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https://www.readbyqxmd.com/read/28209736/pejvakin-a-candidate-stereociliary-rootlet-protein-regulates-hair-cell-function-in-a-cell-autonomous-manner
#1
Marcin Kazmierczak, Piotr Kazmierczak, Anthony W Peng, Suzan L Harris, Prahar Shah, Jean-Luc Puel, Marc Lenoir, Santos J Franco, Martin Schwander
Mutations in the Pejvakin (PJVK) gene are thought to cause auditory neuropathy and hearing loss of cochlear origin by affecting noise-induced peroxisome proliferation in auditory hair cells and neurons. Here we demonstrate that loss of pejvakin in hair cells, but not in neurons, causes profound hearing loss and outer hair cell degeneration in mice. Pejvakin binds to and co-localizes with the rootlet component TRIOBP at the base of stereocilia in injectoporated hair cells, a pattern that is disrupted by deafness-associated PJVK mutations...
February 16, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28202466/time-varying-auditory-gain-control-in-response-to-double-pulse-stimuli-in-harbour-porpoises-is-not-mediated-by-a-stapedial-reflex
#2
Asger Emil Munch Schrøder, Kristian Beedholm, Peter Teglberg Madsen
Echolocating animals reduce their output level and hearing sensitivity, presumably to stabilize the perceived echo intensity during target approaches. In bats this variation in hearing sensitivity is formed by a call-induced stapedial reflex that tapers off over time after the call. Here we test the hypothesis that a similar mechanism exists in toothed whales by subjecting a trained harbour porpoise to a series of double sound pulses varying in delay and frequency, while measuring the magnitudes of the evoked ABRs...
February 15, 2017: Biology Open
https://www.readbyqxmd.com/read/28196606/imaging-and-cell-count-in-cleared-intact-cochlea-in-the-mongolian-gerbil-using-laser-scanning-confocal-microscopy
#3
M Risoud, J Sircoglou, G Dedieu, M Tardivel, C Vincent, N-X Bonne
OBJECTIVES: To draw up a clearing protocol for Mongolian gerbil cochlea, and to assess the feasibility of quantifying and analyzing 3D cell architecture in the transparent cochleae. MATERIALS AND METHODS: Freshly dissected inner ears were prepared on a 13-day protocol: fixation, microdissection, post-fixation, decalcification, pretreatment (signal enhancement, permeabilization and blocking), fluorescent labeling (indirect immunolabeling and direct labeling), dehydration, clearing in Spalteholz solution (MSBB: methyl salicylate and benzyl benzoate) and mounting...
February 9, 2017: European Annals of Otorhinolaryngology, Head and Neck Diseases
https://www.readbyqxmd.com/read/28179572/spectrin-%C3%AE-v-adaptive-mutations-and-changes-in-subcellular-location-correlate-with-emergence-of-hair-cell-electromotility-in-mammalians
#4
Matteo Cortese, Samantha Papal, Francisco Pisciottano, Ana Belén Elgoyhen, Jean-Pierre Hardelin, Christine Petit, Lucia Florencia Franchini, Aziz El-Amraoui
The remarkable hearing capacities of mammals arise from various evolutionary innovations. These include the cochlear outer hair cells and their singular feature, somatic electromotility, i.e., the ability of their cylindrical cell body to shorten and elongate upon cell depolarization and hyperpolarization, respectively. To shed light on the processes underlying the emergence of electromotility, we focused on the βV giant spectrin, a major component of the outer hair cells' cortical cytoskeleton. We identified strong signatures of adaptive evolution at multiple sites along the spectrin-βV amino acid sequence in the lineage leading to mammals, together with substantial differences in the subcellular location of this protein between the frog and the mouse inner ear hair cells...
February 8, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28170296/muse-cells-derived-from-dermal-tissues-can-differentiate-into-melanocytes
#5
Ting Tian, Ru-Zhi Zhang, Yu-Hua Yang, Qi Liu, Di Li, Xiao-Ru Pan
The objective of the authors has been to obtain multilineage-differentiating stress-enduring cells (Muse cells) from primary cultures of dermal fibroblasts, identify their pluripotency, and detect their ability to differentiate into melanocytes. The distribution of SSEA-3-positive cells in human scalp skin was assessed by immunohistochemistry, and the distribution of Oct4, Sox2, Nanog, and SSEA-3-positive cells was determined by immunofluorescence staining. The expression levels of Sox2, Oct4, hKlf4, and Nanog mRNAs and proteins in Muse cells were determined by reverse transcription polymerase chain reaction (RT-PCR) analyses and Western blots, respectively...
