Reversal of an existing hearing loss by gene activation in mutant mice.

Proc Natl Acad Sci U S A

Wolfson Centre for Age-Related Diseases, King's College London, Guy's Campus, London SE1 1UL, United Kingdom.

Published: August 2023

AI Article Synopsis

  • Hearing loss can result from an inability to maintain the proper ionic environment in sensory hair cells, leading to a lower endocochlear potential.
  • Researchers used a genetic strategy in a mouse model to investigate if they could reverse this type of hearing loss by activating gene transcription at different ages.
  • Their findings suggest that early intervention can restore normal auditory responses and protect hair cells from further damage, indicating a critical window for effective treatment that could potentially apply to other conditions as well.

Article Abstract

Hearing loss is highly heterogeneous, but one common form involves a failure to maintain the local ionic environment of the sensory hair cells reflected in a reduced endocochlear potential. We used a genetic approach to ask whether this type of pathology can be reversed, using the mouse mutant known to show this defect. By activating gene transcription at different ages after the onset of hearing loss, we found that an existing auditory impairment can be reversed to give close to normal thresholds for an auditory brainstem response (ABR), at least at low to mid stimulus frequencies. Delaying the activation of led to less effective recovery of ABR thresholds, suggesting that there is a critical period for intervention. Early activation of not only led to improvement in auditory function but also to protection of sensory hair cells from secondary degeneration. The genetic approach we have used to establish that this type of hearing loss is in principle reversible could be extended to many other diseases using available mouse resources.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10450448PMC
http://dx.doi.org/10.1073/pnas.2307355120DOI Listing

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