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auditory neuropathy

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https://www.readbyqxmd.com/read/27891689/deficit-in-acoustic-signal-in-noise-detection-in-glycine-receptor-%C3%AE-3-subunit-knockout-mice
#1
Konstantin Tziridis, Stefanie Buerbank, Volker Eulenburg, Julia Dlugaiczyk, Holger Schulze
Hearing is an essential sense for communication in animals and humans. Normal function of the cochlea of higher vertebrates relies on a fine-tuned interplay of afferent and efferent innervation of both inner and outer hair cells. Efferent inhibition is controlled via olivocochlear feedback loops, mediated mainly by acetylcholine, γ-aminobutyric acid (GABA) and glycine, and is one of the first sites affected by synapto- and neuropathy in the development of hearing loss. While the functions of acetylcholine, GABA, and other inhibitory transmitters within these feedback loops are at least partially understood, especially the function of glycine still remains elusive...
November 27, 2016: European Journal of Neuroscience
https://www.readbyqxmd.com/read/27885979/gender-differences-in-audiological-findings-and-hearing-aid-benefit-in-255-individuals-with-auditory-neuropathy-spectrum-disorder-a-retrospective-study
#2
Vijaya Kumar Narne, Prashanth Prabhu, Hunsur S Chandan, Mahadeva Deepthi
BACKGROUND: There are many studies reported in the literature that have summarized audiological findings and possible rehabilitation in individuals with auditory neuropathy spectrum disorder (ANSD). However, there are very few studies that have attempted to delineate the gender differences in audiological characteristics and hearing aid benefit in individuals with ANSD. PURPOSE: The study aimed to explore the differences between males and females in terms of demographic details, audiogram, speech identification scores, otoacoustic emissions, acoustic reflexes, long latency responses, and hearing aid benefit...
November 2016: Journal of the American Academy of Audiology
https://www.readbyqxmd.com/read/27865214/-auditory-neuropathy-clinical-evaluation-and-diagnostic-approach
#3
Guilherme Machado Carvalho, Beatriz Prista Leão, Priscila Zonzini Ramos, Alexandre Caixeta Guimarães, Arthur Menino Castilho, Edi Lúcia Sartorato
INTRODUCTION: Auditory neuropathy is a condition in which there is a change in the neuronal transmission of the auditory stimuli. Our objective was to describe the patients' series within the clinical spectrum of auditory neuropathy. MATERIAL AND METHODS: We designed a transversal, retrospective study, with a description of a consecutive case series. Auditory neuropathy was defined by the presence of acoustic otoemissions plus absent/abnormal auditory brainstem responses with cochlear microphonism...
June 2016: Acta Médica Portuguesa
https://www.readbyqxmd.com/read/27843504/relationship-between-patients-with-clinical-auditory-neuropathy-spectrum-disorder-and-mutations-in-gjb2-gene
#4
Guilherme M de Carvalho, Priscila Z Ramos, Arthur M Castilho, Alexandre C Guimarães, Edi L Sartorato
: The auditory neuropathy is a condition which there is a dyssynchrony in the nerve conduction of the auditory nerve fibers. There is no evidence about the relationship between patients with clinical auditory neuropathy spectrum disorder and mutations in GJB2 gene. There are only two studies about this topic in the medical literature. Connexin 26 (GJB2 gene) mutations are common causes of genetic deafness in many populations and we also being reported in subjects with auditory neuropathy...
2016: Open Neurology Journal
https://www.readbyqxmd.com/read/27761598/seeing-the-sound-after-visual-loss-functional-mri-in-acquired-auditory-visual-synesthesia
#5
Zixin Yong, Po-Jang Hsieh, Dan Milea
Acquired auditory-visual synesthesia (AVS) is a rare neurological sign, in which specific auditory stimulation triggers visual experience. In this study, we used event-related fMRI to explore the brain regions correlated with acquired monocular sound-induced phosphenes, which occurred 2 months after unilateral visual loss due to an ischemic optic neuropathy. During the fMRI session, 1-s pure tones at various pitches were presented to the patient, who was asked to report occurrence of sound-induced phosphenes by pressing one of the two buttons (yes/no)...
