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Exploring the Molecular, Cellular, and Physiological Basis of Hearing Disorders
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Exploring the Molecular, Cellular, and Physiological Basis of Hearing Disorders

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Pathology, Diagnostics, and Therapeutics".

Deadline for manuscript submissions: 30 August 2024 | Viewed by 2986

Special Issue Editor

Dr. Jing Zheng

E-Mail Website
Guest Editor
Department of Otolaryngology-Head and Neck Surgery, Feinberg School of Medicine Northwestern University, Chicago, IL, USA
Interests: hearing impairment; mechanisms underlying deafness; biomarker for cochlear damage

Special Issue Information

Dear Colleagues,

Hearing loss is the most common sensory defect and affects millions of people worldwide, ranging from newborns to older adults. Despite extensive efforts, we still do not fully understand the mechanisms underlying hearing loss, nor do we possess effective methods with which to detect and prevent hearing loss. This Special Issue is designed to (1) explore the mechanisms underlying sensorineural hearing loss from different research perspectives ranging from molecular, cellular, and in vivo physiological levels; and (2) provide a platform via which to exchange novel concepts and ideas for developing enhanced strategies or biomarkers to detect and prevent hearing loss caused by cochlear stress inducers, including noise, ototoxic chemicals, and age. I am delighted to invite you to publish your research articles and review papers in this Special Issue focused on the following topics.

We look forward to publishing your exciting and thought-provoking research communications.

Dr. Jing Zheng
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. International Journal of Molecular Sciences is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. There is an Article Processing Charge (APC) for publication in this open access journal. For details about the APC please see here. Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • outer hair cells
  • biomarker
  • cochlear stress
  • hearing loss

Published Papers (3 papers)

