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https://www.readbyqxmd.com/read/28651255/visual-cues-contribute-differentially-to-audiovisual-perception-of-consonants-and-vowels-in-improving-recognition-and-reducing-cognitive-demands-in-listeners-with-hearing-impairment-using-hearing-aids
#1
Shahram Moradi, Björn Lidestam, Henrik Danielsson, Elaine Hoi Ning Ng, Jerker Rönnberg
Purpose: We sought to examine the contribution of visual cues in audiovisual identification of consonants and vowels-in terms of isolation points (the shortest time required for correct identification of a speech stimulus), accuracy, and cognitive demands-in listeners with hearing impairment using hearing aids. Method: The study comprised 199 participants with hearing impairment (mean age = 61.1 years) with bilateral, symmetrical, mild-to-severe sensorineural hearing loss...
June 23, 2017: Journal of Speech, Language, and Hearing Research: JSLHR
https://www.readbyqxmd.com/read/28650353/the-effect-of-hearing-loss-on-novel-word-learning-in-infant-and-adult-directed-speech
#2
V Susie Robertson, Deborah von Hapsburg, Jessica S Hay
OBJECTIVES: Relatively little is known about how young children with hearing impairment (HI) learn novel words in infant- and adult-directed speech (ADS). Infant-directed speech (IDS) supports word learning in typically developing infants relative to ADS. This study examined how children with normal hearing (NH) and children with HI learn novel words in IDS and ADS. It was predicted that IDS would support novel word learning in both groups of children. In addition, children with HI were expected to be less proficient word learners as compared with their NH peers...
June 22, 2017: Ear and Hearing
https://www.readbyqxmd.com/read/28649563/symptomatic-congenital-cytomegalovirus-infection-in-children-of-seropositive-women
#3
Ines Mack, Marie-Anne Burckhardt, Ulrich Heininger, Friederike Prüfer, Sven Schulzke, Sven Wellmann
Cytomegalovirus (CMV) is the most frequent congenital virus infection worldwide. The risk of congenital CMV (cCMV) transmission is highest in seronegative women who acquire primary CMV infection during pregnancy. A growing body of evidence indicates that secondary CMV infections in pregnant women with preconceptual immunity (either through reactivation of latent virus or re-infection with a new strain of CMV) contribute to a much greater proportion of symptomatic cCMV than was previously thought. Here, we describe a case of symptomatic cCMV infection in the newborn of a woman with proven immunity prior to pregnancy...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28648811/a-case-of-improved-hearing-with-cochlear-implantation-in-gaucher-disease-type-1
#4
Shiori Endo, Kunihiro Mizuta, Takashi Yamatodani, Hiroshi Nakanishi, Kumiko Hosokawa, Kiyoshi Misawa, Seiji Hosokawa, Hiroyuki Mineta
Gaucher disease is a lysosomal storage disorder that is caused by congenital defective function of the enzyme glucocerebrosidase. Glucocerebroside that is not hydrolyzed by glucocerebrosidase mainly accumulates in the reticular tissue. We describe a Japanese boy with Gaucher disease type 1 who developed bilateral profound sensorineural hearing loss within approximately 4years. We performed cochlear implantation initially on his right ear and again on his left ear 5 months later. The cochlear implants were successfully utilized with a speech discrimination score of 95% on a Japanese sentence recognition test...
June 22, 2017: Auris, Nasus, Larynx
https://www.readbyqxmd.com/read/28648633/identification-of-evidence-for-autoimmune-pathology-of-bilateral-sudden-sensorineural-hearing-loss-using-proteomic-analysis
#5
Jeon Mi Lee, Jin Young Kim, Jinwoong Bok, Kyu-Sung Kim, Jae Young Choi, Sung Huhn Kim
Sudden sensorineural hearing loss (S-SNHL) is an inner ear disorder with an abrupt hearing loss occurring <3days. The pathologic mechanism of the disease remains unclear, although autoimmunity has been regarded as one of the suggested causes, especially in bilateral form. In this study, we aimed to provide evidence for the involvement of autoimmunity in bilateral S-SNHL using proteomic approaches such as ProtoArray®, western blotting, immunoprecipitation, and liquid column mass spectrometry for mass screening of candidate antigens and autoantibodies based on the hypothesis that multiple autoantibodies and target antigens must exist in order for autoimmune bilateral S-SNHL to develop...
