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.
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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
Morton CC, Nance WE (2006) Newborn hearing screening—a silent revolution. N Engl J Med 354(20):2151–2164
Smith RJH, Bale JF Jr, White KR (2005) Sensorineural hearing loss in children. Lancet 365(9462):879–890
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
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
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
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
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
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
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
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
Hatzopoulos S, Ciorba A, Sliwa L, Skarzynski P (2013) Technological advances in universal neonatal hearing screening (UNHS). Hear Balance Commun 11:104–109
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
Clark JG (1981) Uses and abuses of hearing loss classification. ASHA 23(7):493–500
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
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
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
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
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
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
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
Molini E, Calzolaro L, Lapenna R, Ricci G (2016) Universal newborn hearing screening in Umbria region, Italy. Int J Pediatr Otorhinolaryngol 82:92–97
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
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
Van Camp G, Smith RJH (2006). Hereditary hearing loss homepage. https://hereditaryhearingloss.org. Accessed 15 August 2021
Findlen UM, Hounam GM, Alexy E, Adunka OF (2019) Early hearing detection and intervention: timely diagnosis, timely management. Ear Hear 40(3):651–658
Wickremasinghe AC, Risley RJ, Kuzniewicz MW et al (2015) Risk of sensorineural hearing loss and bilirubin exchange transfusion thresholds. Pediatrics 136(3):505–512
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
Yoshinaga-Itano C, Manchaiah V, Hunnicutt C (2021) Outcomes of universal newborn screening programs: systematic review. J Clin Med 10(13):2784
Korver AMH, Smith RJH, Van Camp G et al (2017) Congenital hearing loss. Nat Rev Dis Primers 3:16094
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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.
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The Ethics Committee of the involved hospital approved the study (EC/2018/0787).
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Not applicable (retrospective chart study approved by the Ethics Committee of the involved hospital).
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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
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DOI: https://doi.org/10.1007/s00405-021-07060-5