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https://www.readbyqxmd.com/read/28742630/unique-case-of-hearing-recovery-after-otic-capsule-destruction-and-complete-sensorineural-hearing-loss-caused-by-langerhans-cell-histiocytosis
#1
Jennifer A Losie, Michael Yong, Frederick K Kozak, Neil K Chadha
: A 14-year-old woman presented with right-sided otologic and vestibular symptoms after presenting with hormonal disturbances earlier that year. Imaging showed a gross destruction of the temporal bone, mastoid air cells, and external acoustic meatus with invasion into the otic capsule. The patient experienced complete sensorineural hearing loss in the right ear. Biopsy diagnosed Langerhans cell histiocytosis (LCH) and the patient was treated with chemotherapy. After 1 year of treatment, the patient's hearing partially recovered and imaging showed reconstitution of the temporal bone including the otic capsule...
July 24, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28733657/a-gene-network-regulated-by-fgf-signalling-during-ear-development
#2
Maryam Anwar, Monica Tambalo, Ramya Ranganathan, Timothy Grocott, Andrea Streit
During development cell commitment is regulated by inductive signals that are tightly controlled in time and space. In response, cells activate specific programmes, but the transcriptional circuits that maintain cell identity in a changing signalling environment are often poorly understood. Specification of inner ear progenitors is initiated by FGF signalling. Here, we establish the genetic hierarchy downstream of FGF by systematic analysis of many ear factors combined with a network inference approach. We show that FGF rapidly activates a small circuit of transcription factors forming positive feedback loops to stabilise otic progenitor identity...
July 21, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28688556/pediatric-langerhans-cell-histiocytosis-of-the-lateral-skull-base
#3
Ananya Majumder, Cameron C Wick, Rebecca Collins, Timothy N Booth, Brandon Isaacson, J Walter Kutz
OBJECTIVE: Describe the presentation, imaging characteristics, management, and outcomes of pediatric patients with Langerhans cell histiocytosis (LCH) of the temporal bone. METHODS: A retrospective chart review was performed between 2000 and 2014 at a single tertiary care children's hospital. Fourteen patients were identified with a diagnosis of LCH and involvement of the temporal bone. RESULTS: Ten patients were female and ten were Caucasian...
August 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28686713/transcriptome-wide-comparison-of-the-impact-of-atoh1-and-mir-183-family-on-pluripotent-stem-cells-and-multipotent-otic-progenitor-cells
#4
Michael Ebeid, Prashanth Sripal, Jason Pecka, Kirk W Beisel, Kelvin Kwan, Garrett A Soukup
Over 5% of the global population suffers from disabling hearing loss caused by multiple factors including aging, noise exposure, genetic predisposition, or use of ototoxic drugs. Sensorineural hearing loss is often caused by the loss of sensory hair cells (HCs) of the inner ear. A barrier to hearing restoration after HC loss is the limited ability of mammalian auditory HCs to spontaneously regenerate. Understanding the molecular mechanisms orchestrating HC development is expected to facilitate cell replacement therapies...
2017: PloS One
https://www.readbyqxmd.com/read/28662075/microarray-analyses-of-otospheres-derived-from-the-cochlea-in-the-inner-ear-identify-putative-transcription-factors-that-regulate-the-characteristics-of-otospheres
#5
Takehiro Iki, Michihiro Tanaka, Shin-Ichiro Kitajiri, Tomoko Kita, Yuri Kawasaki, Akifumi Mizukoshi, Wataru Fujibuchi, Takayuki Nakagawa, Tatsutoshi Nakahata, Juichi Ito, Koichi Omori, Megumu K Saito
Various tissues possess tissue-specific stem/progenitor cells, including the inner ears. Stem/progenitor cells of the inner ear can be isolated as so-called otospheres from differentiated cells using a sphere forming assay. Although recent studies have demonstrated the characteristics of otospheres to some extent, most of the features of these cells are unknown. In this report, we describe the findings of transcriptome analyses with a cDNA microarray of otospheres derived from the cochleae of the inner ears of neonatal mice in order to clarify the gene expression profile of otic stem/progenitor cells...
