The “quivering” mice carrying recessively inherited loss-of-function mutations of murine SPTBN3 homolog present auditory and motor neuropathies, similar to symptoms observed in SPTBN3 homozygous or compound heterozygous patients suffering from congenital muscular hypotonia, neuropathy, and deafness [11,12]. Here, SPTBN4 is linked to deafness.