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Sensorineural hearing loss

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https://www.readbyqxmd.com/read/28534734/the-impact-of-single-sided-deafness-upon-music-appreciation
#1
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/28534732/evaluation-of-adaptive-noise-management-technologies-for-school-age-children-with-hearing-loss
#2
Jace Wolfe, Mila Duke, Erin Schafer, Christine Jones, Lori Rakita
BACKGROUND: Children with hearing loss experience significant difficulty understanding speech in noisy and reverberant situations. Adaptive noise management technologies, such as fully adaptive directional microphones and digital noise reduction, have the potential to improve communication in noise for children with hearing aids. However, there are no published studies evaluating the potential benefits children receive from the use of adaptive noise management technologies in simulated real-world environments as well as in daily situations...
May 2017: Journal of the American Academy of Audiology
https://www.readbyqxmd.com/read/28534728/hearing-loss-and-age-induced-changes-in-the-central-auditory-system-measured-by-the-p3-response-to-small-changes-in-frequency
#3
Kathy R Vander Werff, Kerrie L Nesbitt
BACKGROUND: Recent behavioral studies have suggested that individuals with sloping audiograms exhibit localized improvements in frequency discrimination in the frequency region near the drop in hearing. Auditory-evoked potentials may provide evidence of such cortical plasticity and reorganization of frequency maps. PURPOSE: The objective of this study was to evaluate electrophysiological evidence of cortical plasticity related to cortical frequency representation and discrimination abilities in older individuals with high-frequency sensorineural hearing loss (SNHL)...
May 2017: Journal of the American Academy of Audiology
https://www.readbyqxmd.com/read/28532971/clinical-and-audiological-characteristics-of-1000hz-audiometric-notch-patients
#4
Hyun-Min Lee, Seok-Hyun Kim, Jae-Hwan Jung, Se-Joon Oh, Soo-Keun Kong, Il-Woo Lee
PURPOSE: Among the hearing loss patients, we can confirm that the hearing loss of the specific frequency decreases, such as the 2000Hz notch in otosclerosis and the 4000Hz notch (c5-dip) in noise-induced hearing loss. The 1000Hz notch (c3-dip), however, is rarely studied. We fortuitously encountered a group of patients with a 1kHz hearing loss and report it with a review of the literature. METHODS: Otological history, audiogram, diagnosis, occupation, and history of noise exposure were reviewed from charts and telephone interview, and compared between c3-dip and c5-dip patients (n=98)...
April 1, 2017: American Journal of Otolaryngology
https://www.readbyqxmd.com/read/28532627/regenerative-medicine-in-hearing-recovery
#5
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/28530253/sensitivity-and-specificity-of-vestibular-bedside-examination-in-detecting-viii-cranial-nerve-schwannoma-with-sensorineural-sudden-unilateral-hearing-loss-as-presenting-symptom
#6
L Califano, F Salafia, M G Melillo, S Mazzone
The objectives of this study were to identify signs of vestibular nerve suffering through a bedside vestibular examination protocol in case of sudden sensorineural unilateral hearing loss without spontaneous signs of vestibular impairment and to propose a bed-side vestibular examination based protocol for the focused execution of gadolinium-enhanced magnetic resonance imaging (MRI) only if a vestibular schwannoma is suspected. 96 patients, 52 men, 44 women, mean age 57.73 +/- 12.85 years, suffering from sudden sensorineural unilateral hearing loss, which presented neither vertigo nor spontaneous nystagmus, were enrolled...
May 22, 2017: Acta Otorhinolaryngologica Italica
https://www.readbyqxmd.com/read/28516965/benefits-of-active-middle-ear-implants-over-hearing-aids-in-patients-with-sloping-high-tone-hearing-loss-comparison-with-hearing-aids
#7
J M Lee, J H Jeon, I S Moon, J Y Choi
In this retrospective chart review we compared the subjective and objective benefits of active middle ear implants (AMEIs) with conventional hearing aids (HAs) in patients with sloping high tone hearing loss. Thirty-four patients with sensorineural hearing loss were treated with AMEIs. Of these, six had sloping high tone hearing loss and had worn an HA for more than 6 months. Objective assessments, a pure-tone audiogram, as well as a word recognition test, and the Korean version of the Hearing in Noise Test (K-HINT), and a subjective assessment, the Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire, were performed...
