Hearing ResearchPub Date : 2025-03-02DOI: 10.1016/j.heares.2025.109228
Tom T. Austin , Christian L. Thomas , Ben Warren
{"title":"Sex differences in auditory function of the desert locust","authors":"Tom T. Austin , Christian L. Thomas , Ben Warren","doi":"10.1016/j.heares.2025.109228","DOIUrl":"10.1016/j.heares.2025.109228","url":null,"abstract":"<div><div>Age-related auditory decline manifests across the animal kingdom, from humans and mice to zebrafish and insects. Sex differences in auditory decline are established for humans, but there is now evidence in mice and even zebrafish. Here, we found sex differences in auditory decline in an insect, the Desert Locust and investigated its biological basis. We profiled gene expression in a dedicated auditory organ, Müller's organ to understand the genetic underpinning of sex differences and measured sound-evoked transduction currents and electrophysiological properties of auditory neurons to quantify auditory decline. We analysed gene expression in Müller's organ of young locusts where sex differences in auditory function were absent and in older, noise-exposed locusts where sex differences in auditory function were maximal. The auditory organs of both male and females changed expression of 1200 and 931 genes, respectively, as they aged and were exposed to repeated bouts of noise exposure. Only 39 genes were differentially expressed between the sexes for young locusts and 9 for aged and noise exposed auditory organs. In young locusts we found sex-differences in genes for juvenile hormone and proteins involved in egg production and catalysis of steroid hormones. The majority of sex differences in Müller's organ manifested as a function of stress with females upregulating more and downregulating less genes compared to males. We hypothesise that sex differences in auditory decline are due to differences in immune responses and metabolic processes.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"460 ","pages":"Article 109228"},"PeriodicalIF":2.5,"publicationDate":"2025-03-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143577761","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}
Hearing ResearchPub Date : 2025-03-01DOI: 10.1016/j.heares.2025.109224
Hong Zhang, Xufeng Qiu, Jonah Mittelstadt, Ulrich Müller
{"title":"Ankyrins are dispensable for mechanotransduction by cochlear hair cells","authors":"Hong Zhang, Xufeng Qiu, Jonah Mittelstadt, Ulrich Müller","doi":"10.1016/j.heares.2025.109224","DOIUrl":"10.1016/j.heares.2025.109224","url":null,"abstract":"<div><div>The mechanotransduction (MET) channels of cochlear hair cells is a heteromeric protein complex consisting of TMC1, TMIE and CIB2. The activity of this ion channel is thought to be regulated by a gating spring, a mechanical element that conveys sound-induced vibrations to the MET channel. In nematodes, orthologs of TMC-1, TMIE and CIB2 similarly assemble into a MET channel mediating light nose-touch. Studies in nematodes have suggested that nematode <em>Unc-44</em>, an ortholog of the mammalian ankyrins <em>Ank1, 2</em>, and <em>3</em>, encodes a gating spring that tethers the nematode MET channel to the cytoskeleton. Here we show that mammalian ankyrins are expressed in cochlear hair cells. Using single and triple conditional knockout mice, we demonstrate that <em>Ank1, 2</em>, and <em>3</em> are dispensable for the function of cochlear hair cells. We concluded that <em>Ank1, 2</em>, and <em>3</em> are unlikely to be components of the gating spring that gates mechanotransduction channels in mammalian cochlear hair cells.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"459 ","pages":"Article 109224"},"PeriodicalIF":2.5,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143519415","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}
Hearing ResearchPub Date : 2025-02-28DOI: 10.1016/j.heares.2025.109226
Eleanor D. Brown, Shada Nassar, Daniel J. Jagger
{"title":"NF2-related schwannomatosis: A view from within the inner ear","authors":"Eleanor D. Brown, Shada Nassar, Daniel J. Jagger","doi":"10.1016/j.heares.2025.109226","DOIUrl":"10.1016/j.heares.2025.109226","url":null,"abstract":"<div><div>NF2-related schwannomatosis (NF2-SWN, formerly known as neurofibromatosis type 2) is an autosomal dominant disorder associated with the growth of bilateral schwannomas on the cochleo-vestibular nerves and meningiomas. NF2-SWN is caused by pathogenic variations in the <em>NF2, moesin-ezrin-radixin-like (MERLIN) tumour suppressor</em> gene. The mostly benign tumours can cause progressive sensorineural hearing loss, tinnitus and balance dysfunction. Outside the inner ear, tumours grow on other intra-cranial nerves, leading to further neurological issues and shortened life-expectancy. Here we re-evaluate some historic cases from our human temporal bone collection, and we review similar instances from the literature to highlight the structural and functional effects of such tumours on the cochlea and vestibular organs. Tumour growth is associated with the remodelling of sensory and ion-transporting epithelia, the loss of afferent neurons and hair cells, and signs of fluid dysregulation. These cases demonstrate the aggressive nature of this disease and the difficulties of surgically excising the bilateral tumours. They also emphasise the need for novel therapies that can slow or prevent tumour growth to preserve sensory function in people living with NF2-SWN.