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Safa Alqudah, Mark Chertoff, Dianne Durham, Jackob Moskovitz, Hinrich Staecker, Marcello Peppi
Methionine sulfoxide reductases (MsrA and MsrB) protect the biological activity of proteins from oxidative modifications to methionine residues and are important for protecting against the pathological effects of neurodegenerative diseases. In the current study, we characterized the auditory phenotype of the MsrA knockout mouse. Young MsrA knockout mice showed small high-frequency threshold elevations for auditory brainstem response and distortion product otoacoustic emission compared to those of wild-type mice, which progressively worsened in older MsrA knockout mice...
June 21, 2018: Audiology & Neuro-otology
Sang-Yeon Lee, Dong-Han Lee, Yun Jung Bae, Jae-Jin Song, Ji Soo Kim, Ja-Won Koo
Background: Superficial siderosis (SS) is a rare condition in which hemosiderin, an iron storage complex, is deposited in neural tissues because of recurrent subarachnoid bleeding. Hemosiderin deposition in the vestibulocochlear nerve (CN VIII), brain, spinal cord and peripheral nerve can cause sensorineural hearing loss (SNHL) and postural imbalance, but much remains unknown about the vestibular manifestations of SS. Objectives: To report the clinical course, cochleovestibular status, and patterns of vestibulopathy during follow-up of a relatively large case series, and to discuss the possible pathophysiological mechanism of vestibular deterioration...
2018: Frontiers in Neurology
Martin J Spencer, Hamish Meffin, Anthony N Burkitt, David B Grayden
Asynchrony among synaptic inputs may prevent a neuron from responding to behaviorally relevant sensory stimuli. For example, "octopus cells" are monaural neurons in the auditory brainstem of mammals that receive input from auditory nerve fibers (ANFs) representing a broad band of sound frequencies. Octopus cells are known to respond with finely timed action potentials at the onset of sounds despite the fact that due to the traveling wave delay in the cochlea, synaptic input from the auditory nerve is temporally diffuse...
2018: Frontiers in Computational Neuroscience
Qing-Qing Hao, Liang Li, Wei Chen, Qing-Qing Jiang, Fei Ji, Wei Sun, Hong Wei, Wei-Wei Guo, Shi-Ming Yang
SRY-box 10 (SOX10) mutation may lead to inner ear deformities. However, its molecular mechanisms on inner ear development are not clear. In this work, the inner ear morphology was investigated at different embryonic stages of the SOX10 mutation miniature porcine model with sensorineural hearing loss, and high-throughput RNA-seq and bioinformatics analyses were applied. Our results indicated that the SOX10 mutation in the miniature pigs led to an incomplete partition (IP) of the cochlea, a cystic apex caused by fusion from middle and apical turns, cochlear modiolar defects and a shortened cochlear duct...
2018: Frontiers in Molecular Neuroscience
Joaquin T Valderrama, Elizabeth Francis Beach, Ingrid Yeend, Mridula Sharma, Bram Van Dun, Harvey Dillon
Recent animal studies have shown that the synapses between inner hair cells and the dendrites of the spiral ganglion cells they innervate are the elements in the cochlea most vulnerable to excessive noise exposure. Particularly in rodents, several studies have concluded that exposure to high level octave-band noise for 2 h leads to an irreversible loss of around 50% of synaptic ribbons, leaving audiometric hearing thresholds unaltered. Cochlear synaptopathy following noise exposure is hypothesized to degrade the neural encoding of sounds at the subcortical level, which would help explain certain listening-in-noise difficulties reported by some subjects with otherwise 'normal' hearing...
June 12, 2018: Hearing Research
Mohamed A Alhussaini, Renee M Banakis Hartl, Victor Benichoux, Daniel J Tollin, Herman A Jenkins, Nathaniel T Greene
HYPOTHESIS: Simulated otitis media with effusion reduces intracochlear pressures comparable to umbo velocity. BACKGROUND: Otitis media with effusion is a common cause of temporary hearing loss, particularly in children, producing deficits of 30 to 40 dB. Previous studies measured the effects of simulated effusion on ossicular mechanics; however, no studies have measured cochlear stimulation directly. Here, we compare pressures in the scala vestibuli and tympani to umbo velocity, before and after induction of simulated effusion in cadaveric human specimens...
