Pub Date : 2026-01-30DOI: 10.1016/j.heares.2026.109553
Brett R. Schofield
The auditory efferent system has been implicated in nearly all aspects of hearing, including adaptations to aging and response to damage or disease. The system encompasses a collection of descending neural pathways that are widespread, supporting modulation of auditory processing from the cochlea to the cortex. To understand efferent function at a cellular and circuit level, it is necessary to determine which of the inputs to a region contact which of the various output pathways. For both inputs and the outputs, the presence or absence of axonal branching has important implications for how these circuits function. Lack of branching can allow projections to two targets to be modulated independently. The presence of branching can allow for efficient delivery of information and coordinated neuronal processing in multiple targets. The present review considers the merits of several methods that have been used to assess branching. These methods reveal that axonal branching is prominent in some efferent pathways and minimal or absent in others.
{"title":"Circuit architecture and axonal branching in the efferent auditory system","authors":"Brett R. Schofield","doi":"10.1016/j.heares.2026.109553","DOIUrl":"10.1016/j.heares.2026.109553","url":null,"abstract":"<div><div>The auditory efferent system has been implicated in nearly all aspects of hearing, including adaptations to aging and response to damage or disease. The system encompasses a collection of descending neural pathways that are widespread, supporting modulation of auditory processing from the cochlea to the cortex. To understand efferent function at a cellular and circuit level, it is necessary to determine which of the inputs to a region contact which of the various output pathways. For both inputs and the outputs, the presence or absence of axonal branching has important implications for how these circuits function. Lack of branching can allow projections to two targets to be modulated independently. The presence of branching can allow for efficient delivery of information and coordinated neuronal processing in multiple targets. The present review considers the merits of several methods that have been used to assess branching. These methods reveal that axonal branching is prominent in some efferent pathways and minimal or absent in others.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"472 ","pages":"Article 109553"},"PeriodicalIF":2.5,"publicationDate":"2026-01-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146118694","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}
Pub Date : 2026-01-30DOI: 10.1016/j.heares.2026.109551
Oren Poliva , Yuanxin Liu , Robert D. Rafal
Background: The acoustic radiations connect the medial geniculate nucleus (MGN) of the thalamus with the auditory cortex in Heschl’s gyrus (HG). Classic histological dissections described a trajectory that courses through the optic radiations and beneath the posterior insula en route to HG. More recent histological work, however, along with probabilistic tractography, has reported a curved pathway composed of a genu, stem, and fan that is inconsistent with the classically described topography. Thus, the precise course of the acoustic radiations is not settled, motivating renewed anatomical characterization.
Methods: In nineteen healthy adults, we used probabilistic diffusion tractography to visualize streamlines connecting the medial geniculate nucleus (MGN) with the supramarginal gyrus (SMG) bordering the ascending ramus of the lateral fissure. We compared these streamlines with the acoustic and optic radiations as defined in the XTRACT white matter atlas. We then qualitatively re-evaluated published neuroimaging figures from eight patients with severe auditory agnosia or cortical deafness caused by bilateral subcortical white matter lesions and overlaid the reported lesion locations onto the tractography-derived pathways.
Results: In contrast to a ventral acoustic radiation (vAR) that courses through the optic radiations and beneath the insula, our tractography analysis revealed a dorsal bundle that we term the dorsal acoustic radiations (dAR). The dAR comprises two components that initially travel together: an ansa acoustica (AA) projecting from ventral MGN to Heschl’s gyrus (HG), and a projection from magnocellular MGN to the supramarginal gyrus (SMG). Both components arch dorsally over the optic radiations before diverging. In all eight historical cases, bilateral lesions overlapped one or both components of the dAR while visual fields were preserved, suggesting relative sparing of the optic radiations and the vAR.
Conclusions: These findings support a model in which the human acoustic radiations comprise at least two neuroanatomically distinct components: an earlier-developing ventral branch (vAR) arising from ventral MGN that courses beneath the insula, and a later-developing dorsal branch (dAR) that includes the AA traveling from ventral MGN to Heschl’s gyrus (HG) as well as a second pathway traveling from magnocellular MGN to the supramarginal gyrus (SMG) and neighboring auditory-responsive cortices.