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Research

18 pages, 3913 KiB  
Article
Functional Studies of Deafness-Associated Pendrin and Prestin Variants
by Satoe Takahashi, Takashi Kojima, Koichiro Wasano and Kazuaki Homma
Int. J. Mol. Sci. 2024, 25(5), 2759; https://doi.org/10.3390/ijms25052759 - 27 Feb 2024
Cited by 1 | Viewed by 606
Abstract
Pendrin and prestin are evolutionary-conserved membrane proteins that are essential for normal hearing. Dysfunction of these proteins results in hearing loss in humans, and numerous deafness-associated pendrin and prestin variants have been identified in patients. However, the pathogenic impacts of many of these [...] Read more.
Pendrin and prestin are evolutionary-conserved membrane proteins that are essential for normal hearing. Dysfunction of these proteins results in hearing loss in humans, and numerous deafness-associated pendrin and prestin variants have been identified in patients. However, the pathogenic impacts of many of these variants are ambiguous. Here, we report results from our ongoing efforts to experimentally characterize pendrin and prestin variants using in vitro functional assays. With previously established fluorometric anion transport assays, we determined that many of the pendrin variants identified on transmembrane (TM) 10, which contains the essential anion binding site, and on the neighboring TM9 within the core domain resulted in impaired anion transport activity. We also determined the range of functional impairment in three deafness-associated prestin variants by measuring nonlinear capacitance (NLC), a proxy for motor function. Using the results from our functional analyses, we also evaluated the performance of AlphaMissense (AM), a computational tool for predicting the pathogenicity of missense variants. AM prediction scores correlated well with our experimental results; however, some variants were misclassified, underscoring the necessity of experimentally assessing the effects of variants. Together, our experimental efforts provide invaluable information regarding the pathogenicity of deafness-associated pendrin and prestin variants. Full article
(This article belongs to the Special Issue Exploring the Molecular, Cellular, and Physiological Basis of Hearing Disorders)
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15 pages, 1317 KiB  
Article
Noise Stress Abrogates Structure-Specific Endonucleases within the Mammalian Inner Ear
by O’neil W. Guthrie
Int. J. Mol. Sci. 2024, 25(3), 1749; https://doi.org/10.3390/ijms25031749 - 01 Feb 2024
Viewed by 544
Abstract
Nucleotide excision repair (NER) is a multistep biochemical process that maintains the integrity of the genome. Unlike other mechanisms that maintain genomic integrity, NER is distinguished by two irreversible nucleolytic events that are executed by the xeroderma pigmentosum group G (XPG) and xeroderma [...] Read more.
Nucleotide excision repair (NER) is a multistep biochemical process that maintains the integrity of the genome. Unlike other mechanisms that maintain genomic integrity, NER is distinguished by two irreversible nucleolytic events that are executed by the xeroderma pigmentosum group G (XPG) and xeroderma pigmentosum group F (XPF) structure-specific endonucleases. Beyond nucleolysis, XPG and XPF regulate the overall efficiency of NER through various protein–protein interactions. The current experiments evaluated whether an environmental stressor could negatively affect the expression of Xpg (Ercc5: excision repair cross-complementing 5) or Xpf (Ercc4: excision repair cross-complementing 4) in the mammalian cochlea. Ubiquitous background noise was used as an environmental stressor. Gene expression levels for Xpg and Xpf were quantified from the cochlear neurosensory epithelium after noise exposure. Further, nonlinear cochlear signal processing was investigated as a functional consequence of changes in endonuclease expression levels. Exposure to stressful background noise abrogated the expression of both Xpg and Xpf, and these effects were associated with pathological nonlinear signal processing from receptor cells within the mammalian inner ear. Given that exposure to environmental sounds (noise, music, etc.) is ubiquitous in daily life, sound-induced limitations to structure-specific endonucleases might represent an overlooked genomic threat. Full article
(This article belongs to the Special Issue Exploring the Molecular, Cellular, and Physiological Basis of Hearing Disorders)
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25 pages, 15126 KiB  
Article
Abnormal Cholesterol Metabolism and Lysosomal Dysfunction Induce Age-Related Hearing Loss by Inhibiting mTORC1-TFEB-Dependent Autophagy
by Yun Yeong Lee, Jungho Ha, Young Sun Kim, Sivasubramanian Ramani, Siung Sung, Eun Sol Gil, Oak-Sung Choo, Jeong Hun Jang and Yun-Hoon Choung
Int. J. Mol. Sci. 2023, 24(24), 17513; https://doi.org/10.3390/ijms242417513 - 15 Dec 2023
Cited by 1 | Viewed by 1242
Abstract
Cholesterol is a risk factor for age-related hearing loss (ARHL). However, the effect of cholesterol on the organ of Corti during the onset of ARHL is unclear. We established a mouse model for the ARHL group (24 months, n = 12) and a [...] Read more.
Cholesterol is a risk factor for age-related hearing loss (ARHL). However, the effect of cholesterol on the organ of Corti during the onset of ARHL is unclear. We established a mouse model for the ARHL group (24 months, n = 12) and a young group (6 months, n = 12). Auditory thresholds were measured in both groups using auditory brainstem response (ABR) at frequencies of 8, 16, and 32 kHz. Subsequently, mice were sacrificed and subjected to histological analyses, including transmission electron microscopy (TEM), H&E, Sudan Black B (SBB), and Filipin staining, as well as biochemical assays such as IHC, enzymatic analysis, and immunoblotting. Additionally, mRNA extracted from both young and aged cochlea underwent RNA sequencing. To identify the mechanism, in vitro studies utilizing HEI-OC1 cells were also performed. RNA sequencing showed a positive correlation with increased expression of genes related to metabolic diseases, cholesterol homeostasis, and target of rapamycin complex 1 (mTORC1) signaling in the ARHL group as compared to the younger group. In addition, ARHL tissues exhibited increased cholesterol and lipofuscin aggregates in the organ of Corti, lateral walls, and spiral ganglion neurons. Autophagic flux was inhibited by the accumulation of damaged lysosomes and autolysosomes. Subsequently, we observed a decrease in the level of transcription factor EB (TFEB) protein, which regulates lysosomal biosynthesis and autophagy, together with increased mTORC1 activity in ARHL tissues. These changes in TFEB and mTORC1 expression were observed in a cholesterol-dependent manner. Treatment of ARHL mice with atorvastatin, a cholesterol synthesis inhibitor, delayed hearing loss by reducing the cholesterol level and maintaining lysosomal function and autophagy by inhibiting mTORC1 and activating TFEB. The above findings were confirmed using stress-induced premature senescent House Ear Institute organ of Corti 1 (HEI-OC1) cells. The findings implicate cholesterol in the pathogenesis of ARHL. We propose that atorvastatin could prevent ARHL by maintaining lysosomal function and autophagy by inhibiting mTORC1 and activating TFEB during the aging process. Full article
(This article belongs to the Special Issue Exploring the Molecular, Cellular, and Physiological Basis of Hearing Disorders)
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