June 15, 2018: Otology & Neurotology
Fiona C E Hill, Alasdair Grenness, Shannon Withers, Claire Iseli, Robert Briggs
OBJECTIVES: To determine the temporal occurrence of fluid signal changes on T2-weighted magnetic resonance imaging (T2MRI) that occur in the cochlear lumen after translabyrinthine surgery. STUDY DESIGN: Retrospective case control study. SETTING: Tertiary referral neurotology center. SUBJECTS AND METHODS: Seventy-one patients were identified who underwent translabyrinthine removal of a vestibular schwannoma between 2010 and 2014; of these 49 were included as they had postoperative T2 MRI at 6, 18, and 48 months available postsurgery...
June 15, 2018: Otology & Neurotology
Ksenia Gnedeva, A J Hudspeth, Neil Segil
The sensory organs of the inner ear are challenging to study in mammals due to their inaccessibility to experimental manipulation and optical observation. Moreover, although existing culture techniques allow biochemical perturbations, these methods do not provide a means to study the effects of mechanical force and tissue stiffness during development of the inner ear sensory organs. Here we describe a method for three-dimensional organotypic culture of the intact murine utricle and cochlea that overcomes these limitations...
June 1, 2018: Journal of Visualized Experiments: JoVE
Mia E Miller, Ivan A Lopez, Fred H Linthicum, Akira Ishiyama
HYPOTHESIS: Connexin-26 (Cx26) expression is diminished in the spiral ligament of subjects with hearing loss and cochlear otosclerosis (CO). BACKGROUND: Human temporal bone (HTB) studies have demonstrated that CO is associated with hyalinization of the spiral ligament. We hypothesize that hyalinization is associated with a loss of fibrocytes with a consequent decline in Cx26 expression. Cx26 and Connexin-30 (Cx30) encode gap junction proteins expressed in supporting cells of the organ of Corti, the spiral limbus, stria vascularis, and in fibrocytes of the spiral ligament...
June 1, 2018: Annals of Otology, Rhinology, and Laryngology
Tobias Moser
This review addresses the advances of our molecular understanding of hearing and how this benefits the hearing impaired. Classical biochemical methods usually fall short in contributing to the analysis of the molecular mechanisms of hearing e. g. in the cochlea, the auditory part of the inner ear, due to the scarcity of the cells of interest. Genetics, molecular cell biology, and physiology, on the other hand, have elucidated the intricate molecular and cellular mechanisms that bring about the outstanding performance of the auditory system...
March 2018: Laryngo- Rhino- Otologie
Kaley Woods, Percy Lee, Tania Kaprealian, Isaac Yang, Ke Sheng
Purpose: This study investigates whether 4π noncoplanar radiation therapy can spare the cochleae and consequently potentially improve hearing preservation in patients with acoustic neuroma who are treated with radiation therapy. Methods and materials: Clinical radiation therapy plans for 30 patients with acoustic neuroma were included (14 stereotactic radiation surgery [SRS], 6 stereotactic radiation therapy [SRT], and 10 intensity modulated radiation therapy [IMRT])...
April 2018: Advances in Radiation Oncology
Wei Zhou, Jintao Du, Di Jiang, Xianren Wang, Kaitian Chen, Haocheng Tang, Xuemei Zhang, Hui Cao, Ling Zong, Chang Dong, Hongyan Jiang
Auditory hair cell regeneration following injury is critical to hearing restoration. The Notch signaling pathway participates in the regulation of inner ear development and cell differentiation. Recent evidence suggests that microRNA (miR)‑183 has a similar role in the inner ear. However, it is unclear how Notch signaling functions in hair cell regeneration in mammals and if there is cross‑talk between Notch signaling and miR‑183. The present study used a gentamicin‑induced cochlear injury mouse model...
June 5, 2018: Molecular Medicine Reports
Bouchra Edderkaoui, Liana Sargsyan, Alisa Hetrick, Hongzhe Li
Cochlear inflammatory response to various environmental insults, including acoustic and ototoxic overexposures, has been increasingly become a topic of interest. As the immune response is associated with both pathology and protection, targeting specific components of the immune response is expected to dissect the relationships between cellular damage and inflammation-associated protection and repair in the cochlea. Duffy antigen receptor for chemokines (DARC) is a member of a group of atypical chemokine receptors, and essential for chemokine-regulated leukocyte/neutrophil trafficking during inflammation...
2018: Frontiers in Molecular Neuroscience
Satu Turunen-Taheri, Per-Inge Carlsson, Ann-Christin Johnson, Sten Hellström
PURPOSE: The purpose of this study was to identify and report demographic data of patients with severe-to-profound hearing loss, assess participation in audiological rehabilitation and analyze the benefits of various rehabilitation methods. MATERIALS AND METHODS: Data on 4286 patients with severe-to-profound hearing impairments registered in the Swedish Quality Register of Otorhinolaryngology over a period from 2006-2015 were studied. Demographic data, gender differences, audiological rehabilitation and benefits of the rehabilitation were analyzed...
