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https://www.readbyqxmd.com/read/27918591/transcriptomic-analysis-of-mouse-cochlear-supporting-cell-maturation-reveals-large-scale-changes-in-notch-responsiveness-prior-to-the-onset-of-hearing
#1
Juan C Maass, Rende Gu, Tiantian Cai, Ying-Wooi Wan, Silvia C Cantellano, Joanna S T Asprer, Hongyuan Zhang, Hsin-I Jen, Renée K Edlund, Zhandong Liu, Andrew K Groves
Neonatal mouse cochlear supporting cells have a limited ability to divide and trans-differentiate into hair cells, but this ability declines rapidly in the two weeks after birth. This decline is concomitant with the morphological and functional maturation of the organ of Corti prior to the onset of hearing. However, despite this association between maturation and loss of regenerative potential, little is known of the molecular changes that underlie these events. To identify these changes, we used RNA-seq to generate transcriptional profiles of purified cochlear supporting cells from 1- and 6-day-old mice...
2016: PloS One
https://www.readbyqxmd.com/read/27917898/incomplete-and-delayed-sox2-deletion-defines-residual-ear-neurosensory-development-and-maintenance
#2
Martina Dvorakova, Israt Jahan, Iva Macova, Tetyana Chumak, Romana Bohuslavova, Josef Syka, Bernd Fritzsch, Gabriela Pavlinkova
The role of Sox2 in neurosensory development is not yet fully understood. Using mice with conditional Islet1-cre mediated deletion of Sox2, we explored the function of Sox2 in neurosensory development in a model with limited cell type diversification, the inner ear. In Sox2 conditional mutants, neurons initially appear to form normally, whereas late- differentiating neurons of the cochlear apex never form. Variable numbers of hair cells differentiate in the utricle, saccule, and cochlear base but sensory epithelium formation is completely absent in the apex and all three cristae of the semicircular canal ampullae...
December 5, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27917401/aao-autoimmune-and-autoinflammatory-disease-in-otology-what-is-new-in-immune-mediated-hearing-loss
#3
REVIEW
Andrea Vambutas, Shresh Pathak
OBJECTIVES: Autoinflammatory diseases are a family of immune-mediated, rare diseases, some of which, exhibit sensorineural hearing loss (SNHL), suggesting potentially similar mechanisms of molecular pathogenesis between autoinflammatory-mediated hearing loss and autoimmune inner ear disease (AIED) may exist. The purpose of this review is to compare the clinical features of autoimmune and autoinflammatory diseases that affect hearing, discuss the limitations of our knowledge, and highlight potential new disease mechanisms and therapeutics...
October 2016: Laryngoscope Investigative Otolaryngology
https://www.readbyqxmd.com/read/27916008/facial-nerve-anomalies-in-paediatric-cochlear-implant-candidates-radiological-evaluation
#4
F B Palabiyik, K Hacikurt, Z Yazici
BACKGROUND: Pre-operative radiological identification of facial nerve anomalies can help prevent intra-operative facial nerve injury during cochlear implantation. This study aimed to evaluate the incidence and configuration of facial nerve anomalies and their concurrence with inner-ear anomalies in cochlear implant candidates. METHODS: Inner-ear and concomitant facial nerve anomalies were evaluated by magnetic resonance imaging and temporal high-resolution computed tomography in 48 children with congenital sensorineural hearing loss who were cochlear implant candidates...
December 5, 2016: Journal of Laryngology and Otology
https://www.readbyqxmd.com/read/27916007/characteristics-and-clinical-use-of-ocular-and-cervical-vestibular-evoked-myogenic-potentials-for-evaluating-paediatric-candidates-for-cochlear-implants
#5
X-D Xu, J Hu, Q Zhang, Y Zhang, X-T Zhang, Y-F Chen, M Xu
OBJECTIVE: This study aimed to define the characteristics and use of ocular and cervical vestibular evoked myogenic potentials for evaluating paediatric cochlear implant candidates. METHODS: Ocular and cervical vestibular evoked myogenic potentials of 34 paediatric cochlear implant candidates were analysed. All patients also underwent a routine audiological examination, including computed tomography. RESULTS: In all, 27 patients with normal inner-ear structures had absent or impaired vestibular evoked myogenic potential responses...
