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Inner ear

E Verheij, L Elden, T B Crowley, F A Pameijer, E H Zackai, D M McDonald-McGinn, H G X M Thomeer
BACKGROUND AND PURPOSE: The 22q11.2 deletion syndrome is characterized by a heterogenic phenotype, including hearing loss. The underlying cause of hearing loss, especially sensorineural hearing loss, is not yet clear. Therefore, our objective was to describe anatomic malformations in the middle and inner ear in patients with 22q11.2 deletion syndrome. MATERIALS AND METHODS: A retrospective case series was conducted in 2 tertiary referral centers. All patients with 22q11...
March 15, 2018: AJNR. American Journal of Neuroradiology
Ricardo Araújo, Vincent Fernandez, Richard D Rabbitt, Eric G Ekdale, Miguel T Antunes, Rui Castanhinha, Jörg Fröbisch, Rui M S Martins
The semicircular canal (SC) system of the inner ear detects head angular accelerations and is essential for navigation and spatial awareness in vertebrates. Because the bony labyrinth encloses the membranous labyrinth SCs, it can be used as a proxy for animal behavior. The bony labyrinth of dicynodonts, a clade of herbivorous non-mammalian synapsids, has only been described in a handful of individuals and remains particularly obscure. Here we describe the bony labyrinth anatomy of three Endothiodon cf. bathystoma specimens from Mozambique based on digital reconstructions from propagation phase-contrast synchrotron micro-computed tomography...
2018: PloS One
Akiko Iizuka-Kogo
The organ of Corti, an acoustic sensory organ, is a specifically differentiated epithelium of the cochlear duct, which is a part of the membranous labyrinth in the inner ear. Cells in the organ of Corti are generally classified into two kinds; hair cells, which transduce the mechanical stimuli of sound to the cell membrane electrical potential differences, and supporting cells. These cells emerge from homogeneous prosensory epithelium through cell fate determination and differentiation. In the organ of Corti organogenesis, cell differentiation and the rearrangement of their position proceed in parallel, resulting in a characteristic alignment of mature hair cells and supporting cells...
March 13, 2018: Medical Molecular Morphology
Lejo Johnson Chacko, Dominik T Schmidbauer, Stephan Handschuh, Alen Reka, Karl D Fritscher, Patrik Raudaschl, Rami Saba, Michael Handler, Peter P Schier, Daniel Baumgarten, Natalie Fischer, Elisabeth J Pechriggl, Erich Brenner, Romed Hoermann, Rudolf Glueckert, Anneliese Schrott-Fischer
Stable posture and body movement in humans is dictated by the precise functioning of the ampulla organs in the semi-circular canals. Statistical analysis of the interrelationship between bony and membranous compartments within the semi-circular canals is dependent on the visualization of soft tissue structures. Thirty-one human inner ears were prepared, post-fixed with osmium tetroxide and decalcified for soft tissue contrast enhancement. High resolution X-ray microtomography images at 15 μm voxel-size were manually segmented...
2018: Frontiers in Neuroscience
Michelle W Antoine, Xiaoxia Zhu, Marianne Dieterich, Thomas Brandt, Sarath Vijayakumar, Nicholas McKeehan, Joseph C Arezzo, R Suzanne Zukin, David A Borkholder, Sherri M Jones, Robert D Frisina, Jean M Hébert
How asymmetries in motor behavior become established normally or atypically in mammals remains unclear. An established model for motor asymmetry that is conserved across mammals can be obtained by experimentally inducing asymmetric striatal dopamine activity. However, the factors that can cause motor asymmetries in the absence of experimental manipulations to the brain remain unknown. Here, we show that mice with inner ear dysfunction display a robust left or right rotational preference, and this motor preference reflects an atypical asymmetry in cortico-striatal neurotransmission...
March 2018: PLoS Biology
Pedrom C Sioshansi, Robert K Jackler, Jennifer C Alyono
OBJECTIVE: To describe the practice of otology in America during the first quarter century of the American Otological Society (AOS). METHODS: Two sources were used to determine the most prevalent disease conditions cared for and surgical procedures undertaken during this era. All articles published in the AOS transactions between 1868 and 1893 were studied as were the otology textbooks published by 6 of the first 10 Presidents of the Society. RESULTS: The primary emphasis of late 19th century American otological scholarship was on chronic ear infection with numerous articles focusing on complications of otitis including frequent descriptions of fatalities...
April 2018: Otology & Neurotology
Hannes Petersen, Alexandra Gliese, Yannick Stober, Stephanie Maier, Nils-Owe Hansen, Sebastian Kruber, Dennis Eggert, Miklós Tóth, Tobias Gosau, Hartmut Schlüter, Klaus Püschel, Udo Schumacher, Robert John Dwayne Miller, Adrian Münscher, Carsten Dalchow
OBJECTIVE: Using a contact-free laser technique for stapedotomy reduces the risk of mechanical damage of the stapes footplate. However, the risk of inner ear dysfunction due to thermal, acoustic, or direct damage has still not been solved. The objective of this study was to describe the first experiences in footplate perforation in cadaver tissue performed by the novel Picosecond-Infrared-Laser (PIRL), allowing a tissue preserving ablation. PATIENTS AND INTERVENTION: Three human cadaver stapes were perforated using a fiber-coupled PIRL...
