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https://www.readbyqxmd.com/read/29161295/cardiac-and-renal-dysfunction-is-associated-with-progressive-hearing-loss-in-patients-with-fabry-disease
#1
Maria Köping, Wafaa Shehata-Dieler, Mario Cebulla, Kristen Rak, Daniel Oder, Jonas Müntze, Peter Nordbeck, Christoph Wanner, Rudolf Hagen, Sebastian Schraven
BACKGROUND: Fabry disease (FD) is an X-linked recessive hereditary lysosomal storage disorder which results in the accumulation of globotriaosylceramid (Gb3) in tissues of kidney and heart as well as central and peripheral nervous system. Besides prominent renal and cardiac organ involvement, cochlear symptoms like high-frequency hearing loss and tinnitus are frequently found with yet no comprehensive data available in the literature. OBJECTIVE: To examine hearing loss in patients with FD depending on cardiac and renal function...
2017: PloS One
https://www.readbyqxmd.com/read/29161200/early-placement-of-ventilation-tubes-in-infants-with-cleft-lip-and-palate-a-systematic-review
#2
Mark Felton, Jong Wook Lee, Darius D Balumuka, Jugpal S Arneja, Neil K Chadha
Objective Studies have shown that the majority of cleft lip and palate (CLP) children have middle ear fluid present at the time of lip repair (3-4 months). Despite hearing loss, the majority of children do not undergo ventilation tube treatment if required until the time of palate repair (9-12 months). We aimed to examine the effectiveness and potential complications of early ventilation tube placement prior to palatoplasty in infants with cleft lip and palate. Data Sources Medline (1946-2015), Embase (1980-2015), and EBM Reviews (Cochrane Central Register of Controlled)...
November 1, 2017: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/29160511/the-importance-of-audiometric-monitoring-in-patients-with-multidrug-resistant-tuberculosis
#3
Karla Anacleto de Vasconcelos, Silvana Maria Monte Coelho Frota, Antonio Ruffino-Netto, Afrânio Lineu Kritski
INTRODUCTION: A total of 771 cases of multidrug-resistant tuberculosis (MDR-TB) were reported in Brazil in 2014. Treatment of MDR-TB with aminoglycosides can produce serious side effects such as permanent and irreversible hearing loss, which occurs in 5-64% of cases, and severely compromise patient quality of life. The goal of this research was to evaluate auditory and vestibular side effects in patients treated for MDR-TB and to identify associations between these complaints and the type of aminoglycoside used...
September 2017: Revista da Sociedade Brasileira de Medicina Tropical
https://www.readbyqxmd.com/read/29160441/psychosocial-hearing-disadvantage-and-associated-factors-among-construction-workers-in-mato-grosso-brazil
#4
Andréia Cristina Munzlinger Dos Santos, Ageo Mário Cândido da Silva, Gabriela Coelho Pereira de Luccia, Clóvis Botelho, Delma Regina Della Riva
OBJECTIVE: To estimate the prevalence and to analyze the factors associated with self-reported hearing handicap by construction workers in the state of Mato Grosso, Brazil. METHODS: A cross-sectional study was conducted with 866 construction and heavy construction workers. Participants responded to an epidemiological survey subdivided in: identification data, sociodemographic data; lifestyle; work environment characteristics; occupational exposure factors; hearing protection measures; and hearing handicap inventory to quantify the psychosocial consequences of work-related hearing loss...
July 2017: Revista Brasileira de Epidemiologia, Brazilian Journal of Epidemiology
https://www.readbyqxmd.com/read/29160150/electrocardiogram-screening-in-children-with-congenital-sensorineural-hearing-loss-prevalence-and-follow-up-of-abnormalities
#5
Zainab Farzal, Jonathan Walsh, Faisal I Ahmad, Jason Roberts, Sunita J Ferns, And Carlton J Zdanski
Objective The purpose is to determine the prevalence of electrocardiogram (ECG) abnormalities, including borderline and prolonged QT, among screened children with sensorineural hearing loss (SNHL) and to analyze their subsequent medical workup. Study Design Institutional Review Board-approved case series with chart review. Setting Tertiary academic center. Subjects and Methods Cases from 1996 to 2014 involving pediatric patients (N = 1994) with SNHL were analyzed. Abnormal ECGs were categorized as borderline/prolonged QT or other...
