Brain structure correlates with auditory function in children diagnosed with auditory neuropathy spectrum disorder
Sign Up to like & getrecommendations! Published in 2022 at "Brain and Behavior"
DOI: 10.1002/brb3.2773
Abstract: Auditory neuropathy spectrum disorder (ANSD) is a term for a collection of test results which indicate disruption of the auditory signal at some point along the neural pathway. This results in a spectrum of functional… read more here.
Keywords: brain structure; auditory neuropathy; brain; neuropathy spectrum ... See more keywords
Effect of acoustic features on discrimination ability in individuals with auditory neuropathy spectrum disorder: an electrophysiological and behavioral study
Sign Up to like & getrecommendations! Published in 2019 at "European Archives of Oto-Rhino-Laryngology"
DOI: 10.1007/s00405-019-05405-9
Abstract: PurposeThe present study aimed to investigate the discrimination ability for acoustic cues in individuals with auditory neuropathy spectrum disorder (ANSD) using both behavioral and neural measures and to compare the result with normal hearing individuals.MethodsFour… read more here.
Keywords: auditory neuropathy; acoustic features; study; individuals auditory ... See more keywords
A new genetic variant causing auditory neuropathy: A CARE case report.
Sign Up to like & getrecommendations! Published in 2021 at "European annals of otorhinolaryngology, head and neck diseases"
DOI: 10.1016/j.anorl.2021.08.007
Abstract: INTRODUCTION Auditory neuropathy refers to impaired synchronization of the auditory signal along the cochlear nerve. The present study, following CARE case report guidelines, describes a case of auditory neuropathy secondary to a genetic variant not… read more here.
Keywords: auditory neuropathy; care case; auditory; genetic variant ... See more keywords
Vestibular dysfunction in patients with auditory neuropathy detected by vestibular evoked myogenic potentials
Sign Up to like & getrecommendations! Published in 2020 at "Clinical Neurophysiology"
DOI: 10.1016/j.clinph.2020.02.002
Abstract: OBJECTIVES This study aimed to determine vestibular involvement in patients with auditory neuropathy (AN) using ocular vestibular evoked myogenic potential (oVEMP), cervical vestibular evoked myogenic potential (cVEMP), caloric tests, video Head Impulse Tests (vHIT), and… read more here.
Keywords: auditory neuropathy; vestibular evoked; patients auditory; test ... See more keywords
Case report of two children with auditory neuropathy spectrum disorder related to a neurofascin (NFASC) gene variant.
Sign Up to like & getrecommendations! Published in 2020 at "International journal of pediatric otorhinolaryngology"
DOI: 10.1016/j.ijporl.2020.109863
Abstract: We present a case of two siblings born to nonconsanguineous parents that presented with hypotonia, respiratory insufficiency, and auditory neuropathy spectrum disorder (ANSD) correlated with NFASC (MIM: 609145) and the homozygous loss of function variant… read more here.
Keywords: auditory neuropathy; neuropathy spectrum; spectrum disorder; case ... See more keywords
Importance of Vestibulo-ocular Reflex Gain and Refixation Saccade Analysis in Individuals with Auditory Neuropathy Spectrum Disorder
Sign Up to like & getrecommendations! Published in 2020 at "International Archives of Otorhinolaryngology"
DOI: 10.1055/s-0039-1697004
Abstract: Introduction Auditory neuropathy spectrum disorder (ANSD) features the presence of otoacoustic emissions, poor speech identification score and absent auditory brainstem response. Objective The present study was designed to evaluate the functioning of all six semicircular… read more here.
Keywords: auditory neuropathy; gain; ocular reflex; vestibulo ocular ... See more keywords
A nonsense TMEM43 variant leads to disruption of connexin-linked function and autosomal dominant auditory neuropathy spectrum disorder
Sign Up to like & getrecommendations! Published in 2021 at "Proceedings of the National Academy of Sciences of the United States of America"
DOI: 10.1073/pnas.2019681118
Abstract: Significance Auditory neuropathy spectrum disorder (ANSD) is a confounding auditory disease in which the subjects respond to sound but have difficulties in speech discrimination. Herein, we examined two Asian families with hereditary late-age–onset ANSD. By… read more here.
Keywords: auditory neuropathy; neuropathy spectrum; tmem43; spectrum disorder ... See more keywords
Multiple Cases of Auditory Neuropathy Illuminate the Importance of Subcortical Neural Synchrony for Speech-in-noise Recognition and the Frequency-following Response
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DOI: 10.1097/aud.0000000000001122
Abstract: Objectives: The role of subcortical synchrony in speech-in-noise (SIN) recognition and the frequency-following response (FFR) was examined in multiple listeners with auditory neuropathy. Although an absent FFR has been documented in one listener with idiopathic… read more here.
Keywords: subcortical neural; auditory neuropathy; speech; noise ... See more keywords
A 15-Year Review of 260 Children With Auditory Neuropathy Spectrum Disorder: I. Demographic and Diagnostic Characteristics.
Sign Up to like & getrecommendations! Published in 2023 at "Ear and hearing"
DOI: 10.1097/aud.0000000000001337
Abstract: OBJECTIVES The aim of the study was to review the demographic and clinical characteristics of all pediatric patients diagnosed with auditory neuropathy spectrum disorder (ANSD) by a pediatric health care system from 2005 to 2020… read more here.
Keywords: spectrum disorder; year; auditory neuropathy; neuropathy spectrum ... See more keywords
A 15-year Review of 260 Children With Auditory Neuropathy Spectrum Disorder: II. Management and Outcomes.
Sign Up to like & getrecommendations! Published in 2023 at "Ear and hearing"
DOI: 10.1097/aud.0000000000001366
Abstract: OBJECTIVES Following a review of the demographic and clinical characteristics of all pediatric patients diagnosed with auditory neuropathy spectrum disorder (ANSD) by a pediatric health care system from 2005 to 2020, the present report highlights… read more here.
Keywords: year; language; auditory neuropathy; speech language ... See more keywords
Prevalence, behavioural, and management outcomes of infants with auditory neuropathy spectrum disorder.
Sign Up to like & getrecommendations! Published in 2021 at "Developmental medicine and child neurology"
DOI: 10.1111/dmcn.15103
Abstract: AIM To provide an overview of electrophysiological and behavioural outcomes from a large UK centres data set on children diagnosed with auditory neuropathy (ANSD) between 2002 and March 2018. METHOD A systematic audit was undertaken,… read more here.
Keywords: auditory neuropathy; behavioural management; prevalence behavioural; outcomes infants ... See more keywords