Implications of dried blood spot testing for congenital CMV on management of children with hearing loss: A preliminary report.

INTRODUCTION

Non-genetic, congenital sensorineural hearing loss (cSNHL) is commonly caused by congenital CMV infection (cCMV). Hearing loss related to cCMV is variable in degree, often progressive, and can affect one or both ears.

OBJECTIVES

We sought to examine the outcomes of DBS testing in California, and the hearing outcomes of cCMV-positive children.

METHODS

This is a retrospective study of patients with SNHL of unknown etiology aged 6 months to 17 years old presenting to a tertiary care pediatric center and evaluated for cCMV by DBS testing.

RESULTS

114 children (228 ears) with SNHL of unknown origin were included. 6/114 (5.3%) tested positive for cCMV versus 108/114 (94.7%), who tested negative. None of the cCMV-positive children had symmetric bilateral hearing loss, compared with 56.5% (61/108) of cCMV-negative children (p < 0.05). cCMV-positive children were more likely to have profound SNHL in the worse-hearing ear (5/6 (83%) vs 16/108 (14.9%) of cCMV-negative children, p < 0.001). 86% (5/6) exhibited progressive hearing loss, including progression or new-onset hearing loss in the previously better hearing ear. 3 of the 6 children with cCMV underwent CI.

CONCLUSION

A small proportion of patients presenting with SNHL tested positive on DBS. Of cCMV-positive children, most presented with profound hearing loss in the worse-hearing ear, and 50% of cCMV-positive children developed progressive hearing loss in the initially better-hearing ear. Prognostic information afforded by etiologic confirmation of cCMV infection informed decision-making concerning cochlear implantation in these cases.