Abstract
Evidence-based guidelines for the prevention, diagnosis and treatment of congenital cytomegalovirus (cCMV) were recently released by two independent expert groups. Of particular emphasis was the relationship between cCMV and sensorineural hearing loss (SNHL), a major component of the virus’ overall disease burden. In this study, a literature review was performed to estimate the proportion of cCMV-related SNHL cases, which might be identified through selective cCMV testing following failed newborn hearing screening. Furthermore, it was of interest to estimate the potential benefit of emerging antiviral therapies. Currently, at most 10% of cCMV-related SNHL is likely to be identified clinically. Through use of a selective cCMV testing protocol, however, a significant improvement in the identification rate can be achieved. Recent expert group statements strongly recommend antiviral therapy in cases of moderate-to-severe disease, especially in the presence of central nervous system involvement. Though differences exist between recommendations in instances of isolated SNHL or SNHL in combination with only mild symptoms, the majority of experts in both groups offered at least a weak recommendation for antiviral treatment. Available results suggest antiviral treatment could therefore benefit a meaningful proportion of newborns referred for cCMV testing following failed newborn hearing screening.
Highlights
Congenital cytomegalovirus is the leading non-genetic cause of sensorineural hearing loss (SNHL) worldwide and a significant contributor to neurodevelopmental delay [1,2,3,4]
A literature review was performed to estimate the proportion of congenital cytomegalovirus (cCMV)-related SNHL cases, which might be identified through selective cCMV testing following failed newborn hearing screening
It was the objective of this article to review the epidemiology of congenital cytomegalovirus infection and its effects in relation to hearing loss, present the potential benefits and drawbacks of targeted cCMV testing, as well as assess the efficacy of selective testing and the potential benefit of additional antiviral therapy according to recently published recommendations
Summary
Congenital cytomegalovirus (cCMV) is the leading non-genetic cause of sensorineural hearing loss (SNHL) worldwide and a significant contributor to neurodevelopmental delay [1,2,3,4]. The first involves limiting screening to those newborns who have already failed a newborn hearing screening, while the second constitutes a universal screening [29,30,31,32,33] It was the objective of this article to review the epidemiology of congenital cytomegalovirus infection and its effects in relation to hearing loss, present the potential benefits and drawbacks of targeted cCMV testing, as well as assess the efficacy of selective testing and the potential benefit of additional antiviral therapy according to recently published recommendations. A conservative estimate of 25% of symptomatic children, which would be diagnosed clinically, (i.e., tested for CMV because of their presenting clinical signs or symptoms) was adopted [20] and is used for the purposes of this study (see Table 1) What is troubling, this result indicates that 75% of symptomatic children will currently not be diagnosed without targeted or universal screening measures. Of the approximately 6000 children with cCMV per 1,000,000 births, a correct CMV diagnosis, based solely on apparent clinical symptoms, is possible in only approximately 3.2% of cases. 9.6% and 87.2% will be missed due to nonspecific symptoms and the complete lack of symptoms, respectively (see Table 1)
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