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Auditory neuropathy spectrum disorder

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https://www.readbyqxmd.com/read/29725841/auditory-function-in-pelizaeus-merzbacher-disease
#1
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/29696047/comparison-of-cochlear-microphonics-magnitude-with-broad-and-narrow-band-stimuli-in-healthy-adult-wistar-rats
#2
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/29671961/tmtc2-variant-associated-with-sensorineural-hearing-loss-and-auditory-neuropathy-spectrum-disorder-in-a-family-dyad
#3
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/29625343/rapid-exhaustion-of-auditory-neural-conduction-in-a-prototypical-mitochondrial-disease-friedreich-ataxia
#4
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/29605339/cochlear-implantation-in-children-with-auditory-neuropathy-spectrum-disorder-a-multicenter-study-on-auditory-performance-and-speech-production-outcomes
#5
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/29371893/evaluation-of-dizziness-handicap-in-adolescents-and-adults-with-auditory-neuropathy-spectrum-disorder
#6
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/29184165/atp1a3-mutations-can-cause-progressive-auditory-neuropathy-a-new-gene-of-auditory-synaptopathy
#7
Kyu-Hee Han, Doo-Yi Oh, Seungmin Lee, Chung Lee, Jin Hee Han, Min Young Kim, Hye-Rim Park, Moo Kyun Park, Nayoung K D Kim, Jaekwang Lee, Eunyoung Yi, Jong-Min Kim, Jeong-Whun Kim, Jong-Hee Chae, Seung Ha Oh, Woong-Yang Park, Byung Yoon Choi
The etiologies and prevalence of sporadic, postlingual-onset, progressive auditory neuropathy spectrum disorder (ANSD) have rarely been documented. Thus, we aimed to evaluate the prevalence and molecular etiologies of these cases. Three out of 106 sporadic progressive hearing losses turned out to manifest ANSD. Through whole exome sequencing and subsequent bioinformatics analysis, two out of the three were found to share a de novo variant, p.E818K of ATP1A3, which had been reported to cause exclusively CAPOS (cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss) syndrome...
November 28, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28965846/fdxr-mutations-cause-sensorial-neuropathies-and-expand-the-spectrum-of-mitochondrial-fe-s-synthesis-diseases
#8
Antoine Paul, Anthony Drecourt, Floriane Petit, Delphine Dupin Deguine, Christelle Vasnier, Myriam Oufadem, Cécile Masson, Crystel Bonnet, Saber Masmoudi, Isabelle Mosnier, Laurence Mahieu, Didier Bouccara, Josseline Kaplan, Georges Challe, Christelle Domange, Fanny Mochel, Olivier Sterkers, Sylvie Gerber, Patrick Nitschke, Christine Bole-Feysot, Laurence Jonard, Souad Gherbi, Oriane Mercati, Ines Ben Aissa, Stanislas Lyonnet, Agnès Rötig, Agnès Delahodde, Sandrine Marlin
Hearing loss and visual impairment in childhood have mostly genetic origins, some of them being related to sensorial neuronal defects. Here, we report on eight subjects from four independent families affected by auditory neuropathy and optic atrophy. Whole-exome sequencing revealed biallelic mutations in FDXR in affected subjects of each family. FDXR encodes the mitochondrial ferredoxin reductase, the sole human ferredoxin reductase implicated in the biosynthesis of iron-sulfur clusters (ISCs) and in heme formation...
October 5, 2017: American Journal of Human Genetics
https://www.readbyqxmd.com/read/28954873/chronic-auditory-toxicity-in-late-preterm-and-term-infants-with-significant-hyperbilirubinemia
#9
MULTICENTER STUDY
Sanjiv B Amin, Satish Saluja, Arvind Saili, Mark Orlando, Hongyue Wang, Nirupama Laroia, Asha Agarwal
BACKGROUND AND OBJECTIVES: Significant hyperbilirubinemia (SHB) may cause chronic auditory toxicity (auditory neuropathy spectrum disorder and/or sensorineural hearing loss); however, total serum bilirubin (TSB) does not discriminate neonates at risk for auditory toxicity. Our objective was to compare TSB, bilirubin albumin molar ratio (BAMR), and unbound bilirubin (UB) for their association with chronic auditory toxicity in neonates with SHB (TSB ≥20 mg/dL or TSB that met criteria for exchange transfusion)...