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"460 ","pages":"Article 109226"},"PeriodicalIF":2.5,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143548523","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}
Hearing ResearchPub Date : 2025-02-25DOI: 10.1016/j.heares.2025.109227
Anusha Yasoda-Mohan , Feifan Chen , Sven Vanneste
{"title":"Unveiling the mind's ear: Understanding the science behind auditory processing using illusions","authors":"Anusha Yasoda-Mohan , Feifan Chen , Sven Vanneste","doi":"10.1016/j.heares.2025.109227","DOIUrl":"10.1016/j.heares.2025.109227","url":null,"abstract":"<div><div>Our perceptual experience is not purely driven by the information our senses receive but is an active combination of prior experience and the sensory information that we receive. This is promisingly demonstrated by an illusory experience. Particularly in the auditory domain, we encounter illusions that serve as a tool to understand the more holistic and bigger umbrella of auditory processing. In the current manuscript, we review spectral, temporal, spatial auditory and multisensory processing through the lens of auditory illusions. We review examples of auditory illusions that could serve as models to better comprehend these sub-domains of auditory processing. We also explore the literature where these illusions have been used as a causal human model to unravel mechanisms behind neuropathology and conclude with futuristic applications of auditory illusions.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"459 ","pages":"Article 109227"},"PeriodicalIF":2.5,"publicationDate":"2025-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143509798","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}
Hearing ResearchPub Date : 2025-02-21DOI: 10.1016/j.heares.2025.109225
Abbie Baricevich, Danielle Bassett, Sophia Chan, Shayna Lavi, Jonathan Siegel
{"title":"Frequency and level dependence of the middle ear acoustic reflex and its decay measured in wideband absorbance with contralateral narrowband noise elicitors","authors":"Abbie Baricevich, Danielle Bassett, Sophia Chan, Shayna Lavi, Jonathan Siegel","doi":"10.1016/j.heares.2025.109225","DOIUrl":"10.1016/j.heares.2025.109225","url":null,"abstract":"<div><div>The acoustic reflex has potential as a diagnostic tool for identifying individuals with selective damage to cochlear sensory neurons (Bramhall et al., 2022; Feeney et al., 2023; Wojtczak et al., 2017). If damage to neurons is localized, then using broadband stimuli to elicit the reflex, as reported in the studies cited above, may not be most effective in revealing spatially restricted lesions. We have measured changes in absorbance with chirps delivered to one ear and contralateral narrowband noise to elicit the reflex. We aim to characterize the frequency and level dependence of changes in absorbance elicited by noise with different center frequencies as well as the decay of the reflex for prolonged elicitors as a baseline for comparison with similar measurements in individuals suspected of having neural damage. Our hypothesis is that elicitors that preferentially activate the damaged part of the reflex pathway will show the largest deviation from normal. We have identified a novel sensitization of the acoustic reflex following repeated exposure to moderate level elicitors such that absorbance changes more than double, regardless of the level of the elicitor, compared with the minimally stimulated contralateral ear. These changes recover slowly. We are measuring the growth of the reflex vs elicitor level and at constant elicitor level vs center frequency. We are also measuring the rate of reflex decay vs elicitor center frequency. Preliminary findings substantiate the measurement protocols in revealing differences between individuals and ears.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"459 ","pages":"Article 109225"},"PeriodicalIF":2.5,"publicationDate":"2025-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143527061","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}
Hearing ResearchPub Date : 2025-02-21DOI: 10.1016/j.heares.2025.109223
