Understanding and treating paediatric hearing impairment
Sensorineural hearing impairment is the most frequent form of hearing impairment affecting 1–2 in 1000 newborns and another 1 in 1000 adolescents. More than 50% of congenital hearing impairment is of genetic origin and some forms of monogenic deafness are likely targets for future gene therapy. Good...
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doaj-df026c1b492645c6bbd84e1bd92c4faa2021-01-22T04:50:13ZengElsevierEBioMedicine2352-39642021-01-0163103171Understanding and treating paediatric hearing impairmentChristian Wrobel0Maria-Patapia Zafeiriou1Tobias Moser2Department of Otolaryngology and InnerEarLab, University Medical Center Göttingen, 37099 Göttingen, Germany; Multiscale Bioimaging Cluster of Excellence (MBExC), University of Göttingen, GermanyMultiscale Bioimaging Cluster of Excellence (MBExC), University of Göttingen, Germany; Institute of Pharmacology and Toxicology, University Medical Center, 37075 Göttingen, GermanyMultiscale Bioimaging Cluster of Excellence (MBExC), University of Göttingen, Germany; Institute for Auditory Neuroscience and InnerEarLab, University Medical Center Göttingen, 37099 Göttingen, Germany; Corresponding author at: Institute for Auditory Neuroscience and InnerEarLab, University Medical Center Göttingen, 37099 Göttingen, GermanySensorineural hearing impairment is the most frequent form of hearing impairment affecting 1–2 in 1000 newborns and another 1 in 1000 adolescents. More than 50% of congenital hearing impairment is of genetic origin and some forms of monogenic deafness are likely targets for future gene therapy. Good progress has been made in clinical phenotyping, genetic diagnostics, and counselling. Disease modelling, e.g. in transgenic mice, has helped elucidate disease mechanisms underlying genetic hearing impairment and informed clinical phenotyping in recent years. Clinical management of paediatric hearing impairment involves hearing aids, cochlear or brainstem implants, signal-to-noise improvement in educational settings, speech therapy, and sign language. Cochlear implants, for example, have much improved the situation of profoundly hearing impaired and deaf children. Nonetheless there remains a major unmet clinical need for improving hearing restoration. Preclinical studies promise that we will witness clinical trials on gene therapy and a next generation of cochlear implants during the coming decade. Moreover, progress in generating sensory hair cells and neurons from stem cells spurs disease modelling, drug screening, and regenerative approaches. This review briefly summarizes the pathophysiology of paediatric hearing impairment and provides an update on the current preclinical development of innovative approaches toward improved hearing restoration.http://www.sciencedirect.com/science/article/pii/S2352396420305478CochleaHearing aidCochlear implantGene therapyOptogeneticsStem cell |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Christian Wrobel Maria-Patapia Zafeiriou Tobias Moser |
spellingShingle |
Christian Wrobel Maria-Patapia Zafeiriou Tobias Moser Understanding and treating paediatric hearing impairment EBioMedicine Cochlea Hearing aid Cochlear implant Gene therapy Optogenetics Stem cell |
author_facet |
Christian Wrobel Maria-Patapia Zafeiriou Tobias Moser |
author_sort |
Christian Wrobel |
title |
Understanding and treating paediatric hearing impairment |
title_short |
Understanding and treating paediatric hearing impairment |
title_full |
Understanding and treating paediatric hearing impairment |
title_fullStr |
Understanding and treating paediatric hearing impairment |
title_full_unstemmed |
Understanding and treating paediatric hearing impairment |
title_sort |
understanding and treating paediatric hearing impairment |
publisher |
Elsevier |
series |
EBioMedicine |
issn |
2352-3964 |
publishDate |
2021-01-01 |
description |
Sensorineural hearing impairment is the most frequent form of hearing impairment affecting 1–2 in 1000 newborns and another 1 in 1000 adolescents. More than 50% of congenital hearing impairment is of genetic origin and some forms of monogenic deafness are likely targets for future gene therapy. Good progress has been made in clinical phenotyping, genetic diagnostics, and counselling. Disease modelling, e.g. in transgenic mice, has helped elucidate disease mechanisms underlying genetic hearing impairment and informed clinical phenotyping in recent years. Clinical management of paediatric hearing impairment involves hearing aids, cochlear or brainstem implants, signal-to-noise improvement in educational settings, speech therapy, and sign language. Cochlear implants, for example, have much improved the situation of profoundly hearing impaired and deaf children. Nonetheless there remains a major unmet clinical need for improving hearing restoration. Preclinical studies promise that we will witness clinical trials on gene therapy and a next generation of cochlear implants during the coming decade. Moreover, progress in generating sensory hair cells and neurons from stem cells spurs disease modelling, drug screening, and regenerative approaches. This review briefly summarizes the pathophysiology of paediatric hearing impairment and provides an update on the current preclinical development of innovative approaches toward improved hearing restoration. |
topic |
Cochlea Hearing aid Cochlear implant Gene therapy Optogenetics Stem cell |
url |
http://www.sciencedirect.com/science/article/pii/S2352396420305478 |
work_keys_str_mv |
AT christianwrobel understandingandtreatingpaediatrichearingimpairment AT mariapatapiazafeiriou understandingandtreatingpaediatrichearingimpairment AT tobiasmoser understandingandtreatingpaediatrichearingimpairment |
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