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Progressive hearing loss

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https://www.readbyqxmd.com/read/28087419/cochlear-synaptopathy-in-acquired-sensorineural-hearing-loss-manifestations-and-mechanisms
#1
REVIEW
M Charles Liberman, Sharon G Kujawa
Common causes of hearing loss in humans - exposure to loud noise or ototoxic drugs and aging - often damage sensory hair cells, reflected as elevated thresholds on the clinical audiogram. Recent studies in animal models suggest, however, that well before this overt hearing loss can be seen, a more insidious, but likely more common, process is taking place that permanently interrupts synaptic communication between sensory inner hair cells and subsets of cochlear nerve fibers. The silencing of affected neurons alters auditory information processing, whether accompanied by threshold elevations or not, and is a likely contributor to a variety of perceptual abnormalities, including speech-in-noise difficulties, tinnitus and hyperacusis...
January 10, 2017: Hearing Research
https://www.readbyqxmd.com/read/28079680/middle-ear-sound-transmission-under-normal-damaged-repaired-and-reconstructed-conditions
#2
Wei Dong, Ying Tian, Xin Gao, Timothy T K Jung
HYPOTHESIS: We hypothesize that current clinical treatment strategies for the disarticulated or eroded incus have the effect of combining the incus and stapes of the human middle ear (ME) into one rigid structure, which, while capable of adequately transmitting lower-frequency sounds, fails for higher frequencies. BACKGROUND: ME damage causes conductive hearing loss (CHL) and while great progress has been made in repairing or reconstructing damaged MEs, the outcomes are often far from ideal...
January 10, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28079432/primary-tumors-of-the-facial-nerve-misdiagnosed-a-case-series-and-review-of-the-literature
#3
Haijin Yi, Bin Hu, Guoping Yin, Xin Li, Jinkun Xu, Jingying Ye, Shi-Ming Yang
CONCLUSION: Unusual primary tumors of the facial nerve should be considered in patients with progressive facial paralysis, especially if this is accompanied by hearing loss or vertigo. Misdiagnosis could increase the difficulties of operation, diminish the chance of facial nerve reconstruction, and increase the likelihood of poor reconstructive outcomes. OBJECTIVE: This paper was to determine the characteristics of facial nerve primary tumors misdiagnosed as tumor-free conditions many years prior, and to describe appropriate treatments...
January 12, 2017: Acta Oto-laryngologica
https://www.readbyqxmd.com/read/28067622/a-homozygous-fitm2-mutation-causes-a-deafness-dystonia-syndrome-with-motor-regression-and-signs-of-ichthyosis-and-sensory-neuropathy
#4
Celia Zazo Seco, Anna Castells-Nobau, Seol-Hee Joo, Margit Schraders, Jia Nee Foo, Monique van der Voet, S Sendhil Velan, Bonnie Nijhof, Jaap Oostrik, Erik de Vrieze, Radoslaw Katana, Atika Mansoor, Martijn Huynen, Radek Szklarczyk, Martin Oti, Lisbeth Tranebjærg, Erwin van Wijk, Jolanda M Scheffer-de Gooyert, Saadat Siddique, Jonathan Baets, Peter de Jonghe, Syed Ali Raza Kazmi, Suresh Anand Sadananthan, Bart P van de Warrenburg, Chiea Chuen Khor, Martin C Göpfert, Raheel Qamar, Annette Schenck, Hannie Kremer, Saima Siddiqi
A consanguineous family from Pakistan was ascertained with a novel deafness-dystonia syndrome with motor regression, ichthyosis-like features and signs of sensory neuropathy. By applying a combined strategy of linkage analysis and whole-exome sequencing in the presented family, a homozygous nonsense mutation, c.4G>T (p.Glu2*), in FITM2 was identified. FITM2 and its paralog FITM1 constitute an evolutionary conserved protein family involved in partitioning of triglycerides into cellular lipid droplets. Despite the role of FITM2 in neutral lipid storage and metabolism, no indications for lipodystrophy were observed in the affected individuals...
