TRPV4 and proximal spinal muscular atrophy: Another study by Oonk et al. (2014) in a Dutch family with spinal muscular atrophy caused by a mutation in TRPV4, found significant differences in the manifestation and severity of hearing loss among family members with the same TRPV4 mutation, suggesting that TRPV4 is essential for maintaining cochlear function under stressful conditions, such as acoustic injury.