Article
Neurosciences
Nicolas I. C. Muller, Isabelle Paulussen, Lina N. Hofmann, Jonas O. Fisch, Abhyudai Singh, Eckhard Friauf
Summary: Sound localization involves information analysis in the lateral superior olive (LSO), a conspicuous nucleus in the mammalian auditory brainstem. This study shows that acoustic experience plays a significant role in the development of reliable neurotransmission in the LSO, which is essential for effective interaural level difference (ILD) detection.
JOURNAL OF PHYSIOLOGY-LONDON
(2022)
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)
Article
Multidisciplinary Sciences
Jie Li, Shuang Liu, Chenmeng Song, Qun Hu, Zhikai Zhao, Tuantuan Deng, Yi Wang, Tong Zhu, Linzhi Zou, Shufeng Wang, Jiaofeng Chen, Lian Liu, Hanqing Hou, Kexin Yuan, Hairong Zheng, Zhiyong Liu, Xiaowei Chen, Wenzhi Sun, Bailong Xiao, Wei Xiong
Summary: The knockout of the mechanosensitive ion channel PIEZO2 in cochlea disrupts ultrasonic hearing in mice, but not low-frequency hearing. Deletion of Piezo2 in outer hair cells specifically abolishes associative learning in mice during hearing exposure at ultrasonic frequencies. The study demonstrates that OHCs serve as effector cells, combining with PIEZO2 as an essential molecule for ultrasonic hearing in mice.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2021)
Article
Neurosciences
Adolfo E. Cuadra, Fuu-Jiun Hwang, Lindsay M. Burt, William C. Edmonds, Anastasia V. Chobany, Geng-Lin Li
Summary: The study found that clearance of Ca2+ dynamics in paired hair cell synapses follows a double exponential function, with the fast component significantly reduced under prolonged stimulation. Inhibiting PMCA, MCU, or SERCA increased both basal Ca2+ levels and the Ca2+ rise during sinusoidal stimulation, while significantly reducing phase-locking of synaptic vesicle releases from hair cells.
JOURNAL OF NEUROSCIENCE
(2021)
Article
Biotechnology & Applied Microbiology
Xiaoqiong Ding, Yangnan Hu, Hong Cheng, Xiaoli Zhang, Ling Lu, Song Gao, Cheng Cheng, Lifen Wang, Xiaoyun Qian, Chen Zhang, Renjie Chai, Xia Gao, Zhichun Huang
Summary: The ideal treatment for sensory hearing loss is to regenerate inner ear hair cells through stem cell therapy. This study found that Lgr5+ supporting cells in the inner ear can regenerate hair cells, which are considered as inner ear progenitor cells. The use of graphene substrates promoted the differentiation of Lgr5+ progenitors into hair cells.
FRONTIERS IN BIOENGINEERING AND BIOTECHNOLOGY
(2022)
Article
Immunology
Gaelle Mawambo, Malika Oubaha, Yusuke Ichiyama, Guillaume Blot, Sergio Crespo-Garcia, Agnieszka Dejda, Francois Binet, Roberto Diaz-Marin, Christina Sawchyn, Mikhail Sergeev, Rachel Juneau, Randal J. Kaufman, El Bachir Affar, Frederick A. Mallette, Ariel M. Wilson, Przemyslaw Sapieha
Summary: Cellular adaptation to low oxygen tension and metabolic stress in injured tissues is crucial for the proper functioning of inflammatory cells. In this study, we show that the inositol-requiring enzyme 1 alpha (IRE1 alpha/XBP1) axis is required for the initiation of HIF1 alpha-dependent cytokine production in innate immune cells. Knockout of either HIF1 alpha or IRE1 alpha improves vascular phenotypes in a model of retinal pathological angiogenesis driven by sterile inflammation. Thus, ER stress pathways, in partnership with HIF1 alpha, may co-regulate immune adaptation to low oxygen.