December 15, 2016: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28060022/otosclerosis-an-update-on-diagnosis-and-treatment
#5
Lora Batson, Denise Rizzolo
Otosclerosis is a complex and progressive disease of pathological bone remodeling that affects the otic capsule of the temporal bone, resulting in hearing loss. Although traditional diagnostic methods are still used, improvements in technology and research have paved the way for additional diagnostic techniques and advancements. The traditional treatment of otosclerosis, stapes surgery, is now being augmented or replaced by innovations in hearing aid technology and cochlear implants. Earlier diagnosis of otosclerosis can occur through understanding of the cause, risk factors, and current diagnostic testing...
January 5, 2017: JAAPA: Official Journal of the American Academy of Physician Assistants
https://www.readbyqxmd.com/read/28054914/detailed-audiological-evaluation-of-a-patient-with-xeroderma-pigmentosum-with-neural-degeneration
#6
Danielle Mercer, Annette Hurley, Fern Tsien
BACKGROUND: Xeroderma pigmentosum (XP) is a rare autosomal recessive condition characterized by extreme sensitivity to ultraviolet light. Individuals with XP lack the ability to repair DNA (deoxyribonucleic acid) damage caused by ultraviolet radiation, leading to sunburn and increased susceptibility to skin cancers. Approximately 25% of patients also exhibit neural degeneration, which includes progressive mental deterioration, cortical thinning, and sensorineural hearing loss. PURPOSE: Herein, we describe the audiological and genetic findings in a patient with XP subtype D with neural degeneration and hearing loss...
January 2017: Journal of the American Academy of Audiology
https://www.readbyqxmd.com/read/28053826/sensorineural-organs-dysfunction-and-cognitive-decline-a-review-article
#7
REVIEW
Supakanya Wongrakpanich, Aisawan Petchlorlian, Andrew Rosenzweig
Vision, hearing, olfaction, and cognitive function are essential components of healthy and successful aging. Multiple studies demonstrate relationship between these conditions with cognitive function. The present article focuses on hearing loss, visual impairment, olfactory loss, and dual sensory impairments in relation to cognitive declination and neurodegenerative disorders. Sensorineural organ impairment is a predictive factor for mild cognitive impairment and neurodegenerative disorders in the elderly. We recommend early detection of sensorineural dysfunction by history, physical examination, and screening tests...
December 2016: Aging and Disease
https://www.readbyqxmd.com/read/28052261/cochlear-cell-modeling-using-disease-specific-ipscs-unveils-a-degenerative-phenotype-and-suggests-treatments-for-congenital-progressive-hearing-loss
#8
Makoto Hosoya, Masato Fujioka, Takefumi Sone, Satoshi Okamoto, Wado Akamatsu, Hideki Ukai, Hiroki R Ueda, Kaoru Ogawa, Tatsuo Matsunaga, Hideyuki Okano
Hearing impairments are the most common symptom of congenital defects, and they generally remain intractable to treatment. Pendred syndrome, the most frequent syndromic form of hereditary hearing loss, is associated with mutations in the anion exchanger pendrin. Loss of pendrin function as an anion exchanger is thought to be causative, but rodent models do not exhibit progressive deafness. Here, we report a degenerative phenotype exhibiting mutant pendrin aggregates and increased susceptibility to cellular stresses in cochlear epithelial cells induced from patient-derived induced pluripotent stem cells (iPSCs)...
January 3, 2017: Cell Reports
https://www.readbyqxmd.com/read/28050212/otoneurological-abnormalities-in-patients-with-friedreich-s-ataxia
#9
Bianca Simone Zeigelboim, Juliana Cristina Mesti, Vinicius Ribas Fonseca, João Henrique Faryniuk, Jair Mendes Marques, Rafaella Cardosa Cardoso, Hélio Afonso Ghizoni Teive
Introduction Friedreich's ataxia is a neurodegenerative disease and progressive by nature. It has autosomal recessive inheritance and early onset in most cases. Nystagmus and hearing loss (in some cases) make up some of the common symptoms seen in this disorder. Objective The objective of this study is to examine vestibular disorders in patients with Friedreich ataxia. Methods We conducted a retrospective cross-sectional study. We evaluated 30 patients with ages ranging from six to 72 years (mean age of 38...
