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Management of Congenital Cytomegalovirus-Related Hearing Loss

  • Hearing Loss in Children (D Horn and H Ou, Section Editors)
  • Published:
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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.

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References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Zuhair M, Smit GSA, Wallis G, Jabbar F, Smith C, Devleesschauwer B, et al. Estimation of the worldwide seroprevalence of cytomegalovirus: a systematic review and meta-analysis. Rev Med Virol. 2019;29(3):e2034. https://doi.org/10.1002/rmv.2034.

    Article  PubMed  Google Scholar 

  2. Fowler KB. Congenital cytomegalovirus infection: audiologic outcome. Clin Infect Dis. 2013;57(Suppl 4):S182–4. https://doi.org/10.1093/cid/cit609.

    Article  PubMed  PubMed Central  Google Scholar 

  3. Mestas E. Congenital cytomegalovirus. Adv Neonatal Care. 2016;16(1):60–5. https://doi.org/10.1097/ANC.0000000000000242.

    Article  PubMed  Google Scholar 

  4. Morton CC, Nance WE. Newborn hearing screening—a silent revolution. N Engl J Med. 2006;354(20):2151–64. https://doi.org/10.1056/NEJMra050700.

    Article  PubMed  CAS  Google Scholar 

  5. Foulon I, Vleurinck L, Kerkhofs K, Gordts F. Hearing configuration in children with cCMV infection and proposal of a flow chart for hearing evaluation. Int J Audiol. 2015;54(10):714–9. https://doi.org/10.3109/14992027.2015.1046506.

    Article  PubMed  Google Scholar 

  6. Teissier N, Bernard S, Quesnel S, Van Den Abbeele T. Audiovestibular consequences of congenital cytomegalovirus infection. Eur Ann Otorhinolaryngol Head Neck Dis. 2016;133(6):413–8. https://doi.org/10.1016/j.anorl.2016.03.004.

    Article  PubMed  CAS  Google Scholar 

  7. Yamaguchi A, Oh-Ishi T, Arai T, Sakata H, Adachi N, Asanuma S, et al. Screening for seemingly healthy newborns with congenital cytomegalovirus infection by quantitative real-time polymerase chain reaction using newborn urine: an observational study. BMJ Open. 2017;7(1):e013810. https://doi.org/10.1136/bmjopen-2016-013810.

    Article  PubMed  PubMed Central  Google Scholar 

  8. Foulon I, Naessens A, Foulon W, Casteels A, Gordts F. A 10-year prospective study of sensorineural hearing loss in children with congenital cytomegalovirus infection. J Pediatr. 2008;153(1):84–8. https://doi.org/10.1016/j.jpeds.2007.12.049.

    Article  PubMed  Google Scholar 

  9. • Lanzieri TM, Chung W, Flores M, Blum P, Caviness AC, Bialek SR, Grosse SD, Miller JA, Demmler-Harrison G, Congenital Cytomegalovirus Longitudinal Study G (2017) Hearing loss in children with asymptomatic congenital cytomegalovirus infection. Pediatrics 139 (3). doi:https://doi.org/10.1542/peds.2016-2610. A study with long-term follow up that found that after age 5, the risk of delayed-onset SNHL in CMV-infected children was no different than uninfected controls.

  10. American Academy of Pediatrics JCoIH. Year 2007 position statement: principles and guidelines for early hearing detection and intervention programs. Pediatrics. 2007;120(4):898–921. https://doi.org/10.1542/peds.2007-2333.

    Article  Google Scholar 

  11. Wentland CJ, Ronner EA, Basonbul RA, Pinnapureddy S, Mankarious L, Keamy D, et al. Utilization of diagnostic testing for pediatric sensorineural hearing loss. Int J Pediatr Otorhinolaryngol. 2018;111:26–31. https://doi.org/10.1016/j.ijporl.2018.05.024.

    Article  PubMed  Google Scholar 

  12. Park AH, Duval M, McVicar S, Bale JF, Hohler N, Carey JC. A diagnostic paradigm including cytomegalovirus testing for idiopathic pediatric sensorineural hearing loss. Laryngoscope. 2014;124(11):2624–9. https://doi.org/10.1002/lary.24752.