JOURNAL OF NEUROINFLAMMATION
(2023)
Article
Oncology
Lee-Ann Van de Velde, E. Kaitlynn Allen, Jeremy Chase Crawford, Taylor L. Wilson, Clifford S. Guy, Marion Russier, Leonie Zeitler, Armita Bahrami, David Finkelstein, Stephane Pelletier, Stacey Schultz-Cherry, Paul G. Thomas, Peter J. Murray
Summary: This study investigated the role of immune cells in tumor formation using a neuroblastoma model, and found that CD4(+) T cells and macrophages play significant roles in the process of tumor formation.
Article
Neurosciences
Pengcheng Xu, Longhao Wang, Hu Peng, Huihui Liu, Hongchao Liu, Qingyue Yuan, Yun Lin, Jun Xu, Xiuhong Pang, Hao Wu, Tao Yang
Summary: Mutations in genes encoding mitochondrial aminoacyl-tRNA synthetases can lead to sensorineural hearing loss in humans. In a mouse model with targeted knockout of histidyl-tRNA synthetase HARS2, progressive hearing loss was observed along with hair cell apoptosis and mitochondrial dysfunction. While inner hair cells demonstrated more prominent mitochondrial abnormalities, outer hair cells experienced more severe cell loss.
FRONTIERS IN CELLULAR NEUROSCIENCE
(2021)
Article
Neurosciences
Nobuyoshi Tsuzuki, Kazunori Namba, Chika Saegusa, Hideki Mutai, Takanori Nishiyama, Naoki Oishi, Tatsuo Matsunaga, Masato Fujioka, Hiroyuki Ozawa
Summary: Otof knockout mice, which lack otoferlin, are used as model mice for auditory neuropathy spectrum disorder characterized by an absent ABR despite preserved DPOAE. The effects of the Otof mutation on spiral ganglion neurons (SGNs) remains unclear. Our study on Otoftm1a/tm1a mice showed a reduction in SGNs and apoptosis of SGN-Is, suggesting that otoferlin deficiency in inner hair cells may result in secondary defects in SGNs.
NEUROSCIENCE LETTERS
(2023)
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
Materials Science, Multidisciplinary
Junyao Zhang, Qianqian Shi, Ruizhi Wang, Xuan Zhang, Li Li, Jianhua Zhang, Li Tian, Lize Xiong, Jia Huang
Summary: Research on photonic synaptic transistors based on 2DPs shows that they can successfully emulate common synaptic functions with low energy consumption and spectrum-dependent synaptic behaviors.
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
Neurosciences
Cheng Cheng, Jiaoyao Ma, Xiaoling Lu, Panpan Zhang, Xiaohan Wang, Luo Guo, Peifan Li, Ying Wei, Geng-Lin Li, Xia Gao, Yuqiu Zhang, Renjie Chai, Huawei Li, Shan Sun
Summary: Aminoglycoside antibiotics (AGAs) are commonly used in life-threatening infections but can cause hearing loss by accumulating in cochlear hair cells (HCs). This study demonstrated that the P2X7 receptor, which is activated by high ATP concentrations, may be involved in the entry of AGAs into HCs. Knocking out the P2X7 receptor protected HCs against neomycin-induced injury and inhibited oxidative stress and apoptosis in the cochlea. Therefore, the P2X7 receptor could be a potential therapeutic target for auditory HC protection.
NEUROBIOLOGY OF DISEASE
(2023)
Article
Neurosciences
Anping Xia, Tomokatsu Udagawa, Patricia M. Quinones, Patrick J. Atkinson, Brian E. Applegate, Alan G. Cheng, John S. Oghalai
Summary: The loss of even a relatively small number of outer hair cells (OHCs) can cause a substantial reduction in cochlear amplification, as these cells play a crucial role in amplifying and tuning the traveling wave in the mammalian cochlea. The selective removal of the third row of OHCs and Deiters' cells in adult mice led to a 79% reduction in gain, demonstrating the importance of OHC forces in cochlear amplification.
