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spiral ganglion neuron

Zhenjie Liu, Yiyun Jiang, Xiaoyang Li, Zhengqing Hu
Integration of stem cell-derived neurons into the central nervous system (CNS) remains a challenge. A co-culture system is developed to understand whether mouse embryonic stem cell (ESC)-derived spiral ganglion neuron (SGN)-like cells (ESNs) synapse with native mouse cochlear nucleus (CN) neurons. A Cre system is used to generate Cop-GFP ESCs, which are induced into ESNs expressing features similar to auditory SGNs. Neural connections are observed between ESNs and CN neurons 4-6 days after co-culturing, which is stimulated by thrombospondin-1 (TSP1)...
June 7, 2018: Stem Cell Reports
Lindsay DeVries, Julie G Arenberg
Speech understanding abilities vary widely among cochlear implant (CI) listeners. A potential source of this variability is the electrode-neuron interface (ENI), which includes peripheral factors such as electrode position and integrity of remaining spiral ganglion neurons. Suboptimal positioning of the electrode array has been associated with poorer speech outcomes; however, postoperative computerized tomography (CT) scans are often not available to clinicians. CT-estimated electrode-to-modiolus distance (distance from the inner wall of the cochlea) has been shown to account for some variability in behavioral thresholds...
June 4, 2018: Journal of the Association for Research in Otolaryngology: JARO
Florian Christov, Erik G Nelson, Michael B Gluth
INTRODUCTION: Normative data on superior olivary nucleus neuron counts derived from human specimens are sparse, and little is known about their coherence with structure and function of the cochlea. The purpose of this study was to quantify the neuron populations of the divisions of the superior olivary nucleus in human subjects with normal hearing and presbycusis and investigate potential relationships between these findings and histopathology in the cochlea and hearing phenotype Methods: Histopathologic examination of temporal bone and brainstem specimens from 13 subjects having normal hearing or presbycusis was undertaken...
June 1, 2018: Annals of Otology, Rhinology, and Laryngology
Jieying Li, Yan Chen, Shan Zeng, Chuijin Lai, Yanping Zhang, Liting Zhang, Yuxuan Shi, Tianyu Zhang, Huawei Li, Peidong Dai
Medial olivocochlear (MOC) efferent feedback is suggested to protect the ear from acoustic injury and to increase its ability to discriminate sounds against a noisy background. We investigated whether type II spiral ganglion neurons participate in the contralateral suppression of the MOC reflex. The application of ouabain to the round window of the mouse cochlea selectively induced the apoptosis of the type I spiral ganglion neurons, left the peripherin-immunopositive type II spiral ganglion neurons intact, and did not affect outer hairs, as evidenced by the maintenance of the distorted product otoacoustic emissions (DPOAEs)...
2018: Neural Plasticity
C F Guo, Y D Jin, Y L Jin
Neurotrophin-3 (NT-3) attracted increasing attention about NTFs researches in recent years. But the mechanism of promoting the development of neurons and neurite extension is not clear. Recombinant human NT-3 or NT-3 gene is commonly used in the treatment of peripheral and central neurons system damage. When rats are born, the cochlear is not mature yet. It is a suitable experimental animal for studying the morphological and functional development of the peripheral auditory pathway. It was found that NT-3 could promote the survival, growth, division and extension of the cochlear neurons in rats...
June 20, 2017: Journal of Clinical Otorhinolaryngology, Head, and Neck Surgery
X Huang, H Y Chen, T J Wei, D X Qin, P Q Liu, W H Ye, J P Su
Objective: To investigate mRNA expression of dopamine receptor subtypes in the rat cochlear spiral ganglion neurons (SGN) following exposure to the sodium salicylate. In addition, we observed the effect of sodium salicylate on N methyl-D-aspartic acid (NMDA) receptor subunit NR1 and gamma aminobutyric acid (GABA)a receptor subunit GABRα2 mRNA under the circumstance of DR activation or blocking. Moreover, we also focused on the the interaction between receptors mediated by SS. Method: Immunofluorescence techniques were applied to detect DR (DR1 and DR2) expression in cultured rat SGN...
October 20, 2017: Journal of Clinical Otorhinolaryngology, Head, and Neck Surgery
Satish R Ghimire, Evan M Ratzan, Michael R Deans
The cochlea is innervated by neurons that relay sound information from hair cells to central auditory targets. A subset of these are the type II spiral ganglion neurons, which have nociceptive features and contribute to feedback circuits providing neuroprotection in extreme noise. Type II neurons make a distinctive 90° turn towards the cochlear base to synapse with 10-15 outer hair cells. We demonstrate that this axon turning event requires planar cell polarity (PCP) signaling and is disrupted in Vangl2 and Celsr1 knockout mice, and that VANGL2 acts non-autonomously from the cochlea to direct turning...