April 2018: Otology & Neurotology
José Ferreira Penêda, Nuno Barros Lima, Francisco Monteiro, Joana Vilela Silva, Rita Gama, Artur Condé
INTRODUCTION: Immune Mediated Inner Ear Disease (IMIED) is a rare form of sensorineural bilateral hearing loss, usually progressing in weeks to months and responsive to immunosuppressive treatment. Despite recent advances, there is no consensus on diagnosis and optimal treatment. METHODS: A review of articles on IMIED from the last 10 years was conducted using PubMed® database. RESULTS: IMIED is a rare disease, mostly affecting middle aged women...
March 7, 2018: Acta Otorrinolaringológica Española
Rebecca Susan Dewey, Deborah A Hall, Hannah Guest, Garreth Prendergast, Christopher J Plack, Susan T Francis
BACKGROUND: Rodent studies indicate that noise exposure can cause permanent damage to synapses between inner hair cells and high-threshold auditory nerve fibers, without permanently altering threshold sensitivity. These demonstrations of what is commonly known as hidden hearing loss have been confirmed in several rodent species, but the implications for human hearing are unclear. OBJECTIVE: Our Medical Research Council-funded program aims to address this unanswered question, by investigating functional consequences of the damage to the human peripheral and central auditory nervous system that results from cumulative lifetime noise exposure...
March 9, 2018: JMIR Research Protocols
Hiromasa Ninomiya, Nobutaka Ohgami, Reina Oshino, Masashi Kato, Kyoko Ohgami, Xiang Li, Dandan Shen, Machiko Iida, Ichiro Yajima, Charalampos E Angelidis, Hiroaki Adachi, Masahisa Katsuno, Gen Sobue, Masashi Kato
Previous studies showed that people in urban areas are possibly exposed to 60-110 dB of low frequency noise (LFN) defined as noise of ≤100 Hz in their daily life. Previous studies also showed increased health risks by exposure to high levels (130-140 dB) of LFN in animals. However, little is known about the health effects of exposure to an ordinary level of LFN. We biochemically and immunohistochemically assessed the effects of exposure to inaudible LFN for mice (12 h/day of 100 Hz LFN at 95 dB for 5 days), at a level to which people are possibly exposed in daily life, on a murine inner ear by targeting 9 stress-reactive molecules...
February 24, 2018: Hearing Research
Clive P Morgan, Hongyu Zhao, Meredith LeMasurier, Wei Xiong, Bifeng Pan, Piotr Kazmierczak, Matthew R Avenarius, Michael Bateschell, Ruby Larisch, Anthony J Ricci, Ulrich Müller, Peter G Barr-Gillespie
Hair cells of the inner ear transduce mechanical stimuli like sound or head movements into electrical signals, which are propagated to the central nervous system. The hair-cell mechanotransduction channel remains unidentified. We tested whether three transient receptor channel (TRP) family members, TRPV6, TRPM6 and TRPM7, were necessary for transduction. TRPV6 interacted with USH1C (harmonin), a scaffolding protein that participates in transduction. Using a cysteine-substitution knock-in mouse line and methanethiosulfonate (MTS) reagents selective for this allele, we found that inhibition of TRPV6 had no effect on transduction in mouse cochlear hair cells...
2018: Frontiers in Cellular Neuroscience
Wen-Wei Luo, Xin-Wei Wang, Rui Ma, Fang-Lu Chi, Ping Chen, Ning Cong, Yu-Yan Gu, Dong-Dong Ren, Juan-Mei Yang
Notch inhibition is known to generate supernumerary hair cells (HCs) at the expense of supporting cells (SCs) in the mammalian inner ear. However, inhibition of Notch activity becomes progressively less effective at inducing SC-to-HC conversion in the postnatal cochlea and balance organs as the animal ages. It has been suggested that the SC-to-HC conversion capacity is inversely correlated with E-cadherin accumulation in postnatal mammalian utricles. However, whether E-cadherin localization is linked to the SC-to-HC conversion capacity in the mammalian inner ear is poorly understood...
2018: Frontiers in Molecular Neuroscience
Jerry D Monroe, Heidi L Hruska, Hannah K Ruggles, Kevin M Williams, Michael E Smith
Unlike cisplatin, which forms bifunctional DNA adducts, monofunctional platinum(II) complexes bind only one strand of DNA and might target cancer without causing auditory side-effects associated with cisplatin treatment. We synthesized the monofunctional triamine-ligated platinum(II) complexes, Pt(diethylenetriamine)Cl, [Pt(dien)Cl]+, and Pt(N,N-diethyldiethylenetriamine)Cl, [Pt(Et2dien)Cl]+, and the monofunctional heterocyclic-ligated platinum(II) complexes, pyriplatin and phenanthriplatin, and compared their 5'-GMP binding rates, cellular compartmental distribution and cellular viability effects...
