Congenital Cytomegalovirus Infection as a Cause of Sensorineural Hearing Loss in a Highly Immune Population

Aparecida Y. Yamamoto, MD; Marisa Marcia Mussi-Pinhata, MD; Myriam de Lima Isaac, MD; Fabiana R. Amaral, MD; Cristina G. Carvalheiro, MD; Davi C. Aragon, MSc; Alessandra K. da Silva Manfredi, MSc; Suresh B. Boppana, MD; William J. Britt, MD.

Disclosures

Pediatr Infect Dis J. 2011;30(12):1043-1046. 

In This Article

Abstract and Introduction

Abstract

Background: The burden of congenital cytomegalovirus (CMV)-associated sensorineural hearing loss (SNHL) in populations with CMV seroprevalence approaching 100% is unknown. The purpose of this study was to assess the rate, associated factors, and predictors of SNHL in CMVinfected infants identified by newborn screening in a highly seropositive maternal population.
Methods: Newborns with positive saliva CMV-DNA that was confirmed by virus isolation in the first 2 weeks of life were enrolled in a prospective follow-up study to monitor hearing outcome.
Results: Of 12,195 infants screened, 121 (1%) were infected with CMV and 12 (10%) had symptomatic infection at birth. Hearing function could be assessed in 102/121 children who underwent at least one auditory brainstem evoked response testing at a median age of 12 months. SNHL was observed in 10/102 (9.8%; 95% confidence interval: 5.1–16.7) children. Median age at the latest hearing evaluation was 47 months (12–84 months). Profound loss (>90 dB) was found in 4/5 children with bilateral SNHL while all 5 children with unilateral loss had moderate to severe deficit. The presence of symptomatic infection at birth (odds ratio, 38.1; 95% confidence interval: 1.6 –916.7) was independently associated with SNHL after adjusting for intrauterine growth restriction, gestational age, gravidity, and maternal age. Among 10 infants with SNHL, 6 (60%) were born to mothers with nonprimary CMV infection.
Conclusions: Even in populations with near universal immunity to CMV, congenital CMV infection is a significant cause of SNHL demonstrating the importance of CMV as a major cause of SNHL in children worldwide. As in other populations, SNHL is more frequently observed in symptomatic CMV infection.

Introduction

Congenital cytomegalovirus (CMV) infection has been reported to be an important cause of hearing loss in infants born in North America and northern Europe. Population-based studies in Sweden,[1] Canada,[2] and United States[3,4] have reported that 9.3% to 17% of infants with congenital CMV infection will have sensorineural hearing loss (SNHL). The rates of SNHL reported by these studies ranged between 22% and 41% in children with clinically apparent or symptomatic infection and between 6% and 16% in those with subclinical or asymptomatic infection.

Recent studies have reported that hearing loss occurs at a similar frequency in children born to mothers who had primary CMV infection during pregnancy and offspring of women with nonprimary infection with documented preconceptional seroimmunity.[5,6] These studies showed that 7% to 10% of infected infants from mothers with nonprimary infection had SNHL, whereas 11% to 15% of infants born to mothers with primary infection had SNHL. However, the prevalence and natural history of CMV-associated SNHL in maternal populations with near universal CMV seroimmunity have not been well defined. This feature of the natural history of congenital CMV infection is particularly relevant in regions of the world with transitional economies such as South America, Africa, and southern Asia where near universal seroimmunity to CMV in maternal population has been reported. In a recent study, we have shown that the birth prevalence of congenital CMV infection (1%) and the proportion of congenitally infected infants with symptomatic infection in our population with maternal seroprevalence rate of 96.7% to be similar to that found in the populations with lower CMV seroprevalence rates.[7] Thus, more precise definition of the role of congenital CMV infection as a cause of hearing loss in offspring of women from highly seroimmune population is of considerable importance because understanding the rates of SNHL is relevant to the issue of vaccine prevention of maternal CMV infection. The objective of the current study was to assess the rate, associated factors, and predictors of CMV-induced SNHL in a highly seropositive maternal population.

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