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https://www.readbyqxmd.com/read/28644112/susac-syndrome-misdiagnosed-as-multiple-sclerosis-with-exacerbation-by-interferon-beta-therapy
#1
Hussein Algahtani, Bader Shirah, Muhammad Amin, Eyad Altarazi, Hashem Almarzouki
Susac syndrome is a rare autoimmune disorder characterised by the clinical triad of encephalopathy, retinopathy (branch retinal artery occlusions) and hearing loss. The diagnosis of Susac syndrome may be difficult initially, and it is not uncommon for patients with Susac syndrome to be misdiagnosed with multiple sclerosis. In this case report, we describe a patient who was diagnosed as having multiple sclerosis for three years, with further deterioration after starting treatment with interferon beta-1a. The patient had the triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss...
January 1, 2017: Neuroradiology Journal
https://www.readbyqxmd.com/read/28643534/transplantation-of-mouse-induced-pluripotent-stem-cells-into-the-cochlea-for-the-treatment-of-sensorineural-hearing-loss
#2
Jing Chen, Lina Guan, Hengtao Zhu, Shan Xiong, Liang Zeng, Hongqun Jiang
CONCLUSION: Mouse-induced pluripotent stem cells (iPSCs) could differentiate into hair cell-like cells and spiral ganglion-like cells after transplantation into mouse cochleae, but it cannot improve the auditory brain response (ABR) thresholds in short term. OBJECTIVE: To evaluate the potential of iPSCs for use as a source of transplants for the treatment of sensorineural hearing loss (SNHL). METHODS: Establishing SNHL mice model, then injecting the iPSCs or equal volume DMEM basic medium into the cochleae, respectively...
June 23, 2017: Acta Oto-laryngologica
https://www.readbyqxmd.com/read/28642245/a-reporter-mouse-model-for-in-vivo-tracing-and-in-vitro-molecular-studies-of-melanocytic-lineage-cells-and-their-diseases
#3
Melissa Crawford, Valerie Leclerc, Lina Dagnino
Alterations in melanocytic lineage cells give rise to a plethora of distinct human diseases, including neurocristopathies, cutaneous pigmentation disorders, loss of vision and hearing, as well as melanoma. Understanding the ontogeny and biology of melanocytic cells, as well as how they interact with their surrounding environment, are key steps in the development of therapies for diseases that involve this cell lineage. Efforts to culture and characterize primary melanocytes from normal or genetically engineered mouse models have at times yielded contrasting observations...
June 22, 2017: Biology Open
https://www.readbyqxmd.com/read/28642064/the-genetic-basis-of-deafness-in-populations-of-african-descent
#4
REVIEW
Jason R Rudman, Rosemary I Kabahuma, Sara E Bressler, Yong Feng, Susan H Blanton, Denise Yan, Xue-Zhong Liu
Hearing loss is the most common sensorineural disorder worldwide and is associated with more than 1000 mutations in more than 90 genes. While mutations in genes such as GJB2 (gap-junction protein β 2) and GJB6 (gap-junction protein β 6) are highly prevalent in Caucasian, Asian, and Middle Eastern populations, they are rare in both native African populations and those of African descent. The objective of this paper is to review the current knowledge regarding the epidemiology and genetics of hearing loss in African populations with a focus on native sub-Saharan African populations...
May 6, 2017: Journal of Genetics and Genomics, Yi Chuan Xue Bao
https://www.readbyqxmd.com/read/28641772/susac-syndrome-a-differential-diagnosis-of-white-matter-lesions
#5
Mariano Marrodan, Jorge Correale, Lucas Alessandro, Mariela Amaya, Maria Eugenia Fracaro, Alejandro Alfredo Köhler, Marcela Fiol
INTRODUCTION: Susac Syndrome is an autoimmune endotheliopathy affecting capillaries and precapillary arterioles of the brain, inner ear and retina. The classic symptom triad includes visual disturbances, hypoacusia, and encephalopathy, but is rarely fully manifest at onset. The syndrome typically follows an active fluctuating monophasic course. Typical imaging findings on brain magnetic resonance show central fiber microischemias/microinfarctions of the corpus callosum known as "snow balls", as well as lineal infarcts and upper callosal fiber involvement...