January 2017: International Archives of Otorhinolaryngology
https://www.readbyqxmd.com/read/28045838/effect-of-context-and-hearing-loss-on-time-gated-word-recognition-in-children
#10
Dawna Lewis, Judy Kopun, Ryan McCreery, Marc Brennan, Kanae Nishi, Evan Cordrey, Pat Stelmachowicz, Mary Pat Moeller
OBJECTIVES: The purpose of this study was to examine word recognition in children who are hard of hearing (CHH) and children with normal hearing (CNH) in response to time-gated words presented in high- versus low-predictability sentences (HP, LP), where semantic cues were manipulated. Findings inform our understanding of how CHH combine cognitive-linguistic and acoustic-phonetic cues to support spoken word recognition. It was hypothesized that both groups of children would be able to make use of linguistic cues provided by HP sentences to support word recognition...
December 30, 2016: Ear and Hearing
https://www.readbyqxmd.com/read/28043290/-research-progress-on-ototoxicity-and-hearing-loss-effects-of-organic-solvents
#11
M Zhang, P Xu, Q Gu
No abstract text is available yet for this article.
November 20, 2016: Chinese Journal of Industrial Hygiene and Occupational Diseases
https://www.readbyqxmd.com/read/28030893/recent-advances-in-understanding-audiovestibular-loss-of-a-vascular-cause
#12
Hyun-Ah Kim, Hyung Lee
Acute audiovestibular loss is characterized by abrupt onset of prolonged (lasting days) vertigo and hearing loss. Acute ischemic stroke in the distribution of the anterior inferior cerebellar artery (AICA) is known to be the leading cause of acute audiovestibular loss. So far, eight subgroups of AICA territory infarction have been identified according to the patterns of audiovestibular dysfunctions, among which the most common pattern is the combined loss of auditory and vestibular functions. Unlike inner ear dysfunction of a viral cause , which can commonly present as an isolated vestibular (i...
December 12, 2016: Journal of Stroke
https://www.readbyqxmd.com/read/28027286/the-relationship-of-the-middle-ear-effusion-microbiome-to-secretory-mucin-production-in-pediatric-patients-with-chronic-otitis-media
#13
Anna Krueger, Stéphanie Val, Marcos Pérez-Losada, Karuna Panchapakesan, Joe Devaney, Vanessa Duah, Christine DeMasson, Marian Poley, Mary Rose, Diego Preciado
BACKGROUND: Acute otitis media (AOM), an infection of the middle ear, can become chronic (COM) following multiple episodes. Microbial influence on COM remains unclear. It's been reported that mucin glycoproteins are required for middle ear immune defense against pathogens. We aim to characterize the middle ear effusion (MEE) microbiome using high-throughput sequencing and assess potential associations in microbiome diversity with the presence of the secretory mucins MUC5B and MUC5AC. We hypothesize that MEEs containing MUC5B will exhibit a microbiome largely devoid of typical AOM bacteria...
December 23, 2016: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28024288/asiaticoside-protects-cochlear-hair-cells-from-high-glucose-induced-oxidative-stress-via-suppressing-ages-rage-nf-%C3%AE%C2%BAb-pathway
#14
Ying Xing, Qiuhe Ji, Xiaomiao Li, Jie Ming, Nana Zhang, Dingjun Zha, Ying Lin
Asiaticoside (AC) has been known to have anti-oxidative activity, however, the effect of AC on the progression of high glucose-induced hearing loss has not been studied. This study aims to analyze the effect of AC on cochlear hair cells under the treatment of high glucose in vitro and the hearing function in vivo. The results of MTT showed that high glucose decreased the activity of HEI-OC1 cells, but AC increased the activity of HEI-OC1 cells compared with high glucose group. The results of flow cytometry showed that AC decreased the degree of apoptosis induced by high levels of glucose...
December 23, 2016: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28010677/cochlear-implantation-in-chronic-demyelinating-inflammatory-polyneuropathy
#15
Sarah E Mowry, Sarah King
OBJECTIVE: To describe a case of chronic inflammatory demyelinating polyneuropathy (CDIP) with bilateral sudden sensorineural hearing loss who subsequently benefited from unilateral cochlear implantation. METHODS: case history review and review of the literature for the terms CDIP, hearing loss, cochleovestibular dysfunction, and cochlear implantation. RESULTS: A 49-year-old woman presented with bilateral rapidly progressive sensorineural hearing loss (SNHL) 1 month after an upper respiratory tract infection...
