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https://www.readbyqxmd.com/read/29780422/cochlear-implantation-in-charcot-marie-tooth-disease-case-report-and-review-of-the-literature
#1
C Lane Anzalone, Sarah Nuhanovic, Amy P Olund, Matthew L Carlson
Introduction: Charcot-Marie-Tooth (CMT) disease is a peripheral hereditary neuropathy associated with motor and sensory impairment and can result in profound sensorineural hearing loss (SNHL). Currently, the role of cochlear implantation in the setting of CMT and other progressive peripheral neurodegenerative disorders is not well established. Methods: Case report and review of the English literature. Results: A 70-year-old male with CMT was referred for evaluation of progressive asymmetric SNHL and reported a 15-year duration of deafness involving the left ear...
2018: Case Reports in Medicine
https://www.readbyqxmd.com/read/29725841/auditory-function-in-pelizaeus-merzbacher-disease
#2
Thierry Morlet, Kyoko Nagao, S Charles Bean, Sara E Mora, Sarah E Hopkins, Grace M Hobson
Pelizaeus-Merzbacher disease (PMD; MIM 312080), an inherited defect of central nervous system myelin formation, affects individuals in many ways, including their hearing and language abilities. The aim of this study was to assess the auditory abilities in 18 patients with PMD by examining the functional processes along the central auditory pathways using auditory brainstem responses (ABR) and cortical auditory evoked potentials (CAEP) in response to speech sounds. The significant ABR anomalies confirm the existence of dyssynchrony previously described at the level of the brainstem in patients with PMD...
May 3, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29724652/a-novel-homozygous-ndrg1-mutation-in-a-chinese-patient-with-charcot-marie-tooth-disease-4d
#3
Bin Chen, Songtao Niu, Na Chen, Hua Pan, Xingao Wang, Zaiqiang Zhang
Charcot-Marie-Tooth disease 4D (CMT4D) is characterized by severe peripheral neuropathy and deafness. It is caused by mutations in the N-myc downstream-regulated gene 1 (NDRG1). We report a Chinese man with a homozygous mutation c.675C > T of NDRG1 that resulted in Q185X, representing the third known CMT4D patient of non-European ancestry. The patient presented with a 15-year-long history of progressive limb weakness accompanied by hearing loss and dysarthria. There was abnormal differentiation and increased interpeak latencies in brainstem auditory evoked potentials...
April 30, 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29708492/cochlear-microphonic-and-summating-potential-responses-from-click-evoked-auditory-brain-stem-responses-in-high-risk-and-normal-infants
#4
Lisa L Hunter, Chelsea M Blankenship, Rebekah G Gunter, Douglas H Keefe, M Patrick Feeney, David K Brown, Kelly Baroch
BACKGROUND: Examination of cochlear and neural potentials is necessary to assess sensory and neural status in infants, especially those cared for in neonatal intensive care units (NICU) who have high rates of hyperbilirubinemia and thus are at risk for auditory neuropathy (AN). PURPOSE: The purpose of this study was to determine whether recording parameters commonly used in click-evoked auditory brain stem response (ABR) are useful for recording cochlear microphonic (CM) and Wave I in infants at risk for AN...
May 2018: Journal of the American Academy of Audiology
https://www.readbyqxmd.com/read/29696047/comparison-of-cochlear-microphonics-magnitude-with-broad-and-narrow-band-stimuli-in-healthy-adult-wistar-rats
#5
Fatemeh Heidari Phd, Akram Pourbakht, Seyed Kamran Kamrava, Mohammad Kamali, Abbas Yousefi
Objective: Cochlear microphonic (CM) is a cochlear AC electric field, recorded within, around, and remote from its sources. Nowadays it can contribute to the differential diagnosis of different auditory pathologies such as auditory neuropathy spectrum disorder (ANSD). This study compared CM waveforms (CMWs) and amplitudes with broad and narrow band stimuli in 25 healthy male young adults Wistar rats. Materials & Methods: This experimental study was accomplished in the School of Rehabilitation Sciences of Iran University of Medical Sciences, Tehran, Iran (April, 2016)...