June 14, 2018: Development
Yen-Fu Cheng
More than 80% of all cases of deafness are related to the death or degeneration of cochlear hair cells and the associated spiral ganglion neurons, and a lack of regeneration of these cells leads to permanent hearing loss. Therefore, the regeneration of lost hair cells is an important goal for the treatment of deafness. Atoh1 is a basic helix-loop-helix (bHLH) transcription factor that is critical in both the development and regeneration of cochlear hair cells. Atoh1 is transcriptionally regulated by several signaling pathways, including Notch and Wnt signalings...
July 13, 2017: Journal of Zhejiang University. Science. B
Viktorija Radotić, Dries Braeken, Petar Drviš, Marta Mattotti, Damir Kovačić
This study investigated micro-patterned, high-density complementary metal-oxide-semiconductor (CMOS) electrode array to be used as biologically permissive environment for organization, guidance and electrical stimulation of spiral ganglion neurons (SGN). SGNs extracted and isolated from cochleae of P5-P7 rat pups and adult guinea pigs were cultured 1, 4 and 7 days in vitro on glass coverslips (control) and CMOS electrode array. The cultures were analyzed visually and immunohistochemically for SGN presence, outgrowth, neurite alignment, neurite length, neurite asymmetry as well as the contact of a neuronal soma and neurites with the micro-electrodes...
May 10, 2018: Scientific Reports
Israt Jahan, Karen L Elliott, Bernd Fritzsch
The mammalian hearing organ is a stereotyped cellular assembly with orderly innervation: two types of spiral ganglion neurons (SGNs) innervate two types of differentially distributed hair cells (HCs). HCs and SGNs evolved from single neurosensory cells through gene multiplication and diversification. Independent regulation of HCs and neuronal differentiation through expression of basic Helix-loop-Helix transcription factors (bHLH TFs: Atoh1, Neurog1, Neurod1) led to the evolution of vestibular HC assembly and their unique type of innervation...
April 26, 2018: Integrative and Comparative Biology
Wei Chen, Qing-Qing Hao, Li-Li Ren, Wei Ren, Hui-Sang Lin, Wei-Wei Guo, Shi-Ming Yang
BACKGROUND: Auditory function and cochlear morphology have previously been described in a porcine model with spontaneous WS2-like phenotype. In the present study, cochlear histopathology was further investigated in the inner ear of the developing spontaneous deafness pig. RESULTS: We found that the stria vascularis transformed into a complex tri-laminar tissue at embryonic 85 days (E85) in normal pigs, but not in the MITF-/- pigs. As the neural crest (NC) of cochlea was derived by melanocytes...
May 2, 2018: BMC Neuroscience
Chao Li, Yilai Shu, Guangqin Wang, He Zhang, Ying Lu, Xiang Li, Gen Li, Lei Song, Zhiyong Liu
Precise mouse genetic studies rely on specific tools that can label specific cell types. In mouse cochlea, previous studies suggest that vesicular glutamate transporter 3 (vGlut3), also known as Slc17a8, is specifically expressed in inner hair cells (IHCs) and loss of vGlut3 causes deafness. To take advantage of its unique expression pattern, here we generate a novel vGlut3-P2A-iCreER knockin mouse strain. The P2A-iCreER cassette is precisely inserted before stop codon of vGlut3, by which the endogenous vGlut3 is intact and paired with iCreER as well...
July 2018: Hearing Research
John N Barrett, Samantha Rincon, Jayanti Singh, Cristina Matthewman, Julio Pasos, Ellen F Barrett, Suhrud M Rajguru
We investigated the effects of pulsed infrared radiation (IR, 1863 nm) stimulation on cytosolic [Ca2+ ] in inner ear spiral ganglion neurons cultured from day 4 postnatal mice and loaded with a fluorescent Ca2+ indicator (fluo-4, -5F or -5N). IR pulse trains (200 µs, 200-250 Hz, 2-5 s) delivered via an optical fiber coupled to IR source produced a rapid, transient temperature increase of 6-11ºC (above a baseline of 24-30 ºC) and evoked transient increases in both nuclear and cytosolic [Ca2+ ] of 0.20 - 1...