October 19, 2016: Experimental Brain Research. Experimentelle Hirnforschung. Expérimentation Cérébrale
https://www.readbyqxmd.com/read/27759031/whole-exome-sequencing-identifies-novel-variants-in-pnpt1-causing-oxidative-phosphorylation-defects-and-severe-multisystem-disease
#6
Ahmad Alodaib, Nara Sobreira, Wendy A Gold, Lisa G Riley, Nicole J Van Bergen, Meredith J Wilson, Bruce Bennetts, David R Thorburn, Corinne Boehm, John Christodoulou
Recent advances in next-generation sequencing strategies have led to the discovery of many novel disease genes. We describe here a non-consanguineous family with two affected boys presenting with early onset of severe axonal neuropathy, optic atrophy, intellectual disability, auditory neuropathy and chronic respiratory and gut disturbances. Whole-exome sequencing (WES) was performed on all family members and we identified compound heterozygous variants (c.[760C>A];[1528G>C];p.[(Gln254Lys);(Ala510Pro)] in the polyribonucleotide nucleotidyltransferase 1 (PNPT1) gene in both affected individuals...
October 19, 2016: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/27755139/performance-intensity-function-and-aided-improvement-in-individuals-with-late-onset-auditory-neuropathy-spectrum-disorder
#7
Jijo Pottackal Mathai, Asha Yathiraj
OBJECTIVES: The purpose of the study was to explore the effect of intensity on speech perception in individuals with late-onset auditory neuropathy spectrum disorder (ANSD) by obtaining their performance-intensity (PI) function. Additionally, the study investigated the effect of PI function on aided speech perception. It was hypothesized that speech perception abilities of individuals with ANSD vary with intensity and may provide information regarding their aided performance. DESIGN: A factorial research design was used to obtain the PI function and aided performance...
October 13, 2016: Ear and Hearing
https://www.readbyqxmd.com/read/27718221/auditory-toxicity-in-late-preterm-and-term-neonates-with-severe-jaundice
#8
Sanjiv B Amin, Satish Saluja, Arvind Saili, Nirupama Laroia, Mark Orlando, Hongyue Wang, Asha Agarwal
AIM: Jaundice may cause auditory toxicity (auditory neuropathy and hearing loss). However, total serum bilirubin (TSB) does not discriminate neonates at risk for auditory toxicity. We compared TSB, bilirubin:albumin molar ratio (BAMR), and unbound bilirubin for their association with auditory toxicity in neonates with severe jaundice (TSB ≥342μmol/L, or that met exchange transfusion). METHOD: Neonates greater or equal to 34 weeks gestational age with severe jaundice during the first 2 postnatal weeks were eligible for prospective cohort study, unless they had craniofacial malformations, chromosomal disorders, toxoplasmosis, other infections, rubella, cytomegalovirus, herpes simplex infections, surgery, or family history of congenital deafness...
October 8, 2016: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/27688594/the-p1-biomarker-for-assessing-cortical-maturation-in-pediatric-hearing-loss-a-review
#9
Anu Sharma, Hannah Glick, Emily Deeves, Erin Duncan
We review evidence for a high degree of neuroplasticity of the central auditory pathways in early childhood, citing evidence of studies of the P1 and N1 cortical auditory evoked potentials in congenitally deaf children receiving cochlear implants at different ages during childhood, children with auditory neuropathy spectrum disorder and children with hearing loss and comorbid multiple disabilities. We discuss neuroplasticity, including cortico-cortical de-coupling and cross-modal re-organization that occurs in deafness...
December 2015: Otorinolaringologia
https://www.readbyqxmd.com/read/27666974/photobiomodulation-by-laser-therapy-rescued-auditory-neuropathy-induced-by-ouabain
#10
Min Young Lee, Seung-Hyun Bae, So-Young Chang, Jae-Hun Lee, Se-Hyung Kim, Jin-Chul Ahn, Phil-Sang Chung, Wesley Moy, Jae Yun Jung
Auditory neuropathy is a hearing disorder caused by impaired auditory nerve function. The lack of information about the pathophysiology of this disease limits early diagnosis and further treatment. Laser therapy is a novel approach to enhance nerve growth or induce axonal regeneration. We induced auditory neural degeneration sparing the sensory epithelium with local ouabain application in an animal model and observed the rescue effect of photobiomodulation (PBM), showing recovered auditory function and favorable histologic outcome...
