OTOA and deafness: ,2 This identified a potential causative variant, c.1765del:p.Gln589Argfs∗55 in OTOA, which is predicted to cause premature termination of the OTOA. 3Given the auditory phenotype and inheritance pattern consistent with DFNB22, we hypothesized a compound heterozygous variant in OTOA. The variant, c.2359G > T:p.Glu787∗, initially considered a likely deafness-causing variant due to its predicted deletion of the C-terminal GPI anchor region,3 has a high minor allele frequency in the Korean population, suggesting it may not be causative (Fig. 1C).