude coupling in cochlear implant users derived from EEG.

Cochlear implants (CIs) allow patients with severe to profound hearing loss to gain or regain their sense of hearing. However, the objective assessment of auditory rehabilitation in CI users remains a challenge. In particular, the utility of phase-amplitude coupling (PAC) for evaluating postoperative rehabilitation of CI users remains unknown. In the present study, we conducted an oddball paradigm with stimuli varying in sample speech syllables and collected electroencephalography (EEG) signals for 10 CI users at the time the implant was activated and 180 days after activation. Twelve normal-hearing subjects served as controls. We explored the oscillatory properties of the neural response to syllable incongruence and the cross-frequency coupling between multiple frequencies in CI users. We found that beta-gamma coupling appeared to be enhanced in CI users compared with normal controls and this difference gradually disappeared with increasing implantation time. The present results suggest that predictively encoded auditory pathways are gradually restored in CI users. In addition, the PAC feature in unilateral CI users was found to be lateralized in the auditory cortex, which was consistent with previous studies of auditory-evoked cortical activity. Therefore, PAC may be a reference biomarker for the rehabilitation of speech discrimination in CI users.

[Analysis of clinical audiological characteristics in 868 children referred from maternal and child institutions].

Objective:To investigate the clinical audiological characteristics of children referred from maternal and child institutions and analyze the high risk factors of hearing loss, so as to provide scientific basis for further improvement of children's ear and hearing care. Methods:The subjects of this study were 868 children who were referred by maternal and child institutions in Beijing to the otology outpatient of Beijing Tongren Hospital, Capital Medical University for hearing diagnosis. All subjects underwent acoustic immittance, auditory brainstem response, distortion products otoacoustic emission and other audiological tests. Children were divided into groups according to the age of diagnosis: 0-<3 months group（242 cases）, 3-<6 months group（328 cases）, 6-<12 months group（180 cases）, ≥12 months group（118 cases）, the results of hearing diagnosis, hearing loss degree and types, the relationship between high risk factors and hearing loss in each group were compared and analyzed. Results:The age of diagnosis of 868 children was（7.13±8.29） months. 488 cases with hearing loss accounted for 56.22% and 380 cases with normal hearing accounted for 43.78%. Proportion of different degree of hearing loss of 792 ears from high to low was as follows: mild, 366 ears（46.21%）; moderate, 214 ears（27.02%）; severe, 151 ears（19.07%）; profound, 61 ears（7.70%）. There were statistically significant differences in the proportion of different hearing loss degree among 0-<3 months group, 3-<6 months group, 6-<12 months group and ≥12 months group（P<0.001）. Pairwise comparison between groups showed that the proportion of mild hearing loss of 0-<3 months group was higher than that in the other three groups（P<0.05）, there was no significant difference of moderate hearing loss among all groups（P>0.05）, the proportion of severe hearing loss of ≥12 months group was higher than that of 0-<3 months group（P<0.05）. The proportion of profound hearing loss with 0-<3 months group was lower than the other three groups（P<0.05）. In 792 ears with hearing loss, sensorineural hearing loss accounted for 67.42%, conductive hearing loss accounted for 20.71% and mixed hearing loss accounted for 11.87%. Among 98 cases with high risk factors for hearing loss, 58 cases（59.18%） were diagnosed with hearing loss. The incidence of hearing loss with high risk factors ranked from high to low was: craniofacial malformation（93.75%）, family history/congenital genetic syndrome（61.11%）, neonatal intensive care unit（NICU） hospitalization（46.43%） and others（20.00%）. Conclusion:Referrals from maternal and child institutions play an important role in the early detection of children with mild to moderate sensorineural hearing loss. Children with craniofacial malformation, family history/congenital genetic syndrome, hospitalization history of NICU and other high risk factors have a high incidence of hearing loss and should be attached with great importance.

Analysis of audiological outcomes of children referred from a universal newborn hearing screening program over 9 years in Beijing, China.