December 5, 2016: Journal of Laryngology and Otology
https://www.readbyqxmd.com/read/27913750/congenital-heart-block-and-immune-mediated-sensorineural-hearing-loss-possible-cross-reactivity-of-immune-response
#6
C Bason, I Pagnini, A Brucato, S Maestroni, A Puccetti, C Lunardi, R Cimaz
Immune-mediated sensorineural hearing loss may complicate systemic autoimmune diseases. We have previously reported the presence of antibodies directed against inner ear antigens in patients with Cogan syndrome, a disease characterized by sudden hearing loss and interstitial keratitis. Such autoantibodies cross-react with an epitope of SSA/Ro60 protein. Anti-Ro/SSA antibodies in pregnant women cross the placenta and reach the fetal tissues inducing an immune-mediated damage of the cardiac conduction system...
December 2, 2016: Lupus
https://www.readbyqxmd.com/read/27910089/bilateral-endolymphatic-hydrops-in-a-patient-with-migraine-variant-without-vertigo-a-case-report
#7
Isabelle Y Liu, Akira Ishiyama, Ali R Sepahdari, Kevin Johnson, Gail Ishiyama
OBJECTIVE: To use modern high-resolution inner ear imaging modalities to evaluate for endolymphatic hydrops (EH) in a patient with migraine-associated fluctuating hearing loss without vertigo spells or dizziness. BACKGROUND: EH has been well described in patients with Meniere's disease on both human temporal bone studies and modern high-resolution imaging; however, there is no study to date, to our knowledge, that examines the presence of EH in a patient with migraine and bilateral hearing loss...
December 2, 2016: Headache
https://www.readbyqxmd.com/read/27898607/effects-of-intratympanic-dexamethasone-on-high-dose-radiation-ototoxicity-in-vivo
#8
Christine T Dinh, Si Chen, John Dinh, Stefania Goncalves, Esperanza Bas, Kyle Padgett, Perry Johnson, Nagy Elsayyad, Fred Telischi, Thomas Van De Water
BACKGROUND: Stereotactic radiosurgery for lateral skull base tumors can cause hearing loss when the cochleae are exposed to high doses of single-fraction radiation. Currently, there are no known nondosimetric preventative treatments for radiation-induced ototoxicity. HYPOTHESIS: Intratympanic (IT) dexamethasone (DXM), a synthetic steroid, protects against radiation-induced auditory hair cell (HC) and hearing losses in rats in vivo. METHODS: Seven rats received radiation (12 Gy) to both cochleae...
November 24, 2016: Otology & Neurotology
https://www.readbyqxmd.com/read/27898202/optimization-of-transcutaneous-vagus-nerve-stimulation-using-functional-mri
#9
Natalia Yakunina, Sam Soo Kim, Eui-Cheol Nam
OBJECTIVE/HYPOTHESIS: Vagus nerve stimulation (VNS) is an established therapy for drug-resistant epilepsy, depression, and a number of other disorders. Transcutaneous stimulation of the auricular branch of the vagus nerve (tVNS) has been considered as a non-invasive alternative. Several functional magnetic resonance imaging (fMRI) studies on the effects of tVNS used different stimulation parameters and locations in the ear, which makes it difficult to determine the optimal tVNS methodology...
November 29, 2016: Neuromodulation: Journal of the International Neuromodulation Society
https://www.readbyqxmd.com/read/27896618/loss-of-liver-kinase-b1-causes-planar-polarity-defects-in-cochlear-hair-cells-in-mice
#10
Yuqin Men, Aizhen Zhang, Liwen Zhang, Yecheng Jin, Zhishuo Wang, Jing Zhao, Xiaolin Yu, Jian Zhang, Jiangang Gao
The tumor suppressor gene liver kinase B1 (LKB1), also called STK11, encodes a serine/threonine kinase. LKB1 plays crucial roles in cell differentiation, proliferation, and polarity. In this study, LKB1 conditional knockout mice (LKB1(Pax2) CKO mice) were generated using Pax2-Cre mice to investigate the function of LKB1 in inner ear hair cells during early embryonic period. LKB1(Pax2) CKO mice died perinatally. Immunofluorescence and scanning electron microscopy revealed that stereociliary bundles in LKB1(Pax2) CKO mice were clustered and misoriented, respectively...
