Review
Neurosciences
Lingna Guo, Wei Cao, Yuguang Niu, Shuangba He, Renjie Chai, Jianming Yang
Summary: Inner ear hair cells and spiral ganglion neurons are crucial components of the auditory system, but they are susceptible to genetic defects, noise exposure, ototoxic drugs, and aging. Efforts to combat hearing loss have included cochlear implants, hair cell regeneration, gene therapy, and antioxidant drugs. Understanding the role of autophagy in sensorineural hearing loss and identifying potential autophagy-related targets could lead to new treatments for hearing loss.
FRONTIERS IN CELLULAR NEUROSCIENCE
(2021)
Review
Biochemistry & Molecular Biology
Tobias Moser, Nare Karagulyan, Jakob Neef, Lina Maria Jaime Tobon
Summary: This review discusses the mechanisms behind processing different sound pressures and intensities in the auditory system, as well as the neural decomposition and coding of sound intensity information.
Article
Biochemistry & Molecular Biology
Chika Saegusa, Wataru Kakegawa, Eriko Miura, Takahiro Aimi, Sachiyo Mogi, Tatsuhiko Harada, Taku Yamashita, Michisuke Yuzaki, Masato Fujioka
Summary: This study investigated the expression and function of Bai3, an adhesion G-protein-coupled receptor, in the cochlea. The results showed that Bai3 plays a crucial role in the development and maintenance of pillar cells, and its loss can lead to abnormal formation of pillar cells and impaired hearing function.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Review
Neurosciences
Sumana Ghosh, Kendra Stansak, Bradley J. Walters
Summary: Plants of the genus Cannabis have been used for various purposes by humans for millennia. The biologically active molecules in these plants, known as cannabinoids, play important roles in both plant and human cells, particularly in neuromodulation. Understanding the significance of cannabinoid signaling in the auditory system is an emerging area of research.
FRONTIERS IN MOLECULAR NEUROSCIENCE
(2021)
Article
Audiology & Speech-Language Pathology
Donna S. Whitlon
Summary: Statins, in addition to their cholesterol-lowering effects, have other beneficial effects such as increased antioxidant activity, decreased inflammatory mediators, and alleviation of blood vessel damage. Research on damaged cochlear structures and hearing loss suggests that statins may have protective effects and potential therapeutic applications. However, there is a lack of clinical studies and inconsistent findings currently.
Article
Biochemistry & Molecular Biology
Qian Li, Liuqian Wang, Di Ji, Wei Yu, Yan Zhang, Yanghong Xiang, Chao Zhou, Liting Wang, Ping Deng, Huifeng Pi, Yonghui Lu, Qinlong Ma, Mindi He, Lei Zhang, Zhengping Yu, Anchun Deng
Summary: This study reveals that autophagy dysfunction plays a significant role in cadmium-induced spiral ganglion neuron degeneration, and metformin can attenuate this degeneration by restoring impaired autophagy flux.
JOURNAL OF INORGANIC BIOCHEMISTRY
(2022)
Article
Cell Biology
Kai Xu, Sen Chen, Xue Bai, Le Xie, Yue Qiu, Xiao-zhou Liu, Xiao-hui Wang, Wei-jia Kong, Yu Sun
Summary: The GJB2 gene mutation leads to congenital deafness or late-onset progressive hearing loss, and it has been found that deficiency of Cx26 accelerates age-related hearing loss. The expression of Cx26 is significantly reduced in the cochleae of aged mice, and it is degraded by lysosomes. Mice with Cx26 knockout in the cochlea show high-frequency hearing loss and degeneration of hair cells and spiral ganglions, similar to age-related hearing loss phenotype.
Review
Pharmacology & Pharmacy
Mami Matsunaga, Takayuki Nakagawa
Summary: Sensorineural hearing loss has been a global burden for decades, but recent progress in experimental studies on hair cell regeneration and protection has rapidly advanced clinical trials for pharmacotherapy. This review focuses on recent clinical trials for hair cell protection and regeneration, outlining mechanisms based on associated experimental studies. The outcomes of these trials have provided valuable data on the safety and tolerability of drug delivery methods. Recent findings in molecular mechanisms of hair cell regeneration suggest the potential for regenerative medicine in the near future.