    Article  PubMed  Google Scholar 

  13. Liming BJ, Carter J, Cheng A, Choo D, Curotta J, Carvalho D, et al. International Pediatric Otolaryngology Group (IPOG) consensus recommendations: hearing loss in the pediatric patient. Int J Pediatr Otorhinolaryngol. 2016;90:251–8. https://doi.org/10.1016/j.ijporl.2016.09.016.

    Article  PubMed  Google Scholar 

  14. Chari DA, Chan DK. Diagnosis and treatment of congenital sensorineural hearing loss. Curr Otorhinolaryngol Rep. 2017;5(4):251–8. https://doi.org/10.1007/s40136-017-0163-3.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Rawlinson WD, Boppana SB, Fowler KB, Kimberlin DW, Lazzarotto T, Alain S, et al. Congenital cytomegalovirus infection in pregnancy and the neonate: consensus recommendations for prevention, diagnosis, and therapy. Lancet Infect Dis. 2017;17(6):e177–88. https://doi.org/10.1016/S1473-3099(17)30143-3.

    Article  PubMed  Google Scholar 

  16. Boppana SB, Ross SA, Shimamura M, Palmer AL, Ahmed A, Michaels MG, et al. Saliva polymerase-chain-reaction assay for cytomegalovirus screening in newborns. N Engl J Med. 2011;364(22):2111–8. https://doi.org/10.1056/NEJMoa1006561.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  17. Ross SA, Ahmed A, Palmer AL, Michaels MG, Sanchez PJ, Bernstein DI, et al. Detection of congenital cytomegalovirus infection by real-time polymerase chain reaction analysis of saliva or urine specimens. J Infect Dis. 2014;210(9):1415–8. https://doi.org/10.1093/infdis/jiu263.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  18. Boppana SB, Ross SA, Novak Z, Shimamura M, Tolan RW Jr, Palmer AL, et al. Dried blood spot real-time polymerase chain reaction assays to screen newborns for congenital cytomegalovirus infection. JAMA. 2010;303(14):1375–82. https://doi.org/10.1001/jama.2010.423.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  19. Cytomegalovirus (CMV) and Congenital CMV Infection - Laboratory Testing. Centers for Disease Control and Prevention. https://www.cdc.gov/cmv/clinical/lab-tests.html. Accessed March 17 2020.

  20. Ross SA, Ahmed A, Palmer AL, Michaels MG, Sanchez PJ, Stewart A, et al. Urine collection method for the diagnosis of congenital cytomegalovirus infection. Pediatr Infect Dis J. 2015;34(8):903–5. https://doi.org/10.1097/INF.0000000000000757.

    Article  PubMed  PubMed Central  Google Scholar 

  21. Cannon MJ, Hyde TB, Schmid DS. Review of cytomegalovirus shedding in bodily fluids and relevance to congenital cytomegalovirus infection. Rev Med Virol. 2011;21(4):240–55. https://doi.org/10.1002/rmv.695.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  22. Therrell BL Jr, Hannon WH. Newborn dried blood spot screening: residual specimen storage issues. Pediatrics. 2012;129(2):365–6. https://doi.org/10.1542/peds.2011-3416.

    Article  PubMed  Google Scholar 

  23. Barbi M, Binda S, Primache V, Luraschi C, Corbetta C. Diagnosis of congenital cytomegalovirus infection by detection of viral DNA in dried blood spots. Clin Diagn Virol. 1996;6(1):27–32. https://doi.org/10.1016/0928-0197(96)00228-0.

    Article  PubMed  CAS  Google Scholar 

  24. Johansson PJ, Jonsson M, Ahlfors K, Ivarsson SA, Svanberg L, Guthenberg C. Retrospective diagnostics of congenital cytomegalovirus infection performed by polymerase chain reaction in blood stored on filter paper. Scand J Infect Dis. 1997;29(5):465–8. https://doi.org/10.3109/00365549709011855.

