Hair cells from mice carrying the dominant M412K, D569N, or recessive D528N deafness-associated mutations (Tmc1M412K/M412K, Tmc1D56N/D569N, or Tmc1D528N/D528N), located at the putative TMC1 pore region (Figure 6A), exhibit constitutive externalization of PS at the apical hair cell region10,11,16 and profound progressive hearing loss12,13,17. This evidence concerns the gene TMC1 and deafness.