{"title":"Auditory radiations: Evidence for dorsal and ventral branches","authors":"Oren Poliva , Yuanxin Liu , Robert D. Rafal","doi":"10.1016/j.heares.2026.109551","DOIUrl":"10.1016/j.heares.2026.109551","url":null,"abstract":"<div><div><strong>Background</strong>: The acoustic radiations connect the medial geniculate nucleus (MGN) of the thalamus with the auditory cortex in Heschl’s gyrus (HG). Classic histological dissections described a trajectory that courses through the optic radiations and beneath the posterior insula <em>en route</em> to HG. More recent histological work, however, along with probabilistic tractography, has reported a curved pathway composed of a genu, stem, and fan that is inconsistent with the classically described topography. Thus, the precise course of the acoustic radiations is not settled, motivating renewed anatomical characterization.</div><div><strong>Methods</strong>: In nineteen healthy adults, we used probabilistic diffusion tractography to visualize streamlines connecting the medial geniculate nucleus (MGN) with the supramarginal gyrus (SMG) bordering the ascending ramus of the lateral fissure. We compared these streamlines with the acoustic and optic radiations as defined in the XTRACT white matter atlas. We then qualitatively re-evaluated published neuroimaging figures from eight patients with severe auditory agnosia or cortical deafness caused by bilateral subcortical white matter lesions and overlaid the reported lesion locations onto the tractography-derived pathways.</div><div><strong>Results</strong>: In contrast to a ventral acoustic radiation (vAR) that courses through the optic radiations and beneath the insula, our tractography analysis revealed a dorsal bundle that we term the dorsal acoustic radiations (dAR). The dAR comprises two components that initially travel together: <em>an ansa acoustica</em> (AA) projecting from ventral MGN to Heschl’s gyrus (HG), and a projection from magnocellular MGN to the supramarginal gyrus (SMG). Both components arch dorsally over the optic radiations before diverging. In all eight historical cases, bilateral lesions overlapped one or both components of the dAR while visual fields were preserved, suggesting relative sparing of the optic radiations and the vAR.</div><div><strong>Conclusions</strong>: These findings support a model in which the human acoustic radiations comprise at least two neuroanatomically distinct components: an earlier-developing ventral branch (vAR) arising from ventral MGN that courses beneath the insula, and a later-developing dorsal branch (dAR) that includes the AA traveling from ventral MGN to Heschl’s gyrus (HG) as well as a second pathway traveling from magnocellular MGN to the supramarginal gyrus (SMG) and neighboring auditory-responsive cortices.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"472 ","pages":"Article 109551"},"PeriodicalIF":2.5,"publicationDate":"2026-01-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146142312","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}
Pub Date : 2026-01-30DOI: 10.1016/j.heares.2026.109554
Qikun Zhang , Xinyang Chen , Chuanxia Cao , Yaqi Zhang , Jing Zhao , Linyao Shi , Shengguang Yan , Zhanguo Jin
This study compared the efficacy of short-duration, high-intensity versus stepwise, progressively lengthened optokinetic drum-chair training in alleviating visually induced motion sickness (VIMS) and modulating autonomic function. Ninety-six VIMS-susceptible university students were randomly assigned to either a short-duration (S-D) group (one training session daily for 3 consecutive days, with each session terminated upon the first report of pronounced nausea) or a stepwise (S-W) group underwent a 6-day graduated protocol (one session daily, duration: 60 s/120 s/180 s). Efficacy was assessed by comparing pre- and post-intervention 90-second chair-rotation challenges. The primary outcome was the Graybiel motion-sickness score (Graybiel score); secondary outcomes included frequency-domain heart rate variability (FD-HRV), electrodermal activity (EDA), and skin temperature (SKT). The S-W group showed a greater reduction in Graybiel scores than the S-D group (median change: -9.0 versus -5.0; between-group Hodges-Lehmann difference: -3.0 points; 95% CI -7.0 to -1.0). Autonomically, the S-W group reduced both low-frequency (LF) and high-frequency (HF) power and increased the LF/HF ratio, while The S-D group reduced only HF power. Skin conductance response amplitude (SCR) increased in the S-W group only. SKT decreased substantially (2-4 °C) in the S-D group but minimally (< 1 °C) in the S-W group (between-group P < 0.01, baseline-adjusted). In conclusion, the stepwise protocol was associated with greater VIMS symptom relief and a more favorable autonomic profile than the short-duration approach, suggesting it may serve as a potential non-pharmacological countermeasure for virtual reality or simulator settings.