2017: PloS One
https://www.readbyqxmd.com/read/28642583/sox2-is-required-for-inner-ear-neurogenesis
#6
Aleta R Steevens, Danielle L Sookiasian, Jenna C Glatzer, Amy E Kiernan
Neurons of the cochleovestibular ganglion (CVG) transmit hearing and balance information to the brain. During development, a select population of early otic progenitors express NEUROG1, delaminate from the otocyst, and coalesce to form the neurons that innervate all inner ear sensory regions. At present, the selection process that determines which otic progenitors activate NEUROG1 and adopt a neuroblast fate is incompletely understood. The transcription factor SOX2 has been implicated in otic neurogenesis, but its requirement in the specification of the CVG neurons has not been established...
June 22, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28641322/scuba-diving-and-otology-a-systematic-review-with-recommendations-on-diagnosis-treatment-and-post-operative-care
#7
REVIEW
Devon M Livingstone, Kristine A Smith, Beth Lange
Scuba diving is a popular recreational and professional activity with inherent risks. Complications related to barotrauma and decompression illness can pose significant morbidity to a diver's hearing and balance systems. The majority of dive-related injuries affect the head and neck, particularly the outer, middle and inner ear. Given the high incidence of otologic complications from diving, an evidence-based approach to the diagnosis and treatment of otic pathology is a necessity. We performed a systematic and comprehensive literature review including the pathophysiology, diagnosis, and treatment of otologic pathology related to diving...
June 2017: Diving and Hyperbaric Medicine: the Journal of the South Pacific Underwater Medicine Society
https://www.readbyqxmd.com/read/28628917/anatomical-characteristics-of-facial-nerve-and-cochlea-interaction
#8
Nadine Schart-Morén, Sune Larsson, Helge Rask-Andersen, Hao Li
OBJECTIVE: The aim was to study the relationship between the labyrinthine portion (LP) of the facial canal and the cochlea in human inner ear molds and temporal bones using micro-CT and 3D rendering. A reduced cochlea-facial distance may spread electric currents from the cochlear implant to the LP and cause facial nerve stimulation. Influencing factors may be the topographic anatomy and otic capsule properties. METHODS: An archival collection of human temporal bones underwent micro-CT and 3D reconstruction...
June 20, 2017: Audiology & Neuro-otology
https://www.readbyqxmd.com/read/28609892/reappraisal-of-i-synagrops-i-g%C3%A3-nther-1887-with-rehabilitation-and-revision-of-i-parascombrops-i-alcock-1889-including-description-of-seven-new-species-and-two-new-genera-perciformes-acropomatidae
#9
Werner W Schwarzhans, Artem M Prokofiev
An ongoing review of the fishes of the basal percoid family Acropomatidae has revealed that the genus Synagrops Günther, 1887 as it is currently understood is not a natural group. Species with a serrated pelvic-fin spine are here placed in the resurrected genus Parascombrops Alcock, 1889 (type-species: Parascombrops pellucidus Alcock, 1889), and the new, monospecific genus Caraibops n. gen. (type-species: Synagrops trispinosus Mochizuki & Sano, 1984). Parascombrops is unique amongst Acropomatidae in the combination of the presence of vacant 8th interneural space, a predorsal formula /0+0/0+2/ and an epaxialis attachment type 1...
May 4, 2017: Zootaxa
https://www.readbyqxmd.com/read/28603711/autophagy-in-the-vertebrate-inner-ear
#10
REVIEW
Marta Magariños, Sara Pulido, María R Aburto, Rocío de Iriarte Rodríguez, Isabel Varela-Nieto
Autophagy is a conserved catabolic process that results in the lysosomal degradation of cell components. During development, autophagy is associated with tissue and organ remodeling, and under physiological conditions it is tightly regulated as it plays a housekeeping role in removing misfolded proteins and damaged organelles. The vertebrate inner ear is a complex sensory organ responsible for the perception of sound and for balance. Cell survival, death and proliferation, as well as cell fate specification and differentiation, are processes that are strictly coordinated during the development of the inner ear in order to generate the more than a dozen specialized cell types that constitute this structure...
