A High Risk of Missing Congenital Cytomegalovirus-Associated Hearing Loss through Newborn Hearing Screening in Japan.

Shujiro Bando Minami,Atsuko Nakano,Tomoko Sugiuchi,Yoshiharu Yamanobe,Sayaka Katsunuma,Kimitaka Kaga,Tetsuya Takiguchi,Noriko Morita,Tatsuo Matsunaga,Hirokazu Sakamoto,Sawako Masuda

doi:10.3390/jcm10215056

Abstract

It remains unclear to what extent newborn hearing screening (NHS) detects congenital cytomegalovirus (cCMV)-associated sensorineural hearing loss (SNHL) in Japan. This study aimed to clarify the NHS results and audiological characteristics of patients with cCMV-associated SNHL. A total of 541 individuals with unilateral or bilateral hearing loss of unknown etiology were examined for cCMV infection. cCMV infection was defined by the presence of CMV DNA in the dried umbilical cord detected using real-time quantitative PCR. NHS results and audiological data were retrospectively obtained from medical records. Forty-four cases (8.1%) were positive for cCMV infection. Of them, 33 cases underwent NHS and 13 cases (39.4%) passed NHS bilaterally. The pure-tone audiograms of 21 patients were obtained. There were seven cases of unilateral SNHL, five cases of asymmetric bilateral SNHL, and nine cases of symmetric bilateral SNHL. cCMV-related hearing loss is highly heterogeneous, and there is a high risk of missing this condition through NHS.

Highlights

Hearing loss due to congenital cytomegalovirus infection was first reported in 1964 [1]. cCMV infection is caused by vertical transmission from pregnant women via a primary cytomegalovirus (CMV) infection, reinfection, or reactivation
We investigated the medical records of sensorineural hearing loss (SNHL) patients with cCMV infection identified by PCR using their dried umbilical cords and clarified their
This study study indicates indicates that that cCMV-related cCMV-related hearing hearing loss loss isishighly highlyheterogeneous heterogeneous and and there is a high risk of missing this condition through cCMV-related hearing loss there is a high risk of missing this condition through newborn hearing screening (NHS). cCMV-related hearing lossisis characterized characterized by by aahigh highfrequency frequencyof ofasymmetric asymmetrichearing hearingand andthe thefrequent frequentoccurrence occurrenceof of late-onset or progressive hearing loss

Summary

Introduction

Hearing loss due to congenital cytomegalovirus (cCMV) infection was first reported in 1964 [1]. cCMV infection is caused by vertical transmission from pregnant women via a primary cytomegalovirus (CMV) infection, reinfection, or reactivation. Dried blood spots (DBSs) taken within 1 week after birth for neonatal mass screening have been used to retrospectively study cCMV infection suspected after 21 days of age [6], the sensitivity of CMV DNA detection in these specimens is low [7]. While these specimens are discarded after 1 year in Japan, the umbilical cord is kept at home as a symbol of the mother–child bond, and the sensitivity of CMV DNA detection is higher with umbilical cords than with DBSs [8]. Reyes et al concluded that dried umbilical cords could play a complementary diagnostic role by comparing CMV viral load values between different samples [15]

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