October 28, 2016: Neuroscience Letters
https://www.readbyqxmd.com/read/27658576/autosomal-dominant-auditory-neuropathy-and-variant-diaph3-c-173c-t
#11
Ana Sánchez-Martínez, José I Benito-Orejas, Juan J Tellería-Orriols, María J Alonso-Ramos
No abstract text is available yet for this article.
September 19, 2016: Acta Otorrinolaringológica Española
https://www.readbyqxmd.com/read/27652356/a-novel-missense-mutation-in-the-c2c-domain-of-otoferlin-causes-profound-hearing-impairment-in-an-omani-family-with-auditory-neuropathy
#12
Nadia M Al-Wardy, Mohammed N Al-Kindi, Mazin J Al-Khabouri, Yahya Tamimi, Guy Van Camp
OBJECTIVES: To identify genetic defects in an Omani family diagnosed with deafness.  METHODS: A cross-sectional association study was conducted at the Department of Biochemistry, College of Medicine and Health Sciences, Sultan Qaboos University, Al-Khoud, Oman and the Centre of Medical Genetics, University of Antwerp, Antwerp, Belgium between August 2010 and September 2014. Microsatellites markers for nine non-syndromic genes were used to genotype the defective locus using the extracted DNA from family members...
October 2016: Saudi Medical Journal
https://www.readbyqxmd.com/read/27643807/muscle-spindle-alterations-precede-onset-of-sensorimotor-deficits-in-charcot-marie-tooth-type-2e
#13
E Villalón, M R Jones, C Sibigtroth, S J Zino, J M Dale, D S Landayan, H Shen, D D W Cornelison, M L Garcia
Charcot-Marie-Tooth (CMT) is the most common inherited peripheral neuropathy, affecting approximately 2.8 million people. The CMT leads to distal neuropathy that is characterized by reduced motor nerve conduction velocity, ataxia, muscle atrophy and sensory loss. We generated a mouse model of CMT type 2E (CMT2E) expressing human neurofilament light E396K (hNF-L(E396K) ), which develops decreased motor nerve conduction velocity, ataxia and muscle atrophy by 4 months of age. Symptomatic hNF-L(E396K) mice developed phenotypes that were consistent with proprioceptive sensory defects as well as reduced sensitivity to mechanical stimulation, while thermal sensitivity and auditory brainstem responses were unaltered...
September 19, 2016: Genes, Brain, and Behavior
https://www.readbyqxmd.com/read/27630546/persistent-thalamic-sound-processing-despite-profound-cochlear-denervation
#14
Anna R Chambers, Juan J Salazar, Daniel B Polley
Neurons at higher stages of sensory processing can partially compensate for a sudden drop in peripheral input through a homeostatic plasticity process that increases the gain on weak afferent inputs. Even after a profound unilateral auditory neuropathy where >95% of afferent synapses between auditory nerve fibers and inner hair cells have been eliminated with ouabain, central gain can restore cortical processing and perceptual detection of basic sounds delivered to the denervated ear. In this model of profound auditory neuropathy, auditory cortex (ACtx) processing and perception recover despite the absence of an auditory brainstem response (ABR) or brainstem acoustic reflexes, and only a partial recovery of sound processing at the level of the inferior colliculus (IC), an auditory midbrain nucleus...
2016: Frontiers in Neural Circuits
https://www.readbyqxmd.com/read/27621663/identification-of-a-novel-pathogenic-otof-variant-causative-of-nonsyndromic-hearing-loss-with-high-frequency-in-the-ashkenazi-jewish-population
#15
Anastasia M Fedick, Chaim Jalas, Ananya Swaroop, Eric E Smouha, Bryn D Webb
Mutations in the OTOF gene have previously been shown to cause nonsyndromic prelingual deafness (DFNB9, OMIM 601071) as well as auditory neuropathy/dys-synchrony. In this study, the OTOF NM_194248.2 c.5332G>T, p.Val1778Phe variant was identified in a large Ashkenazi Jewish family as the causative variant in four siblings with hearing loss. Our analysis reveals a carrier frequency of the OTOF c.5332G>T, p.Val1778Phe variant of 1.27% in the Ashkenazi Jewish population, suggesting that this variant may be a significant contributor to nonsyndromic sensorineural hearing loss and should be considered for inclusion in targeted hearing loss panels for this population...
