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auditory brainstem response

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https://www.readbyqxmd.com/read/28731162/a-homozygous-myo7a-mutation-associated-to-usher-syndrome-and-unilateral-auditory-neuropathy-spectrum-disorder
#1
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/28730834/resveratrol-alleviates-ethanol-induced-hormonal-and-metabolic-disturbances-in-the-rat
#2
J Popelář, M Díaz Gómez, J Lindovský, N Rybalko, J Burianová, T Oohashi, J Syka
Brain-specific link protein Bral2 represents a substantial component of perineuronal nets (PNNs) enwrapping neurons in the central nervous system. To elucidate the role of Bral2 in auditory signal processing, the hearing function in knockout Bral2(-/-) (KO) mice was investigated using behavioral and electrophysiological methods and compared with wild type Bral2(+/+) (WT) mice. The amplitudes of the acoustic startle reflex (ASR) and the efficiency of the prepulse inhibition of ASR (PPI of ASR), produced by prepulse noise stimulus or gap in continuous noise, was similar in 2-week-old WT and KO mice...
July 18, 2017: Physiological Research
https://www.readbyqxmd.com/read/28723600/detailed-analysis-of-high-frequency-auditory-brainstem-response-in-patients-with-tinnitus-a-preliminary-study
#3
Joseph Pinkl, Matthew J Wilson, Danica Billingsly, Raymundo Munguia-Vazquez
Increased spontaneous activity and aberrant neural synchrony is thought to be the underlying cause of tinnitus. The perceived pitch of tinnitus may be dictated by frequency specific neural fibers of the subcortical pathway, or the projection of altered cortical activity by-way-of tonotopic reorganizations. Subcortical neural activity in relation to tinnitus was characterized using ABR measurements. In the present study, 11 patients (21 ears) with constant tonal tinnitus underwent a two-part experiment. Experiment 1 involved click ABR measurements and included two experimental groups: tinnitus with normal hearing from 2000-4000 Hz (GI) and tinnitus with hearing loss within the range of 2000-4000 Hz (GII)...
June 1, 2017: International Tinnitus Journal
https://www.readbyqxmd.com/read/28720776/dissonance-encoding-in-human-inferior-colliculus-covaries-with-individual-differences-in-dislike-of-dissonant-music
#4
Seung-Goo Kim, Jöran Lepsien, Thomas Hans Fritz, Toralf Mildner, Karsten Mueller
Harmony is one of the most fundamental elements of music that evokes emotional response. The inferior colliculus (IC) has been known to detect poor agreement of harmonics of sound, that is, dissonance. Electrophysiological evidence has implicated a relationship between a sustained auditory response mainly from the brainstem and unpleasant emotion induced by dissonant harmony. Interestingly, an individual's dislike of dissonant harmony of an individual correlated with a reduced sustained auditory response. In the current paper, we report novel evidence based on functional magnetic resonance imaging (fMRI) for such a relationship between individual variability in dislike of dissonance and the IC activation...
July 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28718886/brainstem-auditory-evoked-responses-in-37-dogs-with-otitis-media-before-and-after-topical-therapy
#5
S Paterson
OBJECTIVES: The objective of this study was to determine whether intra-aural administration of aqueous solutions of marbofloxacin, gentamicin, tobramycin and ticarcillin (used off-licence) was associated with changes in hearing as measured by brainstem auditory evoked responses. MATERIALS AND METHODS: Dogs diagnosed with otitis media (n=37) underwent brainstem auditory evoked response testing and then were treated for their ear disease. First, the external ear canal and middle ear were flushed with sterile saline followed by EDTA tris with 0·15% chlorhexidine...
July 18, 2017: Journal of Small Animal Practice
https://www.readbyqxmd.com/read/28712672/noise-induced-cochlear-synaptopathy-in-rhesus-monkeys-macaca-mulatta
#6
M D Valero, J A Burton, S N Hauser, T A Hackett, R Ramachandran, M C Liberman
Cochlear synaptopathy can result from various insults, including acoustic trauma, aging, ototoxicity, or chronic conductive hearing loss. For example, moderate noise exposure in mice can destroy up to ∼50% of synapses between auditory nerve fibers (ANFs) and inner hair cells (IHCs) without affecting outer hair cells (OHCs) or thresholds, because the synaptopathy occurs first in high-threshold ANFs. However, the fiber loss likely impairs temporal processing and hearing-in-noise, a classic complaint of those with sensorineural hearing loss...
