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https://www.readbyqxmd.com/read/28434002/in-situ-hearing-tests-for-the-purpose-of-a-self-fit-hearing-aid
#1
Monique Boymans, Wouter A Dreschler
This study investigated the potential and limitations of a self-fit hearing aid. This can be used in the "developing" world or in countries with large distances between the hearing-impaired subjects and the professional. It contains an on-board tone generator for in situ user-controlled, automated audiometry, and other tests for hearing aid fitting. Twenty subjects with mild hearing losses were involved. In situ audiometry showed a test-retest reliability (SD <3.7 dB) that compared well with the precision of diagnostic audiometry using headphones...
April 22, 2017: Audiology & Neuro-otology
https://www.readbyqxmd.com/read/28433849/clinical-significance-of-long-term-follow-up-of-children-with-posttraumatic-skull-base-fracture
#2
Sharon Leibu, Guy Rosenthal, Yigal Shoshan, Mony Benifla
BACKGROUND: To assess in children with skull base fractures, incidences of cerebrospinal fluid (CSF) leak, meningitis, and the need for prophylactic antibiotics, anti-pneumococcal vaccination, and surgical interventions. METHODS: We reviewed the records of children with skull base fractures who were admitted to our tertiary care center during 2009-2014. RESULTS: 196 children (153 male) were hospitalized with skull base fractures, aged 1 month to 18 years (mean 6 ± 4 years)...
April 19, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28432874/delayed-changes-in-auditory-status-in-cochlear-implant-users-with-preserved-acoustic-hearing
#3
Rachel A Scheperle, Viral D Tejani, Julia K Omtvedt, Carolyn J Brown, Paul J Abbas, Marlan R Hansen, Bruce J Gantz, Jacob J Oleson, Marie V Ozanne
This retrospective review explores delayed-onset hearing loss in 85 individuals receiving cochlear implants designed to preserve acoustic hearing at the University of Iowa Hospitals and Clinics between 2001 and 2015. Repeated measures of unaided behavioral audiometric thresholds, electrode impedance, and electrically evoked compound action potential (ECAP) amplitude growth functions were used to characterize longitudinal changes in auditory status. Participants were grouped into two primary categories according to changes in unaided behavioral thresholds: (1) stable hearing or symmetrical hearing loss and (2) delayed loss of hearing in the implanted ear...
April 12, 2017: Hearing Research
https://www.readbyqxmd.com/read/28432353/severe-hearing-loss-and-outer-hair-cell-death-in-homozygous-foxo3-knockout-mice-after-moderate-noise-exposure
#4
Felicia Gilels, Stephen T Paquette, Holly J Beaulac, Anwen Bullen, Patricia M White
Noise induced hearing loss (NIHL) is a disease that affects millions of Americans. Identifying genetic pathways that influence recovery from noise exposure is an important step forward in understanding NIHL. The transcription factor Foxo3 integrates the cellular response to oxidative stress and plays a role in extending lifespan in many organisms, including humans. Here we show that Foxo3 is required for auditory function after noise exposure in a mouse model system, measured by ABR. Absent Foxo3, outer hair cells are lost throughout the middle and higher frequencies...
April 21, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28432340/phenome-wide-association-study-for-cyp2a6-alleles-rs113288603-is-associated-with-hearing-loss-symptoms-in-elderly-smokers
#5
Renato Polimanti, Kevin P Jensen, Joel Gelernter
To identify novel phenotypic associations related to Cytochrome P450 Family 2 Subfamily A Member 6 (CYP2A6), we investigated the human phenome in a total of 11,271 individuals. Initially, we conducted a phenome-wide association study in 3,401 nicotine-exposed elderly subjects considering 358 phenotypic traits. We identified a significant association between CYP2A6 rs113288603 and hearing loss symptoms (p = 5.75 × 10(-5)). No association was observed in a sample of 3,245 nicotine-unexposed individuals from the same discovery cohort, consistent with the conclusion that the finding is related to CYP2A6 involvement in nicotine metabolism...