November 1, 2017: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/29160113/superficial-siderosis-of-central-nervous-system-with-unknown-cause-report-of-2-cases-and-review-of-the-literature
#6
Hao Chen, Hafiz Khuram Raza, Jia Jing, Xinchun Ye, Zuohui Zhang, Fang Hua, Guiyun Cui
OBJECTIVE: To report 2 cases of superficial siderosis of central nervous system (SS-CNS) and a review of the literature. METHODS: We have analyzed the clinical data and relevant features of two patients with SS-CNS who were presented with ataxia and slurred speech. Both patients undertook blood tests, lumbar puncture, head CT (computer tomography) scans, and brain and spinal cord magnetic resonance (MR) scans. In addition, the first patient also undewent enhanced susceptibility-weighted angiography (ESWAN) and the second patient undertook susceptibility weighted imaging (SWI) scan...
November 21, 2017: British Journal of Neurosurgery
https://www.readbyqxmd.com/read/29159795/impact-of-chemotherapy-induced-neurotoxicities-on-adult-cancer-survivors-symptom-burden-and-quality-of-life
#7
Christine Miaskowski, Judy Mastick, Steven M Paul, Gary Abrams, Steven Cheung, Jennifer Henderson Sabes, Kord M Kober, Mark Schumacher, Yvette P Conley, Kimberly Topp, Betty Smoot, Grace Mausisa, Melissa Mazor, Margaret Wallhagen, Jon D Levine
PURPOSE: Limited information is available on the impact of chemotherapy (CTX)-induced neurotoxicity on adult survivors' symptom experience and quality of life (QOL). Purposes were to describe occurrence of hearing loss and tinnitus and evaluate for differences in phenotypic characteristics and measures of sensation, balance, perceived stress, symptom burden, and QOL between survivors who received neurotoxic CTX and did (i.e., neurotoxicity group) and did not (i.e., no neurotoxicity group) develop neurotoxicity...
November 20, 2017: Journal of Cancer Survivorship: Research and Practice
https://www.readbyqxmd.com/read/29159459/epg5-related-vici-syndrome-a-primary-defect-of-autophagic-regulation-with-an-emerging-phenotype-overlapping-with-mitochondrial-disorders
#8
Shanti Balasubramaniam, Lisa G Riley, Anand Vasudevan, Mark J Cowley, Velimir Gayevskiy, Carolyn M Sue, Caitlin Edwards, Edward Edkins, Reimar Junckerstorff, C Kiraly-Borri, P Rowe, J Christodoulou
Vici syndrome is a rare, under-recognised, relentlessly progressive congenital multisystem disorder characterised by five principal features of callosal agenesis, cataracts, cardiomyopathy, combined immunodeficiency and oculocutaneous hypopigmentation. In addition, three equally consistent features (profound developmental delay, progressive failure to thrive and acquired microcephaly) are highly supportive of the diagnosis. Since its recognition as a distinct entity in 1988, an extended phenotype with sensorineural hearing loss, skeletal myopathy and variable involvement of virtually any organ system, including the lungs, thyroid, liver and kidneys, have been described...
November 21, 2017: JIMD Reports
https://www.readbyqxmd.com/read/29158884/a-mitochondrial-disorder-in-a-middle-age-iranian-patient-report-of-a-rare-case
#9
Mostafa Almasi, Mohammad Reza Motamed, Masoud Mehrpour, Bahram Haghi-Ashtiani, Fahimeh Haji Akhondi, Yalda Nilipour, Seyed-Mohammad Fereshtehnejad
Introduction: Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) can involve multiple systems and cause stroke-like episodes and status epilepticus. Case Presentation: A 48-year-old female with history of early fatigability, migraine-type headaches, and bilateral sensory-neural hearing loss presented 3 episodes of serial seizures. On admission she was affected by Wernicke aphasia and, then, right hemiparesis. Investigations showed elevated arterial lactate and ragged red fibers on muscle biopsy...
July 2017: Basic and Clinical Neuroscience
https://www.readbyqxmd.com/read/29158869/otitis-media-with-effusion-and-atopy-is-there-a-causal-relationship
#10
REVIEW
Mario E Zernotti, Ruby Pawankar, Ignacio Ansotegui, Hector Badellino, Juan Sebastian Croce, Elham Hossny, Motohiro Ebisawa, Nelson Rosario, Mario Sanchez Borges, Yuan Zhang, Luo Zhang
Otitis Media with Effusion (OME) is an inflammatory condition of the middle ear cleft, acute or chronic, with collection of fluid in the middle ear with an intact tympanic membrane. It is a very common disease in childhood, the most frequent cause of hearing loss in childhood and often requiring surgery. OME is called chronic when the fluid in the middle ear persists for more than three months or when the episodes recur six or more times in one year. The current article covers various aspects of OME including definition, epidemiology...
