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auditory neuropathy

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https://www.readbyqxmd.com/read/28794847/neuro-otological-and-peripheral-nerve-involvement-in-fabry-disease
#1
Sergio Carmona, Romina Weinschelbaum, Ana Pardal, Cintia Marchesoni, Paz Zuberbuhler, Patricia Acosta, Guillermo Cáceres, Isaac Kisinovsky, Luciana Bayón, Ricardo Reisin
Fabry disease (FD) is an X-linked lysosomal storage disease, with multisystemic glycosphingolipids deposits. Neuro-otological involvement leading to hearing loss and vestibular dysfunctions has been described, but there is limited information about the frequency, site of lesion, or the relationship with peripheral neuropathy. The aim was to evaluate the presence of auditory and vestibular symptoms, and assess neurophysiological involvement of the VIII cranial nerve, correlating these findings with clinical and neurophysiological features of peripheral neuropathy...
July 18, 2017: Audiology Research
https://www.readbyqxmd.com/read/28769753/intraoperative-electrocochleographic-characteristics-of-auditory-neuropathy-spectrum-disorder-in-cochlear-implant-subjects
#2
William J Riggs, Joseph P Roche, Christopher K Giardina, Michael S Harris, Zachary J Bastian, Tatyana E Fontenot, Craig A Buchman, Kevin D Brown, Oliver F Adunka, Douglas C Fitzpatrick
Auditory neuropathy spectrum disorder (ANSD) is characterized by an apparent discrepancy between measures of cochlear and neural function based on auditory brainstem response (ABR) testing. Clinical indicators of ANSD are a present cochlear microphonic (CM) with small or absent wave V. Many identified ANSD patients have speech impairment severe enough that cochlear implantation (CI) is indicated. To better understand the cochleae identified with ANSD that lead to a CI, we performed intraoperative round window electrocochleography (ECochG) to tone bursts in children (n = 167) and adults (n = 163)...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28766844/timing-of-cochlear-implantation-in-auditory-neuropathy-patients-with-otof-mutations-our-experience-with-10-patients
#3
C C Wu, C J Hsu, F L Huang, Y H Lin, Y H Lin, T C Liu, C M Wu
Auditory neuropathy spectrum disorder (ANSD) can be caused by a variety of pathologies ranging from inner hair cells, synapses, spiral ganglion neurons, the auditory nerve, to brainstem auditory nuclei.(1) For ANSD patients who derive limited benefit from hearing aids, cochlear implantation constitutes the treatment of last resort. However, corresponding to the heterogeneity in pathology, the outcomes with cochlear implants (CIs) vary significantly among ANSD patients.(1) This article is protected by copyright...
August 2, 2017: Clinical Otolaryngology
https://www.readbyqxmd.com/read/28743950/auditory-neuropathy-after-damage-to-cochlear-spiral-ganglion-neurons-in-mice-resulting-from-conditional-expression-of-diphtheria-toxin-receptors
#4
Haolai Pan, Qiang Song, Yanyan Huang, Jiping Wang, Renjie Chai, Shankai Yin, Jian Wang
Auditory neuropathy (AN) is a hearing disorder characterized by normal cochlear amplification to sound but poor temporal processing and auditory perception in noisy backgrounds. These deficits likely result from impairments in auditory neural synchrony; such dyssynchrony of the neural responses has been linked to demyelination of auditory nerve fibers. However, no appropriate animal models are currently available that mimic this pathology. In this study, Cre-inducible diphtheria toxin receptor (iDTR (+/+) ) mice were cross-mated with mice containing Cre (Bhlhb5-Cre (+/-) ) specific to spiral ganglion neurons (SGNs)...