November 28, 2016: Frontiers of Medicine
https://www.readbyqxmd.com/read/27895556/neurosensory-differentiation-and-innervation-patterning-in-the-human-fetal-vestibular-end-organs-between-the-gestational-weeks-8-12
#11
Lejo Johnson Chacko, Elisabeth J Pechriggl, Helga Fritsch, Helge Rask-Andersen, Michael J F Blumer, Anneliese Schrott-Fischer, Rudolf Glueckert
Balance orientation depends on the precise operation of the vestibular end organs and the vestibular ganglion neurons. Previous research on the assemblage of the neuronal network in the developing fetal vestibular organ has been limited to data from animal models. Insights into the molecular expression profiles and signaling moieties involved in embryological development of the human fetal inner ear have been limited. We present an investigation of the cells of the vestibular end organs with specific focus on the hair cell differentiation and innervation pattern using an uninterrupted series of unique specimens from gestational weeks 8-12...
2016: Frontiers in Neuroanatomy
https://www.readbyqxmd.com/read/27895484/the-effect-of-dexamethasone-cell-penetrating-peptide-nanoparticles-on-gene-delivery-for-inner-ear-therapy
#12
Ji Young Yoon, Keum-Jin Yang, Shi-Nae Park, Dong-Kee Kim, Jong-Duk Kim
Dexamethasone (Dex)-loaded PHEA-g-C18-Arg8 (PCA) nanoparticles (PCA/Dex) were developed for the delivery of genes to determine the synergistic effect of Dex on gene expression. The cationic PCA nanoparticles were self-assembled to create cationic micelles containing an octadecylamine (C18) core with Dex and an arginine 8 (Arg8) peptide shell for electrostatic complexation with nucleic acids (connexin 26 [Cx26] siRNA, green fluorescent protein [GFP] DNA or brain-derived neurotrophic factor [BDNF] pDNA). The PCA/Dex nanoparticles conjugated with Arg8, a cell-penetrating peptide that enhances permeability through a round window membrane in the inner ear for gene delivery, exhibited high uptake efficiency in HEI-OC1 cells...
2016: International Journal of Nanomedicine
https://www.readbyqxmd.com/read/27895287/sudden-sensorineural-hearing-loss-is-there-a-connection-with-inner-ear-electrolytic-disorders-a-literature-review
#13
EDITORIAL
Andrea Ciorba, Virginia Corazzi, Chiara Bianchini, Claudia Aimoni, Henryk Skarzynski, Piotr Henryk Skarzynski, Stavros Hatzopoulos
Electrolytic disorders of the inner ear represent a model that could be implicated in partially explaining the pathogenesis of sudden sensorineural hearing loss (SSNHL). Different types of electrolytes and different inner-ear loci are involved in cochlear homeostasis physiologically, to ensure the maintenance of an ion-balanced cochlear environment allowing a normal hair cell function. It has been hypothesized that a sudden loss of endocochlear potential, due to a rapid disruption of the inner ear fluid osmolality, could be responsible for a deterioration of the hearing function caused by damaged hair cells...
December 2016: International Journal of Immunopathology and Pharmacology
https://www.readbyqxmd.com/read/27891421/imaging-modality-of-choice-for-pre-operative-cochlear-imaging-hrct-vs-mri-temporal-bone
#14
Poornima Digge, Rajendra N Solanki, Dipali C Shah, Rajesh Vishwakarma, Sandeep Kumar
INTRODUCTION: Congenital inner ear malformations occur as a result of the arrest or aberrance of inner ear development due to the heredity, gene mutation or other factors. Ever since the availability of cochlear implants, pre-operative evaluation by imaging of temporal bone has gained much attention. Precise selection of the candidate for cochlear implant dependent on preoperative radiological investigations. Only CT (Computed Tomography) and MRI (Magnetic Resonance Imaging) can provide a better picture of anatomy and pathology...
October 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27890677/time-course-of-organ-of-corti-degeneration-after-noise-exposure
#15
Barbara A Bohne, Melissa Kimlinger, Gary W Harding
From our permanent collection of plastic-embedded flat preparations of chinchilla cochleae, 22 controls and 199 ears from noise-exposed animals were used to determine when, postexposure, hair cell (HC) and supporting cell (SC) degeneration were completed. The exposed ears were divided into four groups based on exposure parameters: 0.5- or 4-kHz octave band of noise at moderate (M) or high (H) intensities. Postexposure survival ranged from < 1 h to 2.5 y. Ears fixed ≤ 0-12 h postexposure were called ‛acute‛...