December 23, 2016: Cochlear Implants International
https://www.readbyqxmd.com/read/28007526/noise-induced-cochlear-synaptopathy-past-findings-and-future-studies
#16
REVIEW
Megan Kobel, Colleen G Le Prell, Jennifer Liu, John W Hawks, Jianxin Bao
For decades, we have presumed the death of hair cells and spiral ganglion neurons are the main cause of hearing loss and difficulties understanding speech in noise, but new findings suggest synapse loss may be the key contributor. Specifically, recent preclinical studies suggest that the synapses between inner hair cells and spiral ganglion neurons with low spontaneous rates and high thresholds are the most vulnerable subcellular structures, with respect to insults during aging and noise exposure. This cochlear synaptopathy can be "hidden" because this synaptic loss can occur without permanent hearing threshold shifts...
December 19, 2016: Hearing Research
https://www.readbyqxmd.com/read/27999687/a-novel-nonsense-mutation-of-pou4f3-gene-causes-autosomal-dominant-hearing-loss
#17
Chi Zhang, Mingming Wang, Yun Xiao, Fengguo Zhang, Yicui Zhou, Jianfeng Li, Qingyin Zheng, Xiaohui Bai, Haibo Wang
POU4F3 gene encodes a transcription factor which plays an essential role in the maturation and maintenance of hair cells in cochlea and vestibular system. Several mutations of POU4F3 have been reported to cause autosomal dominant nonsyndromic hearing loss in recent years. In this study, we describe a pathogenic nonsense mutation located in POU4F3 in a four-generation Chinese family. Target region capture sequencing was performed to search for the candidate mutations from 81 genes related to nonsyndromic hearing loss in this family...
2016: Neural Plasticity
https://www.readbyqxmd.com/read/27995350/cytomegalovirus-cmv-infection-causes-degeneration-of-cochlear-vasculature-and-hearing-loss-in-a-mouse-model
#18
Mattia Carraro, Ali Almishaal, Elaine Hillas, Matthew Firpo, Albert Park, Robert V Harrison
Cytomegalovirus (CMV) infection is one of the most common causes of congenital hearing loss in children. We have used a murine model of CMV infection to reveal functional and structural cochlear pathogenesis. The cerebral cortex of Balb/c mice (Mus musculus) was inoculated with 2000 pfu (plaque forming units) of murine CMV on postnatal day 3. At 6 weeks of age, cochlear function was monitored using auditory brainstem response (ABR) and distortion product otoacoustic emission (DPOAE) measures. Histological assessment of cochlear vasculature using a corrosion cast technique was made at 8 weeks...
December 19, 2016: Journal of the Association for Research in Otolaryngology: JARO
https://www.readbyqxmd.com/read/27991672/self-reported-hearing-impairment-and-incident-frailty-in-english-community-dwelling-older-adults-a-4-year-follow-up-study
#19
Ann E M Liljas, Livia A Carvalho, Efstathios Papachristou, Cesar De Oliveira, S Goya Wannamethee, Sheena E Ramsay, Kate Walters
OBJECTIVES: To examine the association between hearing impairment and incident frailty in older adults. DESIGN: Cross-sectional and longitudinal analyses with 4-year follow-up using data from the English Longitudinal Study of Ageing. SETTING: Community. PARTICIPANTS: Community-dwelling individuals aged 60 and older with data on hearing and frailty status (N = 2,836). MEASUREMENTS: Hearing impairment was defined as poor self-reported hearing...
December 19, 2016: Journal of the American Geriatrics Society
https://www.readbyqxmd.com/read/27989950/plastic-changes-along-auditory-pathway-during-salicylate-induced-ototoxicity-hyperactivity-and-cf-shifts
#20
Chen Jiang, Bin Luo, Senthilvelan Manohar, Guang-Di Chen, Richard Salvi
High dose of salicylate, the active ingredient in aspirin, has long been known to induce transient hearing loss, tinnitus and hyperacusis making it a powerful experimental tool. These salicylate-induced perceptual disturbances are associated with a massive reduction in the neural output of the cochlea. Paradoxically, the diminished neural output of the cochlea is accompanied by a dramatic increase in sound-evoked activity in the auditory cortex (AC) and several other parts of the central nervous system. Exactly where the increase in neural activity begins and builds up along the central auditory pathway are not fully understood...
October 27, 2016: Hearing Research
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