April 21, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28431308/hyperexcitability-of-inferior-colliculus-and-acoustic-startle-reflex-with-age-related-hearing-loss
#6
Binbin Xiong, Ana'am Alkharabsheh, Senthilvelan Manohar, Guang-Di Chen, Ning Yu, Xiaoming Zhao, Richard Salvi, Wei Sun
Chronic tinnitus and hyperacusis often develop with age-related hearing loss presumably due to aberrant neural activity in the central auditory system (CAS) induced by cochlear pathologies. However, the full spectrum of physiological changes that occur in the CAS as a result age-related hearing loss are still poorly understood. To address this issue, neurophysiological measures were obtained from the cochlea and the inferior colliculus (IC) of 2, 6 and 12 month old C57BL/6J mice, a mouse model for early age-related hearing loss...
March 27, 2017: Hearing Research
https://www.readbyqxmd.com/read/28430993/a-multi-systemic-mitochondrial-disorder-due-to-a-dominant-p-y955h-disease-variant-in-dna-polymerase-gamma
#7
Triinu Siibak, Paula Clemente, Ana Bratic, Helene Bruhn, Timo E S Kauppila, Bertil Macao, Florian A Schober, Nicole Lesko, Rolf Wibom, Karin Naess, Inger Nennesmo, Anna Wedell, Bradley Peter, Christoph Freyer, Maria Falkenberg, Anna Wredenberg
Mutations in the mitochondrial DNA polymerase, POLG, are associated with a variety of clinical presentations, ranging from early onset fatal brain disease in Alpers syndrome to chronic progressive external ophthalmoplegia. The majority of mutations are linked with disturbances of mitochondrial DNA (mtDNA) integrity and maintenance. On a molecular level, depending on their location within the enzyme, mutations either lead to mtDNA depletion or the accumulation of multiple mtDNA deletions, and in some cases these molecular changes can be correlated to the clinical presentation...
April 17, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28430293/three-cases-of-ectopic-sphenoid-sinus-pituitary-adenoma
#8
Ernest Jan Bobeff, Karol Wiśniewski, Wielisław Papierz, Ludomir Stefańczyk, Dariusz Jan Jaskólski
<i><b>Introduction</b>: Ectopic sphenoid sinus pituitary adenoma is a rare tumour originating from embryologic remnants of Rathke's pouch. Although it is considered a clinically benign neoplasm, necrosis is encountered in 25% of cases and it can invade adjacent bone structures. <b>Aims</b>: To establish clinical, radiological and histopathological features of ectopic sphenoid sinus pituitary adenoma. <b>Material and methods: </b> Analysis of three cases: two females and one man, aged 61-70...
2017: Folia Neuropathologica
https://www.readbyqxmd.com/read/28429364/application-of-gene-detection-technique-in-the-antenatal-diagnosis-of-hereditary-hearing-loss
#9
Y Fang, M-S Gu, F Suo, C-X Wang, X-H Liu, F-M Liu
OBJECTIVE: Gene chip and gene sequencing techniques were used to detect the main pathogenic genes in pregnant women with hereditary hearing loss. PATIENTS AND METHODS: From May 2015 to May 2016, 1080 pregnant in Xuzhou Maternal and Child Health Hospital were enrolled in this study. Women age range was 18 to 40 years. 4 genes and 9 mutation sites, including 4 sites (35delG, 176, 235delC and 299) in GJB2 gene, 2 sites (2168A>G and IVS-7-2A>G) in SLC26A4 (PDS) gene, 2 sites (1494C>T and 1555A>G) in 12s rRNA gene and 1 site (538C>T) in GJB3 gene, were detected using the GeeDom® 9-item hereditary hearing loss gene detection kit...
April 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/28428906/tbc1d24-mutations-in-a-sibship-with-multifocal-polymyoclonus
#10
Adeline Ngoh, Jose Bras, Rita Guerreiro, Amy McTague, Joanne Ng, Esther Meyer, W Kling Chong, Stewart Boyd, Linda MacLellan, Martin Kirkpatrick, Manju A Kurian
BACKGROUND: Advances in molecular genetic technologies have improved our understanding of genetic causes of rare neurological disorders with features of myoclonus. CASE REPORT: A family with two affected siblings, presenting with multifocal polymyoclonus and neurodevelopmental delay, was recruited for whole-exome sequencing following unyielding diagnostic neurometabolic investigations. Compound heterozygous mutations in TBC1D24, a gene previously associated with various epilepsy phenotypes and hearing loss, were identified in both siblings...
