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https://www.readbyqxmd.com/read/28535131/detection-of-deafness-in-puppies-using-a-hand-held-otoacoustic-emission-screener
#1
Michael H Sims, Erin Plyler, Ashley Harkrider, Karen McLucas
The purpose of this study was to evaluate the use of a hand-held otoacoustic emissions screener to detect deafness in puppies. Specifically, distortion product otoacoustic emissions were recorded from 34 puppies (both sexes) of a variety of breeds, from 6-10 wk of age, and the results were compared to brainstem auditory evoked responses (BAER) recorded from the same puppies. Recordings were obtained from both ears in awake or lightly anesthetized puppies, and the results from each ear were compared. In all 62 ears that had normal BAERs, the distortion product otoacoustic emissions screener gave a response of "Pass...
May 23, 2017: Journal of the American Animal Hospital Association
https://www.readbyqxmd.com/read/28534737/integration-of-tmc1-2-into-the-mechanotransduction-complex-in-zebrafish-hair-cells-is-regulated-by-transmembrane-o-methyltransferase-tomt
#2
Timothy Erickson, Clive P Morgan, Jennifer Olt, Katherine Hardy, Elisabeth M Busch-Nentwich, Reo Maeda, Rachel Clemens-Grisham, Jocelyn F Krey, Alex V Nechiporuk, Peter G Barr-Gillespie, Walter Marcotti, Teresa Nicolson
Transmembrane O-methyltransferase (TOMT / LRTOMT) is responsible for non-syndromic deafness DFNB63. However, the specific defects that lead to hearing loss have not been described. Using a zebrafish model of DFNB63, we show that the auditory and vestibular phenotypes are due to a lack of mechanotransduction (MET) in Tomt-deficient hair cells. GFP-tagged Tomt is enriched in the Golgi of hair cells, suggesting that Tomt might regulate the trafficking of other MET components to the hair bundle. We found that Tmc1/2 proteins are specifically excluded from the hair bundle in tomt mutants, whereas other MET complex proteins can still localize to the bundle...
May 23, 2017: ELife
https://www.readbyqxmd.com/read/28534734/the-impact-of-single-sided-deafness-upon-music-appreciation
#3
Sarah Meehan, Elizabeth A Hough, Gemma Crundwell, Rachel Knappett, Mark Smith, David M Baguley
BACKGROUND: Many of the world's population have hearing loss in one ear; current statistics indicate that up to 10% of the population may be affected. Although the detrimental impact of bilateral hearing loss, hearing aids, and cochlear implants upon music appreciation is well recognized, studies on the influence of single-sided deafness (SSD) are sparse. PURPOSE: We sought to investigate whether a single-sided hearing loss can cause problems with music appreciation, despite normal hearing in the other ear...
May 2017: Journal of the American Academy of Audiology
https://www.readbyqxmd.com/read/28534117/a-multicenter-study-on-objective-and-subjective-benefits-with-a-transcutaneous-bone-anchored-hearing-aid-device-first-nordic-results
#4
Dan Dupont Hougaard, Soren Kjaergaard Boldsen, Anne Marie Jensen, Soren Hansen, Per Cayé Thomassen
Examination of objective as well as subjective outcomes with a new transcutaneous bone-anchored hearing aid device. The study was designed as a prospective multicenter consecutive case-series study involving tertiary referral centers at two Danish University Hospitals. A total of 23 patients were implanted. Three were lost to follow-up. Patients had single-sided deafness, conductive or mixed hearing loss. INTERVENTION: Rehabilitative. Aided and unaided sound field hearing was evaluated objectively using (1) pure warble tone thresholds, (2) pure-tone average (PTA4), (3) speech discrimination score (SDS) in quiet, and (4) speech reception threshold 50% at 70 dB SPL noise level (SRT50%)...
May 22, 2017: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/28534045/compound-heterozygous-mutations-in-masp1-in-a-deaf-child-with-absent-cochlear-nerves
#5
Elina Kari, Isabelle Schrauwen, Lorida Llaci, Laurel M Fisher, John L Go, Marcus Naymik, James A Knowles, Matthew J Huentelman, Rick A Friedman
No abstract text is available yet for this article.
