European Archives of Oto-Rhino-Laryngology

, Volume 266, Issue 2, pp 207–212

Pedaudiologic findings after severe neonatal hyperbilirubinemia

  • Andreas Nickisch
  • Claudia Massinger
  • Birgit Ertl-Wagner
  • Hubertus von Voss
Otology

Abstract

Neonatal hyperbilirubinemia (NHB) above 20 mg/dl (NHB20) has been shown to increase the risk of hearing impairments. Up to now, audiological findings based on behavioural audiometry (BA), otoacoustic emissions (TEOAE) and auditory brainstem responses (ABR) from children after being diagnosed with NHB20 have not been thoroughly compared to those with lower NHB-levels. We, therefore, aimed to assess the presence and characteristics of auditory dysfunction in children with NHB20. The audiological data of 15 children aged 11 months to 9 years with a NHB level between 22.6 and 45.6 mg/dl and/or MRI-confirmed bilirubin encephalopathy (NHBG) were compared retrospectively to 15 children with NHB levels between 12.5 and 19.4 mg/dl (CG). After matching by weeks of gestation at birth, BA, TEOAE and ABR were performed in all the children. Subsequently the groups were compared. Only two children of the NHBG had consistently normal audiologic findings. Hearing function disorders were detected in 87% (13/15) of the NHBG-children, ranging from total deafness to normal BA, including unilateral and bilateral deafness as well as cochlear hearing loss. Auditory neuropathy/dys-synchrony (AN) was found in a total of eight children (53%) of the NHBG. In addition, it was found that after the occurrence of NHB20, initially detected TEOAE can disappear in some cases. In the comparison group (CG) only two children demonstrated a hearing dysfunction, both of which were cochlear hearing impairments, whereas no child had AN. A bias towards hearing impairments has to be taken into account for both groups. Detailed pedaudiologic testing should be mandatory for all children after the occurrence of NHB20 including follow-up during the first 12 months. Audiological diagnostic work-up in the affected children requires objective investigations of hearing functions, while BA is recommended to evaluate the adequate therapeutic procedure.

Keywords

Neonatal hyperbilirubinemia Bilirubin encephalopathy Kernicterus Hearing disorders Auditory neuropathy 