July 8, 2017: Hearing Research
https://www.readbyqxmd.com/read/28710948/brainstem-auditory-evoked-potentials-for-diagnosing-autism-spectrum-disorder-adhd-and-schizophrenia-spectrum-disorders-in-adults-a-blinded-study
#7
Irina Manouilenko, Mats B Humble, Jeanette Georgieva, Susanne Bejerot
The aim of the present study was to examine the clinical utility of complex auditory brainstem response (c-ABR) and investigate if c-ABR is helpful in the diagnostic procedure. Thirty-one adult psychiatric patients, thoroughly diagnosed with autism spectrum disorder (ASD) (n=16), ADHD (n=8), or schizophrenia spectrum disorder (SSD) (n=7) and 15 healthy controls (HC), were blindly assessed with SensoDetect BERA. This c-ABR correctly identified psychiatric diagnoses in 4 patients (13%) and provided partially correct diagnoses in 11 more patients...
July 6, 2017: Psychiatry Research
https://www.readbyqxmd.com/read/28704898/a-case-of-auditory-neuropathy-caused-by-pontine-hemorrhage-in-an-adult
#8
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/28697607/wideband-acoustic-immittance-for-assessing-middle-ear-functioning-for-preterm-neonates-in-the-neonatal-intensive-care-unit
#9
Nandel Gouws, De Wet Swanepoel, Leigh Biagio De Jager
BACKGROUND: The primary aim of newborn hearing screening is to detect permanent hearing loss. Because otoacoustic emissions (OAEs) and automated auditory brainstem response (AABR) are sensitive to hearing loss, they are often used as screening tools. On the other hand, false-positive results are most often because of transient outer- and middle ear conditions. Wideband acoustic immittance (WAI), which includes physical measures known as reflectance and absorbance, has shown potential for accurate assessment of middle ear function in young infants...
June 28, 2017: South African Journal of Communication Disorders. die Suid-Afrikaanse Tydskrif Vir Kommunikasieafwykings
https://www.readbyqxmd.com/read/28689296/development-of-auditory-sensitivity-in-the-barn-owl
#10
Anna Kraemer, Caitlin Baxter, Alayna Hendrix, Catherine E Carr
Adult barn owl hearing is acute, but development of this sense is not well understood. We, therefore, measured auditory brainstem responses in barn owls from before the onset of hearing (posthatch day 2, or P2) to adulthood (P69). The first consistent responses were detected at P4 for 1 and 2 kHz, followed by responses to 0.5 and 4 kHz at P9, and 5 kHz at P13. Sensitivity to higher frequencies increased with age, with responses to 12 kHz appearing about 2 months after hatching, once the facial ruff was mature...
July 8, 2017: Journal of Comparative Physiology. A, Neuroethology, Sensory, Neural, and Behavioral Physiology
https://www.readbyqxmd.com/read/28688560/speech-evoked-auditory-brainstem-responses-in-children-with-hearing-loss
#11
Amineh Koravand, Rida Al Osman, Véronique Rivest, Catherine Poulin
OBJECTIVE: The main objective of the present study was to investigate subcortical auditory processing in children with sensorineural hearing loss. METHODS: Auditory Brainstem Responses (ABRs) were recorded using click and speech/da/stimuli. Twenty-five children, aged 6-14 years old, participated in the study: 13 with normal hearing acuity and 12 with sensorineural hearing loss. RESULTS: No significant differences were observed for the click-evoked ABRs between normal hearing and hearing-impaired groups...
