Medical and Surgical Interventions for Hearing Loss Associated with Congenital Cytomegalovirus: A Systematic Review

Background. Hearing loss associated with congenital cytomegalovirus (CMV) infection occurs in 0.2 to 0.6 per 1000 neonates. Objective. The primary goal of this systemic review was to test the following null hypotheses: (1) antiviral therapy has no impact on congenital CMV-related sensorineural hearing loss and (2) surgical therapy has no impact on congenital CMV-related sensorineural hearing loss. Data Sources. Computerized searches of MEDLINE and EMBASE databases through September 2010 were performed, supplemented with manual searches and inquiries to topic experts. Review Methods. Studies were included based on review of 387 studies according to criteria developed a priori. Data extraction was performed by independent reviewers and focused on relevant audiologic measurements, study designs, and potential confounders. Results. Criterion-meeting studies (n = 19) included a total of 446 participants. The largest randomized controlled trial (RCT) suggested a significant protective effect of intravenous ganciclovir against deterioration of hearing in neonates with central nervous system manifestations of CMV infection. It also, however, suggested a 3-fold increase in neutropenia. The second RCT suggested that there may be no significant benefit of intravenous ganciclovir for normal-hearing infants with asymptomatic congenital CMV. Additional prospective and retrospective data evaluated the impact of oral therapy and cochlear implantation in affected patients. Conclusion. Although results are mixed, the highest level of evidence suggests that antiviral therapy confers a protective benefit on neonates with hearing loss and symptomatic CMV. Cochlear implantation can result in advancement of speech and language skills, but there are mixed results compared with non-CMV-infected patients.