Sensorineural hearing loss is the most frequent sequela of congenital cytomegalovirus (CMV) infection, and epidemiological evidence also suggests that congenital CMV infection is responsible for a substantial proportion of sensorineural hearing loss in children. Hearing loss due to congenital CMV infection can be present at birth or can appear later, usually during the first year of life; it usually worsens as the infant or child ages. Follow-up of children with congenital CMV infection should include repeated audiological testing. Based on the benefits of early detection of hearing loss, one could propose screening all infants for congenital CMV infection so that those with hearing impairment can be identified as early as possible by appropriate audiological follow-up. Antiviral treatment that could improve hearing outcome, with a safety profile suitable for use in minimally ill infants, would clearly increase the benefit of universal screening for congenital CMV infection.