Cochlear Implantation in Infants Deafened by Congenital Cytomegalovirus

Abstract

Congenital cytomegalovirus (cCMV) is the most common intra-uterine viral infection in most developed countries. In Flanders (Belgium), Foulon and colleagues (2008) found a prevalence of approximately 0.53 per cent. Moreover, cCMV has been identified as the leading cause of non-genetic congenital sensorineural hearing loss (SNHL) in infants. The vast majority of infected newborns are asymptomatic at birth (90%), although 7–21 per cent of patients are at risk for developing SNHL, neurologic deficits, and behavioural problems later on in life (Dahle et al., 2000; Foulon et al., 2008). Research has shown that progression, fluctuation, improvement, and late onset SNHL are commonly seen within asymptomatic cCMV children (Fowler et al., 1997). Approximately 10 per cent of the infected infants (symptomatic cCMV children) are born with clinically apparent sequelae, including SNHL, mental retardation, microcephaly, and severe neurologic deficits. Cochlear implantation is of value in the setting of cCMV-associated severe SNHL, although the outcome is less predictive. Case series indicate benefit with good speech and language development (Ramirez-Inscoe et al., 2004; Lee et al., 2005).