Auditory screening of newborn infants has been recommended on the basi
s of the presence of risk criteria, including congenital infection. We
assessed the ability of risk criteria-based neonatal auditory brain s
tem response to identify infants with hearing loss resulting from cong
enital cytomegalovirus (CMV) infection. Data from 6 1/2 years of risk
criteria-based neonatal auditory screening were compared with the resu
lts of screening of all newborn infants for congenital CMV infection.
Infants with congenital CMV infection received follow-up hearing evalu
ations. Congenital CMV infection was found in 167 (1.3%) of 12,371 inf
ants; 134 had follow-up hearing evaluations, and 14 (10.4%) had confir
med sensorineural hearing loss. The rate of sensorineural hearing loss
resulting from congenital CMV infection was 14 per 12,371 infants, of
1.1 per 1000 live births; the rate of bilateral loss greater-than-or-
equal-to 50 dB was 0.6 per 1000. Although 2036 infants received audito
ry screening because of risk criteria, only 34 (20%) of 167 infants wi
th congenital CMV infection were included. Only 2 (14%) of 14 children
with sensorineural hearing loss caused by CMV were identified by risk
criteria-based screening. We conclude that congenital CMV infection i
s an important cause of hearing impairment. Neonatal auditory screenin
g based on the presence of risk criteria will fail to identify the maj
ority of cases of sensorineural hearing loss caused by congenital CMV
infection.