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the review, researchers suggested that in order to restore hearing loss, the common strategies
include Cochlear Implants, gene therapy for genetic deafness, gene therapy for developed
deafness, and gene therapy for spiral ganglion neurons, stem cell therapies to generate of hair
cells and generation of auditory neurons and molecular therapies to generate new hair cells
(Géléoc and Holt 2014). On the other hand, genome editing is one of the most effective
approaches that could offer hope for restoration of hearing loss by restoring wild type sequence
of native DNA. As KCNQ4 mutation induces a dominant-progressive hearing loss amongst
human, implementation of these strategies may require significant overexpression of the wild-
type protein. The implication of the practice is that it will enable health care professionals to
recommend molecular and gene therapies for restoring hearing loss.
Third article:
Mizutari et al. (2013), conducted an experimental study on the notch signalling pathway
that induced regeneration of cochlear hair cell as well as recovery of hearing after severe trauma.
The researchers manipulated the notch signalling pathway for generating new hair cells in a
deafened animal. Moreover, researchers conducted research by identifying a potent γ-secretase
inhibitor by an assay of stem cells. They used a lineage tag for determining the source of the new
hair cells. The result of the study suggested that new hair cells can be produced and it can cause
fractional recovery of hearing which was damaged by severe noise. The underlying mechanism
is that when notch singling pathway is inhibited by y a γsecretase inhibitor chosen for
stimulating the differential of hair cells from inner stem cells, the generation resulted in
overexpression of the bHLH transcription factor, Atoh1 and inhibit the Notch pathway.
Consequently, notch pathway inhibition resulted in cochlear hair cell generation and restoration

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