July 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28641636/-short-and-long-term-clinical-efficacy-of-imrt-and-crt-for-treatment-of-patients-with-enktl
#6
Jian-Xiong Cui, Wu-Hua Lin
OBJECTIVE: To compare the short and long term clinical efficacy for treatment of patients with extranodal nasal-type NK/T-cell lymphoma(ENKTL) by intensity modulated radiotherapy(IMRT) and conventional radiotheraphy(CRT). METHODS: Sixty-three cases of early ENKTL were divided into the IMRT group of (33 cases) and CRT group(30 cases). The short effects, overall survival(OS) of 3 years, progression free survival(PFS) of 3 years, acute radiation injury and late adverse reactions to radiation were compared between 2 group...
June 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28640090/efficiency-of-microarray-and-snpscan-for-the-detection-of-hearing-loss-gene-in-71-cases-with-nonsyndromic-hearing-loss
#7
Rui Han, Linge Li, Ling Duan, Yan Xia, Pilidong Kuyaxi, Juan Zhao, Qi Zhao, Hua Zhang, Yu Chen
We aim to screen the mutations of 3 hearing loss (HL) genes (GJB2, SLC26A4, and 12S rRNA) in 71 cases with nonsyndromic hearing loss (NSHL) using microarray and SNPscan, and identify the roles of nonhotspot mutation of these genes in the screening of NSHL. Seventy-one cases with moderate or severe neurosensory deafness confirmed in our department from July 2014 to December 2015 including 25 Uyghur minorities and 46 Han Chinese were included in this study. The type of mutations in GJB2, SLC26A4, and 12S rRNA genes were detected using microarray and SNPscan, respectively...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28640038/impact-of-noise-and-noise-reduction-on-processing-effort-a-pupillometry-study
#8
Dorothea Wendt, Renskje K Hietkamp, Thomas Lunner
OBJECTIVES: Speech perception in adverse listening situations can be exhausting. Hearing loss particularly affects processing demands, as it requires increased effort for successful speech perception in background noise. Signal processing in hearing aids and noise reduction (NR) schemes aim to counteract the effect of noise and reduce the effort required for speech recognition in adverse listening situations. The present study examined the benefit of NR schemes, applying a combination of a digital NR and directional microphones, for reducing the processing effort during speech recognition...
June 20, 2017: Ear and Hearing
https://www.readbyqxmd.com/read/28639843/characteristics-of-children-with-unilateral-hearing-loss
#9
Elizabeth M Fitzpatrick, Rakan S Al-Essa, JoAnne Whittingham, Jessica Fitzpatrick
OBJECTIVE: The purpose of this study was to describe the clinical characteristics of children with unilateral hearing loss (UHL), examine deterioration in hearing, and explore amplification decisions. DESIGN: Population-based data were collected prospectively from time of diagnosis. Serial audiograms and amplification details were retrospectively extracted from clinical charts to document the trajectory and management of hearing loss. SAMPLE: The study included all children identified with UHL in one region of Canada over a 13-year period (2003-2015) after implementation of universal newborn hearing screening...
June 22, 2017: International Journal of Audiology
https://www.readbyqxmd.com/read/28639554/evaluation-of-procalcitonin-and-hs-crp-levels-in-sudden-sensorineural-hearing-loss
#10
Sercan Göde, Göksel Turhal, İsa Kaya, Halil İbrahim Mavili, Tayfun Kirazlı
OBJECTIVE: The aim of this study was to evaluate procalcitonin and high sensitive c-reactive protein (hs-CRP) levels in idiopathic sudden sensorineural hearing loss (ISSNHL) patients and assess their correlations with the clinical prognosis. MATERIALS AND METHODS: Twenty-three ISSNHL patients were included in the study (group A). The control group was consisted of 19 patients (group B). Procalcitonin and hs-CRP levels were compared between the groups. The relationship between procalcitonin and hs-CRP levels and the configuration of the audiogram, degree of hearing loss [partial or total (>90 dB)], and status of improvement (improvement of > 15 dB in the first month PTA) were evaluated...
