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OBJECTIVE:In this study, we determined the prevalence of hearing loss in 157 children with proven congenital cytomegalovirus (cCMV) infection. We looked at possible risk determinants for developing hearing loss and proposed recommendations for screening and follow-up in the newborn.METHODS: In a prospective 22-year study, 157 children with proven cCMV infection were evaluated for sensorineural hearing loss (SNHL). The development of SNHL was correlated with the type of maternal infection (primary versus nonprimary), the gestational age of maternal primary infection, imaging findings at birth, and the presence of symptomatic or asymptomatic infection in the newborn.RESULTS: Of all children, 12.7% had SNHL, and 5.7% needed hearing amplification because of SNHL. Improvement, progression, and fluctuations of hearing thresholds were seen in 45%, 53.8%, and 5.7% of the children, respectively. Hearing loss was more common in the case of a symptomatic infection at birth (P 5 .017), after a maternal primary infection in the first trimester of pregnancy (P 5 .029), and in the presence of abnormalities on a neonatal brain ultrasound and/or MRI (P , .001).CONCLUSION SNHL is a common sequela in children with cCMV infection. Risk factors for SNHL were primary maternal infections before the 14th week of pregnancy, the presence of a disseminated infection at birth, and imaging abnormalities in the newborn. These children may benefit from a more thorough investigation for SNHL than children who do not present with those risk factors.WHAT'S KNOWN ON THIS SUBJECT: Hearing loss is 1 of the most common sequelae in children with a congenital cytomegalovirus infection.WHAT THIS STUDY ADDS: Incidences of hearing loss and the need for hearing rehabilitation in children with congenital cytomegalovirus infection are discussed. We describe risk indicators for late-onset hearing loss.

• In cCMV, isolated periventricular T2-weighted signal hyperintensity has a good postnatal prognosis. • In cCMV, SNHL and neurological impairment can be predicted at 27 or 33 weeks. • In cCMV, fetal MR has a high NPV in predicting SNHL. • In cCMV, fetal MR has a high NPV in predicting neurological impairment.