2017: Tremor and Other Hyperkinetic Movements
https://www.readbyqxmd.com/read/28428741/assessing-auditory-processing-deficits-in-tinnitus-and-hearing-impaired-patients-with-the-auditory-behavior-questionnaire
#11
Isabel Diges, Francisco Simón, Pedro Cobo
Background and Purpose: Auditory processing disorders (APD), tinnitus and hearing loss (HL) are typical issues reported by patients in audiologic clinics. These auditory impairments can be concomitant or mutually excluding. APD are not necessarily accompanied by significant HL, whereas many adults exhibit peripheral HL and typical cognitive deficits often associated with APD. Since HL, tinnitus and APD affects to several parts of the ascending auditory pathway from the periphery to the auditory cortex, there could be some interrelationship between them...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28428247/induction-of-cell-death-and-gain-of-function-properties-of-connexin26-mutants-predict-severity-of-skin-disorders-and-hearing-loss
#12
Eric R Press, Qing Shao, John J Kelly, Katrina Chin, Anton Alaga, Dale W Laird
Connexin26 (Cx26) is a gap junction protein that oligomerizes in the cell to form hexameric transmembrane channels called connexons. Cell surface connexons dock between adjacent cells to allow for gap junctional intercellular communication. Numerous autosomal dominant mutations in the Cx26 encoding GJB2 gene lead to many skin disorders and sensorineural hearing loss. Although some insights have been gained into the pathogenesis of these diseases, it is not fully understood how distinct GJB2 mutations result in hearing loss alone or in skin pathologies with comorbid hearing loss...
April 20, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28428227/mechanisms-in-endocrinology-the-multiple-facets-of-gh-lessons-from-lifetime-untreated-isolated-gh-deficiency-due-to-ghrh-receptor-mutation
#13
Manuel Aguiar-Oliveira, Anita Ho Souza, Carla R Oliveira, Viviane C Campos, Luiz A Oliveira-Neto, Roberto Salvatori
Twenty years ago, we described a kindred of 105 individuals with isolated GH deficiency (IGHD) in Itabaianinha County, in Brazilian northeast, due to a homozygous mutation in the GH-releasing hormone receptor gene. These subjects exhibit markedly reduced GH responsiveness to stimulatory tests, and anterior pituitary hypoplasia. Serum concentrations of IGF-I, IGF binding protein type 3, and the acid-labile subunit are markedly reduced, with a lesser reduction of IGF-II. The most striking physical findings of these IGHD individuals are the proportionate short stature, doll facies, high-pitched voice, and visceral obesity with reduced fat free mass...
April 20, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28426889/concurrent-and-longitudinal-predictors-of-reading-for-deaf-and-hearing-children-in-primary-school
#14
Margaret Harris, Emmanouela Terlektsi, Fiona Elizabeth Kyle
Forty-one children with severe-profound prelingual hearing loss were assessed on single word reading, reading comprehension, English vocabulary, phonological awareness and speechreading at three time points, 1 year apart (T1-T3). Their progress was compared with that of a group of hearing children of similar nonverbal IQ, initially reading at the same level. Single word reading improved at each assessment point for the deaf children but there was no growth in reading comprehension from T2 to T3. There were no differences between children with cochlear implants and those with hearing aids on either reading measure but orally educated children had higher scores than children who signed in the classroom...
April 1, 2017: Journal of Deaf Studies and Deaf Education
https://www.readbyqxmd.com/read/28426851/unilateral-hearing-loss-and-otorrhea
#15
Mark S Costello, Shawn Stevens, Ravi N Samy
No abstract text is available yet for this article.
April 20, 2017: JAMA Otolaryngology—Head & Neck Surgery
https://www.readbyqxmd.com/read/28426234/the-otogl-p-arg925-variant-is-associated-with-moderate-hearing-loss-in-a-syrian-nonconsanguineous-family
#16
Rana Barake, Samer Abou-Rizk, Georges Nemer, Marc Bassim
AIMS: To screen for the genetic basis of congenital hearing loss in a Syrian family. METHODS: A Syrian patient living in Lebanon presented with moderate congenital hearing loss. The patient's large nonconsanguineous family was recruited. DNA was extracted from blood samples and sent for whole-exome sequencing. A detailed clinical examination along with audiograms was obtained for all subjects. RESULTS: Hearing loss was noted to be mild to moderate in the low and mid frequencies, sloping to moderate to severe in the high frequencies for all affected members...
