Hearing Research最新文献

{"title":"Optimizing the stimulus used to elicit the acoustic change complex: Evaluation of the pre-transition stimulus duration and stimulus complexity in normal hearing adults","authors":"Laura I.A. Schellekens ,&nbsp;Marloes L. Adank ,&nbsp;Sarah Meehan ,&nbsp;Marc P. van der Schroeff ,&nbsp;Jantien L. Vroegop","doi":"10.1016/j.heares.2025.109251","DOIUrl":"10.1016/j.heares.2025.109251","url":null,"abstract":"<div><h3>Introduction</h3><div>The Acoustic Change Complex (ACC), a cortical auditory evoked potential elicited by sound changes, is a promising measure of speech discrimination for populations unable to perform speech perception tests. However, its clinical utility is limited by long measurement times, which could be reduced by optimizing the signalto-noise ratio (SNR).</div></div><div><h3>Objectives</h3><div>To study the effect of 1) varying the pre-transition duration (PTD) and 2) tonal complexity on ACC outcomes, including N1-P2 amplitude, baseline noise, SNR, and efficiency (SNR divided by measurement time).</div></div><div><h3>Methods</h3><div>ACC responses were measured in 18 normal-hearing adults using pure-tone stimuli with a frequency change (1 to 1.1 kHz) and PTDs of 0.25, 0.5, 1, 2 and 3 s, as well as a complex tone with a 1-second PTD.</div></div><div><h3>Results</h3><div>N1-P2 amplitude increased with PTD up to 2 s. PTD 0.25 s was excluded due to response overlap. Increasing PTD from 0.5 s to 1 s increased efficiency and SNR, reducing estimated measurement time by 78.8 %. ACC presence increased with PTD (100 % for PTD 3 s), but SNR and efficiency gains were absent beyond PTD 1 s. The complex tone showed no difference in N1-P2 amplitude or SNR compared to the pure tone, but increased ACC presence by 22.5 % points.</div></div><div><h3>Conclusion</h3><div>A PTD of 1 s is recommended over 0.5 s. Increasing tonal complexity and the PTD beyond 1 s seems promising to enhance ACC specificity without compromising SNR or efficiency. These findings support stimulus optimization to improve clinical feasibility of ACC measurements.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"461 ","pages":"Article 109251"},"PeriodicalIF":2.5,"publicationDate":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143681743","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"On spontaneous oscillations of hair bundles in the amphibian papilla","authors":"Dzmitry Vaido ,&nbsp;Martín A. Toderi ,&nbsp;Dolores Bozovic","doi":"10.1016/j.heares.2025.109238","DOIUrl":"10.1016/j.heares.2025.109238","url":null,"abstract":"<div><div>Hair cells play a critical role in the auditory system, acting as key agents in active sound detection. Studying living hair cells <em>ex vivo</em> provides valuable insights into the mechanisms underlying sound detection. In this study, we investigated the nonlinear dynamics of hair bundle oscillations. We developed a robust <em>ex vivo</em> preparation of the amphibian papilla, a bullfrog’s auditory organ, and observed spontaneous oscillations of hair bundles. These oscillations were classified into three distinct types: regular, bursting, and spiking. Regular oscillators demonstrated stable oscillations with a well-defined dominant frequency. Bursting oscillators alternated between periods of stable oscillatory activity and quiescence, while spiking oscillators were mostly quiescent, interrupted by brief oscillatory bursts. The oscillation frequencies ranged from 1 to 90 Hz, with approximately 95% of cells oscillating below 40 Hz.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"460 ","pages":"Article 109238"},"PeriodicalIF":2.5,"publicationDate":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143680348","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The relationship between interaural coherence, interaural level differences, and binaural fusion","authors":"Justin M. Aronoff,&nbsp;Josephine R. LaPapa,&nbsp;Jordan Deutsch","doi":"10.1016/j.heares.2025.109241","DOIUrl":"10.1016/j.heares.2025.109241","url":null,"abstract":"<div><div>Binaural fusion is the perception of a single auditory image when signals are presented to the two ears. Decreasing the interaural coherence of the signals often degrades binaural fusion. However, perception of interaural level differences (ILDs) is minimally affected by interaural coherence changes. This study aims to determine if there is an interaction between ILDs and binaural fusion in the presence of reduced interaural coherence.