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Sensorineural hearing loss

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https://www.readbyqxmd.com/read/27914370/concurrent-measures-of-contralateral-suppression-of-transient-evoked-otoacoustic-emissions-and-of-auditory-steady-state-responses
#1
Ian B Mertes, Marjorie R Leek
Contralateral suppression of otoacoustic emissions (OAEs) is frequently used to assess the medial olivocochlear (MOC) efferent system, and may have clinical utility. However, OAEs are weak or absent in hearing-impaired ears, so little is known about MOC function in the presence of hearing loss. A potential alternative measure is contralateral suppression of the auditory steady-state response (ASSR) because ASSRs are measurable in many hearing-impaired ears. This study compared contralateral suppression of both transient-evoked otoacoustic emissions (TEOAEs) and ASSRs in a group of ten primarily older adults with either normal hearing or mild sensorineural hearing loss...
September 2016: Journal of the Acoustical Society of America
https://www.readbyqxmd.com/read/27913750/congenital-heart-block-and-immune-mediated-sensorineural-hearing-loss-possible-cross-reactivity-of-immune-response
#2
C Bason, I Pagnini, A Brucato, S Maestroni, A Puccetti, C Lunardi, R Cimaz
Immune-mediated sensorineural hearing loss may complicate systemic autoimmune diseases. We have previously reported the presence of antibodies directed against inner ear antigens in patients with Cogan syndrome, a disease characterized by sudden hearing loss and interstitial keratitis. Such autoantibodies cross-react with an epitope of SSA/Ro60 protein. Anti-Ro/SSA antibodies in pregnant women cross the placenta and reach the fetal tissues inducing an immune-mediated damage of the cardiac conduction system...
December 2, 2016: Lupus
https://www.readbyqxmd.com/read/27911912/comprehensive-genetic-analysis-of-japanese-autosomal-dominant-sensorineural-hearing-loss-patients
#3
Yoh-Ichiro Iwasa, Shin-Ya Nishio, Shin-Ichi Usami
BACKGROUND: In general, autosomal dominant inherited hearing loss does not have a founder mutation, with the causative mutation different in each family. For this reason, there has been a strong need for efficient diagnosis methods for autosomal dominant sensorineural hearing loss (ADSNHL) patients. This study sought to verify the effectiveness of our analysis algorithm for the screening of ADSNHL patients as well as the usefulness of the massively parallel DNA sequencing (MPS). SUBJECTS AND METHODS: Seventy-five Japanese ADSNHL patients from 53 ENT departments nationwide participated in this study...
2016: PloS One
https://www.readbyqxmd.com/read/27910108/a-day-in-the-life-of-a-m%C3%A3-ni%C3%A3-re-s-patient-understanding-the-lived-experiences-and-mental-health-impacts-of-m%C3%A3-ni%C3%A3-re-s-disease
#4
Sarah L Bell, Jessica Tyrrell, Cassandra Phoenix
Concepts of social practice are increasingly being used to understand experiences of everyday life, particularly in relation to consumption and healthy lifestyles. This article builds on this in the context of lives disrupted and reshaped by chronic illness. It uses social practice theory to examine the lived experiences of individuals with Ménière's disease; a long-term progressive vestibular disorder, defined by episodes of severe and debilitating vertigo, aural fullness, tinnitus and sensorineural hearing loss...
December 2, 2016: Sociology of Health & Illness
https://www.readbyqxmd.com/read/27910089/bilateral-endolymphatic-hydrops-in-a-patient-with-migraine-variant-without-vertigo-a-case-report
#5
Isabelle Y Liu, Akira Ishiyama, Ali R Sepahdari, Kevin Johnson, Gail Ishiyama
OBJECTIVE: To use modern high-resolution inner ear imaging modalities to evaluate for endolymphatic hydrops (EH) in a patient with migraine-associated fluctuating hearing loss without vertigo spells or dizziness. BACKGROUND: EH has been well described in patients with Meniere's disease on both human temporal bone studies and modern high-resolution imaging; however, there is no study to date, to our knowledge, that examines the presence of EH in a patient with migraine and bilateral hearing loss...