June 2017: Neurology. Genetics
https://www.readbyqxmd.com/read/28534044/hsp-and-deafness-neurocristopathy-caused-by-a-novel-mosaic-sox10-mutation
#6
Sandra Donkervoort, Diana Bharucha-Goebel, Pomi Yun, Ying Hu, Payam Mohassel, Ahmet Hoke, Wadih M Zein, Daniel Ezzo, Andrea M Atherton, Ann C Modrcin, Majed Dasouki, A Reghan Foley, Carsten G Bönnemann
OBJECTIVE: To identify the underlying genetic cause in 2 sisters affected with progressive lower extremity spasticity, neuropathy, and early-onset deafness. METHODS: Whole-exome sequencing was performed, and segregation testing of variants was investigated using targeted Sanger sequencing. An inherited paternal mosaic mutation was further evaluated through quantitative analysis of the ratio of mutant vs wild-type allele in genomic DNA from various tissues, including blood, dermal fibroblasts, and saliva...
June 2017: Neurology. Genetics
https://www.readbyqxmd.com/read/28532627/regenerative-medicine-in-hearing-recovery
#7
REVIEW
Edi Simoni, Giulia Orsini, Milvia Chicca, Simone Bettini, Valeria Franceschini, Alessandro Martini, Laura Astolfi
Hearing loss, or deafness, affects 360 million people worldwide of which about 32 million are children. Deafness is irreversible when it involves sensory hair cell death because the regenerative ability of these cells is lost in mammals after embryo development. The therapeutic strategies for deafness include hearing aids and/or implantable devices. However, not all patients are eligible or truly benefit from these medical devices. Regenerative medicine based on stem cell application could play a role in both improvement of extant medical devices and in vivo recovery of auditory function by regeneration of inner ear cells and neurons...
May 19, 2017: Cytotherapy
https://www.readbyqxmd.com/read/28531343/job-satisfaction-of-teachers-of-students-who-are-deaf-or-hard-of-hearing
#8
John L Luckner, Brittany Dorn
The pressure that educators are experiencing to educate more students, with more challenges, to higher levels of learning than any time in the past is significantly changing their working conditions. The purpose of this study was to examine the perceptions of a national sample of teachers of students who are deaf or hard of hearing to ascertain their overall job satisfaction as well as to identify the specific factors that positively and negatively affect their ability to do their jobs well. In addition, responses of different subsets of teachers (e...
May 20, 2017: Journal of Deaf Studies and Deaf Education
https://www.readbyqxmd.com/read/28529479/in-patients-undergoing-cochlear-implantation-psychological-burden-affects-tinnitus-and-the-overall-outcome-of-auditory-rehabilitation
#9
Petra Brüggemann, Agnieszka J Szczepek, Katharina Klee, Stefan Gräbel, Birgit Mazurek, Heidi Olze
Cochlear implantation (CI) is increasingly being used in the auditory rehabilitation of deaf patients. Here, we investigated whether the auditory rehabilitation can be influenced by the psychological burden caused by mental conditions. Our sample included 47 patients who underwent implantation. All patients were monitored before and 6 months after CI. Auditory performance was assessed using the Oldenburg Inventory (OI) and Freiburg monosyllable (FB MS) speech discrimination test. The health-related quality of life was measured with Nijmegen Cochlear implantation Questionnaire (NCIQ) whereas tinnitus-related distress was measured with the German version of Tinnitus Questionnaire (TQ)...
2017: Frontiers in Human Neuroscience
https://www.readbyqxmd.com/read/28524840/a-novel-de-novo-gata-binding-protein-3-mutation-in-a-patient-with-hypoparathyroidism-sensorineural-deafness-and-renal-dysplasia-syndrome
#10
Xue-Ying Chu, Yue-Peng Li, Min Nie, Ou Wang, Yan Jiang, Mei Li, Wei-Bo Xia, Xiao-Ping Xing
No abstract text is available yet for this article.
