However, the absence of an abnormal auditory phenotype in individuals with Pseudohypoaldosteronism type I (PHA 1), which are homozygous for null alleles of ENaC subunits [14], suggests that the DFNB8/B10 deafness phenotype is due to aberrant proteolytic processing by TMPRSS3 of some other substrate in the inner ear. The gene discussed is TMPRSS3; the disease is Pelger-Huet anomaly.