2017: Frontiers in Cell and Developmental Biology
https://www.readbyqxmd.com/read/28583486/ototoxicity-associated-with-topical-administration-of-diclofenac-sodium-as-an-otic-drop-an-experimental-animal-study
#11
Engin Acioglu, Ozgur Yigit, Firat Onur, Ahmet Atas, Ela Araz Server, Eyup Kara
OBJECTIVES: The aim of the study is to evaluate the ototoxicity of topical diclofenac sodium in comparison to positive and negative controls prior to the investigation of analgesic and anti-inflammatory efficacy of the agent in otic administration. METHODS: Twenty four ears of 12 guinea pigs were included in the study. Wide myringotomy was performed on all tympanic membranes under general anesthesia and auditory brainstem responses (ABR) were evaluated. The subjects were separated into four groups, two groups received diclofenac sodium at low and high doses, positive controls received gentamicin and negative controls received isotonic sodium chloride topically for 14 days and ABRs were reevaluated...
July 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28573817/imaging-of-labyrinthine-fistula-after-repair-with-bone-pate
#12
Jeffrey T Vrabec
OBJECTIVES/HYPOTHESIS: To demonstrate imaging of labyrinthine fistula due to cholesteatoma and illustrate outcome following repair with bone pate. STUDY DESIGN: Retrospective review. METHODS: Patients with labyrinthine fistula due to cholesteatoma were identified, and pre- and postoperative imaging was assessed. Method of repair was recorded. The fistula site was examined for patency and bone density after repair. RESULTS: Twenty-nine fistulae were seen in a cohort of 375 cholesteatoma cases (8%)...
June 2, 2017: Laryngoscope
https://www.readbyqxmd.com/read/28549773/cochlear-implantation-in-patients-with-otosclerosis-of-the-otic-capsule
#13
Jay Burmeister, Susan Rathgeb, Jacques Herzog
OBJECTIVE: To evaluate outcomes of cochlear implantation of patients with otosclerosis of the otic capsule. STUDY DESIGN: A retrospective case series of 6 patients (7 ears). PATIENTS: 6 patients (7 ears), 5 patients with severe to profound sensorineural hearing loss; 1 patient with mild to profound sensorineural hearing loss, with radiologic evidence of otosclerosis. All patients were adult males, with or without history of stapes surgery...
May 19, 2017: American Journal of Otolaryngology
https://www.readbyqxmd.com/read/28537554/pioneer-neurog1-expressing-cells-ingress-into-the-otic-epithelium-and-instruct-neuronal-specification
#14
Esteban Hoijman, L Fargas, Patrick Blader, Berta Alsina
Neural patterning involves regionalised cell specification. Recent studies indicate that cell dynamics play instrumental roles in neural pattern refinement and progression, but the impact of cell behaviour and morphogenesis on neural specification is not understood. Here we combine 4D analysis of cell behaviours with dynamic quantification of proneural expression to uncover the construction of the zebrafish otic neurogenic domain. We identify pioneer cells expressing neurog1 outside the otic epithelium that migrate and ingress into the epithelialising placode to become the first otic neuronal progenitors...
May 24, 2017: ELife
https://www.readbyqxmd.com/read/28535123/topical-otic-drugs-in-a-multi-purpose-manufacturing-facility-a-guide-on-determination-and-application-of-permitted-daily-exposure-pde
#15
Lisa Wiesner, Maarten Prause, Ester Lovsin Barle
Due to newly introduced EU GMP (Good Manufacturing Practice) guideline for Medicinal Products for Human and Veterinary use, product specific permitted daily exposure (PDE) for toxicological evaluation in multi-purpose facilities are required within a documented process for risk assessment. European Medicines Agency (EMA) guidance on setting PDE limits so far focused on systemic administration routes such as intravenous (IV), oral or inhalation. This article provides guidance on setting PDE values for risk management purposes in multi-purpose facilities for active pharmaceutical ingredients (APIs) applied as topical otic drugs to the outer ear canal...