June 21, 2017: Journal of International Advanced Otology
https://www.readbyqxmd.com/read/28637591/salicylate-induced-frequency-map-reorganization-in-four-subfields-of-the-mouse-auditory-cortex
#11
Yasutaka Yanagawa, Kengo Takasu, Hisayuki Osanai, Takashi Tateno
Salicylate is the active ingredient in aspirin, and in high-doses it is used as an experimental tool to induce transient hearing loss, tinnitus, and hyperacusis. These salicylate-induced perceptual disturbances are associated with tonotopic-map reorganization and neural activity modulation, and such neural correlates have been examined in the central auditory pathway, including the auditory cortex (AC). Although previous studies have reported that salicylate induces increases in noise-burst-evoked neural responses and reorganization of tonotopic maps in the primary AC, little is known about the effects of salicylate on other frequency-organized AC subfields such as the anterior auditory, secondary auditory, and dorsomedial fields...
June 10, 2017: Hearing Research
https://www.readbyqxmd.com/read/28637148/pure-tone-hearing-profiles-in-children-with-otitis-media-with-effusion
#12
Ting Cai, Bradley McPherson, Caiwei Li, Feng Yang
INTRODUCTION: Otitis media with effusion (OME) is a common middle ear disease in children. The associated conductive hearing loss is a major concern for hearing health professionals. The aim of the present study was to describe the configuration of pure tone audiograms of children with OME and to design a statistical stratification algorithm to facilitate hearing loss profiling in children with OME. METHODS: School age children with OME were recruited. Bone and air conduction thresholds were obtained using standard procedures...
February 25, 2017: Disability and Rehabilitation
https://www.readbyqxmd.com/read/28636935/the-sensory-striatum-is-permanently-impaired-by-transient-developmental-deprivation
#13
Todd M Mowery, Kristina B Penikis, Stephen K Young, Christopher E Ferrer, Vibhakar C Kotak, Dan H Sanes
Corticostriatal circuits play a fundamental role in regulating many behaviors, and their dysfunction is associated with many neurological disorders. In contrast, sensory disorders, like hearing loss (HL), are commonly linked with processing deficits at or below the level of the auditory cortex (ACx). However, HL can be accompanied by non-sensory deficits, such as learning delays, suggesting the involvement of regions downstream of ACx. Here, we show that transient developmental HL differentially affected the ACx and its downstream target, the sensory striatum...
June 20, 2017: Cell Reports
https://www.readbyqxmd.com/read/28636783/normal-neonatal-hearing-screening-did-not-preclude-sensorineural-hearing-loss-in-two-year-old-very-preterm-infants
#14
Inge L van Noort-van der Spek, André Goedegebure, Nico G Hartwig, René F Kornelisse, Marie-Christine J P Franken, Nynke Weisglas-Kuperus
AIM: Very preterm infants are at risk of neonatal hearing loss. However, it is unknown whether infants with a normal neonatal hearing screening result risk sensorineural hearing loss (SNHL) at a later age. METHODS: This cohort study was conducted at the Erasmus Medical University Center Rotterdam, the Netherlands, on 77 very preterm infants born between October 2005 and September 2008. All infants underwent auditory brainstem response audiometry during neonatal hearing screening and at two years of corrected age...
June 21, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/28636736/the-protective-effect-of-intratympanic-dexamethasone-on-streptomycin-ototoxicity-in-rats
#15
Aylin Gül, Engin Şengül, Beyhan Yılmaz, Fazıl Emre Özkurt, Mehmet Akdağ, Ayşenur Keleş, İsmail Topçu
The purpose of this experimental study was to investigate the protective role of intratympanically administered dexamethasone on the inner ears of rats that were exposed to streptomycin ototoxicity. Twenty-four adult Wistar albino rats were separated into 4 groups: Group 1 (only streptomycin), Group 2 (only intratympanic dexamethasone), Group 3 (streptomycin and intratympanic dexamethasone), and Group 4 (streptomycin and intratympanic saline). All rats were evaluated with distortion product otoacoustic emissions (DPOAE) tests before the start of treatment and on the day it ended...