2017: World Allergy Organization Journal
https://www.readbyqxmd.com/read/29158168/identification-and-functional-analysis-of-a-novel-mutation-in-the-pax3-gene-associated-with-waardenburg-syndrome-type-i
#11
Zhijie Niu, Jiada Li, Fen Tang, Jie Sun, Xueping Wang, Lu Jiang, Lingyun Mei, Hongsheng Chen, Yalan Liu, Xinzhang Cai, Yong Feng, Chufeng He
Waardenburg syndrome type 1 (WS1) is a rare autosomal dominant genetic disorder of neural crest cells (NCC) characterized by congenital sensorineural hearing loss, dystopia canthorum, and abnormal iris pigmentation. WS1 is due to loss-of-function mutations in paired box gene 3 (PAX3). Here, we identified a novel PAX3 mutation (c.808C>G, p.R270G) in a three-generation Chinese family with WS1, and then analyzed its in vitro activities. The R270G PAX3 retained nuclear distribution and normal DNA-binding ability; however, it failed to activate MITF promoter, suggesting that haploinsufficiency may be the underlying mechanism for the mild WS1 phenotype of the study family...
November 17, 2017: Gene
https://www.readbyqxmd.com/read/29157806/trigonelline-promotes-auditory-function-through-nerve-growth-factor-signaling-on-diabetic-animal-models
#12
Rodrigo Castañeda, Isabel Rodriguez, Youn Hee Nam, Bin Na Hong, Tong Ho Kang
BACKGROUND: Protection of cochlear function and reconstruction of neuronal networks in damaged auditory sensory structures is crucial for therapeutic treatment of diabetic hearing loss. Nerve growth factor (NGF) has been used as a novel therapeutic target to protect against the neurodegenerative effects of Diabetes Mellitus (DM). PURPOSE: We aimed to evaluate the potential effect of trigonelline (TRG) on reducing auditory damage produced by DM using NGF as a potential marker...
December 1, 2017: Phytomedicine: International Journal of Phytotherapy and Phytopharmacology
https://www.readbyqxmd.com/read/29157165/successful-cochlear-implantation-in-langerhans-cell-histiocytosis-a-rare-case
#13
Gaurav Gupta, Avani Jain, Mohnish Grover
An 8-year-old girl presented with a 1-year history of bilateral progressive hearing loss and vertigo for 6 months. High-resolution computed tomography of the temporal bones demonstrated multiple lytic lesions. Histopathology examination confirmed a diagnosis of Langerhans cell histiocytosis (LCH). She underwent chemotherapy for 12 months. Following treatment, she was in remission. However, the bilateral profound sensorineural hearing loss persisted. She underwent right cochlear implantation with very good functional audiological outcomes...
November 20, 2017: Cochlear Implants International
https://www.readbyqxmd.com/read/29157038/applying-u-s-national-guidelines-for-ototoxicity-monitoring-in-adult-patients-perspectives-on-patient-populations-service-gaps-barriers-and-solutions
#14
Dawn Konrad-Martin, Gayla L Poling, Angela C Garinis, Candice E Ortiz, Jennifer Hopper, Keri O'Connell Bennett, Marilyn F Dille
OBJECTIVES: To promote establishment of effective ototoxicity monitoring programs (OMPs), this report reviews the U.S. national audiology guidelines in relation to "real world" OMP application. Background is provided on the mechanisms, risks and clinical presentation of hearing loss associated with major classes of ototoxic medications. DESIGN: This is a non-systematic review using PubMed, national and international agency websites, personal communications between ototoxicity experts, and results of unpublished research...
November 20, 2017: International Journal of Audiology
https://www.readbyqxmd.com/read/29154248/the-late-results-of-4000-hz-frequency-bone-conduction-after-tympanoplasty
#15
Joanna Janiak-Kiszka, Wojciech Kaźmierczak
INTRODUCTION: Surgical treatment of conductive hearing loss runs the risk of damage to the inner ear in the mechanism of acoustic trauma. AIM: The aim of this study was to evaluate the function of the organ of Corti, expressed as bone conduction threshold at the frequency of 4000 Hz for selected operations: mastoidectomy and canal-wall-down procedure. MATERIAL AND METHODS: The material was collected from patients with chronic otitis media in the Department of Otolaryngology and ENT Oncology, Collegium Medicum of Nicolaus Copernicus University in Bydgoszcz in 2004-2009...