{"title":"Vestibular habituation is associated with distinct autonomic signatures and greater reduction in visually induced motion sickness: A randomized controlled trial","authors":"Qikun Zhang , Xinyang Chen , Chuanxia Cao , Yaqi Zhang , Jing Zhao , Linyao Shi , Shengguang Yan , Zhanguo Jin","doi":"10.1016/j.heares.2026.109554","DOIUrl":"10.1016/j.heares.2026.109554","url":null,"abstract":"<div><div>This study compared the efficacy of short-duration, high-intensity versus stepwise, progressively lengthened optokinetic drum-chair training in alleviating visually induced motion sickness (VIMS) and modulating autonomic function. Ninety-six VIMS-susceptible university students were randomly assigned to either a short-duration (S-D) group (one training session daily for 3 consecutive days, with each session terminated upon the first report of pronounced nausea) or a stepwise (S-W) group underwent a 6-day graduated protocol (one session daily, duration: 60 s/120 s/180 s). Efficacy was assessed by comparing pre- and post-intervention 90-second chair-rotation challenges. The primary outcome was the Graybiel motion-sickness score (Graybiel score); secondary outcomes included frequency-domain heart rate variability (FD-HRV), electrodermal activity (EDA), and skin temperature (SKT). The S-W group showed a greater reduction in Graybiel scores than the S-D group (median change: -9.0 versus -5.0; between-group Hodges-Lehmann difference: -3.0 points; 95% <em>CI</em> -7.0 to -1.0). Autonomically, the S-W group reduced both low-frequency (LF) and high-frequency (HF) power and increased the LF/HF ratio, while The S-D group reduced only HF power. Skin conductance response amplitude (SCR) increased in the S-W group only. SKT decreased substantially (2-4 °C) in the S-D group but minimally (< 1 °C) in the S-W group (between-group <em>P</em> < 0.01, baseline-adjusted). In conclusion, the stepwise protocol was associated with greater VIMS symptom relief and a more favorable autonomic profile than the short-duration approach, suggesting it may serve as a potential non-pharmacological countermeasure for virtual reality or simulator settings.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"472 ","pages":"Article 109554"},"PeriodicalIF":2.5,"publicationDate":"2026-01-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146124773","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}
Pub Date : 2026-01-30DOI: 10.1016/j.heares.2026.109552
Fei Xie (谢飞) , Xiaozhou Liu (刘晓宙) , Lulu Wang (王露露) , Cong Zhang (张聪) , Chuanhong Liu (刘传宏) , Zhenqing Huo (霍振庆) , Zhengdong Zhao (赵正东) , Qingyuan Zhao (赵清远) , Qiuyue He (贺秋月) , Kenan Guo (郭科男) , Yu Sun (孙宇) , Yong Wang (王勇)
GJB2, the primary gene responsible for DFNB1, the most prevalent non-syndromic hearing loss (NSHL), has variants that account for over 50% of all prelingual hearing loss (HL). Mice are the main model for congenital hearing loss (CHL) research, but they have delayed auditory maturation postnatally, and unconditional Gjb2 knockout in mice causes embryonic lethality. Pigs have similar inner-ear anatomy to humans and, like humans, have matured auditory function and fully differentiated cochlea at birth. Currently, there is no GJB2 unconditional knockout animal model for GJB2-related CHLs research, and whether unconditional GJB2 deletion causes embryonic lethality in pigs or if GJB2-deficient pigs can recapitulate typical clinical pathological characteristics remains unclear. In this study, we employed CRISPR/Cas9 to establish the first unconditional GJB2 knockout pig model. The mutant GJB2 alleles in the founder pig were stably germline-transmitted to subsequent generations. Homozygous GJB2 knockout pigs exhibited no embryonic lethality and showed profound hearing loss, cochlear hair cell depletion and impaired Organ of Corti’s development. This GJB2 unconditional knockout pig model has not been reported before and demonstrates GJB2 mutation pathological characteristics consistent with clinical patients, validating its potential in investigating the pathogenic mechanisms and therapeutic interventions of GJB2-deficient CHLs.