November 24, 2016: Hearing Research
https://www.readbyqxmd.com/read/27890673/functional-validation-of-abhd12-mutations-in-the-neurodegenerative-disease-pharc
#16
Angèle Tingaud-Sequeira, Demetrio Raldúa, Julie Lavie, Guilaine Mathieu, Magali Bordier, Anja Knoll-Gellida, Pierre Rambeau, Isabelle Coupry, Michèle André, Eva Malm, Claes Möller, Sten Andreasson, Nanna D Rendtorff, Lisbeth Tranebjærg, Michel Koenig, Didier Lacombe, Cyril Goizet, Patrick J Babin
ABHD12 mutations have been linked to neurodegenerative PHARC (polyneuropathy, hearing loss, ataxia, retinitis pigmentosa, and early-onset cataract), a rare, progressive, autosomal, recessive disease. Although ABHD12 is suspected to play a role in the lysophosphatidylserine and/or endocannabinoid pathways, its precise functional role(s) leading to PHARC disease had not previously been characterized. Cell and zebrafish models were designed to demonstrate the causal link between an identified new missense mutation p...
November 23, 2016: Neurobiology of Disease
https://www.readbyqxmd.com/read/27889918/inner-ear-labyrinth-anatomy-of-monotremes-and-implications-for-mammalian-inner-ear-evolution
#17
Julia A Schultz, Ulrich Zeller, Zhe-Xi Luo
The monophyletic clade Monotremata branches early from the rest of the mammalian crown group in the Jurassic and members of this clade retain many ancestral mammalian traits. Thus, accurate and detailed anatomical descriptions of this group can offer unique insight into the early evolutionary history of Mammalia. In this study, we examine the inner ear anatomy of two extant monotremes, Ornithorhynchus anatinus and Tachyglossus aculeatus, with the primary goals of elucidating the ancestral mammalian ear morphology and resolving inconsistencies found within previous descriptive literature...
November 27, 2016: Journal of Morphology
https://www.readbyqxmd.com/read/27888337/claudins-vital-partners-in-transcellular-and-paracellular-transport-coupling
#18
REVIEW
Dorothee Günzel
Tight junction (TJ) strands between epithelial or endothelial cells are formed by claudins, a protein family comprising up to 27 members in mammals. Although many more proteins are involved in the formation of TJ complexes, claudins are the only TJ proteins that are able to form TJ-like strands when overexpressed in cells that are normally devoid of TJs (e.g., fibroblasts). Within the paracellular cleft, the extracellular domains of claudins provide the matrix that seals the paracellular pathway. However, within this matrix, some claudins act as channels that specifically allow certain ions to cross this barrier...
November 25, 2016: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/27886803/relapsing-polychondritis-a-2016-update-on-clinical-features-diagnostic-tools-treatment-and-biological-drug-use
#19
REVIEW
Alexis Mathian, Makoto Miyara, Fleur Cohen-Aubart, Julien Haroche, Miguel Hie, Micheline Pha, Philippe Grenier, Zahir Amoura
Relapsing polychondritis (RP) is a very rare autoimmune disease characterised by a relapsing inflammation of the cartilaginous tissues (joints, ears, nose, intervertebral discs, larynx, trachea and cartilaginous bronchi), which may progress to long-lasting atrophy and/or deformity of the cartilages. Non-cartilaginous tissues may also be affected, such as the eyes, heart, aorta, inner ear and skin. RP has a long and unpredictable course. Because no randomised therapeutic trials are available, the treatment of RP remains mainly empirical...
April 2016: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/27885980/a-revision-surgery-for-cochlear-implantation-in-a-case-of-incomplete-partition-type-i
#20
Habib G Rizk, Francesca Hagood, Meredith A Holcomb, Ted A Meyer
BACKGROUND: Patients with cochlear malformations were long considered poor candidates for cochlear implantation (CI), and surgical approaches different than the standard facial recess approach were used to access the inner ear. There is no previous long-term follow-up of a patient with significantly malformed inner ear operated through an untraditional route and requiring a revision surgery. PURPOSE: This case provides a long-term follow-up from the initial surgery, a short-term follow-up from the revision surgery, and it illustrates the evolving classification of inner ear malformations as well as the potential problems associated with nonstandard approaches to the cochlea...
November 2016: Journal of the American Academy of Audiology
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