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https://www.readbyqxmd.com/read/29774994/-comparative-study-of-intratympanic-dexamethasone-injection-for-sudden-deafness-at-different-time-intervals
#1
P L Yang, Z P Zhang
Objective: Comparative study of intratympanic Dexamethasone injection for sudden deafness at different time intervals. Method: One hundred and sixty cases which had been diagnosed sudden deafness were treated by vasodilatortrophic nerve drugs and high pressure oxygen and the same time dexamethasone injective in the middle ear. Injection time intervals were divided into qd, qod, and twice a week. Contral group is not use dexamethasone. Result: The cure rate of qd and qod groups were higher than twice a week group, and the difference was statistically significant ( P <0...
June 5, 2017: Journal of Clinical Otorhinolaryngology, Head, and Neck Surgery
https://www.readbyqxmd.com/read/29774890/genetic-and-clinical-aspects-of-wolfram-syndrome-1-a-severe-neurodegenerative-disease
#2
REVIEW
Luciana Rigoli, Placido Bramanti, Chiara Di Bella, Filippo De Luca
Wolfram syndrome 1 (WS1) is a rare autosomal recessive neurodegenerative disease characterized by diabetes insipidus, diabetes mellitus, optic atrophy, deafness, and other abnormalities. WS1 usually results in death before the age of 50 years. The pathogenesis of WS1 is ascribed to mutations of human WFS1 gene on chromosome 4p encoding a transmembrane protein called wolframin which has physiological functions in membrane trafficking, secretion, processing, and/or regulation of ER calcium homeostasis. Different types of WFS1 mutations have been identified, and some of these have been associated with a dominant, severe type of WS...
May 2018: Pediatric Research
https://www.readbyqxmd.com/read/29773983/somatosensory-cross-modal-reorganization-in-adults-with-age-related-early-stage-hearing-loss
#3
Garrett Cardon, Anu Sharma
Under conditions of profound sensory deprivation, the brain has the propensity to reorganize. For example, intact sensory modalities often recruit deficient modalities' cortices for neural processing. This process is known as cross-modal reorganization and has been shown in congenitally and profoundly deaf patients. However, much less is known about cross-modal cortical reorganization in persons with less severe cases of age-related hearing loss (ARHL), even though such cases are far more common. Thus, we investigated cross-modal reorganization between the auditory and somatosensory modalities in older adults with normal hearing (NH) and mild-moderate ARHL in response to vibrotactile stimulation using high density electroencephalography (EEG)...
2018: Frontiers in Human Neuroscience
https://www.readbyqxmd.com/read/29772954/electric-acoustic-stimulation-suppresses-tinnitus-in-a-subject-with-high-frequency-single-sided-deafness
#4
Griet Mertens, Vincent Van Rompaey, Paul Van de Heyning
BACKGROUND: A suggested solution to suppress tinnitus is to restore the normal sensory input. This is based on the auditory deprivation hypothesis. It is known that hearing aids can provide sufficient activation of the auditory nervous system and reduce tinnitus in subjects with mild to moderate hearing loss and that cochlear implantation can reduce tinnitus in subjects with severe to profound hearing loss. This applies to subjects with single-sided deafness (SSD) or bilateral hearing loss...
May 17, 2018: Cochlear Implants International
https://www.readbyqxmd.com/read/29771080/-analysis-of-related-factors-of-coins-foreign-bodies-crossing-the-esophagus-in-204-cases-of-children
#5
M M Ni, L Liu, R Zhao, M Nie, X H Yang
Objective: To evaluate the effect of systemic and intratympanic dexamethasone combination therapy on sudden deafness. Method: A total of 98 patients randomly divided into three groups: an intravenous corticosteroid (systemic corticosteroid monotherapy group); an intratympanic corticosteroid (IT monotherapy group); and a combination therapy group (IT plus systemic combination group). All patients were treated additionally with ginkgo-damole and mecobalamine and proton pomp inhibitor. An audiometry was performed before and after the therapies in all patients...
May 2018: Journal of Clinical Otorhinolaryngology, Head, and Neck Surgery
https://www.readbyqxmd.com/read/29771077/-observation-of-flupentixol-and-melitracen-on-curative-effect-of-sudden-deafness-patients-with-anxiety-and-depression-in-different-stages
#6
T Zhang, G D Yu
Objective: To investigate the efficacy of flupentixol melitracen on different stages of sudden deafness patients with anxiety and depression. Method: Totally one hundred and sixty-three sudden deafness patients with anxiety and depression were randomly divided into two groups: experimental group(81 cases) and control group (82 cases). All patients were given routine treatment for 2 weeks. The experimental group was given oral flupentixol melitracen (1 tablet/day)for a period of 3 months. The hearing, tinnitus effect and vertigo treatment course were observed and compared...
