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Vestibular Deficits in Deafness: Clinical Presentation, Animal Modeling, and Treatment Solutions

The inner ear is responsible for both hearing and balance. These functions are dependent on the correct functioning of mechanosensitive hair cells, which convert sound- and motion-induced stimuli into electrical signals conveyed to the brain. During evolution of the inner ear, the major changes occu...

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Autores principales: Maudoux, Audrey, Vitry, Sandrine, El-Amraoui, Aziz
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9013928/
https://www.ncbi.nlm.nih.gov/pubmed/35444606
http://dx.doi.org/10.3389/fneur.2022.816534
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author Maudoux, Audrey
Vitry, Sandrine
El-Amraoui, Aziz
author_facet Maudoux, Audrey
Vitry, Sandrine
El-Amraoui, Aziz
author_sort Maudoux, Audrey
collection PubMed
description The inner ear is responsible for both hearing and balance. These functions are dependent on the correct functioning of mechanosensitive hair cells, which convert sound- and motion-induced stimuli into electrical signals conveyed to the brain. During evolution of the inner ear, the major changes occurred in the hearing organ, whereas the structure of the vestibular organs remained constant in all vertebrates over the same period. Vestibular deficits are highly prevalent in humans, due to multiple intersecting causes: genetics, environmental factors, ototoxic drugs, infections and aging. Studies of deafness genes associated with balance deficits and their corresponding animal models have shed light on the development and function of these two sensory systems. Bilateral vestibular deficits often impair individual postural control, gaze stabilization, locomotion and spatial orientation. The resulting dizziness, vertigo, and/or falls (frequent in elderly populations) greatly affect patient quality of life. In the absence of treatment, prosthetic devices, such as vestibular implants, providing information about the direction, amplitude and velocity of body movements, are being developed and have given promising results in animal models and humans. Novel methods and techniques have led to major progress in gene therapies targeting the inner ear (gene supplementation and gene editing), 3D inner ear organoids and reprograming protocols for generating hair cell-like cells. These rapid advances in multiscale approaches covering basic research, clinical diagnostics and therapies are fostering interdisciplinary research to develop personalized treatments for vestibular disorders.
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spelling pubmed-90139282022-04-19 Vestibular Deficits in Deafness: Clinical Presentation, Animal Modeling, and Treatment Solutions Maudoux, Audrey Vitry, Sandrine El-Amraoui, Aziz Front Neurol Neurology The inner ear is responsible for both hearing and balance. These functions are dependent on the correct functioning of mechanosensitive hair cells, which convert sound- and motion-induced stimuli into electrical signals conveyed to the brain. During evolution of the inner ear, the major changes occurred in the hearing organ, whereas the structure of the vestibular organs remained constant in all vertebrates over the same period. Vestibular deficits are highly prevalent in humans, due to multiple intersecting causes: genetics, environmental factors, ototoxic drugs, infections and aging. Studies of deafness genes associated with balance deficits and their corresponding animal models have shed light on the development and function of these two sensory systems. Bilateral vestibular deficits often impair individual postural control, gaze stabilization, locomotion and spatial orientation. The resulting dizziness, vertigo, and/or falls (frequent in elderly populations) greatly affect patient quality of life. In the absence of treatment, prosthetic devices, such as vestibular implants, providing information about the direction, amplitude and velocity of body movements, are being developed and have given promising results in animal models and humans. Novel methods and techniques have led to major progress in gene therapies targeting the inner ear (gene supplementation and gene editing), 3D inner ear organoids and reprograming protocols for generating hair cell-like cells. These rapid advances in multiscale approaches covering basic research, clinical diagnostics and therapies are fostering interdisciplinary research to develop personalized treatments for vestibular disorders. Frontiers Media S.A. 2022-04-04 /pmc/articles/PMC9013928/ /pubmed/35444606 http://dx.doi.org/10.3389/fneur.2022.816534 Text en Copyright © 2022 Maudoux, Vitry and El-Amraoui. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Neurology
Maudoux, Audrey
Vitry, Sandrine
El-Amraoui, Aziz
Vestibular Deficits in Deafness: Clinical Presentation, Animal Modeling, and Treatment Solutions
title Vestibular Deficits in Deafness: Clinical Presentation, Animal Modeling, and Treatment Solutions
title_full Vestibular Deficits in Deafness: Clinical Presentation, Animal Modeling, and Treatment Solutions
title_fullStr Vestibular Deficits in Deafness: Clinical Presentation, Animal Modeling, and Treatment Solutions
title_full_unstemmed Vestibular Deficits in Deafness: Clinical Presentation, Animal Modeling, and Treatment Solutions
title_short Vestibular Deficits in Deafness: Clinical Presentation, Animal Modeling, and Treatment Solutions
title_sort vestibular deficits in deafness: clinical presentation, animal modeling, and treatment solutions
topic Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9013928/
https://www.ncbi.nlm.nih.gov/pubmed/35444606
http://dx.doi.org/10.3389/fneur.2022.816534
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