Auditory and Vestibular Involvement in Congenital Cytomegalovirus Infection

Congenital cytomegalovirus infection (cCMV) is a frequent cause of non-hereditary sensorineural hearing loss (SNHL) and developmental disabilities. The contribution of cCMV to childhood hearing loss has been estimated to be about 25% of all hearing loss in children at 4 years of age. Although the vestibular insufficiency (VI) in cCMV has not been well-characterized and therefore, underestimated, recent studies suggest that VI is also frequent in children with cCMV and can lead to adverse neurodevelopmental outcomes. The pathogenesis of SNHL and VI in children with cCMV has been thought to be from direct viral cytopathic effects as well as local inflammatory responses playing a role. Hearing loss in cCMV can be of varying degrees of severity, unilateral or bilateral, present at birth or develop later (late-onset), and can progress or fluctuate in early childhood. Therefore, newborn hearing screening fails to identify a significant number of children with CMV-related SNHL. Although the natural history of cCMV-associated VI has not been well characterized, recent data suggests that it is likely that VI also varies considerably with respect to the laterality, timing of onset, degree of the deficit, and continued deterioration during early childhood. This article summarizes the current understanding of the natural history and pathogenesis of auditory and vestibular disorders in children with cCMV.