    Article  PubMed  CAS  Google Scholar 

  25. Yamamoto AY, Mussi-Pinhata MM, Pinto PC, Figueiredo LT, Jorge SM. Usefulness of blood and urine samples collected on filter paper in detecting cytomegalovirus by the polymerase chain reaction technique. J Virol Methods. 2001;97(1–2):159–64. https://doi.org/10.1016/s0166-0934(01)00347-0.

    Article  PubMed  CAS  Google Scholar 

  26. • Ross SA, Ahmed A, Palmer AL, Michaels MG, Sanchez PJ, Stewart A, et al. Newborn dried blood spot polymerase chain reaction to identify infants with congenital cytomegalovirus-associated sensorineural hearing loss. J Pediatr. 2017;184:57–61 e51. https://doi.org/10.1016/j.jpeds.2017.01.047Dried blood spot testing PCR is not an effective screening tool for CMV-related hearing loss.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  27. Dobbie AM. Evaluation and management of cytomegalovirus-associated congenital hearing loss. Curr Opin Otolaryngol Head Neck Surg. 2017;25(5):390–5. https://doi.org/10.1097/MOO.0000000000000401.

    Article  PubMed  Google Scholar 

  28. Giannattasio A, Bruzzese D, Di Costanzo P, Capone E, Romano A, D’Amico A, et al. Neuroimaging profiles and neurodevelopmental outcome in infants with congenital cytomegalovirus infection. Pediatr Infect Dis J. 2018;37(10):1028–33. https://doi.org/10.1097/INF.0000000000001974.

    Article  PubMed  Google Scholar 

  29. 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. https://doi.org/10.1542/peds.2014-1173.

    Article  PubMed  Google Scholar 

  30. Kimberlin DW (2017) Asymptomatic congenital CMV treatment. ClinicalTrials.gov. https://clinicaltrials.gov/ct2/show/NCT03301415. Accessed 26 March 2020.

  31. Park A (2017) Randomized controlled trial of valganciclovir for cytomegalovirus infected hearing impaired infants (ValEAR). ClinicalTrials.gov. https://clinicaltrials.gov/ct2/show/NCT03107871. Accessed 26 March 2020.

  32. National Institute of Allergy and Infectious Diseases (NIAID) (2012) Valganciclovir therapy in infants and children with congenital CMV infection and hearing loss. ClinicalTrials.gov. https://clinicaltrials.gov/ct2/show/NCT01649869. Accessed 25 April 2020.

  33. Kimberlin DW, Lin CY, Sanchez PJ, Demmler GJ, Dankner W, Shelton M, et al. 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. https://doi.org/10.1016/s0022-3476(03)00192-6.

    Article  PubMed  CAS  Google Scholar 

  34. Kimberlin DW, Acosta EP, Sanchez PJ, Sood S, Agrawal V, Homans J, et al. Pharmacokinetic and pharmacodynamic assessment of oral valganciclovir in the treatment of symptomatic congenital cytomegalovirus disease. J Infect Dis. 2008;197(6):836–45. https://doi.org/10.1086/528376.

    Article  PubMed  CAS  Google Scholar 

  35. Kimberlin DW, Jester PM, Sanchez PJ, Ahmed A, Arav-Boger R, Michaels MG, et al. Valganciclovir for symptomatic congenital cytomegalovirus disease. N Engl J Med. 2015;372(10):933–43. https://doi.org/10.1056/NEJMoa1404599.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  36. McCrary H, Sheng X, Greene T, Park A. Long-term hearing outcomes of children with symptomatic congenital CMV treated with valganciclovir. Int J Pediatr Otorhinolaryngol. 2019;118:124–7. https://doi.org/10.1016/j.ijporl.2018.12.027.

    Article  PubMed  Google Scholar 

  37. Torrecillas V, Allen CM, Greene T, Park A, Chung W, Lanzieri TM, et al. Should you follow the better-hearing ear for congenital cytomegalovirus infection and isolated sensorineural hearing loss? Otolaryngol Head Neck Surg. 2020;162(1):114–20. https://doi.org/10.1177/0194599819880348.

