Limited Potential to Reverse Deafness Caused by Mutation of Myo7a

PURPOSE: MYO7A is involved in several forms of deafness in humans and mice, and in this study we aimed to investigate if the hearing loss could be reversed after its onset. METHODS: homozygotes at 4 weeks old by excising the transcription disruption cassette in the tm1a allele allowing expression of the Myo7a gene, and ABRs were recorded before and after activation of the gene. RESULTS: mice at 12 and 18 kHz at 6 and 8 weeks old but there were no responses to sound by 14 weeks old. CONCLUSIONS: It is likely to be challenging to reverse hearing loss due to very early developmental defects of stereocilia organisation.