Management of Congenital Cytomegalovirus-Related Hearing Loss
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HEARING LOSS IN CHILDREN (D HORN AND H OU, SECTION EDITORS)
Management of Congenital Cytomegalovirus-Related Hearing Loss Abel P. David 1 & Dylan K. Chan 1
# Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose of Review The aim of this report is to review the current literature regarding the diagnosis, treatment, management, and rehabilitation of congenital cytomegalovirus (cCMV)–associated sensorineural hearing loss (SNHL). Recent Findings Hearing-targeted CMV screening is successful in identifying cases of cCMV-related hearing loss. However, a significant number of children who develop cCMV-related SNHL may not be detected on newborn hearing screening, and it may be cost-effective to implement a universal CMV screening program. We also broadly review the management and audiologic rehabilitation of cCMV-associated hearing loss, including cochlear implantation. Summary Congenital CMV is a common cause of childhood SNHL. CMV testing is important for the workup and management of unknown SNHL. Antiviral therapies are currently only indicated in those with symptomatic cCMV infection and is currently under investigation in children with isolated SNHL. Hearing status is closely followed, and rehabilitation strategies are similar to other etiologies of congenital SNHL, and may include hearing amplification, speech therapy, and cochlear implantation. Keywords Congenital hearing loss . Cytomegalovirus . Cochlear implantation . Sensorineural hearing loss
Introduction Congenital cytomegalovirus (cCMV) infection is the most common intrauterine infection in humans. The global seroprevalence of CMV is estimated to be 83% in the general population and 86% in women of reproductive age. In the Americas, the seroprevalence rates in those groups are estimated to be 75% and 79%, respectively [1]. It is associated with many congenital conditions, including microcephaly, chorioretinitis, cognitive impairment, and/or cerebral palsy [2, 3]. Sensorineural hearing loss (SNHL) is the most common sequela of cCMV infection, with approximately half of symptomatic infants and 10–15% of otherwise asymptomatic cCMV infants developing SNHL. For otolaryngologists, cCMV infection is of particular importance, because it is the
This article is part of the Topical Collection on Hearing Loss in Children * Abel P. David [email protected] * Dylan K. Chan [email protected] 1
Department of Otolaryngology-Head & Neck Surgery, University of California, San Francisco, 2233 Post Street Box 1225, San Francisco, CA 94115, USA
most common cause of non-genetic SNHL in young children, accounting for 21% of all SNHL at birth and 25% at 4 years of age [2, 4]. Here, we discuss the role of CMV testing in the workup of childhood SNHL, screening newborns for cCMV, and the audiologic evaluation and rehabilitation of cCMVassociated SNHL.
Audiologic Characteristics Hearing loss in children affected by cCMV infection is sensorineural in nature, and a small study found that the most common audiologic configuration is a flat SNHL, with
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