JOURNAL OF NEUROPHYSIOLOGY
(2022)
Article
Cell Biology
Xiaoyan Zhai, Haibo Du, Yuxin Shen, Xiujuan Zhang, Zhengjun Chen, Yanfei Wang, Zhigang Xu
Summary: This study demonstrates that FCHSD2 is a crucial protein for maintaining F-actin-based protrusions in inner-ear hair cells, and its deficiency leads to the degeneration of these protrusions and hearing loss.
JOURNAL OF CELL SCIENCE
(2022)
Article
Biology
Alena Maul, Antje Kathrin Huebner, Nicola Strenzke, Tobias Moser, Rudolf Ruebsamen, Sasa Jovanovic, Christian A. Huebner
Summary: This study reveals the importance of Kolliker's organ activity for the refinement of central auditory connectivity. The study also suggests the involvement of TMEM16A in the propagation of Ca2+ waves.
Article
Otorhinolaryngology
Christian Wrobel, Nicholas F. Bevis, Astrid Klinge-Strahl, Nicola Strenzke, Dirk Beutner
Summary: Patients with definite Meniere's disease showed significant improvement in speech perception at higher sound pressure levels after cochlear implantation, but underperformed at lower sound pressure levels compared to non-MD patients. Self-perceived hearing impairment was more pronounced in MD patients compared to non-MD controls.
LARYNGOSCOPE INVESTIGATIVE OTOLARYNGOLOGY
(2022)
Article
Biology
Tobias J. Buscham, Maria A. Eichel-Vogel, Anna M. Steyer, Olaf Jahn, Nicola Strenzke, Rakshit Dardawal, Tor R. Memhave, Sophie B. Siems, Christina Muller, Martin Meschkat, Ting Sun, Torben Ruhwedel, Wiebke Mobius, Eva-Maria Kramer-Albers, Susann Boretius, Klaus-Armin Nave, Hauke B. Werner, Kelly Monk
Summary: CMTM5 plays a role in maintaining axonal integrity rather than myelin biogenesis, as its deficiency leads to early-onset progressive axonopathy without affecting the development or ultrastructure of myelin.
Review
Medicine, Research & Experimental
Bettina Julia Wolf, Kathrin Kusch, Victoria Hunniford, Barbara Vona, Robert Kuhler, Daniel Keppeler, Nicola Strenzke, Tobias Moser
Summary: Hearing impairment is a common sensory deficit affecting millions of people worldwide, and there is a lack of effective treatment. Collaboration with cochlear implant users and the development of new therapies offer potential solutions for improving hearing restoration. Gene therapy and major innovations in cochlear implants are likely to be clinically translated within this decade.
EMBO MOLECULAR MEDICINE
(2022)
Article
Anatomy & Morphology
Homero L. Cantu-Guerra, Michael R. Papazian, Anna L. Gorsky, Nathalie S. Alekos, Adam Caccavano, Nare Karagulyan, Jakob Neef, Stefano Vicini, Tobias Moser, Thomas M. Coate
Summary: SEMA3A may regulate the excitability of SGNs in the cochlea, which could influence the morphology and synaptic arrangement of type I SGNs.
DEVELOPMENTAL DYNAMICS
(2023)
Article
Clinical Neurology
Artur Mittring, Tobias Moser, Antoine Tarquin Huet
Summary: Optogenetic control allows for innovative approaches to restore function in diseased sensory and motor systems. Customization of channelrhodopsins, emitters, and coding strategies is necessary to achieve success in clinical translation. This study provides a framework for parametrizing optogenetic neural control and applies it to the auditory pathway.