{"title":"A porcine congenital deafness model with unconditional knockout of GJB2 generated by CRISPR/Cas9 genomic editing","authors":"Fei Xie (谢飞) , Xiaozhou Liu (刘晓宙) , Lulu Wang (王露露) , Cong Zhang (张聪) , Chuanhong Liu (刘传宏) , Zhenqing Huo (霍振庆) , Zhengdong Zhao (赵正东) , Qingyuan Zhao (赵清远) , Qiuyue He (贺秋月) , Kenan Guo (郭科男) , Yu Sun (孙宇) , Yong Wang (王勇)","doi":"10.1016/j.heares.2026.109552","DOIUrl":"10.1016/j.heares.2026.109552","url":null,"abstract":"<div><div><em>GJB2</em>, the primary gene responsible for DFNB1, the most prevalent non-syndromic hearing loss (NSHL), has variants that account for over 50% of all prelingual hearing loss (HL). Mice are the main model for congenital hearing loss (CHL) research, but they have delayed auditory maturation postnatally, and unconditional <em>Gjb2</em> knockout in mice causes embryonic lethality. Pigs have similar inner-ear anatomy to humans and, like humans, have matured auditory function and fully differentiated cochlea at birth. Currently, there is no <em>GJB2</em> unconditional knockout animal model for <em>GJB2</em>-related CHLs research, and whether unconditional <em>GJB2</em> deletion causes embryonic lethality in pigs or if <em>GJB2</em>-deficient pigs can recapitulate typical clinical pathological characteristics remains unclear. In this study, we employed CRISPR/Cas9 to establish the first unconditional <em>GJB2</em> knockout pig model. The mutant <em>GJB2</em> alleles in the founder pig were stably germline-transmitted to subsequent generations. Homozygous <em>GJB2</em> knockout pigs exhibited no embryonic lethality and showed profound hearing loss, cochlear hair cell depletion and impaired Organ of Corti’s development. This <em>GJB2</em> unconditional knockout pig model has not been reported before and demonstrates <em>GJB2</em> mutation pathological characteristics consistent with clinical patients, validating its potential in investigating the pathogenic mechanisms and therapeutic interventions of <em>GJB2</em>-deficient CHLs.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"472 ","pages":"Article 109552"},"PeriodicalIF":2.5,"publicationDate":"2026-01-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146118709","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}
Pub Date : 2026-01-29DOI: 10.1016/j.heares.2026.109548
Sean Lang , John J. Galvin III , Isaac Cooley , Natalia Stupak , David M. Landsberger
Despite the poor sound quality provided by cochlear implants (CIs), single-sided deaf (SSD) CI users prefer to listen to music with the acoustic hearing (AH) and CI ears together rather than with the AH ear alone. The source of this binaural benefit remains unclear. In the present study, sound quality ratings were collected in SSD CI users for music excerpts from different genres (pop, rock, and classical). A novel vocoder-to-the-CI (VCI) approach was used to control the spectral and temporal information delivered to the CI ear. Custom sine-wave vocoders were designed for each participant according to the frequency allocation in their clinical map. Sound quality ratings were collected with CI-only, AH-only, and CI+AH listening. CI+AH ratings were significantly higher than AH-only ratings when unprocessed stimuli or vocoded stimuli with spectro-temporal information were delivered to the CI ear. There were no significant differences among CI+AH ratings for the unprocessed stimuli, vocoded stimuli with spectro-temporal information, and vocoded stimuli with greatly reduced temporal cues, suggesting that the binaural benefit was largely driven by similar spectral information across ears. Effects of genre were minimal. CI+AH ratings for unprocessed music were significantly correlated with CI-only ratings (r = 0.57, p < 0.001), with the slope (0.97) suggesting that the binaural benefit was largely additive between the AH and CI ears. VCI appears to be a fruitful approach to control the spectral and temporal information delivered to the CI ear without directly manipulating CI users’ clinical processors.
尽管人工耳蜗（CIs）提供的音质较差，但单侧耳聋（SSD） CI用户更喜欢用声学听力（AH）和CI耳朵一起听音乐，而不是单独使用AH耳朵。这种双耳益处的来源尚不清楚。在本研究中，我们收集了SSD CI用户对不同类型音乐（流行、摇滚和古典）的音质评分。提出了一种新的声码器-声码器（VCI）方法来控制传递到声码器耳的频谱和时间信息。根据临床图谱中的频率分配，为每位参与者设计定制的正弦波声码器。通过仅CI、仅AH和CI+AH收听收集音质评分。当将未经处理的刺激或带有光谱时间信息的声音编码刺激传递到CI耳时，CI+AH评分明显高于AH评分。未处理的刺激、带有光谱-时间信息的声编码刺激和时间线索大大减少的声编码刺激的CI+AH评分无显著差异，表明双耳获益主要是由双耳间相似的频谱信息驱动的。体裁的影响很小。未处理音乐的CI+AH评分与仅CI评分显著相关（r = 0.57, p < 0.001），斜率（0.97）表明双耳收益在很大程度上是AH和CI耳朵之间的相加性。VCI似乎是一种有效的方法来控制传递到CI耳的频谱和时间信息，而无需直接操纵CI用户的临床处理器。
