Congenital CMV Infection is a Leading Cause of Hearing Loss in Children
Congenital CMV Infection is a Leading Cause of Hearing Loss in Children
ABSTRACT & COMMENTARY
Synopsis: A cohort of 307 children with clinically inapparent congenital cytomegalovirus (CMV) infection was studied prospectively by audiologic evaluation. Sensorineural hearing loss was documented in 22 (7.2%) of these children at a median age of 18 months. Asymptomatic congenital CMV infection appears to be a leading cause of sensorineural hearing loss.
Source: Fowler KB, et al. Progressive and fluctuating sensorineural hearing loss in children with asymptomatic congenital cytomegalovirus infection. J Pediatr 1997;130: 624-630.
Cytomegalovirus (cmv) infection occurs in 0.5-2% of newborns infants, although approximately 95% of infected newborns will have no symptoms or clinical signs of infection in the newborn period. Central nervous system involvement including microcephaly and periventricular calcifications are frequent findings in newborns with symptomatic congenital CMV infection. This study identified children with clinically asymptomatic CMV infection by screening for virus from urine or saliva obtained from the infant during the first two weeks of life. Children identified with congenital infection were followed by audiologic evaluation, using auditory brain-stem response, at 3-8 weeks, 6-12 months, and annually thereafter.
Sensorineural hearing loss occurred in 22 (7.2%) children with asymptomatic congenital CMV infection. None of the children with hearing loss had another identifiable cause. No hearing loss was found in any of the 76 unaffected siblings or the 201 children without congenital CMV infection. The severity of hearing loss in the 22 affected children included unilateral loss (50%) and bilateral loss (50%) and varied significantly in degree with: high frequency loss (32%); mild loss (5%); moderate loss (9%); severe loss (18%); and profound (36%). Profound bilateral hearing loss occurred in five of the 22 children (23%). Four children (18%) had late onset of hearing loss with a median age of 27 months (range, 25-62 months). Progressive deterioration of hearing loss occurred in 50% of children, and the deterioration was observed more often among children initially presenting with unilateral loss (73%) than with bilateral loss (27%).
COMMENT BY HAL JENSON, MD, FAAP
This study and other prospective studies suggest that hearing loss from congenital CMV infection may account for a least one-third of all cases of sensorineural hearing loss in children. Because congenital CMV infection can be definitively diagnosed only in the newborn period, it is not possible to retrospectively attribute hearing loss found later in life to inapparent congenital CMV infection. Testing of newborn saliva or urine for CMV is feasible for screening of congenital CMV infection, but the testing is not routine or widely available. Recognition of the broader effect of congenital CMV infection should give added impetus to screening programs as well as to development of effective CMV vaccines. In the interim, children who are diagnosed with congenital CMV infection, even if asymptomatic at birth, should be followed by repeated auditory evaluation at least during the first three years of life.
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