December 2, 2016: Headache
https://www.readbyqxmd.com/read/27909890/does-severity-of-cerebral-mri-lesions-in-congenital-cmv-infection-correlates-with-the-outcome-of-cochlear-implantation
#6
Stefan Lyutenski, Friedrich Götz, Alexandros Giourgas, Omid Majdani, Eva Bültmann, Heinrich Lanfermann, Thomas Lenarz, Anja M Giesemann
The objective is to investigate whether there is a correlation between the severity of typical brain lesions in congenital cytomegalovirus (cCMV) infection and cochlear implant (CI) outcome. The design of the study is a retrospective single-institutional chart review (2005-2015), performed in a tertiary academic referral center. 23 children with typical signs of cCMV infection on cerebral magnetic resonance imaging (MRI) and bilateral severe-to-profound sensorineural hearing loss were retrospectively evaluated...
December 1, 2016: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/27909636/imatinib-induced-ototoxicity-in-a-patient-with-gastrointestinal-stromal-tumor-gist
#7
Komal Wasif, Nawal Wasif, Muhammad W Saif
Imatinib (Gleevec) is a biological agent that is approved for the treatment of chronic myeloid leukemia (CML) as well as gastrointestinal stromal tumor (GIST). The most frequently seen adverse effects in patients treated with imatinib include superficial edema, muscle cramps, musculoskeletal pain, rash, fatigue, headache, abdominal pain, and joint pain. Ototoxicity has rarely been reported except in two cases. We report a case of bilateral irreversible sensorineural hearing loss (SNHL) caused by imatinib in a patient receiving this agent in the adjuvant setting...
October 26, 2016: Curēus
https://www.readbyqxmd.com/read/27906862/correlational-study-of-diabetic-retinopathy-and-hearing-loss
#8
Caroline Ooley, Weon Jun, Kim Le, Allen Kim, Nathan Rock, Molly Cardenal, Rebecca Kline, Drew Aldrich, John Hayes
PURPOSE: Our research goal was to complete a retrospective chart review to determine if there is a correlation between the level of diabetic retinopathy and diabetic neurosensory hearing loss. METHODS: A retrospective analysis of 175 Department of Veterans Affairs Computerized Patient Record System charts was completed at the VA Portland Health Care System. Subjects were classified by degree of diabetic retinopathy as follows: no diabetic retinopathy (n = 80), mild nonproliferative diabetic retinopathy (n = 51), moderate nonproliferative diabetic retinopathy (n = 25), and combined severe nonproliferative diabetic retinopathy and proliferative diabetic retinopathy (PDR) (n = 17)...
November 30, 2016: Optometry and Vision Science: Official Publication of the American Academy of Optometry
https://www.readbyqxmd.com/read/27905190/the-diagnostic-accuracy-of-non-imaging-screening-protocols-for-vestibular-schwannoma-in-patients-with-asymmetrical-hearing-loss-and-or-unilateral-audiovestibular-dysfunction-a-diagnostic-review-and-meta-analysis
#9
M Hentschel, M Scholte, S Steens, H Kunst, M Rovers
BACKGROUND: Currently, all patients presenting with asymmetrical sensorineural hearing loss and/or unilateral audiovestibular dysfunction (i.e. tinnitus, dizziness) undergo MRI, leading to a substantial amount of MRIs with negative findings as the incidence of vestibular schwannoma (VS) in this screening population varies between 1% and 4.7% (i.e. more than 95% of MRIs are negative for VS). OBJECTIVE OF REVIEW: The aim was to assess the diagnostic accuracy of different non-imaging screening protocols that can be used prior to MRI to select patients at high risk of VS...
November 5, 2016: Clinical Otolaryngology
https://www.readbyqxmd.com/read/27900916/electrically-evoked-compound-action-potentials-are-different-depending-on-the-site-of-cochlear-stimulation
#10
Paul van de Heyning, Santiago L Arauz, Marcus Atlas, Wolf-Dieter Baumgartner, Marco Caversaccio, Ronel Chester-Browne, Patricia Estienne, Javier Gavilan, Benoit Godey, Wolfgang Gstöttner, Demin Han, Rudolph Hagen, Martin Kompis, Vlad Kuzovkov, Luis Lassaletta, Franc Lefevre, Yongxin Li, Joachim Müller, Lorne Parnes, Andrea Kleine Punte, Christopher Raine, Gunesh Rajan, Adriana Rivas, José Antonio Rivas, Nicola Royle, Georg Sprinzl, Kurt Stephan, Adam Walkowiak, Yuri Yanov, Kim Zimmermann, Patrick Zorowka, Henryk Skarzynski
One of the many parameters that can affect cochlear implant (CI) users' performance is the site of presentation of electrical stimulation, from the CI, to the auditory nerve. Evoked compound action potential (ECAP) measurements are commonly used to verify nerve function by stimulating one electrode contact in the cochlea and recording the resulting action potentials on the other contacts of the electrode array. The present study aimed to determine if the ECAP amplitude differs between the apical, middle, and basal region of the cochlea, if double peak potentials were more likely in the apex than the basal region of the cochlea, and if there were differences in the ECAP threshold and recovery function across the cochlea...