</div><div>In Experiment 1, vocoded stimuli with varying envelope interaural coherences were presented containing a range of ILDs. Participants indicated the perceived size and lateralization of the auditory image they perceived. Stimuli with reduced interaural coherence were binaurally fused when there was a large ILD, even when participants indicated that the same stimulus was unfused when there was a 0 dB ILD. This suggests that signals with low interaural coherence can become binaurally fused when the stimulus has large ILDs.</div><div>To determine if participants could choose to attend to only one ear for stimuli that were reported to be fused in the presence of ILDs in Experiment 1 (i.e. if complete binaural fusion occurred), participants indicated the perceived loudness in their right ear for stimuli with varying right-ear intensities in Experiment 2. When the stimulus had a left-biased ILD, participants had a reduced ability to report changes in right ear signal intensity, suggesting that largely complete binaural fusion was fostered by the presence of large ILDs.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"460 ","pages":"Article 109241"},"PeriodicalIF":2.5,"publicationDate":"2025-03-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143673456","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hierarchical deviant processing in auditory cortex of awake mice","authors":"Dan Luo ,&nbsp;Ji Liu ,&nbsp;Ryszard Auksztulewicz ,&nbsp;Tony Ka Wing Yip ,&nbsp;Patrick O. Kanold ,&nbsp;Jan W.H. Schnupp","doi":"10.1016/j.heares.2025.109242","DOIUrl":"10.1016/j.heares.2025.109242","url":null,"abstract":"<div><div>Detecting patterns, and noticing unexpected pattern changes, in the environment is a vital aspect of sensory processing. Adaptation and prediction error responses are two components of neural processing related to these tasks, and previous studies in the auditory system in rodents show that these two components are partially dissociable in terms of the topography and latency of neural responses to sensory deviants. However, many previous studies have focused on repetitions of single stimuli, such as pure tones, which have limited ecological validity. In this study, we tested whether the auditory cortical activity shows adaptation to repetition of more complex sound patterns (disyllabic pairs). Specifically, we compared neural responses to violations of sequences based on single stimulus probability only, against responses to more complex violations based on stimulus order. We employed an auditory oddball paradigm and monitored the auditory cortex (AC) activity of awake mice (<em>N</em> = 8) using wide-field calcium imaging. We found that cortical responses were sensitive both to single stimulus probabilities and to more global stimulus patterns, as mismatch signals were elicited following both substitution deviants and transposition deviants. Notably, higher order AC area elicited larger mismatch signaling to those deviants than primary AC, which suggests a hierarchical gradient of prediction error signaling in the auditory cortex. Such a hierarchical gradient was observed for late but not early peaks of calcium transients to deviants, suggesting that the late part of the deviant response may reflect prediction error signaling in response to more complex sensory pattern violations.</div></div><div><h3>Significance statement</h3><div>Detecting the unexpected change of patterns from the dynamic environment is vital for sensory processing, as it is essential to survival for humans and animals. Using wide-field calcium imaging, we investigated whether the auditory cortex of awake mice exhibits a hierarchical gradient of prediction error signaling and its sensitivity to violations of sequences based on stimulus features and stimulus order. We discovered the high-order auditory cortex elicited more significant mismatch signaling to those deviants than primary auditory cortex in substitution and transposition deviants. Calcium transients to deviants showed a hierarchical gradient for late but not for early peaks, indicating that the late part of the deviant response may reflect prediction error signaling in response to more complex sensory pattern violations.