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Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports

Auditory neuropathy spectrum disorder (ANSD) refers to a range of hearing impairments characterized by deteriorated speech perception, despite relatively preserved pure-tone detection thresholds. Affected individuals usually present with abnormal auditory brainstem responses (ABRs), but normal otoac...

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Autores principales: De Siati, Romolo Daniele, Rosenzweig, Flora, Gersdorff, Guillaume, Gregoire, Anaïs, Rombaux, Philippe, Deggouj, Naïma
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7230308/
https://www.ncbi.nlm.nih.gov/pubmed/32290039
http://dx.doi.org/10.3390/jcm9041074
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author De Siati, Romolo Daniele
Rosenzweig, Flora
Gersdorff, Guillaume
Gregoire, Anaïs
Rombaux, Philippe
Deggouj, Naïma
author_facet De Siati, Romolo Daniele
Rosenzweig, Flora
Gersdorff, Guillaume
Gregoire, Anaïs
Rombaux, Philippe
Deggouj, Naïma
author_sort De Siati, Romolo Daniele
collection PubMed
description Auditory neuropathy spectrum disorder (ANSD) refers to a range of hearing impairments characterized by deteriorated speech perception, despite relatively preserved pure-tone detection thresholds. Affected individuals usually present with abnormal auditory brainstem responses (ABRs), but normal otoacoustic emissions (OAEs). These electrophysiological characteristics have led to the hypothesis that ANSD may be caused by various dysfunctions at the cochlear inner hair cell (IHC) and spiral ganglion neuron (SGN) levels, while the activity of outer hair cells (OHCs) is preserved, resulting in discrepancies between pure-tone and speech comprehension thresholds. The exact prevalence of ANSD remains unknown; clinical findings show a large variability among subjects with hearing impairment ranging from mild to profound hearing loss. A wide range of prenatal and postnatal etiologies have been proposed. The study of genetics and of the implicated sites of lesion correlated with clinical findings have also led to a better understanding of the molecular mechanisms underlying the various forms of ANSD, and may guide clinicians in better screening, assessment and treatment of ANSD patients. Besides OAEs and ABRs, audiological assessment includes stapedial reflex measurements, supraliminal psychoacoustic tests, electrocochleography (ECochG), auditory steady-state responses (ASSRs) and cortical auditory evoked potentials (CAEPs). Hearing aids are indicated in the treatment of ANSD with mild to moderate hearing loss, whereas cochlear implantation is the first choice of treatment in case of profound hearing loss, especially in case of IHC presynaptic disorders, or in case of poor auditory outcomes with conventional hearing aids.
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spelling pubmed-72303082020-05-22 Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports De Siati, Romolo Daniele Rosenzweig, Flora Gersdorff, Guillaume Gregoire, Anaïs Rombaux, Philippe Deggouj, Naïma J Clin Med Review Auditory neuropathy spectrum disorder (ANSD) refers to a range of hearing impairments characterized by deteriorated speech perception, despite relatively preserved pure-tone detection thresholds. Affected individuals usually present with abnormal auditory brainstem responses (ABRs), but normal otoacoustic emissions (OAEs). These electrophysiological characteristics have led to the hypothesis that ANSD may be caused by various dysfunctions at the cochlear inner hair cell (IHC) and spiral ganglion neuron (SGN) levels, while the activity of outer hair cells (OHCs) is preserved, resulting in discrepancies between pure-tone and speech comprehension thresholds. The exact prevalence of ANSD remains unknown; clinical findings show a large variability among subjects with hearing impairment ranging from mild to profound hearing loss. A wide range of prenatal and postnatal etiologies have been proposed. The study of genetics and of the implicated sites of lesion correlated with clinical findings have also led to a better understanding of the molecular mechanisms underlying the various forms of ANSD, and may guide clinicians in better screening, assessment and treatment of ANSD patients. Besides OAEs and ABRs, audiological assessment includes stapedial reflex measurements, supraliminal psychoacoustic tests, electrocochleography (ECochG), auditory steady-state responses (ASSRs) and cortical auditory evoked potentials (CAEPs). Hearing aids are indicated in the treatment of ANSD with mild to moderate hearing loss, whereas cochlear implantation is the first choice of treatment in case of profound hearing loss, especially in case of IHC presynaptic disorders, or in case of poor auditory outcomes with conventional hearing aids. MDPI 2020-04-10 /pmc/articles/PMC7230308/ /pubmed/32290039 http://dx.doi.org/10.3390/jcm9041074 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
De Siati, Romolo Daniele
Rosenzweig, Flora
Gersdorff, Guillaume
Gregoire, Anaïs
Rombaux, Philippe
Deggouj, Naïma
Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports
title Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports
title_full Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports
title_fullStr Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports
title_full_unstemmed Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports
title_short Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports
title_sort auditory neuropathy spectrum disorders: from diagnosis to treatment: literature review and case reports
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7230308/
https://www.ncbi.nlm.nih.gov/pubmed/32290039
http://dx.doi.org/10.3390/jcm9041074
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