    Article  PubMed  Google Scholar 

  38. Stehel EK, Shoup AG, Owen KE, Jackson GL, Sendelbach DM, Boney LF, et al. Newborn hearing screening and detection of congenital cytomegalovirus infection. Pediatrics. 2008;121(5):970–5. https://doi.org/10.1542/peds.2006-3441.

    Article  PubMed  Google Scholar 

  39. Rawlinson WD, Palasanthiran P, Hall B, Al Yazidi L, Cannon MJ, Cottier C, et al. Neonates with congenital cytomegalovirus and hearing loss identified via the universal newborn hearing screening program. J Clin Virol. 2018;102:110–5. https://doi.org/10.1016/j.jcv.2018.03.006.

    Article  PubMed  Google Scholar 

  40. Boppana SB, Ross SA, Fowler KB. Congenital cytomegalovirus infection: clinical outcome. Clin Infect Dis. 2013;57(Suppl 4):S178–81. https://doi.org/10.1093/cid/cit629.

    Article  PubMed  PubMed Central  Google Scholar 

  41. •• Fowler KB, McCollister FP, Sabo DL, Shoup AG, Owen KE, Woodruff JL, Cox E, Mohamed LS, Choo DI, Boppana SB, Study C (2017) A targeted approach for congenital cytomegalovirus screening within newborn hearing screening. Pediatrics 139 (2). Doi:https://doi.org/10.1542/peds.2016-2128. Targeted hearing loss CMV screening identified a majority of congenital CMV cases, but 43% of those with CMV-related SNHL were not identified by hearing screening.

  42. Barkai G, Ari-Even Roth D, Barzilai A, Tepperberg-Oikawa M, Mendelson E, Hildesheimer M, et al. Universal neonatal cytomegalovirus screening using saliva—report of clinical experience. J Clin Virol. 2014;60(4):361–6. https://doi.org/10.1016/j.jcv.2014.04.024.

    Article  PubMed  Google Scholar 

  43. Koyano S, Inoue N, Oka A, Moriuchi H, Asano K, Ito Y, et al. Screening for congenital cytomegalovirus infection using newborn urine samples collected on filter paper: feasibility and outcomes from a multicentre study. BMJ Open. 2011;1(1):e000118. https://doi.org/10.1136/bmjopen-2011-000118.

    Article  PubMed  PubMed Central  Google Scholar 

  44. Cannon MJ, Griffiths PD, Aston V, Rawlinson WD. Universal newborn screening for congenital CMV infection: what is the evidence of potential benefit? Rev Med Virol. 2014;24(5):291–307. https://doi.org/10.1002/rmv.1790.

    Article  PubMed  PubMed Central  Google Scholar 

  45. Bergevin A, Zick CD, McVicar SB, Park AH. Cost-benefit analysis of targeted hearing directed early testing for congenital cytomegalovirus infection. Int J Pediatr Otorhinolaryngol. 2015;79(12):2090–3. https://doi.org/10.1016/j.ijporl.2015.09.019.

    Article  PubMed  Google Scholar 

  46. Gantt S, Dionne F, Kozak FK, Goshen O, Goldfarb DM, Park AH, et al. Cost-effectiveness of universal and targeted newborn screening for congenital cytomegalovirus infection. JAMA Pediatr. 2016;170(12):1173–80. https://doi.org/10.1001/jamapediatrics.2016.2016.

    Article  PubMed  Google Scholar 

  47. Williams EJ, Gray J, Luck S, Atkinson C, Embleton ND, Kadambari S, et al. First estimates of the potential cost and cost saving of protecting childhood hearing from damage caused by congenital CMV infection. Arch Dis Child Fetal Neonatal Ed. 2015;100(6):F501–6. https://doi.org/10.1136/archdischild-2014-306756.

    Article  PubMed  Google Scholar 

  48. 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. https://doi.org/10.1016/s0022-3476(97)70248-8.

    Article  PubMed  CAS  Google Scholar 

  49. Dahle AJ, Fowler KB, Wright JD, Boppana SB, Britt WJ, Pass RF. Longitudinal investigation of hearing disorders in children with congenital cytomegalovirus. J Am Acad Audiol. 2000;11(5):283–90.