June 7, 2017: Pharmaceutical Development and Technology
https://www.readbyqxmd.com/read/28535077/sticky-situations-cyanoacrylate-exposures-reported-to-a-poison-control-system
#16
Shaun D Carstairs, Cynthia Koh, Lily Qian, Mariam Qozi, Grant Seivard, F Lee Cantrell
INTRODUCTION: Cyanoacrylate (Super Glue(®)) exposures are commonly reported to poison control centers, but little has been published in the medical literature regarding these exposures. We sought to characterize cyanoacrylate exposures reported to a poison control system. METHODS: We performed a retrospective review of a poison system's database for all cases of single-substance human exposure to cyanoacrylate-containing products from 2005 to 2015. Data collected included age, gender, route of exposure, clinical effects, treatments recommended and medical outcome...
May 23, 2017: Clinical Toxicology
https://www.readbyqxmd.com/read/28502255/a-moist-edge-environment-aids-the-regeneration-of-traumatic-tympanic-membrane-perforations
#17
Z C Lou, Z H Lou
OBJECTIVE: To review the history of moist therapy used to regenerate traumatic tympanic membrane perforations. STUDY DESIGN: Literature review. METHODS: The literature on topical agents used to treat traumatic tympanic membrane perforations was reviewed, and the advantages and disadvantages of moist therapy were analysed. RESULTS: A total of 76 studies were included in the analysis. Topical applications of certain agents (e...
July 2017: Journal of Laryngology and Otology
https://www.readbyqxmd.com/read/28498272/paget-s-disease-of-the-temporal-bone-a-single-institution-contemporary-review-of-27-patients
#18
Nicholas L Deep, Jake G Besch-Stokes, John I Lane, Colin L W Driscoll, Matthew L Carlson
OBJECTIVES: To report a contemporary review from a single-institution series on Paget's disease of the temporal bone (PDTB). STUDY DESIGN: Retrospective chart review of patients evaluated from 1998 to 2016. SETTING: Quaternary referral center. PATIENTS: Patients with radiographically confirmed PDTB. MAIN OUTCOME MEASURES: Clinical, audiological, and radiological features and management strategies of PDTB...
July 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28492243/identification-of-mouse-cochlear-progenitors-that-develop-hair-and-supporting-cells-in-the-organ-of-corti
#19
Jinshu Xu, Hiroo Ueno, Chelsea Y Xu, Binglai Chen, Irving L Weissman, Pin-Xian Xu
The adult mammalian cochlear sensory epithelium houses two major types of cells, mechanosensory hair cells and underlying supporting cells, and lacks regenerative capacity. Recent evidence indicates that a subset of supporting cells can spontaneously regenerate hair cells after ablation only within the first week postparturition. Here in vivo clonal analysis of mouse inner ear cells during development demonstrates clonal relationship between hair and supporting cells in sensory organs. We report the identification in mouse of a previously unknown population of multipotent stem/progenitor cells that are capable of not only contributing to the hair and supporting cells but also to other cell types, including glia, in cochlea undergoing development, maturation and repair in response to damage...
May 11, 2017: Nature Communications
https://www.readbyqxmd.com/read/28459451/generation-of-inner-ear-organoids-containing-functional-hair-cells-from-human-pluripotent-stem-cells
#20
Karl R Koehler, Jing Nie, Emma Longworth-Mills, Xiao-Ping Liu, Jiyoon Lee, Jeffrey R Holt, Eri Hashino
The derivation of human inner ear tissue from pluripotent stem cells would enable in vitro screening of drug candidates for the treatment of hearing and balance dysfunction and may provide a source of cells for cell-based therapies of the inner ear. Here we report a method for differentiating human pluripotent stem cells to inner ear organoids that harbor functional hair cells. Using a three-dimensional culture system, we modulate TGF, BMP, FGF, and WNT signaling to generate multiple otic-vesicle-like structures from a single stem-cell aggregate...
June 2017: Nature Biotechnology
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