July 25, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28731162/a-homozygous-myo7a-mutation-associated-to-usher-syndrome-and-unilateral-auditory-neuropathy-spectrum-disorder
#5
Hong Xia, Pengzhi Hu, Lamei Yuan, Wei Xiong, Hongbo Xu, Junhui Yi, Zhijian Yang, Xiong Deng, Yi Guo, Hao Deng
Usher syndrome (USH) is an autosomal recessive disorder characterized by sensorineural hearing loss, progressive visual loss and night blindness due to retinitis pigmentosa (RP), with or without vestibular dysfunction. The purpose of this study was to detect the causative gene in a consanguineous Chinese family with USH. A c.3696_3706del (p.R1232Sfs*72) variant in the myosin VIIa gene (MYO7A) was identified in the homozygous state by exome sequencing. The co‑segregation of the MYO7A c.3696_3706del variant with the phenotype of deafness and progressive visual loss in the USH family was confirmed by Sanger sequencing...
July 21, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28708635/the-management-of-pediatric-hearing-loss-caused-by-auditory-neuropathy-spectrum-disorder
#6
Nguyen S Pham
PURPOSE OF REVIEW: Auditory neuropathy spectrum disorder (ANSD) is a condition in which auditory testing reveals normal otoacoustic emissions, but auditory brainstem testing is abnormal or absent and speech discrimination is poor. This constellation of findings ostensibly suggests that the cochlea is healthy and an abnormality of conduction or processing of sound occurs along the nerve fibers. As more is learned about this condition, it is becoming clear that ANSD describes heterogeneous, distinct clinical entities that must be taken into account when devising treatment modalities...
July 13, 2017: Current Opinion in Otolaryngology & Head and Neck Surgery
https://www.readbyqxmd.com/read/28704898/a-case-of-auditory-neuropathy-caused-by-pontine-hemorrhage-in-an-adult
#7
Seung-Hyun Chung, Sung Wook Jeong, Lee-Suk Kim
A pontine hemorrhage can evoke several neurological symptoms because the pons contains various nuclei and nerve fibers. Hearing loss can develop as a result of a pontine hemorrhage because there is an auditory conduction pathway in the cochlear nucleus of the pons. However, very few cases of hearing loss caused by pontine lesions have been reported, and there have been no reports of auditory neuropathy that developed following a pontine hemorrhage. Recently we had a patient who experienced a nontraumatic pontine hemorrhage who was diagnosed with auditory neuropathy...
July 2017: Journal of Audiology & Otology
https://www.readbyqxmd.com/read/28688553/clinical-role-of-electrocochleography-in-children-with-auditory-neuropathy-spectrum-disorder
#8
Tatyana E Fontenot, Christopher K Giardina, Holly F Teagle, Lisa R Park, Oliver F Adunka, Craig A Buchman, Kevin D Brown, Douglas C Fitzpatrick
OBJECTIVES: To assess electrocochleography (ECochG) to tones as an instrument to account for CI speech perception outcomes in children with auditory neuropathy spectrum disorder (ANSD). MATERIALS & METHODS: Children (<18 years) receiving CIs for ANSD (n = 30) and non-ANSD (n = 74) etiologies of hearing loss were evaluated with ECochG using tone bursts (0.25-4 kHz). The total response (TR) is the sum of spectral peaks of responses across frequencies. The compound action potential (CAP) and the auditory nerve neurophonic (ANN) in ECochG waveforms were used to estimate nerve activity and calculate nerve score...
August 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28687782/human-cochlear-histopathology-reflects-clinical-signatures-of-primary-neural-degeneration
#9
Jessica E Sagers, Lukas D Landegger, Steven Worthington, Joseph B Nadol, Konstantina M Stankovic
Auditory neuropathy is a significant and understudied cause of human hearing loss, diagnosed in patients who demonstrate abnormal function of the cochlear nerve despite typical function of sensory cells. Because the human inner ear cannot be visualized during life, histopathological analysis of autopsy specimens is critical to understanding the cellular mechanisms underlying this pathology. Here we present statistical models of severe primary neuronal degeneration and its relationship to pure tone audiometric thresholds and word recognition scores in comparison to age-matched control patients, spanning every decade of life...