October 30, 2017: Otolaryngologia Polska
https://www.readbyqxmd.com/read/29154247/diagnostic-evaluation-of-neck-torsion-test-in-objective-examination-in-patients-with-vertigo-and-or-hearing-impairment
#16
Piotr Niewiadomski, Marzena Bielińska, Piotr Pietkiewicz, Jurek Olszewski
INTRODUCTION: The aim of the study was to evaluate the neck torsion test in objective examinations of patients with vertigo and/or hearing loss. MATERIAL AND METHODS: The study was conducted in 100 patients, including 54 women and 46 men aged 17-79 years, who were divided into two groups: I - 50 patients, including 30 women and 20 men aged 17-79 years (mean age 49.92 years) with dizziness and/or hearing impairments, and confirmed asymmetry of intracranial vessels, II - 50 patients - the control group, including 24 women and 26 men aged 20-71 years without dizziness and/or hearing disorders and without disturbance in the construction of intracranial vessels...
October 30, 2017: Otolaryngologia Polska
https://www.readbyqxmd.com/read/29153369/retrospective-comparison-of-three-dimensional-imaging-sequences-in-the-visualization-of-posterior-fossa-cranial-nerves
#17
Suna Ors, Ercan Inci, Rustu Turkay, Atilla Kokurcan, Elif Hocaoglu
PURPOSE: To compare efficancy of three-dimentional SPACE (sampling perfection with application-optimized contrasts using different flip-angle evolutions) and CISS (constructive interference in steady state) sequences in the imaging of the cisternal segments of cranial nerves V-XII. METHODS: Temporal MRI scans from 50 patients (F:M ratio, 27:23; mean age, 44.5±15.9 years) admitted to our hospital with vertigo, tinnitus, and hearing loss were retrospectively analyzed...
December 2017: European Journal of Radiology
https://www.readbyqxmd.com/read/29153260/diagnostic-criteria-for-patulous-eustachian-tube-a-proposal-by-the-japan-otological-society
#18
Toshimitsu Kobayashi, Masahiro Morita, Satoshi Yoshioka, Kunihiro Mizuta, Shigeto Ohta, Toshiaki Kikuchi, Tatsuya Hayashi, Akihiro Kaneko, Nobumasa Yamaguchi, Sho Hashimoto, Hiromi Kojima, Shingo Murakami, Haruo Takahashi
Patulous Eustachian Tube (PET) is of increasing importance in otology. However, despite the abundance of diseases requiring a differential diagnosis from PET, such as superior semicircular canal dehiscence syndrome, perilymphatic fistula, acute low-tone sensorineural hearing loss, etc., there are currently no established diagnostic criteria for PET. In view of these circumstances, the Japan Otological Society (JOS) Eustachian Tube Committee proposed the diagnostic criteria for Patulous Eustachian Tube in 2012, in order to promote clinical research on PET...
November 15, 2017: Auris, Nasus, Larynx
https://www.readbyqxmd.com/read/29153256/intraoperative-force-and-electrocochleography-measurements-in-an-animal-model-of-cochlear-implantation
#19
J Lo, C Bester, A Collins, C Newbold, A Hampson, S Chambers, H Eastwood, S O'Leary
To preserve residual hearing, techniques for monitoring and reducing the effects of trauma during cochlear implant surgery are being developed. This study examines the relationships between intraoperative recordings (electrode insertion force and electrocochleography), trauma, and hearing loss after cochlear implantation. The study also evaluated the efficacy of intravenous steroids for reducing hearing loss after implantation. Thirty-two normal-hearing guinea pigs were randomly implanted with electrode arrays of differing stiffness ('hard' or 'soft')...
November 9, 2017: Hearing Research
https://www.readbyqxmd.com/read/29152771/prevalence-of-hearing-loss-among-noise-exposed-workers-within-the-agriculture-forestry-fishing-and-hunting-sector-2003-2012
#20
Elizabeth A Masterson, Christa L Themann, Geoffrey M Calvert
BACKGROUND: The purpose of this study was to estimate the prevalence of hearing loss among noise-exposed US workers within the Agriculture, Forestry, Fishing, and Hunting (AFFH) sector. METHODS: Audiograms for 1.4 million workers (17 299 within AFFH) from 2003 to 2012 were examined. Prevalence, and the adjusted risk for hearing loss as compared with the reference industry (Couriers and Messengers), were estimated. RESULTS: The overall AFFH sector prevalence was 15% compared to 19% for all industries combined, but many of the AFFH sub-sectors exceeded the overall prevalence...
November 20, 2017: American Journal of Industrial Medicine
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