August 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28687812/blast-induced-tinnitus-and-elevated-central-auditory-and-limbic-activity-in-rats-a-manganese-enhanced-mri-and-behavioral-study
#12
Jessica Ouyang, Edward Pace, Laura Lepczyk, Michael Kaufman, Jessica Zhang, Shane A Perrine, Jinsheng Zhang
Blast-induced tinitus is the number one service-connected disability that currently affects military personnel and veterans. To elucidate its underlying mechanisms, we subjected 13 Sprague Dawley adult rats to unilateral 14 psi blast exposure to induce tinnitus and measured auditory and limbic brain activity using manganese-enhanced MRI (MEMRI). Tinnitus was evaluated with a gap detection acoustic startle reflex paradigm, while hearing status was assessed with prepulse inhibition (PPI) and auditory brainstem responses (ABRs)...
July 7, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28686716/gsr-is-not-essential-for-the-maintenance-of-antioxidant-defenses-in-mouse-cochlea-possible-role-of-the-thioredoxin-system-as-a-functional-backup-for-gsr
#13
Chul Han, Mi-Jung Kim, Dalian Ding, Hyo-Jin Park, Karessa White, Logan Walker, Tongjun Gu, Masaru Tanokura, Tatsuya Yamasoba, Paul Linser, Richard Salvi, Shinichi Someya
Glutathione reductase (GSR), a key member of the glutathione antioxidant defense system, converts oxidized glutathione (GSSG) to reduced glutathione (GSH) and maintains the intracellular glutathione redox state to protect the cells from oxidative damage. Previous reports have shown that Gsr deficiency results in defects in host defense against bacterial infection, while diquat induces renal injury in Gsr hypomorphic mice. In flies, overexpression of GSR extended lifespan under hyperoxia. In the current study, we investigated the roles of GSR in cochlear antioxidant defense using Gsr homozygous knockout mice that were backcrossed onto the CBA/CaJ mouse strain, a normal-hearing strain that does not carry a specific Cdh23 mutation that causes progressive hair cell degeneration and early onset of hearing loss...
2017: PloS One
https://www.readbyqxmd.com/read/28685837/proteomic-analyses-of-nucleus-laminaris-identified-candidate-targets-of-the-fragile-x-mental-retardation-protein
#14
Hitomi Sakano, Diego A R Zorio, Xiaoyu Wang, Ying S Ting, William S Noble, Michael J MacCoss, Edwin W Rubel, Yuan Wang
The avian nucleus laminaris (NL) is a brainstem nucleus necessary for binaural processing, analogous in structure and function to the mammalian medial superior olive. In chickens (Gallus gallus), NL is a well-studied model system for activity-dependent neural plasticity. Its neurons have bipolar extension of dendrites, which receive segregated inputs from two ears and display rapid and compartment-specific reorganization in response to unilateral changes in auditory input. More recently, FMRP (fragile X mental retardation protein), an RNA-binding protein that regulates local protein translation, has been shown to be enriched in NL dendrites, suggesting its potential role in the structural dynamics of these dendrites...
July 7, 2017: Journal of Comparative Neurology
https://www.readbyqxmd.com/read/28683731/nephron-development-and-extrarenal-features-in-a-child-with-congenital-nephrotic-syndrome-caused-by-null-lamb2-mutations
#15
Jiro Kino, Hiroyasu Tsukaguchi, Takahisa Kimata, Huan Thanh Nguyen, Yorika Nakano, Noriko Miyake, Naomichi Matsumoto, Kazunari Kaneko
BACKGROUND: Congenital nephrotic syndrome (CNS) is a rare disorder caused by various structural and developmental defects of glomeruli. It occurs typically as an isolated kidney disorder but associates sometimes with other systemic, extrarenal manifestations. CASE PRESENTATIONS: An infant presented with severe CNS, which progressed rapidly to renal failure at age of 3 months and death at 27 months. The clinical phenotypes and genetic causes were studied, including the renal pathology at autopsy...