June 5, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28521875/exome-sequencing-reveals-a-de-novo-pold1-mutation-causing-phenotypic-variability-in-mandibular-hypoplasia-deafness-progeroid-features-and-lipodystrophy-syndrome-mdpl
#11
Sahar Elouej, Ana Beleza-Meireles, Richard Caswell, Kevin Colclough, Sian Ellard, Jean Pierre Desvignes, Christophe Béroud, Nicolas Lévy, Shehla Mohammed, Annachiara De Sandre-Giovannoli
BACKGROUND: Mandibular hypoplasia, deafness, progeroid features, and lipodystrophy syndrome (MDPL) is an autosomal dominant systemic disorder characterized by prominent loss of subcutaneous fat, a characteristic facial appearance and metabolic abnormalities. This syndrome is caused by heterozygous de novo mutations in the POLD1 gene. To date, 19 patients with MDPL have been reported in the literature and among them 14 patients have been characterized at the molecular level. Twelve unrelated patients carried a recurrent in-frame deletion of a single codon (p...
June 2017: Metabolism: Clinical and Experimental
https://www.readbyqxmd.com/read/28520991/effects-of-a-picture-racetrack-game-on-the-expressive-vocabulary-of-deaf-preschoolers
#12
Carrie A Davenport, Sheila R Alber-Morgan, Shannon M Clancy, Michael P Kranak
This study examined the effects of a picture racetrack game on the acquisition, maintenance, and generalization of picture labeling for 2 preschool students who are deaf. The game consisted of placing photographs representing individualized target vocabulary around a racetrack board and prompting the participant to sign each photo. A multiple baseline design across picture sets demonstrated that playing the picture racetrack game was functionally related to acquisition of vocabulary to 100% mastery on at least 3 consecutive sessions for each participant...
May 18, 2017: Journal of Deaf Studies and Deaf Education
https://www.readbyqxmd.com/read/28516959/adaptive-psychological-structure-in-childhood-hearing-impairment-audiological-correlations
#13
A Serra, G Spinato, S Cocuzza, L Licciardello, P Pavone, L Maiolino
The present research deals with the clinical and social problems present during linguistic and cognitive development of deaf children. Currently, the development of Theory of Mind represents an important research field in deafness studies. These international studies highlighted a significant alteration in the development of Theory of Mind in deaf children compared to normal hearing children, especially in cases of congenital or preverbal hearing loss. In particular, the research focuses on the skills of deaf children in recognising emotions and desires, through both perceptive and cognitive methods, by evaluation of psycho-cognitive skills of children with severe hearing loss using a set of questions to be administered to hearing loss patients...
June 2017: Acta Otorhinolaryngologica Italica
https://www.readbyqxmd.com/read/28516283/developmental-window-of-sensorineural-deafness-in-biotinidase-deficient-mice
#14
Kathleen June Maheras, Kirit Pindolia, Barry Wolf, Alexander Gow
Biotinidase deficiency is an autosomal recessively inherited disorder that results in the inability to recycle the vitamin, biotin. If untreated, the disorder can result in a range of neurological and cutaneous symptoms, including sensorineural deficits and deafness. To understand early mechanistic abnormalities that may precede more generalized and nonspecific effects of metabolic deficits such as weight loss and acidosis, we have analyzed auditory brainstem responses (ABRs) in biotinidase-deficient knockout (Btd (-/-) ) mice in the periweaning period with or without dietary biotin supplementation...
May 17, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28511890/intratympanic-steroid-delivery-by-an-indwelling-catheter-in-refractory-severe-sudden-sensorineural-hearing-loss
#15
Diego Zanetti, Federica Di Berardino, Nader Nassif, Luca Oscar Redaelli De Zinis
OBJECTIVE: Many studies over the last decade showed favorable outcomes with intratympanic (IT) steroid treatment, alone as salvage treatment or in combination with conventional systemic therapy (ST). However, in severe to profound sensorineural hearing loss resistant to ST, the optimal infusion mode, the type and concentration of the solution, the preferable drug, its total amount, and the duration and fractionation of the treatment are still debated. Aim of the study was to investigate the feasibility and the outcomes of a direct and constant IT delivery of dexamethasone (DEX) by means of a new indwelling catheter...
