Interestingly, both in FSHD1 and FSHD2 but not in BAMS, we observed a decreased expression of several genes such as ACTA1, ACTN2, TNNC1, TNNT2, TPM1, or TCAP that have been associated with hearing impairment and a decreased perception of sounds, one of the extra‐muscular symptoms reported in many FSHD patients. Here, SMCHD1 is linked to hearing loss disorder.