Background/Objectives: Congenital cytomegalovirus (cCMV) infection is a significant cause of pediatric hearing loss. However, the prevalence and characteristics of cCMV-related hearing loss remain unclear. Methods: A comprehensive search of major databases (PubMed, Scopus, and Web of Science) was conducted to identify studies and reviews published between 2000 and 2024 that investigated cCMV infection, testing for cCMV, and pediatric hearing loss. Studies were included if they reported on the prevalence, characteristics, current policies, and recommendations for universal cCMV testing in newborns. Results: This review highlights key issues: cCMV is a significant and treatable cause of pediatric hearing loss; most cCMV-related hearing loss cases presented with bilateral and profound hearing impairment; and cCMV testing is performed only for babies who fail newborn hearing screening. Conclusions: This narrative review highlights the significant association between cCMV infection and pediatric hearing loss. Currently, targeted cCMV testing is recommended for newborns who fail newborn hearing screening. Universal cCMV testing may facilitate early diagnosis and directed intervention and could be cost-effective in the long run.