However, patients carrying the mt-DNA mutation m.A1555G, previously identified as a cause of deafness and located in proximity to the two adenines methylated by TFB1M, did not manifest changes in mt-12S rRNA methylation levels compared to controls, thus putting into question the role of TFB1M in the pathogenesis of this disorder (O’Sullivan et al., 2015). Here, TFB1M is linked to deafness.