Congenital Cytomegalovirus (CMV) Related Hearing Loss
Congenital infection with cytomegalovirus (CMV) causes 10-20% of all cases of sensorineural hearing loss (SNHL). [Goderis: 2014] One third of children with symptomatic CMV, characterized by any combination of the following: low-birth weight, microcephaly, thrombocytopenia, jaundice, hepatosplenomegaly, seizures) and 10% children with asymptomatic CMV will develop sensorineural hearing loss. [Goderis: 2014]
Screening and Testing
Although symptomatic CMV may be suspected from a constellation of
clinical signs/symptoms, CMV culture or PCR testing of the urine or saliva before 3
weeks of life is still considered the gold standard for diagnosing congenital CMV
infection. Recent studies have reported false positive results from saliva CMV PCR
or culture. [Leruez-Ville: 2017]
[Puhakka: 2018] For that reason, we recommend a
confirmatory urine CMV PCR or culture within the first 3 weeks of life for any child
with a positive saliva CMV test. [Dedhia: 2018] Lab studies
obtained after 3 weeks of age often cannot differentiate between congenital and
postnatal infection. Testing of neonatal archived dry blood spots for CMV offers an
option to diagnose the older child although a landmark study by Boppana et al.
detected only 34% of infected children. [Boppana: 2010]
There is ongoing controversy regarding universal or targeted CMV screening in
pregnant women and/or infants. We have recently reported our state-wide results for
hearing targeted early CMV screening. [Diener: 2017]
Fourteen infants were diagnosed and we noted improvements in the time to diagnosis
the hearing status of all infants who failed their newborn hearing
screening.
CMV-related hearing loss is often progressive and may develop after the neonatal period. Fluctuating hearing loss is also common. Children with congenital CMV infection should have diagnostic hearing testing and regular hearing testing to identify and treat hearing impairment in a timely manner.
Treatment
Research supports treatment of newborns with symptomatic congenital
CMV infection to prevent or mitigate sensorineural hearing loss.[Kimberlin: 2015] Current recommendations are to start oral
valganciclovir within the first month of life and careful monitoring of dose to
avoid adverse side effects (e.g. neutropenia). [Rawlinson: 2017]
Prevention
Prevention of congenital CMV through pre-conception vaccination has been
an elusive goal for decades. [Schleiss: 2017] Perhaps a
more practical recommendation is to provide information to potential mothers and to
have them follow simple handwashing measures to reduce infection risk. [Din: 2011]
[Stowell: 2012]
Resources
Information & Support
For Professionals
Cytomegalovirus (CMV) and Congenital CMV Infection (CDC)
A clinical overview, testing, and management information for clinicians treating CMV infection; Centers for Disease Control
and Prevention.
For Parents and Patients
Congenital CMV Infection (CDC)
From the Centers for Disease Control and Prevention, information about congenital CMV infection for families.
Studies
Treatment of CMV-related hearing loss (ClinicalTrials.gov)
Studies looking at better understanding, diagnosing, and treating this condition; from the National Library of Medicine.
Helpful Articles
Pass RF.
Cytomegalovirus infection.
Pediatr Rev.
2002;23(5):163-70.
PubMed abstract
Lagasse N, Dhooge I, Govaert P.
Congenital CMV-infection and hearing loss.
Acta Otorhinolaryngol Belg.
2000;54(4):431-6.
PubMed abstract
Williamson WD, Demmler GJ, Percy AK, Catlin FI.
Progressive hearing loss in infants with asymptomatic congenital cytomegalovirus infection.
Pediatrics.
1992;90(6):862-6.
PubMed abstract
Misono S, Sie KC, Weiss NS, Huang ML, Boeckh M, Norton SJ, Yueh B.
Congenital cytomegalovirus infection in pediatric hearing loss.
Arch Otolaryngol Head Neck Surg.
2011;137(1):47-53.
PubMed abstract / Full Text
Authors & Reviewers
| Author: | Albert H. Park, MD |
| Contributing Author: | Jennifer Goldman, MD, MRP, FAAP |
| 2010: update: Richard Harward, AuDA |
| 2008: first version: Karl White, Ph DA |
Page Bibliography
Boppana SB, Ross SA, Novak Z, Shimamura M, Tolan RW Jr, Palmer AL, Ahmed A, Michaels MG, Sánchez PJ, Bernstein DI, Britt WJ,
Fowler KB.