{"title":"Using vocoders to the implanted ear to investigate the binaural benefit for music sound quality in single-sided deaf cochlear implant users","authors":"Sean Lang , John J. Galvin III , Isaac Cooley , Natalia Stupak , David M. Landsberger","doi":"10.1016/j.heares.2026.109548","DOIUrl":"10.1016/j.heares.2026.109548","url":null,"abstract":"<div><div>Despite the poor sound quality provided by cochlear implants (CIs), single-sided deaf (SSD) CI users prefer to listen to music with the acoustic hearing (AH) and CI ears together rather than with the AH ear alone. The source of this binaural benefit remains unclear. In the present study, sound quality ratings were collected in SSD CI users for music excerpts from different genres (pop, rock, and classical). A novel vocoder-to-the-CI (VCI) approach was used to control the spectral and temporal information delivered to the CI ear. Custom sine-wave vocoders were designed for each participant according to the frequency allocation in their clinical map. Sound quality ratings were collected with CI-only, AH-only, and CI+AH listening. CI+AH ratings were significantly higher than AH-only ratings when unprocessed stimuli or vocoded stimuli with spectro-temporal information were delivered to the CI ear. There were no significant differences among CI+AH ratings for the unprocessed stimuli, vocoded stimuli with spectro-temporal information, and vocoded stimuli with greatly reduced temporal cues, suggesting that the binaural benefit was largely driven by similar spectral information across ears. Effects of genre were minimal. CI+AH ratings for unprocessed music were significantly correlated with CI-only ratings (<em>r</em> = 0.57, <em>p</em> < 0.001), with the slope (0.97) suggesting that the binaural benefit was largely additive between the AH and CI ears. VCI appears to be a fruitful approach to control the spectral and temporal information delivered to the CI ear without directly manipulating CI users’ clinical processors.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"472 ","pages":"Article 109548"},"PeriodicalIF":2.5,"publicationDate":"2026-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146124821","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}
Pub Date : 2026-01-29DOI: 10.1016/j.heares.2026.109550
Payton E. Charlton , Kali Burke , Sevda Abdavinejad , Mariam Ashour , Zachary Zaharkin , Riley McLaughlin , Srijita Paul , Amanda M. Lauer , Micheal L. Dent
Alzheimer’s disease (AD) is a brain condition with heterogeneity in disease progression due to genetic and environmental variables. There is a critical need to better understand the relationship of AD pathologies and potential modifiable factors like hearing loss. Auditory processing measurements in AD mouse models have been reported, but the results are mixed. Most were conducted using evoked potential recordings measured under anesthesia, unlike typical hearing assessments on aging adult humans. Here, we used operant conditioning and signal detection theory to measure daily pure tone behavioral detection throughout the lifespan of trained mutant and wild-type (WT) control APP/PS1 (on a C57BL/6J background) and 5xFAD (on a C57/SJL background) mice. At the conclusion of operant testing, auditory brainstem response (ABR) measures were taken on the same subjects to determine if evoked potentials provided accurate estimates of perceptual abilities. Behavioral detection worsened significantly across the lifespan in APP/PS1 mice, but there were no differences between mutant and WT mice. For 5xFAD mice, behavioral thresholds generally worsened over the lifespan but with substantial variability, which may be explained by genetic heterogeneity among the background strain. There were no differences between 5xFAD mutant and WT mice. ABR hearing threshold estimates generally matched behavioral findings, with APP/PS1 having significantly worse thresholds than 5xFAD mice but no within-model differences between mutants and WTs. The within-subject differences between behavioral and ABR thresholds ranged from <1 dB to over 40 dB across subjects, suggesting physiological measurements of auditory function are not necessarily reflective of an animal’s acoustic perception.