May 2018: Journal of Clinical Otorhinolaryngology, Head, and Neck Surgery
https://www.readbyqxmd.com/read/29770645/atoh1-regulation-in-the-cochlea-more-than-just-transcription
#7
REVIEW
Yen-Fu Cheng
More than 80% of all cases of deafness are related to the death or degeneration of cochlear hair cells and the associated spiral ganglion neurons, and a lack of regeneration of these cells leads to permanent hearing loss. Therefore, the regeneration of lost hair cells is an important goal for the treatment of deafness. Atoh1 is a basic helix-loop-helix (bHLH) transcription factor that is critical in both the development and regeneration of cochlear hair cells. Atoh1 is transcriptionally regulated by several signaling pathways, including Notch and Wnt signalings...
July 13, 2017: Journal of Zhejiang University. Science. B
https://www.readbyqxmd.com/read/29768731/cochlear-implant-performance-in-children-deafened-by-congenital-cytomegalovirus-a-systematic-review
#8
V J C Kraaijenga, F Van Houwelingen, S F Van der Horst, J Visscher, J M L Huisman, E J Hollman, I Stegeman, A L Smit
BACKGROUND: Congenital cytomegalovirus (cCMV) infection is a major cause of sensorineural hearing loss in children. OBJECTIVE OF REVIEW: The objective of this systematic review was to compare performance in pediatric cochlear implant users with SNHL caused by cCMV compared to non-cCMV implantees. TYPE OF REVIEW: Systematic review. SEARCH STRATEGY: Pubmed, EMBASE and the Cochrane databases were searched from inception up to May 15th 2017 for children, cochlear implant, performance and their synonyms...
May 16, 2018: Clinical Otolaryngology
https://www.readbyqxmd.com/read/29768325/is-nucleate-cell-count-and-neutrophil-to-lymphocyte-ratio-related-to-patients-with-audiographically-distinct-sudden-sensorineural-hearing-loss
#9
Yuanyuan Sun, Liang Xia, Hui Wang, Zhengnong Chen, Yaqin Wu, Bin Chen, Haibo Shi, Yanmei Feng, Shankai Yin
The aim of this study was to investigate the possible relationships between sudden sensorineural hearing loss (SSNHL) patients with distinct audiographic data and nucleate cell count and neutrophil to lymphocyte ratio (NLR).SSNHL patients differed in audiographic curves were included, 40 with low-frequency SSNHL (LF-SSNHL), 33 with high-frequency SSNHL (HF-SSNHL), 36 with all-frequency SSNHL (AF-SSNHL), 34 with total-deafness SSNHL (TD-SSNHL), and 31 age- and sex-matched healthy controls. Peripheral venous blood samples were collected and nucleate cell such as white blood cell (WBC), neutrophil and lymphocyte, and NLR were measured...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29767748/glial-activation-and-central-synapse-loss-but-not-motoneuron-degeneration-are-prevented-by-the-sigma-1-receptor-agonist-pre-084-in-the-smn2b-mouse-model-of-spinal-muscular-atrophy
#10
Clàudia Cerveró, Alba Blasco, Olga Tarabal, Anna Casanovas, Lídia Piedrafita, Xavier Navarro, Josep E Esquerda, Jordi Calderó
Spinal muscular atrophy (SMA) is characterized by the loss of α-motoneurons (MNs) with concomitant muscle denervation. MN excitability and vulnerability to disease are particularly regulated by cholinergic synaptic afferents (C-boutons), in which Sigma-1 receptor (Sig1R) is concentrated. Alterations in Sig1R have been associated with MN degeneration. Here, we investigated whether a chronic treatment with the Sig1R agonist PRE-084 was able to exert beneficial effects on SMA. We used a model of intermediate SMA, the Smn2B/- mouse, in which we performed a detailed characterization of the histopathological changes that occur throughout the disease...