April 18, 2018: Journal of Neurophysiology
Xi Shi, Yan Zhang, Shiwei Qiu, Wei Zhuang, Na Yuan, Tiantian Sun, Jian Gao, Yuehua Qiao, Ke Liu
OBJECTIVE: To investigate whether a novel compound heterozygous mutations c.257C>G (p.T86R)/c.176del16 (p.G59A fs*18) in GJB2 result in hearing loss. METHODS: Allele-specific PCR-based universal array (ASPUA) screening and sequence analysis were applied to identify these mutations. 3D model was built to perform molecular dynamics (MD) simulation to verify the susceptibility of the mutations. Furthermore, WT- and Mut-GJB2 DNA fragments, containing the mutation of c...
April 17, 2018: Journal of Clinical Laboratory Analysis
Tian Yang, Ning Hu, Tina Pangršič, Steven Green, Marlan Hansen, Amy Lee
CaBPs are a family of Ca2+ binding proteins related to calmodulin. Two CaBP family members, CaBP1 and CaBP2, are highly expressed in the cochlea. Here, we investigated the significance of CaBP1 and CaBP2 for hearing in mice lacking expression of these proteins (CaBP1 KO and CaBP2 KO) using auditory brain responses (ABRs) and distortion product otoacoustic emissions (DPOAEs). In CaBP1 KO mice, ABR wave I was larger in amplitude, and shorter in latency and faster in decay, suggestive of enhanced synchrony of auditory nerve fibers...
July 2018: Hearing Research
Francisco Carricondo, Bárbara Romero-Gómez
The VIII nerve is formed by sensory neurons that innervate the inner ear, i.e., the vestibular and the auditory receptors. Neurons of the auditory portion, the cochlear afferent fibers that innervate the sensory hair cells of the organ of Corti, have their somas in the cochlear spiral ganglion where two types of neurons can be distinguished. Afferent Type-I neurons are the 95% of the total population. Bipolar and myelinated fibers, each one innervates only one cochlear inner hair cell (IHC). In contrast, afferent Type-II neurons are only the 5% of the spiral ganglion population...
April 16, 2018: Anatomical Record: Advances in Integrative Anatomy and Evolutionary Biology
Ophir Handzel, Joseph B Nadol
: Cockayne syndrome (CS) is a rare autosomal recessive syndrome resulting in defective DNA repair. Its features include cachectic dwarfism, hearing loss, skin hypersensitivity to sunlight, premature aging, and dementia. Presented is a right temporal bone of a patient who died at the age of 29 years. The clinical course was compatible with type 1 CS, the classical form. Homozygous missense variant in the ERCC6 gene (Excision Repair Cross-Complementation group 6) was found, compatible with CS complementation group B...
June 2018: Otology & Neurotology
Steven J Meas, Chun-Li Zhang, Alain Dabdoub
Disabling hearing loss affects over 5% of the world's population and impacts the lives of individuals from all age groups. Within the next three decades, the worldwide incidence of hearing impairment is expected to double. Since a leading cause of hearing loss is the degeneration of primary auditory neurons (PANs), the sensory neurons of the auditory system that receive input from mechanosensory hair cells in the cochlea, it may be possible to restore hearing by regenerating PANs. A direct reprogramming approach can be used to convert the resident spiral ganglion glial cells into induced neurons to restore hearing...
2018: Frontiers in Molecular Neuroscience
Rizwan Yousaf, Chunfang Gu, Zubair M Ahmed, Shaheen N Khan, Thomas B Friedman, Sheikh Riazuddin, Stephen B Shears, Saima Riazuddin
Autosomal recessive nonsyndromic hearing loss is a genetically heterogeneous disorder. Here, we report a severe-to-profound sensorineural hearing loss locus, DFNB100 on chromosome 5q13.2-q23.2. Exome enrichment followed by massive parallel sequencing revealed a c.2510G>A transition variant in PPIP5K2 that segregated with DFNB100-associated hearing loss in two large apparently unrelated Pakistani families. PPIP5Ks enzymes interconvert 5-IP7 and IP8, two key members of the inositol pyrophosphate (PP-IP) cell-signaling family...
March 2018: PLoS Genetics
Wenwen Liu, Xiaochen Xu, Zhaomin Fan, Gaoying Sun, Yuechen Han, Daogong Zhang, Lei Xu, Mingming Wang, Xue Wang, Shasha Zhang, Mingliang Tang, Jianfeng Li, Renjie Chai, Haibo Wang
AIMS: Cisplatin can damage spiral ganglion neurons (SGNs) and cause sensorineural hearing loss. Wnt activation protects against neomycin-induced hair cell damage in the mouse cochlea, but the role of Wnt signaling in protecting SGNs from cisplatin treatment has not yet been elucidated. This study was designed to investigate the neuroprotective effects of Wnt signaling against cisplatin-induced SGN damage. RESULTS: First, we found that Wnt signaling was activated in SGNs after cisplatin treatment...
May 4, 2018: Antioxidants & Redox Signaling
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