April 20, 2017: Genetic Testing and Molecular Biomarkers
https://www.readbyqxmd.com/read/28425563/differences-in-interregional-brain-connectivity-in-children-with-unilateral-hearing-loss
#17
Matthew E Jung, Miranda Colletta, Rebecca Coalson, Bradley L Schlaggar, Judith E C Lieu
OBJECTIVES: To identify functional network architecture differences in the brains of children with unilateral hearing loss (UHL) using resting-state functional-connectivity magnetic resonance imaging (rs-fcMRI). STUDY DESIGN: Prospective observational study. METHODS: Children (7 to 17 years of age) with severe to profound hearing loss in one ear, along with their normal hearing (NH) siblings, were recruited and imaged using rs-fcMRI. Eleven children had right UHL; nine had left UHL; and 13 had normal hearing...
April 20, 2017: Laryngoscope
https://www.readbyqxmd.com/read/28424585/efr3a-insufficiency-attenuates-the-degeneration-of-spiral-ganglion-neurons-after-hair-cell-loss
#18
Haixia Hu, Bin Ye, Le Zhang, Quan Wang, Zhiwei Liu, Suying Ji, Qiuju Liu, Jingrong Lv, Yan Ma, Ying Xu, Hao Wu, Fude Huang, Mingliang Xiang
Sensorineural hearing loss (SNHL) is caused by an irreversible impairment of cochlear hair cells and subsequent progressive degeneration of spiral ganglion neurons (SGNs). Eighty-five requiring 3 (Efr3) is a plasma membrane protein conserved from yeast to human, and knockout of Efr3a was reported to facilitate the survival of hippocampal newborn neurons in adult mice. Previously, we found Efr3a expression in the auditory neural pathway is upregulated soon after the destruction of hair cells. Here we conducted a time-course analysis of drug-caused damage to hearing ability, hair cells and SGNs in Efr3a knocking down mice (Efr3a(-/+), Efr3a KD) and their wild type littermates...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28423374/hyperbaric-oxygen-therapy-as-adjuvant-treatment-for-idiopathic-sudden-sensorineural-hearing-loss-after-failure-of-systemic-steroids
#19
Seiji Hosokawa, Ken-Ichi Sugiyama, Goro Takahashi, Yu-Ichi Hashimoto, Kumiko Hosokawa, Satoru Takebayashi, Hiroyuki Mineta
We evaluated the outcomes of and prognostic factors for idiopathic sudden sensorineural hearing loss (ISSNHL) treated with adjuvant hyperbaric oxygen therapy (HBOT). A retrospective review of clinical data was performed for 167 patients with ISSNHL who failed to respond to systemic steroids and were treated by adjuvant HBOT at Shizuoka Saiseikai General Hospital. We analysed the clinical outcomes, the averaged 5-frequency hearing level after systemic steroids, patient age, the interval between post-steroids and pre-HBOT, vertigo as a complication, the presence of diabetes mellitus, smoking history, and hypertension...
April 20, 2017: Audiology & Neuro-otology
https://www.readbyqxmd.com/read/28423240/biochemical-cellular-physiological-and-pathological-consequences-of-human-loss-of-n-glycolylneuraminic-acid
#20
Jonathan Okerblom, Ajit Varki
About 2-3 million years ago, Alu-mediated deletion of a critical exon in the CMAH gene became fixed in the hominin lineage ancestral to humans, possibly through a stepwise process of selection by pathogen targeting of the CMAH product (the sialic acid Neu5Gc), followed by reproductive isolation via female anti-Neu5Gc antibodies. Loss of CMAH has occurred independently in some other lineages, but is functionally intact in Old World primates, including our closest relatives, the chimpanzees. While the biophysical and biochemical ramifications of losing tens of millions of Neu5Gc hydroxyl groups at most cell surfaces remains poorly understood, there are multi-scale effects functionally relevant to both sides of the host-pathogen interface...
April 19, 2017: Chembiochem: a European Journal of Chemical Biology
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