July 7, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28680492/effectiveness-of-low-cut-modified-amplification-using-receiver-in-the-canal-hearing-aid-in-individuals-with-auditory-neuropathy-spectrum-disorder
#10
Prashanth Prabhu, Animesh Barman
Introduction  The studies on hearing aid benefit in individuals with auditory neuropathy spectrum disorder (ANSD) shows limited benefit. Low cut modified amplification is found to be effective in few individuals with ANSD. With advancement in technology, receiver in the canal (RIC) hearing aids have proven to be more effective than traditional behind the ear (BTE) hearing aids. Objective  Thus, the present study attempts to determine the effectiveness of low cut modified amplification using RIC and BTE...
July 2017: International Archives of Otorhinolaryngology
https://www.readbyqxmd.com/read/28601886/auna2-a-novel-type-of-non-syndromic-slowly-progressive-auditory-synaptopathy-auditory-neuropathy-with-autosomal-dominant-inheritance
#11
Ruth Lang-Roth, Eva Fischer-Krall, Cornelia Kornblum, Gudrun Nürnberg, Dieter Meschede, Ingrid Goebel, Peter Nürnberg, Dirk Beutner, Christian Kubisch, Martin Walger, Alexander E Volk
BACKGROUND: Auditory synaptopathy/neuropathy (AS/AN) is a heterogeneous disorder, which may be caused by environmental factors like postnatal hyperbilirubinemia or by genetic factors. The genetic forms are subdivided into syndromic and non-syndromic types, and show different inheritance patterns with a strong preponderance of autosomal-recessive forms. To date, only a single locus for non-syndromic autosomal-dominant AS/AN (AUNA1) has been reported in a single family, in which a non-coding DIAPH3 mutation was subsequently described as causative...
2017: Audiology & Neuro-otology
https://www.readbyqxmd.com/read/28555600/audiological-findings-in-charcot-marie-tooth-disease-type-4c
#12
Rafael Sivera, Laura Cavalle, Juan J Vílchez, Carmen Espinós, Herminio Pérez Garrigues, Teresa Sevilla
OBJECTIVE: Charcot-Marie-Tooth disease type 4C (CMT4C) is a hereditary demyelinating early onset neuropathy with prominent unsteadiness and occasional cranial nerve involvement. Vestibulopathy caused by the dysfunction of cranial nerve VIII has been demonstrated in a high percentage of these patients, but the presence and degree of auditory neuropathy are unknown. The aim of the study was to characterize the hearing abnormalities of a series of patients with CMT4C and to determine the presence and severity of auditory neuropathy (AN) in these patients...
April 2017: Journal of International Advanced Otology
https://www.readbyqxmd.com/read/28555599/speech-perception-in-quiet-and-in-noise-condition-in-individuals-with-auditory-neuropathy-spectrum-disorder
#13
Kumari Apeksha, Ajith U Kumar
OBJECTIVE: The study investigated the effect of noise on syllable perception in individuals with Auditory Neuropathy Spectrum Disorder (ANSD) and compared that with the normal hearing individuals. MATERIALS AND METHODS: A total of 54 participants were considered, out of which 26 individuals were diagnosed with ANSD and 28 with normal hearing sensitivity. Syllable identification and discrimination were assessed in both the groups in quiet as well as +10 dB SNR. RESULTS: All the individuals with ANSD performed poorer on syllable identification and syllable discrimination tasks compared to individuals with normal hearing...
April 2017: Journal of International Advanced Otology
https://www.readbyqxmd.com/read/28503992/dose-dependent-effects-of-ouabain-on-spiral-ganglion-neurons-and-schwann-cells-in-mouse-cochlea
#14
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/28503341/peripheral-and-central-nervous-system-involvement-in-recently-diagnosed-cases-of-hypothyroidism-an-electrophysiological-study
#15
N Gupta, M Arora, R Sharma, K S Arora
BACKGROUND: Hypothyroidism, one of the most common endocrine disorders, may induce neurological abnormalities at an early stage of the disease. AIM: The study was designed to assess the electrophysiological alterations of some selected variables of nerve conduction, brainstem auditory evoked potentials (BAEPs), and visual evoked potentials (VEPs) in hypothyroid patients. SUBJECTS AND METHODS: Sixty patients of newly diagnosed hypothyroidism and an equal number of age-matched controls were selected for the study...
