Skip to main content

Advertisement

Log in

Twelve years of neonatal hearing screening: audiological and etiological results

  • Otology
  • Published:
European Archives of Oto-Rhino-Laryngology Aims and scope Submit manuscript

Abstract

Purpose

Most developed countries have implemented some form of universal newborn hearing screening program. Early identification and rehabilitation of congenital hearing loss is important in functional outcome, and the need to identify the cause of hearing impairment has become clear. We aimed to evaluate audiological and etiological outcomes in a large group of patients with failed neonatal hearing screening.

Methods

We performed a retrospective chart analysis of patients who were referred to our tertiary referral center after failing neonatal hearing screening during a 12-year period (2007–2019). Screening was based on automated auditory brainstem response (AABR) or a combined approach of AABR and auditory steady-state response (ASSR) with chirp stimulus. Extensive audiometric testing was performed to confirm and determine the type and degree of hearing loss. In case of permanent hearing loss, a standardized etiological protocol was followed to determine the cause.

Results

Of the 802 referred newborns, hearing loss was confirmed by diagnostic ABR in 78%. Main causes of hearing loss included otitis media with effusion (56%, higher in patients screened by AABR/ASSR compared to AABR), a genetic disorder (12%), congenital cytomegalovirus infection (cCMV, 5%) and atresia/stenosis of the external ear canal (5%). Of the patients with permanent hearing loss, 15% showed changes in hearing loss severity over time.

Conclusion

In the majority of newborns referred after failing universal neonatal hearing screening, hearing loss could be confirmed. The leading cause was reversible hearing loss due to otitis media with effusion, but hearing loss proved permanent in about 35% of referred newborns, with genetics as predominant cause. Follow-up of congenital hearing loss patients is important as deterioration as well as improvement was observed over time.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

Data availability

The raw data are available on request.

Abbreviations

AABR:

Automated auditory brainstem response

ANSD:

Auditory neuropathy spectrum disorder

ASSR:

Auditory steady-state response

cCMV:

Congenital cytomegalovirus infection

dBnHL:

Decibel above normal hearing level

EAC:

External ear canal

NICU:

Neonatal intensive care unit

OAE:

Otoacoustic emissions

OME:

Otitis media with effusion

TTD:

Transtympanic drain

UNHS:

Universal newborn hearing screening

References

  1. Morton CC, Nance WE (2006) Newborn hearing screening—a silent revolution. N Engl J Med 354(20):2151–2164

    Article  CAS  Google Scholar 

  2. Smith RJH, Bale JF Jr, White KR (2005) Sensorineural hearing loss in children. Lancet 365(9462):879–890

    Article  Google Scholar 

  3. American Academy of Pediatrics, Joint Committee on Infant Hearing (2007) Year 2007 position statement: principles and guidelines for early hearing detection and intervention programs. Pediatrics 120(4):898–921

    Article  Google Scholar 

  4. Morzaria S, Westerberg BD, Kozak FK (2004) Systematic review of the etiology of bilateral sensorineural hearing loss in children. Int J Pediatr Otorhinolaryngol 68(9):1193–1198

    Article  Google Scholar 

  5. American Academy of Pediatrics, Joint Committee on Infant Hearing (2019) Year 2019 Position statement: principles and guidelines for early hearing detection and intervention programs. J Early Hear Detect Interv 4(2):1–44

    Google Scholar 

  6. Konukseven O, Genc A, Muderris T et al (2010) Can automated auditory brainstem response be used as an initial stage screening test in newborn hearing screening programs? J Int Adv Otol 6:231–238

    Google Scholar 

  7. Stürzebecher E, Cebulla M, Neumann K (2003) Click-evoked ABR at high stimulus repetition rates for neonatal hearing screening. Int J Audiol 42(2):59–70

    Article  Google Scholar 

  8. Declau F, Boudewyns A, Van den Ende J, Peeters A, van den Heyning P (2008) Etiologic and audiologic evaluations after universal neonatal hearing screening: analysis of 170 referred neonates. Pediatrics 121(6):1119–1126

    Article  Google Scholar 

  9. Van Kerschaver E, Stappaerts L (2012) Jaarrapport gehoor 2009–2010–2011—universele gehoorscreening in vlaanderen—doelgroepbereik, testresultaten en resultaten van de verwijzingen. http://www.kindengezin.be/img/rapportGehoor2009-2010-2011.pdf. Accessed 29 March 2021

  10. Lammens F, Verhaert N, Devriendt K, Debruyne F, Desloovere C (2013) Aetiology of congenital hearing loss: a cohort review of 569 subjects. Int J Pediatr Otorhinolaryngol 77(9):1385–1391

    Article  CAS  Google Scholar 

  11. Hatzopoulos S, Ciorba A, Sliwa L, Skarzynski P (2013) Technological advances in universal neonatal hearing screening (UNHS). Hear Balance Commun 11:104–109

    Article  Google Scholar 

  12. Deklerck A, Acke FR, Janssens S, De Leenheer EMR (2015) Etiological approach in patients with unidentified hearing loss. Int J Pediatr Otorhinolaryngol 79(2):216–222

