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Congenital Cytomegalovirus (CMV)-Related Hearing Loss

Congential infection with cytomegalovirus (CMV) is a major cause of sensorineural hearing loss, with 20-30% of all cases of SNHL due to prenatal CMV infection. [Barbi: 2003] Sixty percent of children with symptomatic CMV (characterized by any combination of the following: low birth weight, microcephaly, thrombocytopenia, jaundice, hepatosplenomegaly, seizures) and 10% of children with asymptomatic CMV will develop sensorineural hearing loss. [Williamson: 1992] Although symptomatic CMV may be suspected from a constellation of clinical signs/symptoms, viral culture of the urine or saliva in the first three weeks of life is still considered the gold standard for diagnosing congenital CMV infection. Cultures obtained after three weeks of age cannot differentiate between congenital and postnatal infection.
CMV-related hearing loss is often progressive and may develop after the neonatal period. [Fowler: 1997] Fluctuating hearing loss is also common. Children with known congenital CMV infection should have serial hearing screens at birth, 3, 6, 9, 12, 18, 24, 30, 36 months, and then annually until school age to identify and treat hearing impairment in a timely manner.
Research increasingly supports treatment of newborns with congenital CMV infection to prevent or mitigate sensorineural hearing loss. Treatment with intravenous gancyclovir and/or oral valgancyclovir have been reported. To be effective, treatment should begin as early as possible. [Kimberlin: 2003] [Lackner: 2009] [Amir: 2010] [Ross: 2008]

Resources

Information & Support

For Professionals

Cytomegalovirus (CMV) and Congenital CMV Infection (CDC)
From the Centers for Disease Control and Prevention, information for clinicians about diagnosing and treating CMV infection.

CMV and Congenital Hearing Loss (UDOH) (PDF Document 648 KB)
From the Utah Department of Health, information for clinicians regarding mitigating hearing loss caused by congenital cytomegalovirus infection; pursuant to House Bill 81, enacted in 2013.

For Parents and Patients

Congenital CMV Infection (CDC)
From the Centers for Disease Control and Prevention, information about congenital CMV infection for families.

Core Facts about CMV (UDOH) (PDF Document 567 KB)
From the Utah Department of Health, pdf brochure for families about cytomegalovirus, particularly related to its association with hearing impairment.

Studies

Studies of treatment of CMV-related hearing loss (clinical trials.gov)
From the National Institutes of Health, a list of studies addressing treatment of hearing loss related to CMV infection.

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

Authors: Richard Harward, AuD - 8/2010
Karl White, Ph D - 9/2008
Content Last Updated: 7/2013

Page Bibliography

Amir J, Wolf DG, Levy I.
Treatment of symptomatic congenital cytomegalovirus infection with intravenous ganciclovir followed by long-term oral valganciclovir.
Eur J Pediatr. 2010. PubMed abstract

Barbi M, Binda S, Caroppo S, Ambrosetti U, Corbetta C, Sergi P.
A wider role for congenital cytomegalovirus infection in sensorineural hearing loss.
Pediatr Infect Dis J. 2003;22(1):39-42. PubMed abstract

Fowler KB, McCollister FP, Dahle AJ, Boppana S, Britt WJ, Pass RF.
Progressive and fluctuating sensorineural hearing loss in children with asymptomatic congenital cytomegalovirus infection.
J Pediatr. 1997;130(4):624-30. PubMed abstract

Kimberlin DW, Lin CY, Sánchez PJ, Demmler GJ, Dankner W, Shelton M, Jacobs RF, Vaudry W, Pass RF, Kiell JM, Soong SJ, Whitley RJ.
Effect of ganciclovir therapy on hearing in symptomatic congenital cytomegalovirus disease involving the central nervous system: a randomized, controlled trial.
J Pediatr. 2003;143(1):16-25. PubMed abstract

Lackner A, Acham A, Alborno T, Moser M, Engele H, Raggam RB, Halwachs-Baumann G, Kapitan M, Walch C.
Effect on hearing of ganciclovir therapy for asymptomatic congenital cytomegalovirus infection: four to 10 year follow up.
J Laryngol Otol. 2009;123(4):391-6. PubMed abstract

Ross DS, Fowler KB.
Cytomegalovirus: A Major Cause of Hearing Loss in Children.
The ASHA Leader; American Speech-Language-Hearing Association; (2008) http://www.asha.org/Publications/leader/2008/080506/f080506b/. Accessed on 7/27/13.

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