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Bilirubin and the Auditory System

Abstract

The auditory system is highly sensitive to bilirubin toxicity. Damage to the auditory nervous system includes auditory neuropathy or auditory dyssynchrony and auditory processing problems which may occur with or without deafness, hearing loss. Auditory dysfunction may occur in children with or without other signs of classical kernicterus. Bilirubin selectively damages the brainstem auditory nuclei, and may also damage the auditory nerve and spiral ganglion containing cell bodies of primary auditory neurons. The inner ear, thalamic and cortical auditory pathways appear to be spared. Noninvasive auditory neurophysiological tests such as the auditory brainstem response (ABR) or brainstem auditory response (BAER) play an important role in the early detection of bilirubin-induced auditory and central nervous system dysfunction in the neonate.

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References

  1. Dublin W Neurological lesions in erythroblastosis fetalis in relation to nuclear deafness Am J Clin Pathol 1951 21 935–9

    Article  CAS  Google Scholar 

  2. Ahdab-Barmada M, Moossy J The neuropathology of kernicterus in the premature neonate: diagnostic problems J Neuropathol Exp Neurol 1984 43 45–56

    Article  CAS  Google Scholar 

  3. Gerrard J Nuclear jaundice and deafness J Laryngol Otol 1952 66 39–46

    Article  CAS  Google Scholar 

  4. Kelemen G Erythroblastosis fetalis. Pathologic report on the hearing organs of a newborn infant AMA Arch Otolaryngol 1956 63 392–8

    Article  Google Scholar 

  5. Belal A Effect of hyperbilirubinemia on the inner ear in Gunn rats J Laryngol Otol 1975 89 259–65

    Article  Google Scholar 

  6. Uziel AM, Marot M, Pujol R The Gunn rat: An experimental model for central deafness Acta Otolaryngol 1983 95 651–6

    Article  CAS  Google Scholar 

  7. Matkin ND, Carhart R Auditory profiles associated with Rh incompatibility Arch Otolaryngol 1966 84 502–13

    Article  CAS  Google Scholar 

  8. Bergman I, Hirsch RP, Fria TJ, Shapiro SM, Holzman I, Painter MJ Cause of hearing loss in the high-risk premature infant J Pediatr 1985 106 95–101

    Article  CAS  Google Scholar 

  9. de Vries LS, Lary S, Dubowitz LMS Relationship of serum bilirubin levels to ototoxicity and deafness in high-risk, low birth-weight infants Pediatrics 1985 76 351–4

    CAS  PubMed  Google Scholar 

  10. de Vries LS, Lary S, Whitelaw AG, Dubowitz LM Relationship of serum bilirubin levels and hearing impairment in newborn infants Early Hum Dev 1987 15 269–77

    Article  CAS  Google Scholar 

  11. Gunn CK Hereditary acholuric jaundice in a new mutant strain of rats J Hered 1938 29 137–9

    Article  Google Scholar 

  12. Strebel L, Odell GB Bilirubin uridine disphosphoglucuronyltransferase in rat liver microsomes: genetic variation and maturation Pediatr Res 1971 5 548–59

    Article  CAS  Google Scholar 

  13. Churn SB, DeLorenzo RJ, Shapiro SM Bilirubin induces a calcium-dependent inhibition of multifunctional Ca 2+/calmodulin-dependent kinase II activity in vitro Pediatr Res 1995 38 949–54

    Article  CAS  Google Scholar 

  14. Shapiro SM, Hecox KE Developmental studies of brainstem auditory evoked potentials in jaundiced Gunn rats Dev Brain Res 1988 41 147–57

    Article  Google Scholar 

  15. Shapiro SM, Hecox KE Brain stem auditory evoked potentials in jaundiced Gunn rats Ann Otol Rhinol Laryngol 1989 98 308–17

    Article  CAS  Google Scholar 

  16. Diamond I, Schmid R Experimental bilirubin encephalopathy: The mode of entry of bilirubin- 14C into the CNS J Clin Invest 1966 45 678–89

    Article  CAS  Google Scholar 

  17. Shapiro SM Acute brainstem auditory evoked potential abnormalities in jaundiced Gunn rats given sulfonamide Pediatr Res 1988 23 306–10

    Article  CAS  Google Scholar 

  18. Shapiro SM Reversible brainstem auditory evoked potential abnormalities in jaundiced Gunn rats given sulfonamide Pediatr Res 1993 34 629–33

    Article  CAS  Google Scholar 

  19. Shapiro SM Binaural effects in brainstem auditory evoked potentials of jaundiced rats Hearing Res 1991 53 41–8

    Article  CAS  Google Scholar 

  20. Chisin R, Perlman M, Sohmer H Cochlear and brain stem responses in hearing loss following neonatal hyperbilirubinemia Ann Otol 1979 88 352–7

    CAS  Google Scholar 

  21. Starr A, Picton TW, Sininger Y, Hood LJ, Berlin CI Auditory neuropathy Brain 1996 119 741–53

    Article  Google Scholar 

  22. Funato M, Tamai H, Shimada S, Nakamura H Vigitiphobia, unbound bilirubin, and auditory brainstem responses Pediatrics 1994 93 50–3

    CAS  PubMed  Google Scholar 

  23. Nakamura H, Takada S, Shimabuku R, Matsuo M, Matsuo T, Negishi H Auditory nerve and brainstem responses in newborn infants with hyperbilirubinemia Pediatrics 1985 75 703–8

    CAS  PubMed  Google Scholar 

  24. Deltenre P, Mansbach AL, Bozet C, Clerex A, Hecox KE Auditory neuropathy: A report on three cases with early onsets and major neonatal illnesses Electroencephalogr Clin Neurophysiol 1997 104 17–22

    Article  CAS  Google Scholar 

  25. Ahlfors CE, Shapiro SM Auditory brainstem response and unbound bilirubin in jaundiced (jj) Gunn rat pups Biol Neonate 2001 80 (2) 158–62 (Aug)

    Article  CAS  Google Scholar 

Download references

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Shapiro, S., Nakamura, H. Bilirubin and the Auditory System. J Perinatol 21 (Suppl 1), S52–S55 (2001). https://doi.org/10.1038/sj.jp.7210635

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