Hearing loss could develop simultaneously with neuropathy, in some patients with pathogenic variants in PRPS1 (n = 1), NEFL (n = 2), MPZ (n = 1), TRPV4 (n = 1); or at a distance in some cases of variants in SH3TC2 (n = 2) and ABHD12 (n = 1). Here, TRPV4 is linked to neuropathy.