May 14, 2018: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/29767737/recent-issues-in-the-use-of-signed-language-assessments-for-diagnosis-of-language-disorders-in-signing-deaf-and-hard-of-hearing-children
#11
Jon Henner, Rama Novogrodsky, Jeanne Reis, Robert Hoffmeister
In recent years, normed signed language assessments have become a useful tool for researchers, practitioners, and advocates. Nevertheless, there are limitations in their application, particularly for the diagnosis of language disorders, and learning disabilities. Here, we discuss some of the available normed, signed language assessments and some of their limitations. We have also provided information related to practices that should lead to improvement in the quality of signed language assessments.
May 15, 2018: Journal of Deaf Studies and Deaf Education
https://www.readbyqxmd.com/read/29766469/proteinuric-glomerulopathy-in-an-adolescent-with-a-distal-partial-trisomy-chromosome-1
#12
Takaya Sasaki, Masahiro Okabe, Takeshi Tosaki, Yu Honda, Masahiro Ishikawa, Nobuo Tsuboi, Takashi Yokoo
We report a case of distal partial trisomy 1 from q32.1 to 41 that have exhibited proteinuric glomerulopathy. The patient was a 17-year-old adolescent with clinical features of low birth weight, mild mental retardation and mild deafness, from the birth. He exhibited non-nephrotic range proteinuria with the mild obesity since the age of sixteen. Image studies did not reveal morphological abnormalities of the kidneys. Renal biopsy findings showed no definitive evidence of primary glomerular diseases, and were characterized by a very low glomerular density, glomerulomegaly and focal effacement of podocyte foot processes...
May 16, 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29764576/-nipbl-gene-mutations-in-two-children-with-cornelia-de-lange-syndrome
#13
Yun-Jing Zhao, Hong-Wei Ma
Both children (one boy and one girl) experienced disease onset in infancy and visited the hospital due to growth retardation. They had unusual facies including thick hair, arched and confluent eyebrows, long and curly eyelashes, short nose, and micrognathia. Patient 1 had congenital heart disease (atrial septal defect and pulmonary stenosis) and special dermatoglyph (a single palmar crease). Patient 2 had cleft palate and moderate-to-severe deafness. Clinical features suggested Cornelia de Lange syndrome in both children...
May 2018: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/29761951/-research-on-cortical-cross-modal-reorganization-of-children-with-congenital-severe-deafness-after-cochlear-implant
#14
Xianjun Wang, Maojin Liang, Junpeng Zhang, Hua Huang, Yiqing Zheng
Cochlear implant (CI) is the only method for efficacious treatment of congenital severe deafness at present. However, for children with congenital severe deafness after CI, the mechanism of the structural and functional changes of their cerebral cortex is not clear. This study was based on the cross-modal reorganization of deaf patients. Event related potential (ERP) and source localization technique were used to visualize the change of cortical activity in children with congenital severe deafness during 1-year period (0, 1, 3, 6, 9 and 12 months after CI)...
October 1, 2017: Sheng Wu Yi Xue Gong Cheng Xue za Zhi, Journal of Biomedical Engineering, Shengwu Yixue Gongchengxue Zazhi
https://www.readbyqxmd.com/read/29761835/attention-to-speech-and-spoken-language-development-in-deaf-children-with-cochlear-implants-a-10-year-longitudinal-study
#15
Yuanyuan Wang, Carissa L Shafto, Derek M Houston
Early auditory/language experience plays an important role in language development. In this study, we examined the effects of severe-to-profound hearing loss and subsequent cochlear implantation on the development of attention to speech in children with cochlear implants (CIs). In addition, we investigated the extent to which attention to speech may predict spoken language development in children with CIs. We tested children with CIs and compared them to chronologically age-matched peers with normal hearing (NH) on their attention to speech at four time points post implantation; specifically, less than 1 month, 3 to 6 months, 12 months, and 18 months post implantation...
May 15, 2018: Developmental Science
https://www.readbyqxmd.com/read/29761735/spatial-release-from-masking-in-2-year-olds-with-normal-hearing-and-with-bilateral-cochlear-implants
#16
Christi L Hess, Sara M Misurelli, Ruth Y Litovsky
This study evaluated spatial release from masking (SRM) in 2- to 3-year-old children who are deaf and were implanted with bilateral cochlear implants (BiCIs), and in age-matched normal-hearing (NH) toddlers. Here, we examined whether early activation of bilateral hearing has the potential to promote SRM that is similar to age-matched NH children. Listeners were 13 NH toddlers and 13 toddlers with BiCIs, ages 27 to 36 months. Speech reception thresholds (SRTs) were measured for target speech in front (0°) and for competitors that were either Colocated in front (0°) or Separated toward the right (+90°)...