September 2016: Annals of Medical and Health Sciences Research
https://www.readbyqxmd.com/read/28502273/modiolar-ossification-in-paediatric-patients-with-auditory-neuropathy
#16
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...
July 2017: Journal of Laryngology and Otology
https://www.readbyqxmd.com/read/28485679/effectiveness-of-low-cut-modified-amplification-strategy-and-channel-free-hearing-aid-in-individuals-with-auditory-neuropathy-spectrum-disorder
#17
Prashanth Prabhu, Animesh Barman
OBJECTIVE: The present study attempted to compare the aided benefit using low-cut modified amplification and channel-free hearing aids in individuals with auditory neuropathy spectrum disorder (ANSD). It was also attempted to determine these effects in good and poor performers with ANSD. DESIGN: Cross-sectional within group pretest, post-test design. STUDY SAMPLE: Twenty-five individuals with acquired ANSD were selected for the study. The study sample included 11 males and 14 females between the age ranges of 17-40 years (mean age of 24...
May 9, 2017: International Journal of Audiology
https://www.readbyqxmd.com/read/28484666/hearing-and-balance-disorders-in-the-state-of-hawai-i-demographics-and-demand-for-services
#18
Mohsin Ahmed M Shaikh, James W Hall, Cindy McManus, Henry L Lew
Hearing and balance disorders affect people of all ages. Among children, hearing loss affects speech and language development, academic performance, and psychosocial development. Hearing loss in adults negatively impacts work productivity, cognitive function, and psychosocial status. Prevalence of hearing loss in children in Hawai'i is higher than the national average. Research indicates that hearing loss is a prevalent condition among veterans and advanced age adults. This is of particular concern in Hawai'i as the state is home to many military training facilities and has a large elderly population...
May 2017: Hawai'i Journal of Medicine & Public Health: a Journal of Asia Pacific Medicine & Public Health
https://www.readbyqxmd.com/read/28483258/universal-newborn-hearing-screening-in-southwestern-iran
#19
Nader Saki, Arash Bayat, Reza Hoseinabadi, Soheila Nikakhlagh, Majid Karimi, Rezvan Dashti
OBJECTIVES: The implementation of Neonatal Hearing Screening (NHS) program is still at the preliminary stage particularly in developing countries despite the burden of permanent congenital and early-onset hearing impairment. The purpose of this study was to report results for universal newborn NHS in a cohort of children born in the southwestern region of Iran, as part of a national screening program set up by the Iranian National Health System. METHODS: During this cross-sectional study, which took place between March 2013 and April 2016, healthy newborns were screened using transient evoked otoacoustic emissions (TEOAEs) and automated auditory brainstem responses (AABRs) methods at several points in time as early as possible after birth...
June 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28473856/recovery-of-abnormal-abr-in-neonates-and-infants-at-risk-of-hearing-loss
#20
Ioannis Psarommatis, Charalampos Voudouris, Ioannis Kapetanakis, Faselida Athanasiadi, Konstantinos Douros
The purpose of this retrospective study is to present the clinical experience of a single institution on the recovery of ABR thresholds in a large population of neonates and infants at risk of hearing loss. Potential prognostic factors associated with this phenomenon were also investigated. Out of 2248 high risk infants, 384 had abnormal ABR at initial hearing evaluation and 168 of them had absent ABR or a threshold ≥80 dBnHL. From this subgroup, a significant percentage showed complete or partial recovery on reexamination (32...
2017: International Journal of Otolaryngology
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