{"title":"Differences in hearing across the lifespan in two strains of Alzheimer’s mouse models as measured using behavioral and physiological techniques","authors":"Payton E. Charlton , Kali Burke , Sevda Abdavinejad , Mariam Ashour , Zachary Zaharkin , Riley McLaughlin , Srijita Paul , Amanda M. Lauer , Micheal L. Dent","doi":"10.1016/j.heares.2026.109550","DOIUrl":"10.1016/j.heares.2026.109550","url":null,"abstract":"<div><div>Alzheimer’s disease (AD) is a brain condition with heterogeneity in disease progression due to genetic and environmental variables. There is a critical need to better understand the relationship of AD pathologies and potential modifiable factors like hearing loss. Auditory processing measurements in AD mouse models have been reported, but the results are mixed. Most were conducted using evoked potential recordings measured under anesthesia, unlike typical hearing assessments on aging adult humans. Here, we used operant conditioning and signal detection theory to measure daily pure tone behavioral detection throughout the lifespan of trained mutant and wild-type (WT) control APP/PS1 (on a C57BL/6J background) and 5xFAD (on a C57/SJL background) mice. At the conclusion of operant testing, auditory brainstem response (ABR) measures were taken on the same subjects to determine if evoked potentials provided accurate estimates of perceptual abilities. Behavioral detection worsened significantly across the lifespan in APP/PS1 mice, but there were no differences between mutant and WT mice. For 5xFAD mice, behavioral thresholds generally worsened over the lifespan but with substantial variability, which may be explained by genetic heterogeneity among the background strain. There were no differences between 5xFAD mutant and WT mice. ABR hearing threshold estimates generally matched behavioral findings, with APP/PS1 having significantly worse thresholds than 5xFAD mice but no within-model differences between mutants and WTs. The within-subject differences between behavioral and ABR thresholds ranged from <1 dB to over 40 dB across subjects, suggesting physiological measurements of auditory function are not necessarily reflective of an animal’s acoustic perception.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"472 ","pages":"Article 109550"},"PeriodicalIF":2.5,"publicationDate":"2026-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146131669","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}
Pub Date : 2026-01-28DOI: 10.1016/j.heares.2026.109549
Breann Krygsman, Alisa Pitre, Sebastien Paquette
Hyperacusis and sound sensitivity are complex phenomena influenced by both auditory and psychological factors. This study investigated the relationship between hearing thresholds, perceptual ratings of natural sounds, attitudes toward noise, and sex-based differences in a sample of healthy young adults. Fifty participants underwent pure-tone audiometry, completed a psychoacoustic test assessing hyperacusis (sound sensitivity), and filled out self-report questionnaires including the Youth Attitudes to Noise Scale (YANS) and the Beliefs About Hearing Protection and Hearing Loss (BAHPHL). Contrary to expectations, greater sound sensitivity was associated with lower hearing thresholds (indicating minimal hearing loss) in the low-frequency range. This suggests that preserved low-frequency hearing may contribute to auditory discomfort. Questionnaire data revealed that individuals with higher sound sensitivity also held more negative beliefs about noise and hearing protection, highlighting the role of cognitive and affective factors. No significant sex differences were found, though prior research suggests biological distinctions may still influence auditory processing. These findings underscore the importance of considering both perceptual and psychological dimensions in understanding hyperacusis.
{"title":"Psychoacoustic and cognitive predictors of sound sensitivity in healthy young adults","authors":"Breann Krygsman, Alisa Pitre, Sebastien Paquette","doi":"10.1016/j.heares.2026.109549","DOIUrl":"10.1016/j.heares.2026.109549","url":null,"abstract":"<div><div>Hyperacusis and sound sensitivity are complex phenomena influenced by both auditory and psychological factors. This study investigated the relationship between hearing thresholds, perceptual ratings of natural sounds, attitudes toward noise, and sex-based differences in a sample of healthy young adults. Fifty participants underwent pure-tone audiometry, completed a psychoacoustic test assessing hyperacusis (sound sensitivity), and filled out self-report questionnaires including the Youth Attitudes to Noise Scale (YANS) and the Beliefs About Hearing Protection and Hearing Loss (BAHPHL). Contrary to expectations, greater sound sensitivity was associated with lower hearing thresholds (indicating minimal hearing loss) in the low-frequency range. This suggests that preserved low-frequency hearing may contribute to auditory discomfort. Questionnaire data revealed that individuals with higher sound sensitivity also held more negative beliefs about noise and hearing protection, highlighting the role of cognitive and affective factors. No significant sex differences were found, though prior research suggests biological distinctions may still influence auditory processing. These findings underscore the importance of considering both perceptual and psychological dimensions in understanding hyperacusis.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"472 ","pages":"Article 109549"},"PeriodicalIF":2.5,"publicationDate":"2026-01-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146136979","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}
Age-related hearing loss (ARHL), the most prevalent sensory disorder worldwide, arises primarily from cochlear hair cells (HCs) degeneration due to aging. Although the molecular mechanisms driving HC senescence are increasingly understood, effective treatments for ARHL remain lacking. This study explores the therapeutic potential role of Pre-B cell leukemia homeobox 1 (Pbx1), a transcription factor involved in inner ear development and pluripotency, in mitigating ARHL. Our results reveal a striking age-dependent reduction in PBX1 expression within mouse cochlear HCs. Using D-galactose (D-gal)/lipopolysaccharide (LPS)-induced aging models in OC-1 cells and cultured cochlear explants, we demonstrated that lentiviral and adeno-associated virus (AAV)-mediated Pbx1 overexpression significantly suppresses senescent markers and preserves HC integrity. Remarkably, in vivo delivery of Pbx1 by AAV improved auditory function and preserved HC structure and function in ARHL mouse model. These results establish Pbx1 as a key mediator of HC aging and a promising therapeutic target for ARHL. Our findings demonstrate that AAV-mediated Pbx1 overexpression represents a potential therapeutic approach to prevent ARHL progression, paving the way for future clinical management of this prevalent sensory disorder.