June 22, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28648509/pendred-syndrome
#6
REVIEW
Jean-Louis Wémeau, Peter Kopp
Pendred syndrome is an autosomal recessive disorder that is classically defined by the combination of sensorineural deafness/hearing impairment, goiter, and an abnormal organification of iodide with or without hypothyroidism. The hallmark of the syndrome is the impaired hearing, which is associated with inner ear malformations such as an enlarged vestibular aqueduct (EVA). The thyroid phenotype is variable and may be modified by the nutritional iodine intake. Pendred syndrome is caused by biallelic mutations in the SLC26A4/PDS gene, which encodes the multifunctional anion exchanger pendrin...
March 2017: Best Practice & Research. Clinical Endocrinology & Metabolism
https://www.readbyqxmd.com/read/28647130/fever-induced-paroxysmal-weakness-and-encephalopathy-a-new-phenotype-of-atp1a3-mutation
#7
Sho T Yano, Kenneth Silver, Richard Young, Suzanne D DeBrosse, Roseànne S Ebel, Kathryn J Swoboda, Gyula Acsadi
BACKGROUND: We identified a group of patients with ATP1A3 mutations at residue 756 who display a new phenotype, distinct from alternating hemiplegia of childhood, rapid-onset dystonia-parkinsonism, and cerebellar ataxia, areflexia, pes cavus, optic atrophy, sensorineural hearing loss syndromes. METHODS: Four patients with c.2267G>A (R756H) mutations from two families and two patients with c.2267G>T (R756L) mutations from one family are described and compared with the previously reported patients with mutations resulting in R756H and R756C protein variants...
April 29, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28646968/congenital-cytomegalovirus-who-when-what-with-and-why-to-treat
#8
Yinru Lim, Hermione Lyall
Congenital cytomegalovirus (CMV) is the commonest cause of congenital infection worldwide and the leading non-genetic cause of sensorineural hearing loss in children. Appropriate investigations and timely decision on treatment is required as studies have shown that treatment with antiviral therapy leads to improved hearing and neurodevelopmental outcomes in the long term when started in the first month of life. This paper outlines the epidemiology, investigations in the diagnosis of congenital CMV infection and current evidence surrounding treatment...
June 2017: Journal of Infection
https://www.readbyqxmd.com/read/28646272/rhesus-cochlear-and-vestibular-functions-are-preserved-after-inner-ear-injection-of-saline-volume-sufficient-for-gene-therapy-delivery
#9
Chenkai Dai, Mohamed Lehar, Daniel Q Sun, Lani Swarthout Rvt, John P Carey, Tim MacLachlan, Doug Brough, Hinrich Staecker, Alexandra M Della Santina, Timothy E Hullar, Charles C Della Santina
Sensorineural losses of hearing and vestibular sensation due to hair cell dysfunction are among the most common disabilities. Recent preclinical research demonstrates that treatment of the inner ear with a variety of compounds, including gene therapy agents, may elicit regeneration and/or repair of hair cells in animals exposed to ototoxic medications or other insults to the inner ear. Delivery of gene therapy may also offer a means for treatment of hereditary hearing loss. However, injection of a fluid volume sufficient to deliver an adequate dose of a pharmacologic agent could, in theory, cause inner ear trauma that compromises functional outcome...
June 23, 2017: Journal of the Association for Research in Otolaryngology: JARO
https://www.readbyqxmd.com/read/28644112/susac-syndrome-misdiagnosed-as-multiple-sclerosis-with-exacerbation-by-interferon-beta-therapy
#10
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
#11
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/28642064/the-genetic-basis-of-deafness-in-populations-of-african-descent
#12
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/28639554/evaluation-of-procalcitonin-and-hs-crp-levels-in-sudden-sensorineural-hearing-loss
#13
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/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/28635225/-modification-factors-associated-with-maternally-inherited-non-syndromic-hearing-loss
#15
W J Hong, B J Zheng, J F Qian, H Wu, H Jin, Y T Zhu
Mutations in the mitochondrial DNA have been certified to be one of the most important causes of maternally inherited sensorineural hearing loss. Among these, mitochondrial 12S rRNA1555A>G, 1494C>T and other mutations are associated with both nonsyndromic and drug induced hearing loss caused by aminoglycosides. Individuals carrying 1555A>G or 1494C>T mutation have a variety of clinical manifestations, which implies that the 1555A>G or 1494C>T mutation is a chief factor underlying the development of deafness but insufficient to produce the clinical phenotype...