May 13, 2017: Auris, Nasus, Larynx
https://www.readbyqxmd.com/read/28510511/corrigendum
#16
(no author information available yet)
Birkeland AC, Owen JH, Prince ME. 2015. Targeting head and neck cancer stem cells: current advances and future challenges. J Dent Res. 94(11):1516-1523. (Original DOI: 10.1177/0022034515601960 ) In this article, the funding source was not declared. The Acknowledgments have been updated in the online article as follows: Andrew Birkeland is a Research Fellow funded on a T32 Advanced Research Training in Otolaryngology Program Training Grant (T32 DC005356) funded by the National Institutes of Health NIDCD (National Institute On Deafness And Other Communication Disorders)...
May 1, 2017: Journal of Dental Research
https://www.readbyqxmd.com/read/28506442/boosting-syntax-training-with-temporally-regular-musical-primes-in-children-with-cochlear-implants
#17
N Bedoin, A-M Besombes, E Escande, A Dumont, P Lalitte, B Tillmann
OBJECTIVES: Previous research has suggested the use of rhythmic structures (implemented in musical material) to improve linguistic structure processing (i.e., syntax processing), in particular for populations showing deficits in syntax and temporal processing (e.g., children with developmental language disorders). The present study proposes a long-term training program to improve syntax processing in children with cochlear implants, a population showing syntax processing deficits in perception and production...
May 11, 2017: Annals of Physical and Rehabilitation Medicine
https://www.readbyqxmd.com/read/28505178/a-reverse-dot-blot-assay-for-the-screening-of-twenty-mutations-in-four-genes-associated-with-nshl-in-a-chinese-population
#18
Siping Li, Qi Peng, Shengyun Liao, Wenrui Li, Qiang Ma, Xiaomei Lu
BACKGROUND: Congenital deafness is one of the most distressing disorders affecting humanity and exhibits a high incidence worldwide. Most cases of congenital deafness in the Chinese population are caused by defects in a limited number of genes. A convenient and reliable method for detecting common deafness-related gene mutations in the Chinese population is required. METHODS: We developed a PCR-reverse dot blot (RDB) assay for screening 20 hotspot mutations of GJB2, GJB3, SLC26A4, and MT-RNR1, which are common non-syndromic hearing loss (NSHL)-associated genes in the Chinese population...
2017: PloS One
https://www.readbyqxmd.com/read/28504979/congenital-deafness-alters-sensory-weighting-for-postural-control
#19
Maxime Maheu, Andréanne Sharp, Sara Pagé, François Champoux
OBJECTIVES: The aim of this study was to examine sensory reweighting for postural control in congenitally deaf individuals. DESIGN: We studied 14 controls and 14 deaf age-matched individuals using a force platform and the modified clinical test of sensory interaction and balance protocol. Both groups performed the postural tasks without auditory cues (with hearing protectors for controls or without hearing devices for the deaf). RESULTS: The results confirmed poorer postural stability in the deaf...
May 12, 2017: Ear and Hearing
https://www.readbyqxmd.com/read/28504928/the-murine-catecholamine-methyltransferase-mtomt-is-essential-for-mechanotransduction-by-cochlear-hair-cells
#20
Christopher L Cunningham, Zizhen Wu, Aria Jafari, Bo Zhao, Kat Schrode, Sarah Harkins-Perry, Amanda Lauer, Ulrich Mueller
Hair cells of the cochlea are mechanosensors for the perception of sound. Mutations in the LRTOMT gene, which encodes a protein with homology to the catecholamine methyltransferase COMT that is linked to schizophrenia, cause deafness. Here we show that Tomt/Comt2, the murine ortholog of LRTOMT, has an unexpected function in the regulation of mechanotransduction by hair cells. The role of mTOMT in hair cells is independent of mTOMT methyltransferase function and mCOMT cannot substitute for mTOMT function. Instead, mTOMT binds to putative components of the mechanotransduction channel in hair cells and is essential for the transport of some of these components into the mechanically sensitive stereocilia of hair cells...
May 15, 2017: ELife
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