Article
Neurosciences
Hao Xiong, Lan Lai, Yongyi Ye, Yiqing Zheng
Summary: The study shows that glucose supplementation can reduce the formation of reactive oxygen species induced by oxidative stress, thereby protecting cochlear hair cells and reducing NIHL. Glucose supplementation increases ATP and NADPH levels, reduces H2O2-induced ROS production and cytotoxicity. Pharmacological inhibition of glucose transporter type 1 activity eliminates the protective effects of glucose against oxidative stress.
NEUROSCIENCE BULLETIN
(2021)
Article
Biochemistry & Molecular Biology
Fei Lu, Pengfei Liang, Bei Fan, Qingwen Zhu, Tao Xue, Zhenzhen Liu, Renfeng Wang, Yukai Zhang, Xinyu Zhang, Wei Li, Shujuan Wang, Jun Chen, Dingjun Zha
Summary: Autosomal recessive nonsyndromic auditory neuropathy is caused by a genetic etiology. Through candidate gene screening and exome sequencing, we identified a compound heterozygous missense variant in the TNN gene of affected patients in a pedigree. Our findings demonstrate that tenascin-W plays an essential role in the development and function of spiral ganglion neurons in the murine auditory system, and abnormal expression of tenascin-W can affect the function of the auditory system.
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS
(2022)
Review
Neurosciences
Li Zhang, Sen Chen, Yu Sun
Summary: Sensorineural hearing loss affects approximately 360 million people worldwide, and the survival of spiral ganglion neurons is crucial for the efficacy of cochlear implantation. The loss of SGNs can lead to irreversible hearing loss, and various factors like noise exposure and ototoxic drugs can cause damage to SGNs. Gene therapy and stem cell therapy are considered as promising strategies for preventing or reversing SGN damage.
FRONTIERS IN CELLULAR NEUROSCIENCE
(2022)
Article
Multidisciplinary Sciences
Jemma L. Webber, John C. Clancy, Yingjie Zhou, Natalia Yraola, Kazuaki Homma, Jaime Garcia-Anoveros
Summary: Through the use of an Insm1 mouse mutant with IHCs in the position of OHCs, a hierarchical sequence of instructions was discovered: first, IHCs attract and OHCs repel type I afferents; second, type II afferents innervate hair cells not contacted by type I afferents; and lastly, the type of afferent fiber determines how efferents innervate, whether axodendritically on the afferent, axosomatically on the hair cell, or not at all.
Article
Cell Biology
Haiying Sun, Tian Wang, Patrick J. Atkinson, Sara E. Billings, Wuxing Dong, Alan G. Cheng
Summary: The study reveals that Gpr125 is dynamically expressed in various cell types in the developing and mature cochlea, but it is not essential for cochlear development and hearing.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Multidisciplinary Sciences
Caroline Siebald, Philippe F. Y. Vincent, Riley T. Bottom, Shuohao Sun, Daniel O. J. Reijntjes, Marco Manca, Elisabeth Glowatzki, Ulrich Muller
Summary: This study reveals three molecularly defined subtypes of type I SGNs using single-cell RNA sequencing technology, but the extent to which they correspond to physiological subtypes is unclear. Using genetically marked SGN subtypes, the researchers found that Lypd1-CreERT2 expressing SGNs have a low SR and preferentially innervate the IHC modiolar side, while Calb2-CreERT2 expressing SGNs have a wider range of SRs and preferentially innervate the IHC pillar side.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2023)
Article
Neurosciences
Ursula Stalmann, Albert Justin Franke, Hanan Al-Moyed, Nicola Strenzke, Ellen Reisinger
Summary: Deficiency of otoferlin leads to developmental deficits and degenerative mechanisms in the auditory system of mice, particularly affecting synapse development and reduced numbers of IHC and SGNs. This degeneration is more pronounced in the basal aspects of the cochlea, with OHCs showing accelerated age-dependent loss associated with a decrease in DPOAE amplitudes for high frequency stimuli.
FRONTIERS IN CELLULAR NEUROSCIENCE
(2021)