June 7, 2017: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
https://www.readbyqxmd.com/read/28631161/the-role-of-eabr-with-intracochlear-test-electrode-in-decision-making-between-cochlear-and-brainstem-implants-preliminary-results
#16
Betul Cicek Cinar, Mehmet Yarali, Gamze Atay, Munir Demir Bajin, Gonca Sennaroglu, Levent Sennaroglu
The objective of the study was to discuss the findings of intraoperative electrically evoked auditory brainstem response (eABR) test results with a recently designed intracochlear test electrode (ITE) in terms of their relation to decisions of cochlear or auditory brainstem implantation. This clinical study was conducted in Hacettepe University, Department of Otolaryngology, Head and Neck Surgery and Department of Audiology. Subjects were selected from inner ear malformation (IEM) database. Eleven subjects with profound sensorineural hearing loss were included in the current study with age range from 1 year 3 months to 4 years 3 months for children with prelingual hearing loss...
June 19, 2017: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/28630884/evaluation-of-a-high-resolution-patient-specific-model-of-the-electrically-stimulated-cochlea
#17
Ahmet Cakir, Robert T Dwyer, Jack H Noble
Cochlear implants (CIs) are surgically implanted medical devices used to treat individuals with severe-to-profound sensorineural hearing loss. Although these devices have been remarkably successful at restoring audibility, many patients experience poor outcomes. Our group has developed the first image-guided CI programming technique where the electrode positions are found in CT images and used to estimate neural activation patterns, which is unique information that audiologists can use to define patient-specific processor settings...
April 2017: Journal of Medical Imaging
https://www.readbyqxmd.com/read/28629849/analysis-of-archived-newborn-dried-blood-spots-dbs-identifies-congenital-cytomegalovirus-as-a-major-cause-of-unexplained-pediatric-sensorineural-hearing-loss
#18
Lucy Meyer, Bazak Sharon, Tina C Huang, Abby C Meyer, Kristin E Gravel, Lisa A Schimmenti, Elizabeth C Swanson, Hannah E Herd, Nelmary Hernandez-Alvarado, Kirsten R Coverstone, Mark McCann, Mark R Schleiss
PURPOSE: Congenital cytomegalovirus (cCMV) infection is the most common non-genetic cause of sensorineural hearing loss (SNHL). However, accurate diagnosis of cCMV as the etiology of SNHL is problematic beyond the neonatal period. This study therefore examined whether cCMV infection could be identified retrospectively in children presenting with unexplained SNHL to a multidisciplinary diagnostic outpatient otolaryngology clinic at an academic medical center in Minnesota. METHODS: Over a 4-year period, 57 patients with an age range of 3months to 10years with unexplained SNHL were recruited to participate in this study...
June 7, 2017: American Journal of Otolaryngology
https://www.readbyqxmd.com/read/28627931/hyperacoustic-hypoacusis-a-new-pontine-syndrome-case-report
#19
Mohammad Hassan A Noureldine, Raghid Kikano, Naji Riachi, Rechdi Ahdab
We herein report a unique case of a lateral pontine demyelinating lesion presenting with unilateral sensorineural hearing loss and paradoxical ipsilateral hyperacusis. The association of unilateral hearing loss and ipsilateral hyperacusis is a rare manifestation of a central nervous system lesion. The paradoxical combination of these symptoms strongly suggests pontine dysfunction and prompts urgent neurological evaluation.
June 19, 2017: Brain Injury: [BI]
https://www.readbyqxmd.com/read/28624835/cbct-location-of-the-fusion-between-the-buccal-and-lingual-cortical-in-the-mandibular-ramus-importance-to-sagittal-split-osteotomy
#20
L Scomparin, M-Q-S Soares, C-M-F Rubira, R-Y-F Yaedú, T-S-N Imada, B-S Centurion, E-S Tolentino, J-R-P Lauris, I-R-F Rubira-Bullen
BACKGROUND: Mandibular Sagittal Split Osteotomy (MSSO) is a popular technique in orthognathic surgery used both to advance and to retreat the mandible. However, MSSO may incur in important complications, such as bad splits and sensorineural injuries. Knowing the location of the fusion between the buccal and lingual cortical (FBLC) in the mandibular ramus and the bone thickness in the region where osteotomies will be performed is determinant in MSSO planning to avoid complications. The aim of this study was to document and evaluate possible differences between sexes regarding the location of the FBLC in relation to the superior cortical of mandibular foramen (MF) and bone thickness in the region of interest for MSSO in a Brazilian population...
June 18, 2017: Medicina Oral, Patología Oral y Cirugía Bucal
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