2018: Iranian Journal of Child Neurology
https://www.readbyqxmd.com/read/29688962/deterioration-in-distortion-product-otoacoustic-emissions-in-auditory-neuropathy-patients-with-distinct-clinical-and-genetic-backgrounds
#6
Kyoko Kitao, Hideki Mutai, Kazunori Namba, Noriko Morimoto, Atsuko Nakano, Yukiko Arimoto, Tomoko Sugiuchi, Sawako Masuda, Yasuhide Okamoto, Noriko Morita, Hirokazu Sakamoto, Tomoko Shintani, Satoshi Fukuda, Kimitaka Kaga, Tatsuo Matsunaga
OBJECTIVES: Auditory neuropathy (AN) is a clinical disorder characterized by the absence of auditory brainstem response and presence of otoacoustic emissions. A gradual loss of otoacoustic emissions has been reported for some cases of AN. Such cases could be diagnosed as cochlear hearing loss and lead to misunderstanding of the pathology when patients first visit clinics after the loss of otoacoustic emissions. The purpose of this study was to investigate the time course of changes in distortion product otoacoustic emissions (DPOAEs) in association with patients' genetic and clinical backgrounds, including the use of hearing aids...
April 23, 2018: Ear and Hearing
https://www.readbyqxmd.com/read/29671961/tmtc2-variant-associated-with-sensorineural-hearing-loss-and-auditory-neuropathy-spectrum-disorder-in-a-family-dyad
#7
Hector Guillen-Ahlers, Christy B Erbe, Frédéric D Chevalier, Maria J Montoya, Kip D Zimmerman, Carl D Langefeld, Michael Olivier, Christina L Runge
BACKGROUND: Sensorineural hearing loss (SNHL) is a common form of hearing loss that can be inherited or triggered by environmental insults; auditory neuropathy spectrum disorder (ANSD) is a SNHL subtype with unique diagnostic criteria. The genetic factors associated with these impairments are vast and diverse, but causal genetic factors are rarely characterized. METHODS: A family dyad, both cochlear implant recipients, presented with a hearing history of bilateral, progressive SNHL, and ANSD...
April 19, 2018: Molecular Genetics & Genomic Medicine
https://www.readbyqxmd.com/read/29662891/transient-abnormalities-in-masking-tuning-curve-in-early-progressive-hearing-loss-mouse-model
#8
Marion Souchal, Ludimila Labanca, Sirley Alves da Silva Carvalho, Luciana Macedo de Resende, Christelle Blavignac, Paul Avan, Fabrice Giraudet
Damage to cochlear outer hair cells (OHCs) usually affects frequency selectivity in proportion to hearing threshold increase. However, the current clinical heuristics that attributes poor hearing performance despite near-normal auditory sensitivity to auditory neuropathy or "hidden" synaptopathy overlooks possible underlying OHC impairment. Here, we document the part played by OHCs in influencing suprathreshold auditory performance in the presence of noise in a mouse model of progressive hair cell degeneration, the CD1 strain, at postnatal day 18-30 stages when high-frequency auditory thresholds remained near-normal...
2018: BioMed Research International
https://www.readbyqxmd.com/read/29625343/rapid-exhaustion-of-auditory-neural-conduction-in-a-prototypical-mitochondrial-disease-friedreich-ataxia
#9
Fabrice Giraudet, Perrine Charles, Thierry Mom, Odile Boespflug-Tanguy, Alexandra Dürr, Paul Deltenre, Paul Avan
OBJECTIVES: In patients with Friedreich ataxia (FRDA), mitochondrial failure leads to impaired cellular energetics. Since many FRDA patients have impaired hearing in noise, we investigated the objective consequences on standard auditory brainstem-evoked responses (ABRs). METHODS: In 37 FRDA patients, among whom 34 with abnormal standard ABRs, hearing sensitivity, speech-in-noise intelligibility and otoacoustic emissions were controlled. ABR recordings were split into four consecutive segments of the total time frame used for data collection, thus allowing the dynamics of ABR averaging to be observed...
March 27, 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/29614552/-retrospective-analysis-of-auditory-neuropathy-patients-after-cochlear-implantation
#10
F Ji, Q Wang, A T Chen, M D Hong, J N Li, H Zhao, S M Yang
Objectives: To study an effects on hearing ability and speech performance of the patients with auditory neuropathy (AN) after cochlear implantation (CI). Methods: Thirty-five AN patients (26 males and 9 females) after CI in our center since 2007 were chosen, including 5 postlingual patients (implanted age from 14.3 to 38.6 years old) and 30 prelingual patients (implanted age from 1.1 to 13.7 years old). Hearing sensitivity and speech performance were estimated via following methods: (1) implanted hearing thresholds by sound field audiometry; (2) speech audiometry, including monosyllable, disyllable and sentences recognition test by computer-controlled software and sentence recognition test under noise condition (signal to noise ratio=+ 10dB); (3) Mandarin Early Speech Perception test (MESP), Mandarin Pediatric Speech Intelligibility test (MPSI), MAIS, or IT-MAIS for prelingual AN implantees...
