Congenital cytomegalovirus (cCMV) infection is a major cause of hearing loss in children. A few cases of cCMV twin pregnancies are reported in the literature. Twins can react differently to maternal infection, but hearing loss is rarely evaluated. Two couples of twins with cCMV infection and different audiologic outcomes are reported. The first couple of twins was composed by two male twins, both affected by cCMV infection. The first born had normal hearing function, and the second born had sensorineural hearing loss (SNHL). In the second couple, a male and a female twin, only the male twin was affected by cCMV infection, and both had normal hearing function. In this case series, an interesting finding was the association between the presence of viral DNA in liquor and hearing loss in one newborn. Further research is needed to better understand the pathophysiology of SNHL caused by cCMV infection.