2018: PloS One
Joshua A Tillinger, Chhavi Gupta, Kadri Ila, Jamal Ahmed, Jeenu Mittal, Thomas R Van De Water, Adrien A Eshraghi
OBJECTIVE: The present study is aimed at determining the efficacy and exploring the mechanisms by which l-N-acetylcysteine (l-NAC) provides protection against tumor necrosis factor-alpha (TNFα)-induced oxidative stress damage and hair cell loss in 3-day-old rat organ of Corti (OC) explants. Previous work has demonstrated a high level of oxidative stress in TNFα-challenged OC explants. TNFα can potentially play a significant role in hair cell loss following an insult to the inner ear...
March 7, 2018: Acta Oto-laryngologica
Wei Zhuang, Caiji Wang, Xi Shi, Shiwei Qiu, Shili Zhang, Bing Xu, Min Chen, Wen Jiang, Hongyan Dong, Yuehua Qiao
Congenital cytomegalovirus (CMV) infection is the most common infectious cause of sensorineural hearing loss in children. While the importance of CMV‑induced SNHL has been described, the mechanisms underlying its pathogenesis and the role of inflammatory responses remain elusive. The present study established an experimental model of hearing loss after systemic infection with murine CMV (MCMV) in newborn mice. Auditory brainstem responses were tested to evaluate hearing at 3 weeks, expression of inflammasome‑-associated factors was assessed by immunofluorescence, western blot analysis, reverse transcription‑quantitative polymerase chain reaction and ELISA...
March 6, 2018: International Journal of Molecular Medicine
Annika Jessen, Andrew D Ho, C Eduardo Corrales, Bevan Yueh, Jennifer J Shin
Objectives (1) To assess the 11-item Inner Effectiveness of Auditory Rehabilitation (Inner EAR) instrument with item response theory (IRT). (2) To determine whether the underlying latent ability could also be accurately represented by a subset of the items for use in high-volume clinical scenarios. (3) To determine whether the Inner EAR instrument correlates with pure tone thresholds and word recognition scores. Design IRT evaluation of prospective cohort data. Setting Tertiary care academic ambulatory otolaryngology clinic...
February 1, 2018: Otolaryngology—Head and Neck Surgery
Michelle L Stoller, Orvelin Roman, Michael R Deans
The organization of polarized stereociliary bundles is critical for the function of the inner ear sensory receptor hair cells that detect sound and motion, and these cells present a striking example of Planar Cell Polarity (PCP); the coordinated orientation of polarized structures within the plane of an epithelium. PCP is best understood in Drosophila where the essential genes regulating PCP were first discovered, and functions for the core PCP proteins encoded by these genes have been deciphered through phenotypic analysis of core PCP gene mutants...
March 3, 2018: Developmental Biology
Mei Xu, Xiaoling Xie, Xuhui Dong, Guoqing Liang, Lin Gan
LHX3, a LIM-homeodomain transcription factor, is broadly expressed in the developing pituitary, spinal cord, medulla, retina and inner ear, and plays essential roles during embryonic development. Mice with homozygous Lhx3 null mutation exhibit failure in the formation of pituitary gland and die perinatally. To facilitate the functional study of Lhx3 in mice, we engineered and characterized two novel Lhx3 mouse strains: Lhx3GFP reporter knock-in and Lhx3loxP conditional knockout mice. Co-immunolabeling of LHX3 and GFP shows that the expression pattern of the knock-in GFP reporter recapitulates that of endogenous LHX3 in cochlea, vestibule, retina, and spinal cord...
March 6, 2018: Genesis: the Journal of Genetics and Development
Arya Sobhakumari, Robert H Poppenga, J Brad Pesavento, Francisco A Uzal
BACKGROUND: Carbon monoxide (CO), a common cause of poisoning in human beings has also been implicated in the death of animals. Though there are multiple studies on CO poisoning and relevant lethal blood COHb concentrations in humans, there are no reliable reports of diagnostic lethal carboxyhemoglobin percentage of saturation (COHb%) in cats. Additionally, due to shared housing environments, exposures to companion animals can be a surrogate for lethal exposures in human beings and provide valuable information in concurrent forensic investigations...
March 5, 2018: BMC Veterinary Research
Elisabeth Mamelle, Naila El Kechai, Victor Adenis, Yann Nguyen, Olivier Sterkers, Florence Agnely, Amélie Bochot, Jean Marc Edeline, Evelyne Ferrary
OBJECTIVES: Intratympanic injection of corticosteroids membrane after noise-induced hearing loss is an accepted alternative to general administration. We investigated the effect on hearing of a hyaluronic acid gel with liposomes loaded with dexamethasone (DexP) administered into the middle ear. METHODS: An acute acoustic trauma was performed to 13 guinea pigs for a period of 1 h on Day -2. Two 2 days after the noise trauma, the animals were then assigned randomly to four experimental groups: control without gel, gel injection, gel-containing free DexP, gel-containing DexP loaded into liposomes...
March 5, 2018: Acta Oto-laryngologica
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