March 7, 2018: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
https://www.readbyqxmd.com/read/29614547/-hereditary-auditory-neuropathies-stepping-into-precision-management-from-the-discovery
#11
Q J Wang, Arnold Starr
No abstract text is available yet for this article.
March 7, 2018: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
https://www.readbyqxmd.com/read/29607003/patients-with-type-2-diabetes-demonstrate-proprioceptive-deficit-in-the-knee
#12
Lucas Richard Ettinger, Ami Boucher, Elisabeth Simonovich
AIM: To investigate proprioceptive discrepancies in the lower extremity in persons with type 2 diabetes mellitus (T2DM). METHODS: In this cross-sectional study, a total of 46 older persons were divided into a T2DM group ( n = 23) and a control group who did not have T2DM ( n = 23). Participants were given a brief warm up with stretching exercises. Diabetic neuropathy scores were collected prior to proprioceptive testing. For proprioceptive testing, participants performed leg extensions to randomized target positions of 15°, 30°, 45, 60° degrees of elevation in the sagittal plane, each target was repeated a total of four times...
March 15, 2018: World Journal of Diabetes
https://www.readbyqxmd.com/read/29605618/progressive-myoclonus-epilepsy-without-renal-failure-in-a-chinese-family-with-a-novel-mutation-in-scarb2-gene-and-literature-review
#13
Wo-Tu Tian, Xiao-Li Liu, Yang-Qi Xu, Xiao-Jun Huang, Hai-Yan Zhou, Ying Wang, Hui-Dong Tang, Sheng-Di Chen, Xing-Hua Luan, Li Cao
PURPOSE: To describe the clinical and genetic features of a Chinese progressive myoclonus epilepsy (PME) patient related with SCARB2 mutation without renal impairment and review 27 SCARB2-related PME patients from 11 countries. METHODS: The patient was a 27-year-old man with progressive action myoclonus, ataxia, epilepsy, dysarthria and absence of cognitive deterioration. Renal functional test was normal. Electroencephalography (EEG) showed progressively slowed background activity and sporadic generalized spike-and-wave discharges...
March 14, 2018: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/29605339/cochlear-implantation-in-children-with-auditory-neuropathy-spectrum-disorder-a-multicenter-study-on-auditory-performance-and-speech-production-outcomes
#14
Ahmad Daneshi, Marjan Mirsalehi, Seyed Basir Hashemi, Mohammad Ajalloueyan, Mohsen Rajati, Mohammad Mahdi Ghasemi, Hesamaldin Emamdjomeh, Alimohamad Asghari, Shabahang Mohammadi, Mohammad Mohseni, Saleh Mohebbi, Mohammad Farhadi
OBJECTIVES: To evaluate the auditory performance and speech production outcome in children with auditory neuropathy spectrum disorder (ANSD). The effect of age on the outcomes of the surgery at the time of implantation was also evaluated. METHODS: Cochlear implantation was performed in 136 children with bilateral severe-to- profound hearing loss due to ANSD, at four tertiary academic centers. The patients were divided into two groups based on the age at the time of implantation; Group I: Children ≤24 months, and Group II: subjects >24 months...
May 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29553844/estimation-of-the-status-of-spiral-ganglion-neurons-and-schwann-cells-in-the-auditory-neural-degeneration-mouse-using-the-auditory-brainstem-response
#15
Zhi-Jian Zhang, Hong-Xia Guan, Kun Yang, Bo-Kui Xiao, Hua Liao, Yang Jiang, Tao Zhou, Qing-Quan Hua
CONCLUSION: The auditory brainstem response (ABR) wave I threshold, latency and amplitude are insensitive to spiral ganglion neurons (SGNs) degeneration, but are sensitive to the degeneration of Schwann cells and can estimate the status of Schwann cells in a neural degeneration mouse model. The thorough pre-operative ABR assessment would be helpful in predicting cochlear implant performance. OBJECTIVES: This study aimed in finding a non-invasive electrophysiological method to evaluate the status of the auditory nerve and the Schwann cells in sensorineural hearing loss (SNHL) and auditory neuropathy (AN) ears, and providing useful information for candidates screening and outcome prediction in cochlear implantation...