    Article  Google Scholar 

  13. Clark JG (1981) Uses and abuses of hearing loss classification. ASHA 23(7):493–500

    CAS  PubMed  Google Scholar 

  14. Stevens JC, Sutton GJ, Wood SA (2013) Guidelines for the early audiological assessment and management of babies referred from the newborn hearing screening programme (v3.1). https://www.thebsa.org.uk/wp-content/uploads/2014/08/NHSP_NeonateAssess_2014.pdf. Accessed 30 Aug 2021

  15. De Leenheer EMR, Janssens S, Padalko E, Loose D, Leroy BP, Dhooge IJ (2011) Etiological diagnosis in the hearing impaired newborn: proposal of a flow chart. Int J Pediatr Otorhinolaryngol 75(1):27–32

    Article  Google Scholar 

  16. Sommen M, Schrauwen I, Vandeweyer G et al (2016) DNA diagnostics of hereditary hearing loss: a targeted resequencing approach combined with a mutation classification system. Hum Mutat 37(8):812–819

    Article  CAS  Google Scholar 

  17. Chin O, Dharsono F, Kuthubutheen J, Thompson A (2020) Is CT necessary for imaging paediatric congenital sensorineural hearing loss? Cochlear Implants Int 21(2):75–82

    Article  CAS  Google Scholar 

  18. Cebulla M, Shehata-Dieler W (2012) ABR-based newborn hearing screening with MB11 BERAphone® using an optimized chirp for acoustical stimulation. Int J Pediatr Otorhinolaryngol 76(4):536–543

    Article  Google Scholar 

  19. Jakubíková J, Kabátová Z, Pavlovcinová G, Profant M (2008) Newborn hearing screening and strategy for early detection of hearing loss in infants. Int J Pediatr Otorhinolaryngol 73(4):607–612

    Article  Google Scholar 

  20. Kennedy C, McCann D, Campbell MJ, Kimm L, Thornton R (2005) Universal newborn screening for permanent childhood hearing impairment: an 8-year follow-up of a controlled trial. Lancet 366(9486):660–662

    Article  Google Scholar 

  21. Molini E, Calzolaro L, Lapenna R, Ricci G (2016) Universal newborn hearing screening in Umbria region, Italy. Int J Pediatr Otorhinolaryngol 82:92–97

    Article  Google Scholar 

  22. Boudewyns A, Declau F, Van den Ende J et al (2011) Otitis media with effusion: an underestimated cause of hearing loss in infants. Otol Neurotol 32(5):799–804

    Article  Google Scholar 

  23. Boone RT, Bower CM, Martin PF (2005) Failed newborn hearing screens as presentation for otitis media with effusion in the newborn population. Int J Pediatr Otorhinolaryngol 69(3):393–397

    Article  Google Scholar 

  24. Van Camp G, Smith RJH (2006). Hereditary hearing loss homepage. https://hereditaryhearingloss.org. Accessed 15 August 2021

  25. Findlen UM, Hounam GM, Alexy E, Adunka OF (2019) Early hearing detection and intervention: timely diagnosis, timely management. Ear Hear 40(3):651–658

    Article  Google Scholar 

  26. Wickremasinghe AC, Risley RJ, Kuzniewicz MW et al (2015) Risk of sensorineural hearing loss and bilirubin exchange transfusion thresholds. Pediatrics 136(3):505–512

    Article  Google Scholar 

  27. Wroblewska-Seniuk KE, Dabrowski P, Szyfter W, Mazela J (2017) Universal newborn hearing screening: methods and results, obstacles, and benefits. Pediatr Res 81(3):415–422

    Article  Google Scholar 

  28. Yoshinaga-Itano C, Manchaiah V, Hunnicutt C (2021) Outcomes of universal newborn screening programs: systematic review. J Clin Med 10(13):2784

    Article  Google Scholar 

  29. Korver AMH, Smith RJH, Van Camp G et al (2017) Congenital hearing loss. Nat Rev Dis Primers 3:16094

    Article  Google Scholar 

Download references

Funding

The authors did not receive funding for this study.

Author information

Authors and Affiliations

Authors

Contributions

FREA designed the study, collected data, carried out the initial analyses and drafted the initial manuscript. CDV analyzed and interpreted the results and wrote part of the manuscript. HVH conceptualized the study, collected data, and reviewed and revised the manuscript. EMRDL conceptualized and designed the study, collected data, supervised data analysis and critically reviewed and revised the manuscript for important intellectual content.

Corresponding author

Correspondence to Frederic R. E. Acke.

Ethics declarations

Conflict of interest

The authors do not disclose any conflict of interest for this study.

Ethical approval

The Ethics Committee of the involved hospital approved the study (EC/2018/0787).

Consent to participate

Not applicable (retrospective chart study approved by the Ethics Committee of the involved hospital).

Consent for publication

Not applicable (retrospective chart study approved by the Ethics Committee of the involved hospital).

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Acke, F.R.E., De Vriese, C., Van Hoecke, H. et al. Twelve years of neonatal hearing screening: audiological and etiological results. Eur Arch Otorhinolaryngol 279, 3371–3378 (2022). https://doi.org/10.1007/s00405-021-07060-5

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00405-021-07060-5

Keywords

Navigation