November 30, 2016: Cochlear Implants International
https://www.readbyqxmd.com/read/27899912/specific-mri-abnormalities-reveal-severe-perrault-syndrome-due-to-clpp-defects
#11
Tom E J Theunissen, Radek Szklarczyk, Mike Gerards, Debby M E I Hellebrekers, Elvira N M Mulder-Den Hartog, Jo Vanoevelen, Rick Kamps, Bart de Koning, S Lane Rutledge, Thomas Schmitt-Mechelke, Carola G M van Berkel, Marjo S van der Knaap, Irenaeus F M de Coo, Hubert J M Smeets
In establishing a genetic diagnosis in heterogeneous neurological disease, clinical characterization and whole exome sequencing (WES) go hand-in-hand. Clinical data are essential, not only to guide WES variant selection and define the clinical severity of a genetic defect but also to identify other patients with defects in the same gene. In an infant patient with sensorineural hearing loss, psychomotor retardation, and epilepsy, WES resulted in identification of a novel homozygous CLPP frameshift mutation (c...
2016: Frontiers in Neurology
https://www.readbyqxmd.com/read/27897130/recommendations-for-potassium-titanyl-phosphate-laser-in-the-treatment-of-cholesteatoma
#12
REVIEW
Gavin John le Nobel, Adrian Lewis James
Potassium-titanyl-phosphage (KTP) lasers possess many characteristics suitable for otologic surgery. The objective of this report is to provide recommendations on the use of KTP laser for cholesteatoma surgery based on a narrative review of currently available evidence. PubMed and the Cochrane Review of randomized control trials were searched for relevant publications on efficacy and adverse effects and relevant articles appraised by the authors using Oxford Centre for Evidence-based Medicine criteria for recommendations...
November 28, 2016: Journal of International Advanced Otology
https://www.readbyqxmd.com/read/27897126/recognition-of-deep-band-modulated-consonants-in-quiet-and-noise-in-older-individuals-with-and-without-hearing-loss
#13
Hemanth Narayan Shetty, Vishal Kooknoor
OBJECTIVE: The purpose of the present study was to investigate the effects of temporal envelope enhancement using deep band modulation (DBM) on consonant identification scores (CIS) and transfer of features such as place of articulation, manner of articulation, and voicing. MATERIALS AND METHODS: Data were collected from four groups of ten participants each. These were grouped into younger (YNH) and older adult (ONH) individuals with normal hearing and younger (YHI) and older adult (OHI) individuals with hearing impairment who had mild to moderate sensorineural hearing loss...
November 28, 2016: Journal of International Advanced Otology
https://www.readbyqxmd.com/read/27896131/case-report-5%C3%A2-year-follow-up-of-adult-late-onset-mitochondrial-encephalomyopathy-with-lactic-acid-and-stroke-like-episodes-melas
#14
Kiri Sunde, Patrick R Blackburn, Anvir Cheema, Jennifer Gass, Jessica Jackson, Sarah Macklin, Paldeep S Atwal
Mitochondrial encephalomyopathy with lactic acid and stroke-like episodes (MELAS) is a multisystem mitochondrial disorder that typically presents in childhood. We describe the follow-up of a patient who was diagnosed with late-onset MELAS at the age of 49. Her clinical course includes sensorineural hearing loss, seizures, and multiple episodes of stroke-like metabolic crises. Molecular genetic testing on whole blood revealed 31% heteroplasmy of a m.3243A > G variant in the mtDNA, the causative variant in approximately 80% of MELAS cases...
December 2016: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/27895351/frequency-of-sensorineural-hearing-loss-in-chronic-suppurative-otitis-media
#15
Syed Sajjad Ali Zaidi, Hamdan Ahmed Pasha, Anwar Suhail, Talha Ahmed Qureshi
Chronic suppurative otitis media (CSOM) is defined as chronic otorrhea (i.e., lasting > 6-12 weeks) through a perforated tympanic membrane. It is generally associated with some degree of conductive hearing loss. However, recurrent ear infections due to perforated eardrum result in absorption of toxins and macromolecules into the cochlea leading to sensorineural hearing loss (SNHL). We planned to determine the frequency of sensorineural hearing loss in chronic suppurative otitis media. A descriptive cross-sectional study was conducted at Aga Kgan University Hospital, Karachi, from October 2013 to March 2014...
