In a recent, retrospective, longitudinal study, Chien-Yu Hsueh, National Yang Ming Chiao Tung University, Taipei, Taiwan, and colleagues sought to investigate the effects of early enzyme-replacement therapy on 19 patients with infantile-onset Pompe disease. Of these patients, 6 had hearing impairment. The authors emphasized that early enzyme replacement therapy within 2 weeks following birth may lead to better hearing outcomes. According to the authors, clinicians should test for hearing issues associated with infantile-onset Pompe disease and intervene if any impairment is detected.