February 7, 2017: Cellular Reprogramming
https://www.readbyqxmd.com/read/28165504/implementation-of-a-method-to-visualize-noise-induced-hearing-loss-in-mass-stranded-cetaceans
#6
Maria Morell, Andrew Brownlow, Barry McGovern, Stephen A Raverty, Robert E Shadwick, Michel André
Assessment of the impact of noise over-exposure in stranded cetaceans is challenging, as the lesions that lead to hearing loss occur at the cellular level and inner ear cells are very sensitive to autolysis. Distinguishing ante-mortem pathology from post-mortem change has been a major constraint in diagnosing potential impact. Here, we outline a methodology applicable to the detection of noise-induced hearing loss in stranded cetaceans. Inner ears from two mass strandings of long-finned pilot whales in Scotland were processed for scanning electron microscopy observation...
February 6, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28165475/a-synthetic-aav-vector-enables-safe-and-efficient-gene-transfer-to-the-mammalian-inner-ear
#7
Lukas D Landegger, Bifeng Pan, Charles Askew, Sarah J Wassmer, Sarah D Gluck, Alice Galvin, Ruth Taylor, Andrew Forge, Konstantina M Stankovic, Jeffrey R Holt, Luk H Vandenberghe
Efforts to develop gene therapies for hearing loss have been hampered by the lack of safe, efficient, and clinically relevant delivery modalities. Here we demonstrate the safety and efficiency of Anc80L65, a rationally designed synthetic vector, for transgene delivery to the mouse cochlea. Ex vivo transduction of mouse organotypic explants identified Anc80L65 from a set of other adeno-associated virus (AAV) vectors as a potent vector for the cochlear cell targets. Round window membrane injection resulted in highly efficient transduction of inner and outer hair cells in mice, a substantial improvement over conventional AAV vectors...
February 6, 2017: Nature Biotechnology
https://www.readbyqxmd.com/read/28163078/manganese-superoxide-dismutase-influences-the-extent-of-noise-induced-hearing-loss-in-mice
#8
Ayinuer Tuerdi, Makoto Kinoshita, Teru Kamogashira, Chisato Fujimoto, Shinnichi Iwasaki, Takahiko Shimizu, Tatsuya Yamasoba
Reactive oxygen species (ROS) generation is one of the mechanisms underlying noise-induced hearing loss (NIHL). Manganese superoxide dismutase (Mn-SOD), an antioxidant enzyme acting within the mitochondria, converts toxic superoxide to hydrogen peroxide. We investigated the role of Mn-SOD in NIHL by examining the extent of hearing loss and hair cell damage after noise exposure in C57BL/6 wild-type (WT) mice and Mn-SOD heterozygous knockout (HET) mice (n=6 each). Both HET and WT mice were exposed to 120dB sound pressure level at 4kHz octave band noise for 4h...
February 3, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28157763/effect-of-procedural-related-variables-on-melanocyte-keratinocyte-suspension-transplantation-in-nonsegmental-stable-vitiligo-a-clinical-and-immunocytochemical-study
#9
Bakr Mohamed El-Zawahry, Samia Esmat, Dalia Bassiouny, Naglaa Sameh Zaki, Rehab Sobhi, Marwah A Saleh, Dalia Abdel-Halim, Rehab Hegazy, Heba Gawdat, Nesrin Samir, Marwa El-Hawary, Zeinab El Maadawi, Heba Gouda, Mervat Khorshied
BACKGROUND: Melanocyte-keratinocyte suspension (M-K susp) is gaining popularity for vitiligo treatment. Few studies have addressed procedure-related variables. OBJECTIVE: To assess the effect of different M-K susp procedure-related variables on the clinical outcome in stable vitiligo. METHODS: This prospective multicenter comparative study included 40 cases with nonsegmental stable vitiligo. Donor site was either a skin graft in noncultured epidermal cell suspension (NCECS) or hair follicle units in outer root sheath hair follicle suspension (ORSHFS)...
February 2017: Dermatologic Surgery: Official Publication for American Society for Dermatologic Surgery [et Al.]
https://www.readbyqxmd.com/read/28143435/genetic-characterization-of-italian-patients-with-bardet-biedl-syndrome-and-correlation-to-ocular-renal-and-audio-vestibular-phenotype-identification-of-eleven-novel-pathogenic-sequence-variants
#10
Gabriella Esposito, Francesco Testa, Miriam Zacchia, Anna Alessia Crispo, Valentina Di Iorio, Giovanna Capolongo, Luca Rinaldi, Marcella D'Antonio, Tiziana Fioretti, Pasquale Iadicicco, Settimio Rossi, Annamaria Franzè, Elio Marciano, Giovanbattista Capasso, Francesca Simonelli, Francesco Salvatore
BACKGROUND: Bardet-Biedl syndrome (BBS) is a rare genetic disorder that features retinal degeneration, obesity, polydactyly, learning disabilities and renal abnormalities. The diagnosis is often missed at birth, the median age at diagnosis being 9 years. In the attempt to shed light on BBS and improve its diagnosis and treatment, we evaluated the genotype-phenotype relationship in patients with a molecular diagnosis of BBS. METHODS: We analyzed three common BBS genes, BBS1, BBS10 and BBS2, in 25 Italian patients fulfilling the clinical criteria of BBS...