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"460 ","pages":"Article 109242"},"PeriodicalIF":2.5,"publicationDate":"2025-03-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143680347","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The relationship between auditory brainstem responses, cognitive ability, and speech-in-noise perception among young adults with normal hearing thresholds","authors":"Mishaela DiNino ,&nbsp;Jenna Crowell ,&nbsp;Ilsa Kloiber ,&nbsp;Melissa J. Polonenko","doi":"10.1016/j.heares.2025.109243","DOIUrl":"10.1016/j.heares.2025.109243","url":null,"abstract":"<div><div>The goal of this research was to determine the contributions of auditory neural processing and cognitive abilities to predict performance on a speech-in-noise perception task in young, normal hearing adults. Two experiments were performed, each with separate cohorts of ∼30 young adults with normal hearing who performed a competing talker task which included a high-pass filtered condition that was designed to be more sensitive to auditory nerve functioning than are commonly used speech-in-noise perception tests. Predictors of performance on this competing talker task included ABR waves I and V metrics and cognitive test scores. Experiment one included click ABRs at a moderate level commensurate with the level of the competing talker task, as well as the cognitive digit span working memory test. Experiment two included high-intensity click clinical ABRs and three cognitive tests from the NIH Toolbox V3 that assessed working memory, cognitive flexibility and attention, and inhibitory control: List Sorting Working Memory, Dimensional Change Card Sort, and Flanker Inhibitory Control and Attention tests, respectively. Performance on the high-pass filtered competing talker task varied across participants in both experiments. This variability was predicted by performance on the test of inhibitory control, but not the tests involving working memory or cognitive flexibility, nor by any of the auditory processing metrics from moderate or high-intensity click ABRs. Among two groups of young adults with normal hearing, cognitive factors with very similar demands to the competing talker task seem to play the greatest role in speech-in-noise perception.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"460 ","pages":"Article 109243"},"PeriodicalIF":2.5,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143641682","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cochlear health in a cohort of cochlear implant users carrying the p.Pro51Ser variant in the COCH gene (DFNA9): A cross-sectional study evaluating the changes in the electrically evoked compound action potential (eCAP)","authors":"Julie Moyaert ,&nbsp;Annick Gilles ,&nbsp;Dyan Ramekers ,&nbsp;Griet Mertens ,&nbsp;Erik Fransen ,&nbsp;Emilie Cardon ,&nbsp;Lana Biot ,&nbsp;Eline Verhelst ,&nbsp;Vincent Van Rompaey ,&nbsp;Marc JW Lammers","doi":"10.1016/j.heares.2025.109240","DOIUrl":"10.1016/j.heares.2025.109240","url":null,"abstract":"<div><div>The present study focuses on DFNA9, an autosomal dominant disorder caused by pathogenic variants in the <em>COCH</em> gene. These mutations induce the formation of aggregates that are toxic to the fibrocytes in the extracellular matrix, ultimately leading to degeneration of spiral ganglion neurons (SGNs), which are crucial for transmitting auditory signals from the cochlea to the brain. An important tool for evaluating the function of the SGNs, which are the target cells of a cochlear implant (CI), is the electrically evoked compound action potential (eCAP). Therefore, the main objective is to evaluate the eCAP to describe the function of the SGNs and study cochlear health in CI patients with DFNA9.</div><div>For this reason, we included 15 carriers of the p.Pro51Ser variant in the <em>COCH</em> gene who received a MED-EL CI (DFNA9 group) and 15 matched control CI subjects without DFNA9 to compare the impedances and subsequently the threshold, amplitude and slope of the eCAP amplitude growth function (AGF). These parameters were evaluated from intraoperative autoART recordings (MED-EL) during CI surgery. Matching of the two groups was based on sex, age at implantation, duration of deafness, and type of implant. The first results, regarding the difference in impedance between DFNA9 and non-DFNA9 patients, show a significant interaction between time and group in the middle and basal electrodes, indicating that electrode impedances were similar in the early phase after implantation between the two groups, but increased significantly more for the DFNA9 group up to one year after implantation. Secondly, the results show that the success rate (present or absent) to record eCAP responses is lower in the DNFA9 group: eCAPs were detectable in 75.5 % of the intraoperative measurements (145/192) in comparison to 96.9 % (186/192) in the group without DFNA9. ECAP absence in the DFNA9 group was observed across the whole electrode array, but more pronounced in the basal region (channels 11 and 12). Additionally, comparing the parameters of the AGF, the maximum eCAP amplitude was consistently smaller and the AGF slope consistently shallower for the DFNA9 group compared to the control group throughout the entirety of the electrode array. Finally, the eCAP thresholds in patients with DFNA9 were higher compared to those in the control patients for all cochlear locations. To our knowledge, this is the first study to investigate the eCAP measurements in patients with DFNA9. As proven in the literature, eCAP measures correlate well with the health and survival of SGC. This means that the results of our study predominantly suggest that DFNA9 leads to an even stronger reduction in excitability and neuronal health than seen in other causes of deafness.