October 2016: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/27895287/sudden-sensorineural-hearing-loss-is-there-a-connection-with-inner-ear-electrolytic-disorders-a-literature-review
#16
EDITORIAL
Andrea Ciorba, Virginia Corazzi, Chiara Bianchini, Claudia Aimoni, Henryk Skarzynski, Piotr Henryk Skarzynski, Stavros Hatzopoulos
Electrolytic disorders of the inner ear represent a model that could be implicated in partially explaining the pathogenesis of sudden sensorineural hearing loss (SSNHL). Different types of electrolytes and different inner-ear loci are involved in cochlear homeostasis physiologically, to ensure the maintenance of an ion-balanced cochlear environment allowing a normal hair cell function. It has been hypothesized that a sudden loss of endocochlear potential, due to a rapid disruption of the inner ear fluid osmolality, could be responsible for a deterioration of the hearing function caused by damaged hair cells...
December 2016: International Journal of Immunopathology and Pharmacology
https://www.readbyqxmd.com/read/27891421/imaging-modality-of-choice-for-pre-operative-cochlear-imaging-hrct-vs-mri-temporal-bone
#17
Poornima Digge, Rajendra N Solanki, Dipali C Shah, Rajesh Vishwakarma, Sandeep Kumar
INTRODUCTION: Congenital inner ear malformations occur as a result of the arrest or aberrance of inner ear development due to the heredity, gene mutation or other factors. Ever since the availability of cochlear implants, pre-operative evaluation by imaging of temporal bone has gained much attention. Precise selection of the candidate for cochlear implant dependent on preoperative radiological investigations. Only CT (Computed Tomography) and MRI (Magnetic Resonance Imaging) can provide a better picture of anatomy and pathology...
October 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27886419/functional-characterization-of-a-novel-loss-of-function-mutation-of-prps1-related-to-early-onset-progressive-nonsyndromic-hearing-loss-in-koreans-dfnx1-potential-implications-on-future-therapeutic-intervention
#18
So Young Kim, Ah Reum Kim, Nayoung K D Kim, Chung Lee, Jin Hee Han, Min Young Kim, Eun-Hee Jeon, Woong-Yang Park, Rahul Mittal, Denise Yan, Xue Zhong Liu, Byung Yoon Choi
BACKGROUND: The symptoms of phosphoribosyl pyrophosphate synthetase 1 (PRPS1) deficiency diseases have been reported to be alleviated by medication. Herein, we report biochemical data to favor PRPS1 deficiency-related hearing loss as a potential target for pharmaceutical treatment. METHODS: We recruited 42 probands from subjects under the age of 15 years with a moderate degree of nonsyndromic autosomal-recessive or sporadic sensorineural hearing loss (SNHL) in at least one side...
November 25, 2016: Journal of Gene Medicine
https://www.readbyqxmd.com/read/27876815/variable-expressivity-and-genetic-heterogeneity-involving-dpt-and-sema3d-genes-in-autosomal-dominant-familial-meniere-s-disease
#19
Carmen Martín-Sierra, Alvaro Gallego-Martinez, Teresa Requena, Lidia Frejo, Angel Batuecas-Caletrío, Jose A Lopez-Escamez
Autosomal dominant (AD) familial Meniere's disease (FMD) is a rare disorder involving the inner ear defined by sensorineural hearing loss, tinnitus and episodic vertigo. Here, we have identified two novel and rare heterozygous variants in the SEMA3D and DPT genes segregating with the complete phenotype that have variable expressivity in two pedigrees with AD-FMD. A detailed characterization of the phenotype within each family illustrates the clinical heterogeneity in the onset and progression of the disease...
November 23, 2016: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/27875418/nonclassic-features-of-pseudohypoparathyroidism-type-1a
#20
Ashley H Shoemaker, Harald Jüppner
PURPOSE OF REVIEW: To provide readers with a review of contemporary literature describing the evolving understanding of the pseudohypoparathyroidism type 1A (PHP1A) phenotype. RECENT FINDINGS: The classic features of PHP1A include multihormone resistance and the Albright Hereditary Osteodystrophy phenotype (round facies, short stature, subcutaneous ossifications, brachydactyly, and early-onset obesity. Obesity may be because of a decrease in resting energy expenditure because most patients do not report significant hyperphagia...
November 21, 2016: Current Opinion in Endocrinology, Diabetes, and Obesity
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