OBJECTIVE: To verify the occurrence of congenital infections in newborns and its possible association with the results of newborn hearing screening (NHS). METHODS: The medical records of neonates who underwent NHS in a public hospital between 2012 September and 2013 April were investigated. The presence of congenital infections and the NHS results were analyzed by referring to the medical records and by evaluating the results of transient otoacoustic emissions testing. RESULTS: Among the 1849 neonates screened during the study period, 27% (n = 500) showed some risk indicators for hearing impairment (HI). Among these, 3% had a congenital maternal infection such as toxoplasmosis (53.33% [n = 8]), syphilis (33.33% [n = 5]), and rubella (13.33% [n = 2]). Among the neonates in this group (n = 15), only 13.33% (n = 2) failed the NHS, and both of these neonates had congenital syphilis. Of these 2 neonates, 1 passed the NHS re-evaluation and is currently undergoing audiological monitoring and the other was referred for an audiological diagnosis. CONCLUSION: Toxoplasmosis was the most prevalent congenital infection during the study period. However, NHS failure was observed in neonates with syphilis. These findings confirm the importance of conducting studies to examine the association between congenital infections and HI, especially since it is suggested that many of these infections have a delayed effect and/or lead to a progression of HI.