January 2018: Trends in Hearing
https://www.readbyqxmd.com/read/29760175/modulation-of-receptor-protein-tyrosine-phosphatase-sigma-increases-chondroitin-sulfate-proteoglycan-degradation-through-cathepsin-b-secretion-to-enhance-axon-outgrowth
#17
Amanda Phuong Tran, Sapna Sundar, Meigen Yu, Bradley T Lang, Jerry Silver
Severed axon tips reform growth cones following spinal cord injury that fail to regenerate, in part, because they become embedded within an inhibitory extracellular matrix. Chondroitin sulfate proteoglycans (CSPGs) are the major axon inhibitory matrix component that is increased within the lesion scar and in perineuronal nets around deafferented neurons. We have recently developed a novel peptide modulator (Intracellular Sigma Peptide or ISP) of the cognate receptor of CSPGs, protein tyrosine phosphatase sigma (RPTPσ), which has been shown to markedly improve sensorimotor function, micturition, and coordinated locomotor behavior in spinal cord contused rats...
May 14, 2018: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/29754738/psychological-follow-up-care-of-neurofibromatosis-type-2-patients-and-their-relatives
#18
REVIEW
C Carillo, M Kiening, S Bergheimer, M Kalamarides
Neurofibromatosis type 2 (NF2) confronts patients and their relatives with the dual issue of a progressive disease and disability. Deafness creates a brutal rupture in the patients' course of life, and other disabilities often follow in addition, that further deteriorates their quality of life. Hearing rehabilitation, via a cochlear implant and auditory brainstem implant, attempts to reduce the feeling of isolation and suffering due to communication impairment. A NF2-specific quality of life questionnaire not only helps to evaluate the impact of the disease but it is also useful therapy proposals (treatment, auditory implants)...
May 10, 2018: Neuro-Chirurgie
https://www.readbyqxmd.com/read/29754270/heterozygous-missense-variants-of-lmx1a-lead-to-nonsyndromic-hearing-impairment-and-vestibular-dysfunction
#19
Mieke Wesdorp, Pia A M de Koning Gans, Margit Schraders, Jaap Oostrik, Martijn A Huynen, Hanka Venselaar, Andy J Beynon, Judith van Gaalen, Vitória Piai, Nicol Voermans, Michelle M van Rossum, Bas P Hartel, Stefan H Lelieveld, Laurens Wiel, Berit Verbist, Liselotte J Rotteveel, Marieke F van Dooren, Peter Lichtner, Henricus P M Kunst, Ilse Feenstra, Ronald J C Admiraal, Helger G Yntema, Lies H Hoefsloot, Ronald J E Pennings, Hannie Kremer
Unraveling the causes and pathomechanisms of progressive disorders is essential for the development of therapeutic strategies. Here, we identified heterozygous pathogenic missense variants of LMX1A in two families of Dutch origin with progressive nonsyndromic hearing impairment (HI), using whole exome sequencing. One variant, c.721G > C (p.Val241Leu), occurred de novo and is predicted to affect the homeodomain of LMX1A, which is essential for DNA binding. The second variant, c.290G > C (p.Cys97Ser), predicted to affect a zinc-binding residue of the second LIM domain that is involved in protein-protein interactions...
May 12, 2018: Human Genetics
https://www.readbyqxmd.com/read/29752989/a-novel-pathogenic-variant-in-the-marveld2-gene-causes-autosomal-recessive-non-syndromic-hearing-loss-in-an-iranian-family
#20
Afsaneh Taghipour-Sheshdeh, Fatemeh Nemati-Zargaran, Narges Zarepour, Parisa Tahmasebi, Nader Saki, Mohammad Amin Tabatabaiefar, Javad Mohammadi-Asl, Morteza Hashemzadeh-Chaleshtori
BACKGROUND AND AIMS: Hearing loss (HL) is the most common sensorineural disorder and one of the most common human defects. HL can be classified according to main criteria, including: the site (conductive, sensorineural and mixed), onset (pre-lingual and post-lingual), accompanying signs and symptoms (syndromic and non-syndromic), severity (mild, moderate, severe and profound) and mode of inheritance (Autosomal recessive, autosomal dominant, X-linked and mitochondrial). Autosomal recessive non-syndromic HL (ARNSHL) forms constitute a major share of the HL cases...
May 9, 2018: Genomics
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