Universal newborn hearing screening (UNHS) and audiological diagnosis are crucial for children with congenital hearing loss (HL). The objective of this study was to analyze hearing screening techniques, audiological outcomes and risk factors among children referred from a UNHS program in Beijing. A retrospective analysis was performed in children who were referred to our hospital after failing UNHS during a 9-year period. A series of audiological diagnostic tests were administered to each case, to confirm and determine the type and degree of HL. Risk factors for HL were collected. Of 1839 cases, 53.0% were referred after only transient evoked otoacoustic emission (TEOAE) testing, 46.1% were screened by a combination of TEOAE and automatic auditory brainstem response (AABR) testing, and 1.0% were referred after only AABR testing. HL was confirmed in 55.7% of cases. Ears with screening results that led to referral experienced a more severe degree of HL than those with results that passed. Risk factors for HL were identified in 113 (6.1%) cases. The main risk factors included craniofacial anomalies (2.7%), length of stay in the neonatal intensive care unit longer than 5 days (2.4%) and birth weight less than 1500 g (0.8%). The statistical data showed that age (P < 0.001) and risk factors, including craniofacial anomalies (P < 0.001) and low birth weight (P = 0.048), were associated with the presence of HL. This study suggested that hearing screening plays an important role in the early detection of HL and that children with risk factors should be closely monitored.

Optimized concurrent hearing and genetic screening in Beijing, China: A cross-sectional study.

Concurrent screening has been proven to provide a comprehensive approach for management of congenital deafness and prevention of ototoxicity. The SLC26A4 gene is associated with late-onset hearing loss and is of great clinical concern. For much earlier detection of newborns with deafness-causing mutations in the SLC26A4 gene, the Beijing Municipal Government launched a chip for optimized genetic screening of 15 variants of 4 genes causing deafness based on a chip to screen for 9 variants of 4 genes, and 6 variants of the SLC26A4 gene have now been added. To ascertain the advantage of a screening chip including 15 variants of 4 genes, the trends in concurrent hearing and genetic screening were analyzed in 2019 and 2020. Subjects were 76,460 newborns who underwent concurrent hearing and genetic screening at 24 maternal and child care centers in Beijing from January 2019 to December 2020. Hearing screening was conducted using transiently evoked otoacoustic emissions (TEOAEs), distortion product otoacoustic emissions (DPOAE), or the automated auditory brainstem response (AABR). Dried blood spots were collected for genetic testing and 15 variants of 4 genes, namely GJB2, SLC26A4, mtDNA 12S rRNA, and GJB3, were screened for using a DNA microarray platform. The initial referral rate for hearing screening decreased from 3.60% (1,502/41,690) in 2019 to 3.23% (1,124/34,770) in 2020, and the total referral rate for hearing screening dropped form 0.57% (236/41,690) in 2019 to 0.54% (187/34,770) in 2020, indicating the reduced false positive rate of newborn hearing screening and policies to prevent hearing loss conducted by the Beijing Municipal Government have had a significant effect. Positivity according to genetic screening was similar in 2019 (4.970%, 2,072/41,690) and 2020 (4.863%,1,691/34,770), and the most frequent mutant alleles were c.235 del C in the GJB2 gene, followed by c.919-2 A > G in the SLC26A4 gene, and c.299 del AT in the GJB2 gene. In this cohort study, 71.43% (5/7) of newborns with 2 variants of the SLC26A4 gene were screened for newly added mutations, and 28.57% (2/7) of newborns with 2 variants of the SLC26A4 gene passed hearing screening, suggesting that a screening chip including 15 variants of 4 genes was superior at early detection of hearing loss, and especially in early identification of newborns with deafness-causing mutations in the SLC26A4 gene. These findings have clinical significance.

Clinical comparison of two automated audiometry procedures.

Objective: Automated pure-tone audiometry has been shown to provide similar hearing threshold estimates to conventional audiometry, but lower correlations were reported at high and low frequencies in audiometric tests than those of manual tests, while the correlations were better in the middle frequencies. In this paper, we used the same equipment and different test procedures for automated testing, and compared the results with manual test results. Design: One hundred subjects aged 18-36 years were randomly divided into two groups to perform air-conduction pure-tone audiometry (0.25, 0.5, 1, 2, 4, 8 kHz) using the ascending and shortened ascending protocols built-in to the automated audiometer, respectively. Recorded testing time, the total number of responses and the subject's preference tests were compared with those of manual tests. Results: Significant difference was found at 250 Hz regarding the distribution of the absolute difference between the two automated and the manual thresholds. The testing time spend in the ascending method (9.8 ± 1.4 min, mean ± SD) was significantly longer than in the shorted ascending method (5.8 ± 0.9 min). The total numbers of responses of the ascending method (90.5 ± 10.8 times) and shorted ascending method (62.0 ± 11.4 times) were significantly different. Finally, no significant difference was found in preferences between automated and manual procedures. Conclusion: The shorted ascending method can save lots of testing time. The difference between the two automated thresholds at 250 Hz is caused by the different test procedures, and the difference at 8,000 Hz between the automated test and the manual test can be due to the transducer types and allowable differences in calibration.