Background: The association between congenital cytomegalovirus (CMV) infection and sensorineural hearing loss (SNHL) was first described in 1964. Studies over the past four decades have further described the relationship between congenital CMV infection and SNHL in children. Objectives: This manuscript will review the current knowledge of CMV-related SNHL and summarize the studies completed at the University of Alabama at Birmingham (UAB). Study Design: A review of the series of studies at UAB that has led to a more detailed characterization of hearing loss due to congenital CMV infection. Results: Approximately, 22%-65% of symptomatic and 6%-23% of asymptomatic children will have hearing loss following congenital CMV infection. CMV-related SNHL may be present at birth or occur later in childhood. Variability in the severity of CMV-related hearing loss ranges from unilateral high frequency losses to profound bilateral losses. Conclusions: Congenital CMV infection significantly contributes to SNHL in many infant populations. Although, most children with congenital CMV infection do not develop hearing loss, it is difficult to predict which children with congenital CMV infection will develop hearing loss and, among those who do develop loss, whether or not the loss will continue to deteriorate. © 2005 Elsevier B.V. All rights reserved.