Understanding and treating paediatric hearing impairment

2021-01 | journal article; overview. A publication with affiliation to the University of Göttingen.

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​Understanding and treating paediatric hearing impairment​
Wrobel, C.; Zafeiriou, M. P.   & Moser, T. ​ (2021) 
EBioMedicine63 art. 103171​.​ DOI: https://doi.org/10.1016/j.ebiom.2020.103171 

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Authors
Wrobel, Christian; Zafeiriou, Maria Patapia ; Moser, Tobias 
Abstract
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.
Issue Date
January-2021
Journal
EBioMedicine 
Project
EXC 2067: Multiscale Bioimaging 
Working Group
RG Moser (Molecular Anatomy, Physiology and Pathology of Sound Encoding) 
RG Zafeiriou (3D Electrically Excitable Cell Networks – Brain and Heart) 
External URL
https://mbexc.uni-goettingen.de/literature/publications/108
ISSN
2352-3964
Language
English

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