2016: Application of Clinical Genetics
https://www.readbyqxmd.com/read/27591005/bilirubin-induced-neurological-dysfunction-a-clinico-radiological-neurophysiological-correlation-in-30-consecutive-children
#16
Ronald van Toorn, Philip Brink, Johan Smith, Christelle Ackermann, Regan Solomons
The clinical expression of bilirubin-induced neurological dysfunction varies according to severity and location of the disease. Definitions have been proposed to describe different bilirubin-induced neurological dysfunction subtypes. Our objective was to describe the severity and clinico-radiological-neurophysiological correlation in 30 consecutive children with bilirubin-induced neurological dysfunction seen over a period of 5 years. Thirty children exposed to acute neonatal bilirubin encephalopathy were included in the study...
September 2, 2016: Journal of Child Neurology
https://www.readbyqxmd.com/read/27587922/detection-rates-of-cortical-auditory-evoked-potentials-at-different-sensation-levels-in-infants-with-sensory-neural-hearing-loss-and-auditory-neuropathy-spectrum-disorder
#17
Kirsty Gardner-Berry, Hsiuwen Chang, Teresa Y C Ching, Sanna Hou
With the introduction of newborn hearing screening, infants are being diagnosed with hearing loss during the first few months of life. For infants with a sensory/neural hearing loss (SNHL), the audiogram can be estimated objectively using auditory brainstem response (ABR) testing and hearing aids prescribed accordingly. However, for infants with auditory neuropathy spectrum disorder (ANSD) due to the abnormal/absent ABR waveforms, alternative measures of auditory function are needed to assess the need for amplification and evaluate whether aided benefit has been achieved...
February 2016: Seminars in Hearing
https://www.readbyqxmd.com/read/27579218/evaluation-of-depression-anxiety-and-stress-in-adolescents-and-young-adults-with-auditory-neuropathy-spectrum-disorder
#18
Prashanth Prabhu
The aim of the present study was to determine the severity of stress, anxiety, and depression using Depression Anxiety Stress Scales (DASS) in adolescents and young adults with auditory neuropathy spectrum disorder (ANSD). DASS was administered to 20 individuals with auditory neuropathy spectrum disorder. The effect of gender on severity of anxiety, stress, and depression on DASS scores was determined. It was attempted to determine the correlation of severity of anxiety, stress, and depression with the reported onset of the problem, degree of hearing loss, and speech identification scores...
2016: Scientifica
https://www.readbyqxmd.com/read/27553899/auditory-thalamic-circuits-and-gabaa-receptor-function-putative-mechanisms-in-tinnitus-pathology
#19
Donald M Caspary, Daniel A Llano
Tinnitus is defined as a phantom sound (ringing in the ears), and can significantly reduce the quality of life for those who suffer its effects. Ten to fifteen percent of the general adult population report symptoms of tinnitus with 1-2% reporting that tinnitus negatively impacts their quality of life. Noise exposure is the most common cause of tinnitus and the military environment presents many challenging high-noise situations. Military noise levels can be so intense that standard hearing protection is not adequate...
August 20, 2016: Hearing Research
https://www.readbyqxmd.com/read/27531021/pathophysiology-of-the-inner-ear-after-blast-injury-caused-by-laser-induced-shock-wave
#20
Katsuki Niwa, Kunio Mizutari, Toshiyasu Matsui, Takaomi Kurioka, Takeshi Matsunobu, Satoko Kawauchi, Yasushi Satoh, Shunichi Sato, Akihiro Shiotani, Yasushi Kobayashi
The ear is the organ that is most sensitive to blast overpressure, and ear damage is most frequently seen after blast exposure. Blast overpressure to the ear results in sensorineural hearing loss, which is untreatable and is often associated with a decline in the quality of life. In this study, we used a rat model to demonstrate the pathophysiological and structural changes in the inner ear that replicate pure sensorineural hearing loss associated with blast injury using laser-induced shock wave (LISW) without any conductive hearing loss...
2016: Scientific Reports
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