{"title":"Pbx1 overexpression delays cochlear hair cells degeneration in an accelerated aging mouse model","authors":"Ruihan Zhu , Gaogan Jia , Yiming Shen , Xian Gao , Yunjie Li , Hua Jiang , Hui Chai , Mingyu Xia","doi":"10.1016/j.heares.2026.109546","DOIUrl":"10.1016/j.heares.2026.109546","url":null,"abstract":"<div><div>Age-related hearing loss (ARHL), the most prevalent sensory disorder worldwide, arises primarily from cochlear hair cells (HCs) degeneration due to aging. Although the molecular mechanisms driving HC senescence are increasingly understood<em>,</em> effective treatments for ARHL remain lacking<em>.</em> This study explores the therapeutic potential role of Pre-B cell leukemia homeobox 1 (<em>Pbx1</em>), a transcription factor involved in inner ear development and pluripotency, in mitigating ARHL. Our results reveal a striking age-dependent reduction in PBX1 expression within mouse cochlear HCs. Using D-galactose (D-gal)/lipopolysaccharide (LPS)-induced aging models in OC-1 cells and cultured cochlear explants, we demonstrated that lentiviral and adeno-associated virus (AAV)-mediated <em>Pbx1</em> overexpression significantly suppresses senescent markers and preserves HC integrity. Remarkably, in vivo delivery of <em>Pbx1</em> by AAV improved auditory function and preserved HC structure and function in ARHL mouse model. These results establish <em>Pbx1</em> as a key mediator of HC aging and a promising therapeutic target for ARHL. Our findings demonstrate that AAV-mediated <em>Pbx1</em> overexpression represents a potential therapeutic approach to prevent ARHL progression, paving the way for future clinical management of this prevalent sensory disorder.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"472 ","pages":"Article 109546"},"PeriodicalIF":2.5,"publicationDate":"2026-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146037430","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}
Pub Date : 2026-01-20DOI: 10.1016/j.heares.2026.109547
N. Shahnaz, V. Ciocca
The goal of this study was to characterize the population-level distributions of hearing thresholds at conventional and extended high frequencies (EHFs). To achieve this goal, distributions of typical hearing thresholds expressed in dB SPL were measured in an otologically normal young-adult sample. Thresholds were obtained using an adaptive procedure with the Sennheiser HDA-200 earphone. Thresholds were measured at 16 frequencies, ranging from 250 Hz to 16 kHz, in a non-random sample of 126 young adults (18-36 years old) with typical hearing (≤ 20 dB HL at conventional frequencies). Using the posterior of a Bayesian multilevel distributional regression model that was fit to the data, we simulated hearing thresholds at conventional and extended high frequencies for 18,000 new participants. The simulated thresholds were used to calculate probabilistic estimates of the reference equivalent threshold sound pressure levels (RETSPLs) and other percentiles of the distributions of hearing thresholds at each frequency. We used these percentile estimates to define the range of typical hearing sensitivity in dB SPL and found that this range varies across frequency, unlike the current fixed 20–25 dB HL cutoffs that are used to classify “normal” hearing. Specifically, at conventional frequencies, the fixed 20–25 dB HL cutoffs overestimated the range of typical thresholds, whereas at extended high frequencies, the fixed cutoffs overestimated this range. The proposed probabilistic approach provides a framework for characterizing the information from pure tone thresholds in terms of the range of hearing sensitivity, rather than categorizing hearing ability on the basis of a frequency-invariant criterion.