February 1, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28133939/reduced-acoustic-startle-response-and-peripheral-hearing-loss-in-the-5xfad-mouse-model-of-alzheimer-s-disease
#11
Timothy P O'Leary, Sooyoun Shin, Emre Fertan, Rachel N Dingle, Awad Almuklass, Rhian K Gunn, Zhiping Yu, Jian Wang, Richard E Brown
Hearing dysfunction has been associated with Alzheimer's disease in humans, but there is little data on the auditory function of mouse models of Alzheimer's disease. Furthermore, characterization of hearing ability in mouse models is needed to ensure that tests of cognition that use auditory stimuli are not confounded by hearing dysfunction. Therefore we assessed acoustic startle response and pre-pulse inhibition in the double transgenic 5xFAD mouse model of Alzheimer's disease from 3-4 to 16 months of age...
January 29, 2017: Genes, Brain, and Behavior
https://www.readbyqxmd.com/read/28118374/dynamic-expression-of-sox2-gata3-and-prox1-during-primary-auditory-neuron-development-in-the-mammalian-cochlea
#12
Koji Nishimura, Teppei Noda, Alain Dabdoub
Primary auditory neurons (PANs) connect cochlear sensory hair cells in the mammalian inner ear to cochlear nucleus neurons in the brainstem. PANs develop from neuroblasts delaminated from the proneurosensory domain of the otocyst and keep maturing until the onset of hearing after birth. There are two types of PANs: type I, which innervate the inner hair cells (IHCs), and type II, which innervate the outer hair cells (OHCs). Glial cells surrounding these neurons originate from neural crest cells and migrate to the spiral ganglion...
2017: PloS One
https://www.readbyqxmd.com/read/28109481/amplified-music-exposure-carries-risks-to-hearing
#13
Valéria Gomes da Silva, Carlos Augusto Costa Pires de Oliveira, Pedro Luíz Tauil, Isabella Monteiro de Castro Silva, André Luiz Lopes Sampaio
OBJECTIVE: To investigate the association between changes in the outer hair cells and exposure to amplified music in a group of high-school students. MATERIALS AND METHODS: In this retrospective, case-control study, 86 subjects underwent audiometry, immittance audiometry, and distortion-product otoacoustic emission tests. The subjects were questioned about their listening habits and divided into 2 groups: exposed and unexposed. RESULTS: Most of the subjects had reduced function in their outer hair cells, mainly beginning at 8 kHz...
February 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28105227/milia-may-originate-from-the-outermost-layers-of-the-hair-bulge-of-the-outer-root-sheath-a-case-report
#14
Ichiro Kurokawa, Ayako Kakuno, Airo Tsubura
It has been hypothesized that milia originate from the hair bulge of the outer root sheath. To elucidate the histogenesis of milia, an immunohistochemical study was performed using anti-keratin and anti-filaggrin antibodies to determine the levels of keratin and filaggrin expression. Keratin expression was evaluated using anti-keratin antibodies against K1, K7, K8, K10, K14, K15, K16, K17, K18, K19 and K20. K1 and K10 expression were detected in the suprabasal layers of the more superficial section of the cyst walls, but not in the deeper section of the cyst walls...
December 2016: Oncology Letters
https://www.readbyqxmd.com/read/28101341/influence-of-high-altitude-hypoxic-environments-on-the-survival-of-cochlear-hair-cells-and-spiral-ganglion-neurons-in-rats
#15
Dongyan Fan, Hailong Ren, Dunzhu Danzeng, Haonan Li, Ping Wang
The aim of the present study was to observe the histological changes in the peripheral auditory system in rats at different time-points after relocating from low altitude to high altitude (3,600 m). The general physical condition of the rats was observed and cochlear tissue samples were obtained every month. The morphology and survival of the cochlear hair cells (HCs) were observed using cochlear surface preparation at 1, 30, 90, 120, 150 and 180 days after moving to the plateau area. Changes in spiral ganglion neurons (SGNs) were detected at different time-points using immunofluorescence technology on frozen sections...