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"460 ","pages":"Article 109240"},"PeriodicalIF":2.5,"publicationDate":"2025-03-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143621260","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Tinnitus, masked speech perception, and auditory event-related potentials in clinically normal-hearing adults","authors":"Ning Gao ,&nbsp;Sheng-Yu Tao ,&nbsp;Qian-Jie Fu ,&nbsp;John Galvin ,&nbsp;Sean Lang ,&nbsp;Ya-Feng Yu ,&nbsp;Ji-Sheng Liu ,&nbsp;Duo-Duo Tao","doi":"10.1016/j.heares.2025.109239","DOIUrl":"10.1016/j.heares.2025.109239","url":null,"abstract":"<div><div>Tinnitus can increase stress and anxiety and reduce quality of life. Compared to listeners with normal hearing and no tinnitus, listeners with tinnitus (with or without hearing loss) often have greater difficulty segregating competing speech. Previous studies have investigated neural correlates of tinnitus using the P3 component of event-related potentials (ERPs). Speech reception thresholds (SRTs) and ERPs were measured in 29 listeners with tinnitus and 25 listeners without tinnitus; all were clinically normal hearing, with pure-tone average (PTA) thresholds &lt;25 dB HL across 500, 1000, 2000, and 4000 Hz. SRTs for a male target talker were measured in speech-shaped noise (SSN; energetic masking) or with a single female speech masker (informational masking). Auditory ERPs were measured for a 1 kHz vs. 2 kHz contrast. SRTs in competing speech were significantly lower for the non-tinnitus than for the tinnitus group (<em>p</em> = 0.042); there was no significant group difference for SRTs in SSN. Latency was significantly longer for the tinnitus than for the non-tinnitus group for N1 (<em>p</em> = 0.034) and P3 (<em>p</em> &lt; 0.001), but not for P2. There were no significant group differences in terms of P2-N1 or P3 amplitude. Multilinear regression analysis showed that age at testing (<em>p</em> = 0.005), N1 latency (<em>p</em> = 0.031), P3 latency (<em>p</em> = 0.020), and P3 amplitude (<em>p</em> = 0.031) were significant predictors of SRTs in competing speech. The present results suggest that among adults with clinically normal PTAs, susceptibility to informational masking may be greater for listeners with than without tinnitus.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"460 ","pages":"Article 109239"},"PeriodicalIF":2.5,"publicationDate":"2025-03-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143577537","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Evaluating the role of age on speech-in-noise perception based primarily on temporal envelope information","authors":"Jonathan Regev ,&nbsp;Andrew J. Oxenham ,&nbsp;Helia Relaño-Iborra ,&nbsp;Johannes Zaar ,&nbsp;Torsten Dau","doi":"10.1016/j.heares.2025.109236","DOIUrl":"10.1016/j.heares.2025.109236","url":null,"abstract":"<div><div>Acoustic amplitude modulation (AM) patterns carry important information, particularly in speech. AM masking, influenced by frequency selectivity in the modulation domain, is considered a crucial factor for speech intelligibility in noisy environments. Based on recent evidence suggesting an age-related decline in AM frequency selectivity, this study investigated whether increased AM masking in older listeners is associated with reduced speech intelligibility. Speech reception thresholds (SRTs) were measured using tone-vocoded speech and maskers with no modulation, broadband AM, or narrowband AM at varying modulation frequencies. AM masked thresholds were assessed for a 4-Hz target modulation frequency. The study included young (<em>N</em> = 14, 19–25 years) and older (<em>N</em> = 14, 57–79 years) listeners with normal hearing. It was hypothesized that SRTs would be higher for the older group with modulated maskers and that the age-related increase in SRT would depend on the masker's modulation frequency content. The speech intelligibility results showed that maskers with broadband AM produced higher SRTs than unmodulated maskers. However, SRTs varied little with masker-modulation center frequency across the range tested (2–32 Hz). While older listeners exhibited lower AM frequency selectivity than young listeners, they did not consistently exhibit higher SRTs than their young counterparts across maskers. However, there was a trend for the effect of age to be greater for maskers with broadband AM than for unmodulated maskers. Overall, despite supportive trends, the results do not conclusively demonstrate that older listeners are more susceptible than young listeners to AM masking of speech.