July 6, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28682820/current-assessment-of-newborn-hearing-screening-protocols
#16
Rosario R Ricalde, Charlotte M Chiong, Patrick John P Labra
PURPOSE OF REVIEW: The objective of this article is to assess current newborn hearing screening protocols. We will focus on technologies or modalities used, protocol steps, training of screeners, timing of first screen, and loss to follow-up. A summary of program reports focusing on protocols from Greece, China, South Africa, France, Spain, South Korea, Denmark, Italy, Turkey, Taiwan, South Korea, Poland and Iran as they are recently reported will also be presented. RECENT FINDINGS: Community-based hearing screening programs in South Africa and efforts in the Asian region are being reported...
July 5, 2017: Current Opinion in Otolaryngology & Head and Neck Surgery
https://www.readbyqxmd.com/read/28681310/brainstem-reflexes-are-hyperactive-in-patients-with-drug-induced-akathisia
#17
Baris Metin, Sinem Zeynep Metin, Aysegul Gunduz, Burc Cagri Poyraz, Mine Ozmen, Gunes Kiziltan, Meral E Kiziltan
Akathisia is a sensori-motor phenomenon which is generally encountered as an adverse effect of antidopaminergic medications suggesting involvement of dopaminergic pathways. We recently showed nociceptive flexor reflex was altered in akathisia as compared to restless legs syndrome and therefore, these findings may indicate co-involvement of pathways other than dopaminergic ones. To examine functional status of different pathways, we investigated auditory startle reflex (ASR), startle response to somatosensory input (SSS), and trigemino-cervical reflex (TCR) in a group of patients with akathisia...
July 5, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28679259/auditory-sensitivity-of-the-tufted-capuchin-sapajus-apella-a-test-of-allometric-predictions
#18
Marissa A Ramsier, Christopher J Vinyard, Nathaniel J Dominy
New World monkeys are a diverse primate group and a model for understanding hearing in mammals. However, comparable audiograms do not exist for the larger monkeys, making it difficult to test the hypothesized relationship between interaural distance and high-frequency hearing limit (i.e., the allometric model). Here, the auditory brainstem response (ABR) method is used to assess auditory sensitivity in four tufted capuchins (Sapajus apella), a large monkey with a large interaural distance. A primate-typical four-peak pattern in the ABR waveforms was found with peak latencies from ca...
June 2017: Journal of the Acoustical Society of America
https://www.readbyqxmd.com/read/28678080/noise-history-and-auditory-function-in-young-adults-with-and-without-type-1-diabetes-mellitus
#19
Christopher Spankovich, Colleen G Le Prell, Edward Lobarinas, Linda J Hood
OBJECTIVES: The purpose of this study was to examine the relationship between noise exposure history, type 1 diabetes mellitus (DM), and suprathreshold measures of auditory function. DESIGN: A cross-sectional study was conducted; 20 normal-hearing participants without type 1 DM were matched on age and sex to 20 normal-hearing participants with type 1 DM (n=40). Participants, all having normal audiometric thresholds, completed noise history questionnaires and a battery of auditory physiological tests including transient evoked otoacoustic emissions, distortion product otoacoustic emissions, and auditory brainstem responses (ABR) at 80 dB nHL and at 2 different stimulus rates in both ears...
July 1, 2017: Academic Medicine: Journal of the Association of American Medical Colleges
https://www.readbyqxmd.com/read/28674175/enhanced-excitatory-connectivity-and-disturbed-sound-processing-in-the-auditory-brainstem-of-fragile-x-mice
#20
Elisabet Garcia-Pino, Nikodemus Gessele, Ursula Koch
Hypersensitivity to sounds is one of the prevalent symptoms in individuals with Fragile X syndrome (FXS). It manifests behaviorally early during development and is often used as a landmark for treatment efficacy. However, the physiological mechanisms and circuit-level alterations underlying this aberrant behavior remain poorly understood. Using the mouse model of FXS (Fmr1 KO) we demonstrate that functional maturation of auditory brainstem synapses is impaired in FXS. Fmr1 KO mice showed a greatly enhanced excitatory synaptic input strength in neurons of the lateral superior olive (LSO), a prominent auditory brainstem nucleus, which integrates ipsilateral excitation and contralateral inhibition to compute interaural level differences (ILDs)...
July 3, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
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