June 2017: Acta Otorhinolaryngologica Italica
https://www.readbyqxmd.com/read/28516283/developmental-window-of-sensorineural-deafness-in-biotinidase-deficient-mice
#8
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/28515283/anisomorphic-cortical-reorganization-in-asymmetric-sensorineural-hearing-loss
#9
Steven W Cheung, Craig Anthony Atencio, Eliott R J Levy, Robert C Froemke, Christoph E Schreiner
Acoustic trauma or inner ear disease may predominantly injure one ear, causing asymmetric sensorineural hearing loss (SNHL). While characteristic frequency (CF) map plasticity of primary auditory cortex (AI) contralateral to the injured ear has been detailed, there is no study that also evaluates ipsilateral AI to compare cortical reorganization across both hemispheres. We assess whether normal isomorphic mirror image relationship between the two hemispheres is maintained or disrupted in mild-to-moderate asymmetric SNHL of adult squirrel monkeys...
May 17, 2017: Journal of Neurophysiology
https://www.readbyqxmd.com/read/28514357/-the-results-of-universal-audiological-screening-of-newborn-infants-in-the-astrakhan-region
#10
N A Daykhes, E A Grigor'eva, Yu V Nazarochkin, V M Davydov, A O Kuznetsov
We have evaluated the results of universal audiological screening of 93 352 newborn infants performed in the Astrakhan region of the Russian Federation during the period from 2009 till 2015. The screening coverage amounted to 95%. The prevalence of hearing disorders was estimated at 1:1111 and that of their severe forms at 1:3000. The clinical significance of the examination during its first stage consists of the exclusion of evoked otoacoustic emission. After the completion of the second stage, it is necessary to carry out the regular medical follow up observation of the children suffering from hearing impairment and to undertake the early rehabilitative treatment based on the application of hearing aids and prosthetic devices...
2017: Vestnik Otorinolaringologii
https://www.readbyqxmd.com/read/28511890/intratympanic-steroid-delivery-by-an-indwelling-catheter-in-refractory-severe-sudden-sensorineural-hearing-loss
#11
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/28511423/bardet-biedl-syndrome-a-report-of-two-cases-with-otolaryngologic-symptoms
#12
Mahendra K Singh, Shrinkhal, Sidharth Pradhan, Priyanko Chakraborty
Bardet-Biedl Syndrome (BBS) is a rare autosomal recessive disorder characterized primarily by rod-cone dystrophy, postaxial polydactyly, central obesity, mental retardation, hypogonadism, and renal dysfunction. We present two cases of this syndrome, both female, who presented with complaints of nyctalopia and mental retardation, and additionally one of them had sensorineural hearing loss while the other had serous otitis media. Hearing loss being a rare presentation is worth reporting. Both the patients were given a course of vitamin A and the parents were counseled regarding the prognosis and additional complications associated with the syndrome...
March 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28505526/peripheral-hearing-loss-reduces-the-ability-of-children-to-direct-selective-attention-during-multi-talker-listening
#13
Emma Holmes, Padraig T Kitterick, A Quentin Summerfield
Restoring normal hearing requires knowledge of how peripheral and central auditory processes are affected by hearing loss. Previous research has focussed primarily on peripheral changes following sensorineural hearing loss, whereas consequences for central auditory processing have received less attention. We examined the ability of hearing-impaired children to direct auditory attention to a voice of interest (based on the talker's spatial location or gender) in the presence of a common form of background noise: the voices of competing talkers (i...
May 10, 2017: Hearing Research
https://www.readbyqxmd.com/read/28504500/infantile-onset-thiamine-responsive-megaloblastic-anemia-syndrome-with-slc19a2-mutation-a-case-report
#14
Nagehan Katipoğlu, Tuba H Karapinar, Korean Demir, Sultan Aydin Köker, Özlem Nalbantoğlu, Yılmaz Ay, Hüseyin A Korkmaz, Yeşim Oymak, Melek Yıldız, Selma Tunç, Filiz Hazan, Canan Vergin, Behzat Ozkan
BACKGROUND: Thiamine-responsive megaloblastic anemia syndrome (TRMA), also known as Rogers syndrome, is characterized by megaloblastic anemia, sensorineural hearing loss, and diabetes mellitus. Disturbances of the thiamine transport into the cells results from homozygous or compound heterozygous mutations in the SLC19A2 gene. CASE PRESENTATION: We report a girl which presented with sensorineural deafness treated with a hearing prosthesis, insulin requiring diabetes, macrocytic anemia, treated with thiamine (100 mg/day)...
June 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28503992/dose-dependent-effects-of-ouabain-on-spiral-ganglion-neurons-and-schwann-cells-in-mouse-cochlea
#15
Zhi-Jian Zhang, Hong-Xia Guan, Kun Yang, Bo-Kui Xiao, Hua Liao, Yang Jiang, Tao Zhou, Qing-Quan Hua
OBJECTIVE: This study aimed in fully investigating the toxicities of ouabain to mouse cochlea and the related cellular environment, and providing an optimal animal model system for cell transplantation in the treatment of auditory neuropathy (AN) and sensorineural hearing loss (SNHL). METHODS: Different dosages of ouabain were applied to mouse round window. The auditory brainstem responses and distortion product otoacoustic emissions were used to evaluate the cochlear function...