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"460 ","pages":"Article 109236"},"PeriodicalIF":2.5,"publicationDate":"2025-03-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143611668","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Influence of visual analogue of speech envelope, formants, and word onsets on word recognition is not pronounced","authors":"Kaja Rosa Benz ,&nbsp;Anne Hauswald ,&nbsp;Nathan Weisz","doi":"10.1016/j.heares.2025.109237","DOIUrl":"10.1016/j.heares.2025.109237","url":null,"abstract":"<div><div>In noisy environments, filtering out the relevant speech signal from the background noise is a major challenge. Visual cues, such as lip movements, can improve speech understanding. This suggests that lip movements carry information about speech features (e.g. speech envelope, formants, word onsets) that can be used to aid speech understanding. Moreover, the isolated visual or tactile presentation of the speech envelope can also aid word recognition. However, the evidence in this area is rather mixed, and formants and word onsets have not been studied in this context.</div><div>This online study investigates the effect of different visually presented speech features (speech envelope, formants, word onsets) during a two-talker audio on word recognition. The speech features were presented as a circle whose size was modulated over time based on the dynamics of three speech features. The circle was either modulated according to the speech features of the target speaker, the distractor speaker or an unrelated control sentence. After each sentence, the participants` word recognition was tested by writing down what they heard. We show that word recognition is not enhanced for any of the visual features relative to the visual control condition.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"460 ","pages":"Article 109237"},"PeriodicalIF":2.5,"publicationDate":"2025-03-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143629143","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Mammalian TMC1 or 2 are necessary for scramblase activity in auditory hair cells","authors":"Thibault Peineau ,&nbsp;Irina Marcovich ,&nbsp;Cristobal von Muhlenbrock Rodriguez ,&nbsp;Sydney O'Malley ,&nbsp;Runjia Cui ,&nbsp;Angela Ballesteros ,&nbsp;Jeffrey R. Holt","doi":"10.1016/j.heares.2025.109229","DOIUrl":"10.1016/j.heares.2025.109229","url":null,"abstract":"<div><div>Sensory transduction in auditory hair cells gates mechanosensitive ion channels, converting sound information into electrical signals (Zheng and Holt, 2021). Previously, we found that Transmembrane channel (TMC) proteins 1 and 2 form the pore of hair cell transduction channels (Pan et al., 2013; 2018). The structure of <em>C. elegans</em> TMC proteins (Jeong et al., 2022; Clark et al., 2024) and predicted mammalian TMC structures (Hahn et al., 2009; Ballesteros et al., 2018; Pan et al., 2018) are reminiscent of TMEM16 proteins, which function as Ca²⁺-activated ion channels and lipid scramblases. Here, we investigated lipid scramblase activity in live auditory hair cells with pharmacologic or genetic disruption of TMC1, extending work reported by Ballesteros and Swartz (2022). We used annexin-V to label phosphatidylserine (PS) localized in the outer leaflet of hair cell stereocilia membranes. PS externalization was triggered by disruption of sensory transduction using the blocker, benzamil, or by genetic mutations that affect TMC1 permeation properties. We found that expression of either TMC1 or TMC2, was essential for PS externalization. <em>Tmc1/Tmc2</em> knockout mice and <em>Tmie</em> mutant mice lacked PS externalization completely. We also determined that expression of exogenous human TMCs (hTMC1 or hTMC2) in <em>Tmc1/Tmc2</em> knockout mice induced PS externalization. Lastly, we demonstrated that expression of a dominant mutation in <em>Tmc1</em> evoked constitutive PS externalization, while a recessive mutation eliminated PS externalization. Our data suggest that disruption of sensory transduction may lead to dysregulation of membrane homeostasis in hair cells and thus may contribute to auditory dysfunction in mice and humans.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"460 ","pages":"Article 109229"},"PeriodicalIF":2.5,"publicationDate":"2025-03-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143592820","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}