December 2016: Biomedical Reports
https://www.readbyqxmd.com/read/28099203/evaluation-of-gene-expression-patterns-in-micrografts-demonstrate-induction-of-catagen-like-processes-during-storage
#16
Walter Krugluger, Karl Stiefsohn, Karl Moser, Claudia Moser, Katarina Laciak
BACKGROUND: Alterations of gene expression patterns may contribute to the commonly observed transient reduction of hair shaft elongation in hair restoration surgery. OBJECTIVE: To elucidate the molecular causes, we evaluated changes in gene expression patterns in hair follicle micrografts during storage. MATERIALS AND METHODS: Micrografts with different amounts of adjacent connective tissue (regular, skinny, and chubby) were stored for different periods, and the expression of key genes was determined: dermal papilla (DP): FGF7, alkaline phosphatase (ALP), versican; outer root sheath: Krt15; inner root sheath: Krt 25; cuticula: Krt85; Henle layer: filaggrin; genes related to apoptosis and growth/differentiation: Caspase 3, Ovol1, and Foxo1...
February 2017: Dermatologic Surgery: Official Publication for American Society for Dermatologic Surgery [et Al.]
https://www.readbyqxmd.com/read/28093303/chlorogenic-acid-rescues-sensorineural-auditory-function-in-a-diabetic-animal-model
#17
Bin Na Hong, Youn Hee Nam, Sang Ho Woo, Tong Ho Kang
Recently, many studies have reported that sensorineural hearing impairment related to neurological disorders may be caused by diabetes mellitus. However, to date, only a small number of studies have investigated the treatment of sensorineural hearing impairment. In the present study, the effects of chlorogenic acid on diabetic auditory pathway impairment were evaluated by neuro-electrical physiological measurements and morphological investigations. We have shown that CA efficiently prevents the progression of auditory pathway dysfunction caused by DM using auditory brainstem responses and auditory middle latency responses in mice...
January 16, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28089576/conditional-deletion-of-pejvakin-in-adult-outer-hair-cells-causes-progressive-hearing-loss-in-mice
#18
Suzan L Harris, Marcin Kazmierczak, Tina Pangršič, Prahar Shah, Nadiya Chuchvara, Alonso Barrantes-Freer, Tobias Moser, Martin Schwander
Mutations in the Pejvakin (Pjvk) gene cause autosomal recessive hearing loss DFNB59 with audiological features of auditory neuropathy spectrum disorder (ANSD) or cochlear dysfunction. The precise mechanisms underlying the variable clinical phenotypes of DFNB59 remain unclear. Here, we demonstrate that mice with conditional ablation of the Pjvk gene in all sensory hair cells or only in outer hair cells (OHCs) show similar auditory phenotypes with early-onset profound hearing loss. By contrast, loss of Pjvk in adult OHCs causes a slowly progressive hearing loss associated with OHC degeneration and delayed loss of inner hair cells (IHCs), indicating a primary role for pejvakin in regulating OHC function and survival...
March 6, 2017: Neuroscience
https://www.readbyqxmd.com/read/28062185/allicin-protects-auditory-hair-cells-and-spiral-ganglion-neurons-from-cisplatin-induced-apoptosis
#19
Xianmin Wu, Xiaofei Li, Yongdong Song, He Li, Xiaohui Bai, Wenwen Liu, Yuechen Han, Lei Xu, Jianfeng Li, Daogong Zhang, Haibo Wang, Zhaomin Fan
Cisplatin is a broad-spectrum anticancer drug that is commonly used in the clinic. Ototoxicity is one of the major side effects of this drug, which caused irreversible sensorineural hearing loss. Allicin, the main biologically active compound derived from garlic, has been shown to exert various anti-apoptotic and anti-oxidative activities in vitro and in vivo studies. We took advantage of C57 mice intraperitoneally injected with cisplatin alone or with cisplatin and allicin combined, to investigate whether allicin plays a protective role in vivo against cisplatin ototoxicity...
January 3, 2017: Neuropharmacology
https://www.readbyqxmd.com/read/28040033/selective-attentional-processes-in-cochlear-implant-recipients-measurements-of-the-attentional-filter
#20
Christofer Bester, Dona M P Jayakody, Geoffrey Hammond, Donald Robertson
In normal hearing subjects, detection of near-threshold tones in noise is influenced by signal certainty. Thus, tones that are presented more frequently than others, and/or are preceded by a clearly audible cue tone of the same frequency (target tones) are detected better than other tones (probe tones). This auditory attentional filter was examined in six cochlear implant (CI) recipients, using acoustic stimuli and direct programmed electrode stimulation. Three of the subjects showed no evidence of an attentional filter...
December 2016: Journal of the Acoustical Society of America
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