May 14, 2017: Acta Oto-laryngologica
https://www.readbyqxmd.com/read/28502595/a-sustained-increase-of-plasma-fibrinogen-in-sudden-sensorineural-hearing-loss-predicts-worse-outcome-independently
#16
Yoon Ah Park, Tae Hoon Kong, Young Joon Seo
OBJECTIVES: A number of etiologies of idiopathic sudden sensorineural hearing loss (ISSNHL) have been proposed. Vascular disturbance is one cause of ISSNHL and has been reported to be associated with fibrinogen. We aimed to determine whether hyperfibrinogenemia is associated with poor outcome and whether a serial change in fibrinogen level is associated with outcome. METHODS: Twenty-two patients with ISSNHL were enrolled. We compared the levels of fibrinogen in ISSNHL groups classified as improved and non-improved according to improvement of hearing...
May 5, 2017: American Journal of Otolaryngology
https://www.readbyqxmd.com/read/28502583/homozygous-ednrb-mutation-in-a-patient-with-waardenburg-syndrome-type-1
#17
Noriko Morimoto, Hideki Mutai, Kazunori Namba, Hiroki Kaneko, Rika Kosaki, Tatsuo Matsunaga
OBJECTIVE: To examine and expand the genetic spectrum of Waardenburg syndrome type 1 (WS1). METHODS: Clinical features related to Waardenburg syndrome (WS) were examined in a five-year old patient. Mutation analysis of genes related to WS was performed in the proband and her parents. Molecular modeling of EDNRB and the p.R319W mutant was conducted to predict the pathogenicity of the mutation. RESULTS: The proband showed sensorineural hearing loss, heterochromia iridis, and dystopia canthorum, fulfilling the clinical criteria of WS1...
May 11, 2017: Auris, Nasus, Larynx
https://www.readbyqxmd.com/read/28502323/a-case-report-on-the-exceptional-coincidence-of-two-inherited-renal-disorders-adpkd-and-alport-syndrome%C3%A2
#18
Kathrin Ebner, Nadine Reintjes, Markus Feldkötter, Friederike Körber, Mato Nagel, Jörg Dötsch, Bernd Hoppe, Lutz Thorsten Weber, Bodo B Beck, Max C Liebau
Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic cause of dialysis-requiring end-stage renal disease in adults and is characterized by the slowly progressing replacement of renal tissue by focal macrocysts. Alport syndrome (AS; hereditary nephritis) is a rare, inherited disorder of the basement membrane associated with hematuria, proteinuria, and loss of kidney function as well as sensorineural hearing loss and ocular abnormalities. Here, we report on a family in which both ADPKD and AS are present...
May 15, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28502273/modiolar-ossification-in-paediatric-patients-with-auditory-neuropathy
#19
L Wang, Z Wang, F Gao, K A Peng
OBJECTIVE: To describe our finding of increased ossification of the modiolus in paediatric patients with auditory neuropathy who met criteria for cochlear implantation. METHODS: A retrospective case series with a comparison group at a tertiary referral centre is described. Seven paediatric patients with auditory neuropathy who met criteria for and underwent cochlear implantation were identified. Fifteen paediatric implantees with bilateral profound sensorineural hearing loss were included as the comparison group...
May 15, 2017: Journal of Laryngology and Otology
https://www.readbyqxmd.com/read/28501927/cytokine-gene-polymorphism-associations-with-congenital-cytomegalovirus-infection-and-sensorineural-hearing-loss
#20
B Kasztelewicz, J Czech-Kowalska, B Lipka, B Milewska-Bobula, M K Borszewska-Kornacka, J Romańska, K Dzierżanowska-Fangrat
Cytomegalovirus (CMV) is the most common viral agent of congenital infections and a leading nongenetic cause of sensorineural hearing loss (SNHL). The host immunologic factors that render a developing foetus prone to intrauterine CMV infection and development of hearing loss are unknown. The aim of this study was to assess the potential associations between the polymorphisms within cytokine and cytokine receptors genes, and the risk of congenital CMV infection, and the hearing outcome. A panel of 11 candidate single nucleotide polymorphisms (SNPs): TNF rs1799964, TNF rs1800629, TNFRSF1A rs4149570, IL1B rs16944, IL1B rs1143634, IL10 rs1800896, IL10RA rs4252279, IL12B rs3212227, CCL2 rs1024611, CCL2 rs13900, CCR5 rs333 was genotyped in 470 infants (72 with confirmed intrauterine CMV infection and 398 uninfected controls), and related to congenital CMV infection, and the outcome...
May 13, 2017: European Journal of Clinical Microbiology & Infectious Diseases
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