October 2017: Pediatrics
https://www.readbyqxmd.com/read/28816694/evaluation-of-hearing-handicap-in-adults-with-auditory-neuropathy-spectrum-disorder
#10
Prashanth Prabhu
OBJECTIVE: The present study attempted to evaluate hearing handicap in adults with auditory neuropathy spectrum disorder (ANSD). The study also attempted to determine if gender, pure tone average, speech identification scores (SIS), and reported duration of hearing loss could predict the hearing handicap in adults with ANSD. MATERIALS AND METHODS: Hearing Handicap Inventory for Adults and Hearing Handicap Questionnaire were administered to 50 adults with ANSD. RESULTS: Using both the scales, there was a significant hearing handicap in both the social and emotional domains in adults with ANSD...
August 2017: Journal of International Advanced Otology
https://www.readbyqxmd.com/read/28816204/deep-band-modulated-phrase-perception-in-quiet-and-noise-in-individuals-with-auditory-neuropathy-spectrum-disorder-and-sensorineural-hearing-loss
#11
Hemanth Narayan Shetty, Vishal Kooknoor
CONTEXT: Deep band modulation (DBM) improves speech perception in individuals with learning disability and older adults, who had temporal impairment in them. However, it is unclear on perception of DBM phrases at quiet and noise conditions in individuals with auditory neuropathy spectrum disorder (ANSD) and sensorineural hearing loss (SNHL), as these individuals suffer from temporal impairment. AIM: The aim is to study the effect of DBM and noise on phrase perception in individuals with normal hearing, SNHL, and ANSD...
July 2017: Noise & Health
https://www.readbyqxmd.com/read/28769753/intraoperative-electrocochleographic-characteristics-of-auditory-neuropathy-spectrum-disorder-in-cochlear-implant-subjects
#12
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
#13
LETTER
C C Wu, C J Hsu, F L Huang, Y H Lin, Y H Lin, T C Liu, C M Wu
No abstract text is available yet for this article.
February 2018: Clinical Otolaryngology
https://www.readbyqxmd.com/read/28731162/a-homozygous-myo7a-mutation-associated-to-usher-syndrome-and-unilateral-auditory-neuropathy-spectrum-disorder
#14
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...
October 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28708635/the-management-of-pediatric-hearing-loss-caused-by-auditory-neuropathy-spectrum-disorder
#15
REVIEW
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...
October 2017: Current Opinion in Otolaryngology & Head and Neck Surgery
https://www.readbyqxmd.com/read/28688553/clinical-role-of-electrocochleography-in-children-with-auditory-neuropathy-spectrum-disorder
#16
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/28680492/effectiveness-of-low-cut-modified-amplification-using-receiver-in-the-canal-hearing-aid-in-individuals-with-auditory-neuropathy-spectrum-disorder
#17
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/28555599/speech-perception-in-quiet-and-in-noise-condition-in-individuals-with-auditory-neuropathy-spectrum-disorder
#18
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/28485679/effectiveness-of-low-cut-modified-amplification-strategy-and-channel-free-hearing-aid-in-individuals-with-auditory-neuropathy-spectrum-disorder
#19
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...
October 2017: International Journal of Audiology
https://www.readbyqxmd.com/read/28357181/prevalence-of-vestibular-symptoms-in-individuals-with-auditory-neuropathy-spectrum-disorder-a-retrospective-study
#20
Prashanth Prabhu, Pratyasha Jamuar
The objective of the study was to retrospectively determine the prevalence of vestibular symptoms in individuals with auditory neuropathy spectrum disorder (ANSD). It was also attempted to determine the prevalence of vestibular symptoms and factors (gender and age of reported hearing loss) that could affect the prevalence in individuals with ANSD. The vestibular symptoms reported in the case history were analyzed in individuals diagnosed with ANSD. The symptoms reported by a total of 316 individuals (185 females and 131 males) with ANSD were analyzed...
February 2017: Intractable & Rare Diseases Research
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