Chhayakanta Patro , Nirmal Kumar Srinivasan , Sadie O'Neill , Morgan Barkhouse , Srikanta Kumar Mishra
{"title":"Investigating the role of extended high-frequency audibility on temporal envelope processing and spatial release from masking","authors":"Chhayakanta Patro , Nirmal Kumar Srinivasan , Sadie O'Neill , Morgan Barkhouse , Srikanta Kumar Mishra","doi":"10.1016/j.heares.2025.109223","DOIUrl":"10.1016/j.heares.2025.109223","url":null,"abstract":"<div><div>Extended high-frequency (EHF) hearing loss offers a model for investigating the onset of auditory dysfunction before changes in standard audiometric thresholds occur. However, the impact on auditory perception remains poorly understood. This study evaluated the relationship between EHF hearing sensitivity and monaural and binaural measures of auditory temporal resolution and spatial release from masking in young adults (aged 20–35 years) with normal hearing thresholds in the clinical frequency range (.25 - 8 kHz) with varying degrees of hearing sensitivity in the EHFs (10 – 16 kHz). Despite considerable individual variability in performance on perceptual tasks, no significant correlations were found between EHF thresholds and measures of temporal processing or speech perception. This suggests that certain aspects of auditory processing within the standard audiometric frequency range may remain unaffected in individuals with EHF hearing loss.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"460 ","pages":"Article 109223"},"PeriodicalIF":2.5,"publicationDate":"2025-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143577536","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}
Hearing ResearchPub Date : 2025-02-17DOI: 10.1016/j.heares.2025.109218
Gavin M. Bidelman
{"title":"Reply to Manley: Is there more to cochlear tuning than meets the ear?","authors":"Gavin M. Bidelman","doi":"10.1016/j.heares.2025.109218","DOIUrl":"10.1016/j.heares.2025.109218","url":null,"abstract":"<div><div>Enhanced psychophysical and cochlear tuning observed in musicians is unlikely to be explained by mere differences in human cochlear length. A parsimonious account of our 2016 data is improved efferent feedback from the medial olivocochlear efferent system that adjusts masking and tuning properties of the cochlea and is subject to attentional modulation—all functions reported to be enhanced in musically trained ears. Still, new experiments are needed to tease out “nature” vs. “nurture” effects in music-related brain plasticity and move beyond cross-sectional studies and definitions of “musicians” based solely on self-report.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"459 ","pages":"Article 109218"},"PeriodicalIF":2.5,"publicationDate":"2025-02-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143419521","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}
Hearing ResearchPub Date : 2025-02-15DOI: 10.1016/j.heares.2025.109222
XianHua Ma , Fei Jiang , Chunchun Wei , Shuang Han , Yuqing Zhang , Lianhua Sun , Jiaxi Qu , Hao Ying , Yuxia Chen , Jie Tang , David Z. He , Weiping J. Zhang , Zhifang Xie
{"title":"Thyroid hormone signaling is essential for the maturation and survival of cochlear root cells in mice","authors":"XianHua Ma , Fei Jiang , Chunchun Wei , Shuang Han , Yuqing Zhang , Lianhua Sun , Jiaxi Qu , Hao Ying , Yuxia Chen , Jie Tang , David Z. He , Weiping J. Zhang , Zhifang Xie","doi":"10.1016/j.heares.2025.109222","DOIUrl":"10.1016/j.heares.2025.109222","url":null,"abstract":"<div><div>Thyroid hormone and its receptors (TRs) are crucial for late-stage cochlear development and the maintenance of endocochlear potential (EP), yet the mechanisms underlying EP reduction in their absence remain unclear. Cochlear outer sulcus root cells undergo significant morphological changes during late-stage development and are thought to play a role in maintaining endolymph homeostasis and EP. Nevertheless, it remains unknown whether thyroid hormone and TRs are essential for root cell differentiation and function. Here, we demonstrate that thyroid hormone or TRs are indispensable for postnatal root cell development and survival in the mouse cochlea. Thyroid hormone deficiency markedly delays root cell differentiation. Otocyst-selective deletion of both <em>Thra</em> and <em>Thrb</em>, but not <em>Thrb</em> alone, leads to a similar impairment, accompanied by early degeneration of root cells, with the stria vascularis unaffected. Furthermore, conditional double knockout of TRs results in a 22 % reduction in mean EP magnitude at 4 months, less severe than the effects observed in global TRs knockout models. Transcriptome analysis reveals that thyroid hormone deficiency downregulates a significant portion of root cell-enriched genes. These findings underscore the redundant roles of TRα and TRβ in promoting the late-stage differentiation and survival of root cells. Additionally, they suggest that the expression of TRs in cochlear epithelium is crucial for maintaining an optimal EP magnitude, while TRs expressed in areas outside cochlear epithelium, particularly in spiral ligament fibrocytes, may also significantly contribute to EP maintenance. This study advances our understanding of thyroid hormone in cochlear outer sulcus development and EP maintenance.