    PubMed  CAS  Google Scholar 

  50. Kadambari S, Williams EJ, Luck S, Griffiths PD, Sharland M. Evidence based management guidelines for the detection and treatment of congenital CMV. Early Hum Dev. 2011;87(11):723–8. https://doi.org/10.1016/j.earlhumdev.2011.08.021.

    Article  PubMed  CAS  Google Scholar 

  51. Lee DJ, Lustig L, Sampson M, Chinnici J, Niparko JK. Effects of cytomegalovirus (CMV) related deafness on pediatric cochlear implant outcomes. Otolaryngol Head Neck Surg. 2005;133(6):900–5. https://doi.org/10.1016/j.otohns.2005.08.013.

    Article  PubMed  Google Scholar 

  52. Ramirez Inscoe JM, Nikolopoulos TP. Cochlear implantation in children deafened by cytomegalovirus: speech perception and speech intelligibility outcomes. Otol Neurotol. 2004;25(4):479–82. https://doi.org/10.1097/00129492-200407000-00014.

    Article  PubMed  Google Scholar 

  53. Viccaro M, Filipo R, Bosco E, Nicastri M, Mancini P. Long-term follow-up of implanted children with cytomegalovirus-related deafness. Audiol Neurootol. 2012;17(6):395–9. https://doi.org/10.1159/000341160.

    Article  PubMed  Google Scholar 

  54. Iwasaki S, Nakanishi H, Misawa K, Tanigawa T, Mizuta K. Cochlear implant in children with asymptomatic congenital cytomegalovirus infection. Audiol Neurootol. 2009;14(3):146–52. https://doi.org/10.1159/000171476.

    Article  PubMed  Google Scholar 

  55. Matsui T, Ogawa H, Yamada N, Baba Y, Suzuki Y, Nomoto M, et al. Outcome of cochlear implantation in children with congenital cytomegalovirus infection or GJB2 mutation. Acta Otolaryngol. 2012;132(6):597–602. https://doi.org/10.3109/00016489.2011.653445.

    Article  PubMed  CAS  Google Scholar 

  56. Philips B, Maes LK, Keppler H, Dhooge I. Cochlear implants in children deafened by congenital cytomegalovirus and matched Connexin 26 peers. Int J Pediatr Otorhinolaryngol. 2014;78(3):410–5. https://doi.org/10.1016/j.ijporl.2013.11.009.

    Article  PubMed  Google Scholar 

  57. Lee ER, Chan DK. Implications of dried blood spot testing for congenital CMV on management of children with hearing loss: a preliminary report. Int J Pediatr Otorhinolaryngol. 2019;119:10–4. https://doi.org/10.1016/j.ijporl.2018.12.029.

    Article  PubMed  Google Scholar 

  58. • Yoshida H, Takahashi H, Kanda Y, Kitaoka K, Hara M. Long-term outcomes of cochlear implantation in children with congenital cytomegalovirus infection. Otol Neurotol. 2017;38(7):e190–4. https://doi.org/10.1097/MAO.0000000000001483Long-term follow up of CMV-infected cochlear implant recipients.

    Article  PubMed  Google Scholar 

  59. • Lyutenski S, Gotz F, Giourgas A, Majdani O, Bultmann E, Lanfermann H, et al. Does severity of cerebral MRI lesions in congenital CMV infection correlates with the outcome of cochlear implantation? Eur Arch Otorhinolaryngol. 2017;274(3):1397–403. https://doi.org/10.1007/s00405-016-4408-4Severity of MRI findings did not correlate with cochlear implantation outcomes.

    Article  PubMed  Google Scholar 

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  1. Department of Otolaryngology-Head & Neck Surgery, University of California, San Francisco, 2233 Post Street Box 1225, San Francisco, CA, 94115, USA

    Abel P. David & Dylan K. Chan

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David, A.P., Chan, D.K. Management of Congenital Cytomegalovirus-Related Hearing Loss. Curr Otorhinolaryngol Rep 8, 243–249 (2020). https://doi.org/10.1007/s40136-020-00291-w

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