June 2017: Ear, Nose, & Throat Journal
https://www.readbyqxmd.com/read/28636124/the-ips-scale-a-new-soft-tissue-assessment-scale-for-percutaneous-and-transcutaneous-implants-for-bone-conduction-devices
#16
Ivo J Kruyt, Rik C Nelissen, Martin L Johansson, Emmanuel A M Mylanus, Myrthe K S Hol
Percutaneous titanium implants for bone conduction devices (BCDs) have offered, since 1977, a solution for patients with hearing loss not treatable by conventional hearing aids, such as patients with chronic ear infections or microtia and/or ear canal atresia. Percutaneous implants imply a continuous breach in the mechanical defensive barrier of the skin. To compensate for this breach, immunological mechanisms in the subcutaneous tissue surrounding the implant become more active. This article is protected by copyright...
June 21, 2017: Clinical Otolaryngology
https://www.readbyqxmd.com/read/28635507/cochlear-implant-effectiveness-in-postlingual-single-sided-deaf-individuals-what-s-the-point
#17
Mareike Finke, Hanna Bönitz, Björn Lyxell, Angelika Illg
OBJECTIVES: By extending the indication criteria for cochlear implants (CI), the population of CI candidates increased in age, as well as range and type of hearing loss. This qualitative study identified factors that contributed to seek CI treatment in single-sided deaf individuals and gained insights how single-sided deafness (SSD) and hearing with a CI affect their lives. DESIGN: An open-ended questionnaire and a standardised inventory (IOI-HA) were used. Qualitative data reflecting the reasons to seek CI treatment and the individual experiences after CI switch-on were collected...
March 5, 2017: International Journal of Audiology
https://www.readbyqxmd.com/read/28635505/social-inequalities-in-pure-tone-hearing-assessed-using-occupational-stratification-schemes
#18
Petra von Gablenz, Inga Holube
OBJECTIVE: The objective of this study is to analyse the performance of two occupational stratification approaches and the impact of social position on adult hearing. DESIGN: The prevalence of hearing impairment, pure-tone averages (PTA) and prevalence ratios (PR) for relative hearing loss, which focuses on the position of one's PTA in the age- and gender-specific distribution, were compared in groups defined by ISCO Skill Level and the International Socio-Economic Index (ISEI)...
March 1, 2017: International Journal of Audiology
https://www.readbyqxmd.com/read/28635501/promoting-global-action-on-hearing-loss-world-hearing-day
#19
Shelly Chadha, Alarcos Cieza
The growing need for hearing care and a general lack of policies to address it poses a significant public health challenge. The World Health Organization aims to address this through evidence-based advocacy along with technical support to countries in provision of hearing care. As part of its activities, WHO is promoting the World Hearing Day which is observed on 3rd March every year. The day is marked by an annual theme and release of information products and key messages. Through this, we aim to raise awareness and promote actions for ear and hearing care across the world...
February 23, 2017: International Journal of Audiology
https://www.readbyqxmd.com/read/28635498/effect-of-age-related-hearing-loss-on-the-click-rate-induced-facilitation-of-acoustic-reflex-thresholds
#20
Vishakha Waman Rawool
OBJECTIVE: This investigation was designed to evaluate the effect of age-related hearing loss on the click-rate-induced improvement in the acoustic reflex thresholds. DESIGN: Case-control study. STUDY SAMPLE: Data from five different adults ear-groups (15 ears each) were included in the study: 1. Younger with normal hearing. 2. Older with normal hearing. 3. Older with mild high-frequency loss. 4. Older with moderate high-frequency loss. 5...
February 15, 2017: International Journal of Audiology
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