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"459 ","pages":"Article 109222"},"PeriodicalIF":2.5,"publicationDate":"2025-02-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143527112","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}
Hearing ResearchPub Date : 2025-02-14DOI: 10.1016/j.heares.2025.109221
Mi-Jung Kim , Shion Simms , Ghazaleh Behnammanesh , Wei-Wen Chen , Yohei Honkura , Jun Suzuki , Hyo-Jin Park , Marcus Milani , Yukio Katori , Jonathan E Bird , Akihiro Ikeda , Shinichi Someya
{"title":"A mutation in Tmem135 causes progressive sensorineural hearing loss","authors":"Mi-Jung Kim , Shion Simms , Ghazaleh Behnammanesh , Wei-Wen Chen , Yohei Honkura , Jun Suzuki , Hyo-Jin Park , Marcus Milani , Yukio Katori , Jonathan E Bird , Akihiro Ikeda , Shinichi Someya","doi":"10.1016/j.heares.2025.109221","DOIUrl":"10.1016/j.heares.2025.109221","url":null,"abstract":"<div><div>Transmembrane protein 135 (TMEM135) is a highly conserved 52 kDa protein with five predicted transmembrane domains that colocalizes with mitochondria and peroxisomes. Previous studies have shown that TMEM135 is involved in mitochondrial dynamics, thermogenesis, and lipid metabolism across multiple tissues and species; however, its role in the inner ear and auditory system remains unknown. We investigated the function of TMEM135 in hearing using wild-type (WT) and <em>Tmem135</em><sup>FUN025/FUN025</sup> (<em>FUN025</em>) mutant mice on a CBA/CaJ background, a normal-hearing mouse strain. Although <em>FUN025</em> mice displayed normal auditory brainstem response (ABR) thresholds at 1 month, we observed significantly elevated ABR thresholds at 8, 16, and 64 kHz by 3 months, which progressed to profound hearing loss by 12 months. Consistent with our auditory testing results, 13-month-old <em>FUN025</em> mice exhibited a severe loss of outer hair cells and more modest changes in inner hair cell survival, spiral ganglion neuron density, and stria vascularis integrity in the cochlea. Our results using BaseScope RNA in situ hybridization indicate that TMEM135 is expressed in the inner hair cells, outer hair cells, supporting cells, and stria vascularis. Using Volocity software and Costes colocalization analysis, we found that TMEM135 closely colocalizes with mitochondria in hair cells. Together, these results demonstrate that the <em>FUN025</em> mutation in <em>Tmem135</em> causes progressive sensorineural hearing loss, and suggest that TMEM135 is crucial for maintaining key cochlear cell types and normal sensory function in the aging cochlea.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"459 ","pages":"Article 109221"},"PeriodicalIF":2.5,"publicationDate":"2025-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143428005","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}
Hearing ResearchPub Date : 2025-02-14DOI: 10.1016/j.heares.2025.109219
Geoffrey A. Manley
{"title":"Music to my ears","authors":"Geoffrey A. Manley","doi":"10.1016/j.heares.2025.109219","DOIUrl":"10.1016/j.heares.2025.109219","url":null,"abstract":"<div><div>Both psychoacoustic and cochlear measurements of frequency selectivity in humans indicate that persons with substantial active musical experience can show higher selectivity values. The influence of experience on neural networks in the brain is compatible with what is known about learning and development and thus changes in frequency selectivity of elements in the auditory pathway. Assumed changes in cochlear selectivity as the result of experience, however, lack both any known anatomical substrate and precedent. Here, the data interpretations of Bidelmann et al. (2016) are questioned as not being parsimonious, and the suggestion is raised that in fact there are no changes in the cochlea of musicians. Instead, within the assumed large range of differences in human cochlear length, those persons with innately better cochlear selectivity are more likely to take up music as a career or pastime.</div></div>","PeriodicalId":12881,"journal":{"name":"Hearing Research","volume":"459 ","pages":"Article 109219"},"PeriodicalIF":2.5,"publicationDate":"2025-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143436702","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}