Restoration of hearing due to TMC1 gene in mice

I am currently doing an EPQ on hearing loss and specifically future treatments and the possibility for a cure in the future.

I came across some research done by scientists which involved inserting the TMC1 by use of AAV1, which is considered safe as a viral vector, into the cochlea of mice with genetic deafness and these were their findings:

  • In the recessive deafness model, gene therapy with TMC1 restored the ability of sensory hair cells to respond to sound—producing a measurable electrical current—and also restored activity in the auditory portion of the brainstem.
  • Most importantly, the deaf mice regained their ability to hear. To test hearing, the researchers placed the mice in a “startle box” and sounded abrupt, loud tones. The mice with TMC1 mutations did not respond, but with gene therapy, they jumped as high as a normal mouse. (The force of their jump was measured by a plate on the floor underneath them and was detectable at sounds beginning around 80 decibels.)
  • In the dominant deafness model, gene therapy with a related gene, TMC2, was successful at the cellular and brain level and was partially successful at restoring actual hearing in the startle test.

Thank you for reading.

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