AI Article Synopsis
- Progressive non-syndromic sensorineural hearing loss (PNSHL) is a prevalent sensory impairment in older adults, impacting over a third of those aged 65 and above, and includes conditions like noise-induced hearing loss and genetic forms such as delayed-onset autosomal dominant hearing loss.
- PNSHL is considered a strong candidate for genetic therapies due to extensive research and the opportunity to intervene before significant hearing loss occurs, with various gene therapy strategies available, including viral and non-viral methods as well as gene editing techniques.
- To effectively develop and test these therapies, creating a reliable in vitro model of the human inner ear from induced pluripotent stem cells (iPSCs) is essential, which could lead to
Article Abstract
Progressive non-syndromic sensorineural hearing loss (PNSHL) is the most common cause of sensory impairment, affecting more than a third of individuals over the age of 65. PNSHL includes noise-induced hearing loss (NIHL) and inherited forms of deafness, among which is delayed-onset autosomal dominant hearing loss (AD PNSHL). PNSHL is a prime candidate for genetic therapies due to the fact that PNSHL has been studied extensively, and there is a potentially wide window between identification of the disorder and the onset of hearing loss. Several gene therapy strategies exist that show potential for targeting PNSHL, including viral and non-viral approaches, and gene editing versus gene-modulating approaches. To fully explore the potential of these therapy strategies, a faithful in vitro model of the human inner ear is needed. Such models may come from induced pluripotent stem cells (iPSCs). The development of new treatment modalities by combining iPSC modeling with novel and innovative gene therapy approaches will pave the way for future applications leading to improved quality of life for many affected individuals and their families.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7943682 | PMC |
| http://dx.doi.org/10.1007/s10162-020-00781-0 | DOI Listing |
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