本研究的目的是表征常规高频和扩展高频（EHFs）听力阈值的人群水平分布。为了实现这一目标，我们在一个耳部正常的年轻人样本中测量了以分贝声压级表达的典型听力阈值的分布。使用Sennheiser HDA-200耳机采用自适应程序获得阈值。在126名听力正常（常规频率≤20 dB HL）的年轻人（18-36岁）的非随机样本中，测量了从250 Hz到16 kHz的16个频率的阈值。使用拟合数据的贝叶斯多水平分布回归模型的后验，我们模拟了18,000名新参与者在常规高频和扩展高频下的听力阈值。模拟阈值用于计算每个频率下参考等效阈值声压级（RETSPLs）和听力阈值分布的其他百分位数的概率估计。我们使用这些百分位数估计值来定义以dB SPL为单位的典型听力灵敏度范围，并发现该范围随频率而变化，而不像目前用于分类“正常”听力的固定20-25 dB HL截止值。具体来说，在常规频率下，固定的20-25 dB HL截止值高估了典型阈值的范围，而在扩展的高频下，固定截止值高估了该范围。所提出的概率方法提供了一个框架，根据听觉灵敏度的范围来描述来自纯音阈值的信息，而不是根据频率不变标准对听觉能力进行分类。
{"title":"Bayesian simulation of hearing thresholds at conventional and extended high frequencies in young adults","authors":"N. Shahnaz, V. Ciocca","doi":"10.1016/j.heares.2026.109547","DOIUrl":"10.1016/j.heares.2026.109547","url":null,"abstract":"<div><div>The goal of this study was to characterize the population-level distributions of hearing thresholds at conventional and extended high frequencies (EHFs). To achieve this goal, distributions of typical hearing thresholds expressed in dB SPL were measured in an otologically normal young-adult sample. Thresholds were obtained using an adaptive procedure with the Sennheiser HDA-200 earphone. Thresholds were measured at 16 frequencies, ranging from 250 Hz to 16 kHz, in a non-random sample of 126 young adults (18-36 years old) with typical hearing (≤ 20 dB HL at conventional frequencies). Using the posterior of a Bayesian multilevel distributional regression model that was fit to the data, we simulated hearing thresholds at conventional and extended high frequencies for 18,000 new participants. The simulated thresholds were used to calculate probabilistic estimates of the reference equivalent threshold sound pressure levels (RETSPLs) and other percentiles of the distributions of hearing thresholds at each frequency. We used these percentile estimates to define the range of typical hearing sensitivity in dB SPL and found that this range varies across frequency, unlike the current fixed 20–25 dB HL cutoffs that are used to classify “normal” hearing. Specifically, at conventional frequencies, the fixed 20–25 dB HL cutoffs overestimated the range of typical thresholds, whereas at extended high frequencies, the fixed cutoffs overestimated this range. The proposed probabilistic approach provides a framework for characterizing the information from pure tone thresholds in terms of the range of hearing sensitivity, rather than categorizing hearing ability on the basis of a frequency-invariant criterion.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"472 ","pages":"Article 109547"},"PeriodicalIF":2.5,"publicationDate":"2026-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146046554","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}
Pub Date : 2026-01-18DOI: 10.1016/j.heares.2026.109545
Masafumi Ueno , Makoto Hosoya , Marie N Shimanuki , Takanori Nishiyama , Naoki Oishi , Hiroyuki Ozawa
Type I spiral ganglion neurons, which play a major role in hearing by conveying electrical sound signals to the brain, are divided into electrophysiological and molecular subtypes. The differences in vulnerability among the subtypes reported in rodents are of pathophysiological importance for age-related hearing loss. However, the changes in spiral ganglion neurons in primates and humans have not been established. In this study, we investigated the age-related changes in the spiral ganglion neurons of the common marmoset, a primate model animal. Here, we show that the overall number of spiral ganglion neurons decreased in middle-aged individuals and was most pronounced during the basal turn. However, no subtype-specific reduction, as reported in rodents, was identified. Our observations indicate that aging-related changes observed in the cochlea of rodents are not always applicable to primates. The usefulness and importance of common marmosets in the study of age-related hearing loss are also highlighted.
{"title":"A primate model revealed specific age-related changes in spiral ganglion neurons in the cochlea","authors":"Masafumi Ueno , Makoto Hosoya , Marie N Shimanuki , Takanori Nishiyama , Naoki Oishi , Hiroyuki Ozawa","doi":"10.1016/j.heares.2026.109545","DOIUrl":"10.1016/j.heares.2026.109545","url":null,"abstract":"<div><div>Type I spiral ganglion neurons, which play a major role in hearing by conveying electrical sound signals to the brain, are divided into electrophysiological and molecular subtypes. The differences in vulnerability among the subtypes reported in rodents are of pathophysiological importance for age-related hearing loss. However, the changes in spiral ganglion neurons in primates and humans have not been established. In this study, we investigated the age-related changes in the spiral ganglion neurons of the common marmoset, a primate model animal. Here, we show that the overall number of spiral ganglion neurons decreased in middle-aged individuals and was most pronounced during the basal turn. However, no subtype-specific reduction, as reported in rodents, was identified. Our observations indicate that aging-related changes observed in the cochlea of rodents are not always applicable to primates. The usefulness and importance of common marmosets in the study of age-related hearing loss are also highlighted.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"471 ","pages":"Article 109545"},"PeriodicalIF":2.5,"publicationDate":"2026-01-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146018311","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}
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