Longitudinal Investigation of Hearing Disorders in Children with Congenital Cytomegalovirus

Abstract

This investigation consisted of a longitudinal study of the effects of congenital cytomegalovirus (CMV) infection on hearing sensitivity in 860 children with documented asymptomatic or symptomatic congenital CMV infection. Of the 651 children with asymptomatic CMV infection, 48 (7.4%) developed sensorineural hearing loss (SNHL), compared to 85 (40.7%) of the children with symptomatic CMV infection. Children in both groups experienced latent effects consisting of delayed onset of loss, threshold fluctuations, and/or progressive loss of hearing. It can be concluded that congenital CMV infection is a leading cause of SNHL in children. The late onset and progression of loss necessitates continued monitoring of hearing sensitivity in this population.

Abbreviations: ABR = auditory brainstem response, CMV = cytomegalovirus, OAEs = otoacoustic emissions, SNHL = sensorineural hearing loss

Key Words

Congenital cytomegalovirus infection - delayed-onset hearing loss - fluctuating hearing loss - progressive hearing loss - sensorineural hearing loss

• REFERENCES

• Balcarek KB, Warren W, Smith RJ, Lyon MD, Pass RF. (1993). Neonatal screening for congenital cytomegalovirus infection by detection of virus in saliva. J Infect Dis 167:1433–1436.
• Bergstrom L. (1977). Viruses that deafen. In: Bess FH, ed. Childhood Deafness: Causation, Assessment and Management. New York: Grune & Stratton, 53–59.
• Boppana SB, Pass RF, Britt WJ, Stagno S, Alford A. (1992). Symptomatic congenital cytomegalovirus infection: neonatal morbidity and mortality. Pediatr Infect Dis J 11:93–99.
• Brookhouser PF, Worthington DW, Kelly WJ. (1994). Fluctuating and/or progressive sensorineural hearing loss in children. Laryngoscope 104:958–964.
• Dahle AJ, McCollister FP, Stagno S, Reynolds DW, Hoffman H. (1979). Progressive hearing impairment in children with congenital cytomegalovirus infection. J Speech Hear Disord 44:220–229.
• Demmler GJ. (1991). Infectious Diseases Society of America and Centers for Disease Control. Summary of a workshop on surveillance for congenital cytomegalovirus disease. Rev Infect Dis 13:315–329.
• Fowler K, McCollister F, Dahle A, Boppana S, Britt W, Pass R. (1997). Progressive and fluctuating sensorineural hearing loss in children with asymptomatic congenital cytomegalovirus infection. J Pediatr 130:624–630.
• Fowler K, Dahle A, Boppana S, Pass R. (1999). Newborn hearing screening: will children with hearing loss caused by congenital cytomegalovirus infection be missed? J Pediatr 135:60–64.
• Hanshaw JB. (1982). On deafness, cytomegalovirus and neonatal screening. Am J Dis Child 136:886–887.
• Hanshaw JB, Moxley AW, Gaev L, Abel V. (1975). CNS sequelae of congenital cytomegalovirus infection. In: Krugman S, Gershon AA, eds. Infections of the Fetus and the Newborn Infant. New York: Liss, 47–54.
• Hanshaw JB, Scheiner AP, Moxley AW, Gaev L, Abel V, Scheiner B. (1976). School failure and deafness after "silent" congenital cytomegalovirus infection. Ν Engl J Med 295:468–470.
• Harris S, Ahfors K, Ivarsson S, Barbro L, Lemmark B, Svanberg L. (1984). Congenital cytomegalovirus infection and sensorineural hearing loss. Ear Hear 5:352–355.
• Harris JP, Fan JT, Keithley EM. (1990). Immunologic responses in experimental cytomegalovirus labyringthi-tis. Am J Otolaryngol 11:304–308.
• Hicks T, Fowler K, Richardson M, Dahle A, Adams L, Pass R. (1993). Congenital cytomegalovirus infection and neonatal auditory screening. J Pediatr 123:779–782.
• Hickson LM, Alcock D. (1991). Progressive hearing loss in children with congenital cytomegalovirus. J Paediatr Child Health 27:105–107.
• Horner KC, CazalsYC. (1991). Contribution of increased endolymphatic pressure to hearing loss in experimental hydrops. Ann Otol Rhinol Laryngol 100:496–502.
• Huygen PLM, Admiraal RJC. (1996). Audiovestibular sequelae of congenital cytomegalovirus infection in 3 children presumably representing 3 symptomatically different types of delayed endolymphatic hydrops. Int J Pediatr Otorhinolaryngol 35:143–154.
• Keithley EM, Harris JP. (1996). Late sequelae of cochlear infection. Laryngoscope 106:341–345.
• McCollister F, Simpson L, Dahle A, Pass R, Fowler K, Amos C, Boll T. (1996). Hearing loss and congenital symptomatic cytomegalovirus infection: a case report of multidisciplinary longitudinal assessment and intervention. J Am Acad Audiol 7:57–62.
• National Institutes of Health. (1993). Early identification of hearing impairment in infants and young children. NIH Consensus Statement ll(l):l-24.
• Pass RF, Stagno S, Myers GJ. (1980). Outcome of symptomatic congenital cytomegalic infection: results of long-term longitudinal follow-up. Pediatrics 66:758–762.
• Ramsay ME, Miller E, Peckham CS. (1991). Outcome of confirmed symptomatic congenital cytomegalovirus infection. Arch Dis Child 66:1068–1069.
• Reynolds DW, Stagno S, Hosty TS, Tiller M, Alford CA. (1973). Maternal cytomegalovirus excretion and perinatal infection. Ν Engl J Med 289:1–5.
• Stagno S, Reynolds DW, Amos CS, Dahle AJ, McCollister FP, Mohindra I, Ermocilla R, Alford CA. (1977). Auditory and visual defects resulting from symptomatic and subclinical congenital cytomegalovirus and toxoplasma infections. Pediatrics 59:669–678.
• Strauss M. (1990). Human cytomegalovirus labyrinthitis. Am J Otolaryngol 11:292–298.
• Williamson WD, Percy AK, Yow MD, Gerson P, Catlin FI, Koppelman ML, Thurber S. (1990). Asymptomatic congenital cytomegalovirus infection. Audiologic, neuroradiologic, and neurodevelopmental abnormalities during the first year. Am J Dis Child 144:1365–1368.
• Williamson WD, Demmler GJ, Percy AK, Catlin FI. (1992). Progressive hearing loss in infants with asymptomatic congenital cytomegalovirus infection. Pediatrics 90:862–866.
• Woolf NK. (1990). Experimental congenital cytomega-lorvirus labyrinthitis and sensorineural hearing loss. Am J Otolaryngol 11:299–303.