March 19, 2018: Acta Oto-laryngologica
https://www.readbyqxmd.com/read/29492085/clinical-relationship-between-auditory-neuropathy-and-nervous-system-diseases
#16
Jingbo Wang, Lanlan Jin, Jun Chen, Xiaobi Fang, Zhisu Liao
Objective: To explore the clinical relationship between auditory neuropathy (AN) and nervous system diseases. Methods: A total of 134 AN patients who were treated in our hospital from December 2011 to April 2016 were selected. Then 120 cases (240 ears) with complete data of pure tone audiometry and acoustic immittance test were selected as an AN1 group, which was compared with 30 patients (49 ears) with general sensorineural hearing loss (SHL) in regard to the results of pure tone audiometry and acoustic immittance test...
November 2017: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/29435658/correction-to-the-capos-mutation-in-atp1a3-alters-na-k-atpase-function-and-results-in-auditory-neuropathy-which-has-implications-for-management
#17
Lisbeth Tranebjærg, Nicola Strenzke, Sture Lindholm, Nanna D Rendtorff, Hanne Poulsen, Himanshu Khandelia, Wojciech Kopec, Troels J Brünnich Lyngbye, Christian Hamel, Cecile Delettre, Beatrice Bocquet, Michael Bille, Hanne H Owen, Toke Bek, Hanne Jensen, Karen Østergaard, Claes Möller, Linda Luxon, Lucinda Carr, Louise Wilson, Kaukab Rajput, Tony Sirimanna, Katherine Harrop-Griffiths, Shamima Rahman, Barbara Vona, Julia Doll, Thomas Haaf, Oliver Bartsch, Hendrik Rosewich, Tobias Moser, Maria Bitner-Glindzicz
The following information was inadvertently omitted in the original publication.
March 2018: Human Genetics
https://www.readbyqxmd.com/read/29433858/hearing-loss-in-type-2-diabetes-in-association-with-diabetic-neuropathy
#18
Huihui Ren, Zhenggang Wang, Zhongyao Mao, Ping Zhang, Chunfang Wang, Aiguo Liu, Gang Yuan
BACKGROUND: Reports assessing hearing abnormalities in diabetes are debated. We aimed to evaluated auditory alterations and their possible associations with vascular and neurological dysfunction in 160 Type 2 diabetes mellitus individuals and 100 age and sex-matched healthy controls. METHODS: Participants underwent pure tone audiometry (PTA). Associations with demographic, metabolic and neuropathic variables were assessed. RESULTS: Compared with healthy controls, diabetic patients had higher mean hearing thresholds at each frequency, with statistical significance at 2-8 kHz (p <0...
October 2017: Archives of Medical Research
https://www.readbyqxmd.com/read/29371893/evaluation-of-dizziness-handicap-in-adolescents-and-adults-with-auditory-neuropathy-spectrum-disorder
#19
Prashanth Prabhu, Pratyasha Jamuar
Introduction  Vestibular symptoms and damage to the vestibular branch of the eighth cranial nerve is reported in individuals with auditory neuropathy spectrum disorder (ANSD). However, the real life handicap caused by these vestibular problems in individuals with ANSD is not studied. Objective  The present study attempted to evaluate the dizziness-related handicap in adolescents and adults with ANSD. Method  The dizziness handicap inventory (DHI) was administered to 40 adolescents and adults diagnosed with ANSD...
January 2018: International Archives of Otorhinolaryngology
https://www.readbyqxmd.com/read/29327279/emotion-recognition-and-psychological-comorbidity-in-friedreich-s-ataxia
#20
Teresa Costabile, Veronica Capretti, Filomena Abate, Agnese Liguori, Francesca Paciello, Chiara Pane, Anna De Rosa, Silvio Peluso, Giuseppe De Michele, Alessandro Filla, Francesco Saccà
Friedreich's ataxia (FRDA) is an autosomal recessive disease presenting with ataxia, corticospinal signs, peripheral neuropathy, and cardiac abnormalities. Little effort has been made to understand the psychological and emotional burden of the disease. The aim of our study was to measure patients' ability to recognize emotions using visual and non-verbal auditory hints, and to correlate this ability with psychological, neuropsychological, and neurological variables. We included 20 patients with FRDA, and 20 age, sex, and education matched healthy controls (HC)...
January 11, 2018: Cerebellum
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