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KMID : 0361020210640040215
Korean Journal of Otolaryngology - Head and Neck Surgery
2021 Volume.64 No. 4 p.215 ~ p.222
Inner Ear Gene Therapy in Mouse Models of Genetic Hearing Loss
Choi Jin-Woong

Abstract
Hearing loss is the most frequent sensory disorder affecting newborns and children. About 1newborn in every 500 suffer from congenital hearing loss, with approximately half of thesehaving a genetic cause. In the last few decades, the study of genetic hearing loss and relatedmouse models has unveiled molecular, cellular, and physiological mechanisms of the disease.
In addition, effective and safe viral vectors for gene delivery to the inner ear have been generated.
A growing number of approaches, including gene replacement, gene silencing, and geneediting, have proved effective in mouse models. This article briefly introduces basic strategiesof gene therapy, viral vectors used and surgical methods for gene delivery, and reviews thecurrent works on mouse modes of genetic hearing loss.
KEYWORD
Animal model, Correction of hearing impairment, Gene therapy, Genetic vectors, Nonsyndromic sensorineural hearing loss, Sensorineural hearing loss
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