Article
Chemistry, Physical
Christian Helke, Markus Reinhardt, Markus Arnold, Falk Schwenzer, Micha Haase, Matthias Wachs, Christian Gossler, Jonathan Goetz, Daniel Keppeler, Bettina Wolf, Jannis Schaeper, Tim Salditt, Tobias Moser, Ulrich Theodor Schwarz, Danny Reuter
Summary: Improved hearing restoration through optical cochlear implants, which excite optogenetically modified spiral ganglion neurons via an optical pulse, is achieved by using flexible polymer-based waveguide probes. These probes are fabricated using micromachining processes and are capable of guiding light with low intensity loss.
Article
Neurosciences
Victoria Hunniford, Robert Kuehler, Bettina Wolf, Daniel Keppeler, Nicola Strenzke, Tobias Moser
Summary: Hearing-impaired individuals using electrical cochlear implants (eCIs) expressed a desire for improved speech comprehension, music appreciation, and more natural sound impression. They showed willingness to engage with new technologies for improved hearing restoration. However, they had concerns about receiving an implant that had not been evaluated in a human clinical trial.
FRONTIERS IN NEUROSCIENCE
(2023)
Article
Engineering, Electrical & Electronic
Tanmoy Sarkar, Katharina Lieberth, Aristea Pavlou, Thomas Frank, Volker Mailaender, Iain McCulloch, Paul W. M. Blom, Fabrizio Torriccelli, Paschalis Gkoupidenis
Summary: This study presents an organic artificial neuron based on a compact nonlinear electrochemical element. The artificial neuron is capable of operating in a liquid and can respond to the concentration of biological species in its surroundings. The system offers in situ operation and spiking behavior in biologically relevant environments.
NATURE ELECTRONICS
(2022)
Article
Medicine, Research & Experimental
Maria Zerche, Christian Wrobel, Kathrin Kusch, Tobias Moser, Thomas Mager
Summary: Optogenetic neurostimulation, using f-Chrimson-TSKir2.1 as a candidate, shows promise for sensory restoration and can be a potential alternative to electrical stimulation methods. Although removing the C-terminal fluorescent protein leads to a reduction in photocurrent amplitude, adjusting the dose of the adenoassociated virus vector and using the Kir2.1 trafficking sequence can restore optogenetic stimulation efficacy with minimal neural loss after a few months. This research holds significance for the clinical translation of optogenetic neurostimulation, particularly in the development of optical cochlear implants.
MOLECULAR THERAPY-METHODS & CLINICAL DEVELOPMENT
(2023)
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
Susann Michanski, Rohan Kapoor, Anna M. Steyer, Wiebke Moebius, Iris Frueholz, Frauke Ackermann, Mehmet Gueltas, Craig C. Garner, F. Kent Hamra, Jakob Neef, Nicola Strenzke, Tobias Moser, Carolin Wichmann
Summary: In this study, the role of piccolino in the structure and function of inner hair cell synapses was investigated using piccolo gene-trap mutant rats. The findings suggest that piccolino is essential for normal hearing and contributes to the organization of the active zone in inner hair cells.
Review
Multidisciplinary Sciences
Lakshay Khurana, Tamas Harczos, Tobias Moser, Lukasz Jablonski
Summary: Hearing loss is a common sensory deficit, and optical cochlear implants have the potential to overcome the limitations of electrical implants by using light stimulation.
Article
Engineering, Electrical & Electronic
Tobias Moser
Article
Biochemistry & Molecular Biology
Lakshay Khurana, Daniel Keppeler, Lukasz Jablonski, Tobias Moser
Summary: Optical cochlear implants (oCIs) provide a more effective restoration of hearing by directly stimulating spiral ganglion neurons (SGNs). Computer modeling was used to predict the outcome of optogenetic hearing restoration by future oCIs in humans. Waveguide-based oCIs outperformed LED-based oCIs in terms of irradiance and spectral selectivity. The effects of source-to-SGN distance, source orientation, and scar tissue were also taken into consideration.
COMPUTATIONAL AND STRUCTURAL BIOTECHNOLOGY JOURNAL
(2022)