June 12, 2018: Disability and Rehabilitation
Aran Yoo, Jonathan Jou, Jeffrey D Klopfenstein, Jorge C Kattah
Background: Infratentorial siderosis (iSS) is a progressive degenerative disorder targeting primarily the cerebellum and cranial nerve eighth; therefore, progressive ataxia and its neuro-otological findings are common. Toxicity from hemosiderin involves selectively vulnerable neurons and glia in these posterior fossa structures. Other neurologic findings may be present, though our focus relates to the cochlea-vestibular cerebellar involvement. Radiographic evidence of siderosis may be the result of recurrent, albeit covert bleeding in the subarachnoid space, or the consequence of an overt post-traumatic or aneurysmal subarachnoid hemorrhage (SAH)...
2018: Frontiers in Neurology
Reuven Ishai, Renata M Knoll, Jenny X Chen, Kevin Wong, Katherine L Reinshagen, Joseph B Nadol, Aaron K Remenschneider, David H Jung, Elliott D Kozin
Objective Hearing loss following temporal bone (TB) fracture may result from direct transection of the middle and inner ear. The pathophysiology of hearing loss due to head injury without TB fracture, however, is not well understood. Few reports describe otopathologic findings. Herein, we investigate the pathologic findings of patients who sustained a head injury without evidence of a TB fracture. Study Design Otopathology study. Setting Otopathology laboratory. Subjects Subjects with a history of head injury without TB fracture...
June 1, 2018: Otolaryngology—Head and Neck Surgery
Renee M Banakis Hartl, Nathaniel T Greene, Herman A Jenkins, Stephen P Cass, Daniel J Tollin
HYPOTHESIS: Insertion of cochlear implant electrodes generates transient pressure spikes within the vestibular labyrinth equivalent to high-intensity acoustic stimuli. BACKGROUND: Though cochlear implant (CI) surgery is regarded as having low risk of impacting the vestibular system, several studies have documented changes in vestibular function after implantation. The mechanism of these changes is not understood. We have previously established that large, potentially damaging pressure transients can be generated in the cochlea during electrode insertion, but whether pressure transients occur within the vestibular labyrinth has yet to be determined...
July 2018: Otology & Neurotology
Jing-Ying Guo, Lu He, Teng-Fei Qu, Yu-Ying Liu, Ke Liu, Guo-Peng Wang, Shu-Sheng Gong
Local delivery of therapeutic drugs into the inner ear is a promising therapy for inner ear diseases. Injection through semicircular canals (canalostomy) has been shown to be a useful approach to local drug delivery into the inner ear. The goal of this article is to describe, in detail, the surgical techniques involved in canalostomy in both adult and neonatal mice. As indicated by fast-green dye and adeno-associated virus serotype 8 with the green fluorescent protein gene, the canalostomy facilitated broad distribution of injected reagents in the cochlea and vestibular end-organs with minimal damage to hearing and vestibular function...
May 25, 2018: Journal of Visualized Experiments: JoVE
Aoshuang Chang, Cuixian Li, Jianfeng Huang, Wenlu Pan, Yinghong Tian, Jie Tang
The outer hair cell is one of the two types of sensory hair cells in the mammalian cochlea. They alter their cell length with the receptor potential to amplify the weak vibration of low-level sound signal. The morphology and electrophysiological property of outer hair cells (OHCs) develop in early postnatal ages. The maturation of outer hair cell may contribute to the development of the auditory system. However, the process of OHCs development is not well studied. This is partly because of the difficulty to measure their function by an electrophysiological approach...
May 24, 2018: Journal of Visualized Experiments: JoVE
Aykut Aksit, Daniel N Arteaga, Miguel Arriaga, Xun Wang, Hirobumi Watanabe, Karen E Kasza, Anil K Lalwani, Jeffrey W Kysar
The cochlea, or inner ear, is a space fully enclosed within the temporal bone of the skull, except for two membrane-covered portals connecting it to the middle ear space. One of these portals is the round window, which is covered by the Round Window Membrane (RWM). A longstanding clinical goal is to reliably and precisely deliver therapeutics into the cochlea to treat a plethora of auditory and vestibular disorders. Standard of care for several difficult-to-treat diseases calls for injection of a therapeutic substance through the tympanic membrane into the middle ear space, after which a portion of the substance diffuses across the RWM into the cochlea...
June 8, 2018: Biomedical Microdevices
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