Dried blood spot real-time polymerase chain reaction assays to screen newborns for congenital cytomegalovirus infection.
JAMA.
2010;303(14):1375-82.
PubMed abstract / Full Text
Dedhia K, Graham E, Park A.
Hearing Loss and Failed Newborn Hearing Screen.
Clin Perinatol.
2018;45(4):629-643.
PubMed abstract
Diener ML, Zick CD, McVicar SB, Boettger J, Park AH.
Outcomes From a Hearing-Targeted Cytomegalovirus Screening Program.
Pediatrics.
2017;139(2).
PubMed abstract
Din ES, Brown CJ, Grosse SD, Wang C, Bialek SR, Ross DS, Cannon MJ.
Attitudes toward newborn screening for cytomegalovirus infection.
Pediatrics.
2011;128(6):e1434-42.
PubMed abstract
Goderis J, De Leenheer E, Smets K, Van Hoecke H, Keymeulen A, Dhooge I.
Hearing loss and congenital CMV infection: a systematic review.
Pediatrics.
2014;134(5):972-82.
PubMed abstract
Kimberlin DW, Aban I, Acosta EP.
Valganciclovir for Congenital Cytomegalovirus.
N Engl J Med.
2015;372(25):2463.
PubMed abstract
Lagasse N, Dhooge I, Govaert P.
Congenital CMV-infection and hearing loss.
Acta Otorhinolaryngol Belg.
2000;54(4):431-6.
PubMed abstract
Leruez-Ville M, Magny JF, Couderc S, Pichon C, Parodi M, Bussières L, Guilleminot T, Ghout I, Ville Y.
Risk Factors for Congenital Cytomegalovirus Infection Following Primary and Nonprimary Maternal Infection: A Prospective Neonatal
Screening Study Using Polymerase Chain Reaction in Saliva.
Clin Infect Dis.
2017;65(3):398-404.
PubMed abstract
Misono S, Sie KC, Weiss NS, Huang ML, Boeckh M, Norton SJ, Yueh B.
Congenital cytomegalovirus infection in pediatric hearing loss.
Arch Otolaryngol Head Neck Surg.
2011;137(1):47-53.
PubMed abstract / Full Text
Pass RF.
Cytomegalovirus infection.
Pediatr Rev.
2002;23(5):163-70.
PubMed abstract
Puhakka L, Lappalainen M, Lönnqvist T, Niemensivu R, Lindahl P, Nieminen T, Seuri R, Nupponen I, Pati S, Boppana S, Saxen
H.
The Burden of Congenital Cytomegalovirus Infection: A Prospective Cohort Study of 20 000 Infants in Finland.
J Pediatric Infect Dis Soc.
2018.
PubMed abstract
Rawlinson WD, Boppana SB, Fowler KB, Kimberlin DW, Lazzarotto T, Alain S, Daly K, Doutré S, Gibson L, Giles ML, Greenlee J,
Hamilton ST, Harrison GJ, Hui L, Jones CA, Palasanthiran P, Schleiss MR, Shand AW, van Zuylen WJ.
Congenital cytomegalovirus infection in pregnancy and the neonate: consensus recommendations for prevention, diagnosis, and
therapy.
Lancet Infect Dis.
2017;17(6):e177-e188.
PubMed abstract
Schleiss MR, Permar SR, Plotkin SA.
Progress toward Development of a Vaccine against Congenital Cytomegalovirus Infection.
Clin Vaccine Immunol.
2017;24(12).
PubMed abstract / Full Text
Stowell JD, Forlin-Passoni D, Din E, Radford K, Brown D, White A, Bate SL, Dollard SC, Bialek SR, Cannon MJ, Schmid DS.
Cytomegalovirus survival on common environmental surfaces: opportunities for viral transmission.
J Infect Dis.
2012;205(2):211-4.
PubMed abstract / Full Text
Williamson WD, Demmler GJ, Percy AK, Catlin FI.
Progressive hearing loss in infants with asymptomatic congenital cytomegalovirus infection.
Pediatrics.
1992;90(6):862-6.
PubMed abstract