References

  1. 1.
    AAP American Academy of Pediatrics (2004) Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics 114:297–316CrossRefGoogle Scholar
  2. 2.
    Agrawal VK, Shukla R, Misra PK, Kapoor RK, Malik GK (1998) Brainstem auditory evoked response in newborns with hyperbilirubinemia. Indian Pediatrics 35:513–518PubMedGoogle Scholar
  3. 3.
    Akman I, Özek E, Kuleci S, Türkdogan D, Cebeci D, Akdas F (2004) Auditory neuropathy in hyperbilirubinemia. Int J Audiol 43:516–522PubMedCrossRefGoogle Scholar
  4. 4.
    Deltenre P, Mansbach AL, Bozet C, Christiaens F, Barthelemy P, Paulissen D, Renglet T (1999) Auditory neuropathy with preserved cochlear microphonics and secondary loss of otoacustic emissions. Audiology 38:187–195PubMedCrossRefGoogle Scholar
  5. 5.
    Deorari AK, Singh M, Ahuja GK, Bisht MS, Verma A, Paul VK, Tandon DA (1994) One year outcome of babies with severe neonatal hyperbilirubinemia and reversible abnormality in brainstem auditory evoked responses. Indian Pediatr 31:915–921PubMedGoogle Scholar
  6. 6.
    Gupta AK, Mann SBS (1998) Is auditory brainstem response a bilirubin neurotoxicity marker? Am J Otolaryngol 19:232–236PubMedCrossRefGoogle Scholar
  7. 7.
    Katona G, Farkas Z, Revai K, Szabo M (1989) Ujszülöttkori icterusos betegek utanvizsgalata akusztikus kivaltott potencial audiometria (BERA) segitsegevel. Orvosi Hetilap 19:1001–1004Google Scholar
  8. 8.
    Madden C, Rutter M, Hilbert L, Greinwald J, Choo D (2002) Clinical and audiological features in auditory neuropathy. Arch Otolaryngol 128:1026–1030Google Scholar
  9. 9.
    Ögün B, Serbetcioglu B, Duman N, Özkan H, Kirkim G (2003) Long-term outcome of neonatal hyperbilirubinemia. Clin Otolaryngol 28:507–513PubMedCrossRefGoogle Scholar
  10. 10.
    Ozcelik T, Onerci M, Ozcelik U, Aksoy S, Sennaroglu L (1997) Audiological findings in kernicteric patients. Acta Otorhinolaryngol Belg 51:31–34PubMedGoogle Scholar
  11. 11.
    Özmert E, Erdem G, Topcu M, Yurdakök M, Tekinalp G, Genc D, Renda Y (1996) Long-term follow-up of direct hyperbilirubinemia in full-term Turkish infants. Acta Paediatr 85:1440–1444PubMedCrossRefGoogle Scholar
  12. 12.
    Pallotta R, Minervino M, Sabatino G, Nardini P, Elicio MR (1984) Effetti a lungo termine della iperbilirubinemia neonatale di modico grado sui potenziali evocati troncoencefalici. Pediatr Med Chir 6:273–276PubMedGoogle Scholar
  13. 13.
    Rance G, Beer DE, Cone-Wesson B, Shepherd RK, Dowell RC, King AM, Rickards FW, Clark GM (1999) Clinical findings for a group of infants and young children with auditory neuropathy. Ear Hear 20:238–252PubMedCrossRefGoogle Scholar
  14. 14.
    Shapiro SM, Rosen JR, Dixon KT (2002) Auditory brainstem responses and auditory neuropathy abnormalities in children with neonatal hyperbilirubinemia who subsequently develop kernicterus. Pediatr Res 51(Part 2):340AGoogle Scholar
  15. 15.
    Shapiro SM, Daymond MJ (2003) Patterns of kernicterus related to neonatal hyperbilirubinemia and gestational age. Pediatr Res 53–54(Part 2):398A–399AGoogle Scholar
  16. 16.
    Shapiro SM (2005) Definition of the clinical spectrum of kernicterus and bilirubin-induced neurologic dysfunction (BIND). J Perinatol 25:54–59PubMedCrossRefGoogle Scholar
  17. 17.
    Stein L, Tremblay K, Pasternak J, Banerjee S, Lindemann K, Kraus N (1996) Brainstem abnormalities in neonates with normal otoacoustic emissions. Semin Hear 17:197–213CrossRefGoogle Scholar
  18. 18.
    Tan KL, Skurr BA, Yip YY (1992) Phototherapy and the brain-stem auditory evoked response in neonatal hyperbilirubinemia. J Pediatr 120:306–308PubMedCrossRefGoogle Scholar
  19. 19.
    Thoma J, Gerull G, Mrowinski D (1986) A long term study of hearing in children following neonatal hyperbilirubinemia. Arch Otorhinolaryngol 243:133–137PubMedCrossRefGoogle Scholar
  20. 20.
    Vohr BR, Karp D, O’Dea C, Darrow D, Coll CG, Lester BM, Brown L, Oh W, Cashore W (1990) Behavioral changes correlated with brain-stem auditory evoked responses in term infants with moderate hyperbilirubinemia. J Pediatr 117:288–291PubMedCrossRefGoogle Scholar
  21. 21.
    Wong V, Chen WX, Wong KY (2005) Short- and long-term outcome of severe neonatal nonhemolytic hyperbilirubinemia. J Child Neurol 21:309–315CrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  • Andreas Nickisch
    • 1
    • 2
  • Claudia Massinger
    • 1
    • 2
  • Birgit Ertl-Wagner
    • 3
  • Hubertus von Voss
    • 1
    • 2
  1. 1.Department of Phoniatrics and PedaudiologyKinderzentrum MunichMunichGermany
  2. 2.The Institute of Social PediatricsLudwig Maximilian University MunichMunichGermany
  3. 3.Institute of Clinical RadiologyUniversity of MunichMunichGermany

Personalised recommendations