Bilirubin encephalopathy causes potentially preventable brain damage and hearing loss. The site of auditory dysfunction is controversial, despite pathologic studies showing damage to brain stem auditory nuclei in humans and experimental animals. We studied the effects of bilirubin toxicity on the auditory system of homozygous jaundiced Gunn rats by use of brain stem auditory evoked potentials. Small but statistically significant abnormalities were found for wave latencies, interwave intervals, and amplitudes.
Ahdab-BarmadaMMoossyJ. The neuropathology of kernicterus in the premature neonate: Diagnostic problems. J Neuropathol Exp Neurol1984; 43: 45–56.
2.
BergmanIHirschRPFriaTJShapiroSMHolzmanIPainterMJ. Cause of hearing loss in the high-risk premature infant. J Pediatr1985; 106: 95–101.
3.
CashoreWJOhW. Unbound bilirubin and kernicterus in low birth weight infants. Pediatrics1982; 69: 481–5.
4.
DeVriesLSLarySDubowitzLMS. Relationship of serum bilirubin levels to ototoxicity and deafness in high-risk low-birth-weight infants. Pediatrics1985; 76: 351–4.
5.
GartnerLMSnyderRNChabonRSBernsteinJ. Kernicterus: High incidence in premature infants with low serum bilirubin concentrations. Pediatrics1970; 45: 906–17.
6.
TurkelSBGuttenbergMGMoynesDRHodgmanJE. Lack of identifiable risk factors for kernicterus. Pediatrics1980; 66: 502–6.
7.
BengtssonBVerneholtJ. A follow-up study of hyperbilirubinemia in healthy full-term infants without iso-immunization. Acta Paediatr Scand1974; 63: 70–80.
8.
HymanCBKeasterJHansonV. CNS abnormalities after neonatal hemolytic disease or hyperbilirubinemia. A prospective study of 405 patients. Am J Dis Child1969; 117: 395–405.
9.
OdellGBStoreyGNRosenbergLA. Studies in kernicterus, III. The saturation of serum proteins with bilirubin during neonatal life and its relationship to brain damage at five years. J Pediatr1970; 76: 12–21.
10.
ScheidtPCMellitsEDHardyJBDrageJSBoggsTR. Toxicity to bilirubin in neonates: Infant development during first year in relation to maximal neonatal serum bilirubin concentration. J Pediatr1977; 92: 292–7.
11.
CrabtreeNGerrardJ. Perceptive deafness associated with severe neonatal deafness. A report of 16 cases. J Laryngol Otol1950; 64: 482–506.
12.
FlottorpGMorleyDESkatvedtM. The localization of hearing impairment in athetoids. Acta Otolaryngol (Stockh)1957; 48: 404–14.
13.
HardyWG. Auditory deficits of the kernicterus child. In: SwinyardCA, ed. Kernicterus and its importance in cerebral palsy. Springfield, Ill: Charles C Thomas, 1961: 255–66.
14.
KeasterJHymanCBHarrisI. Hearing problems subsequent to neonatal hemolytic disease or hyperbilirubinemia. Am J Dis Child1969; 117: 406–10.
15.
DublinW. Central auditory pathology. Otolaryngol Head Neck Surg1986; 95: 363–424.
16.
DublinW. Cytoarchitecture of the cochlear nuclei: Report of an illustrative case of erythroblastosis. Arch Otolaryngol1974; 100: 355–9.
17.
DublinW. Neurological lesions in erythroblastosis fetalis in relation to nuclear deafness. Am J Clin Pathol1951; 21: 935–9.
18.
GerrardJ. Nuclear jaundice and deafness. J Laryngol Otol1952; 66: 39–46.
19.
HaymakerWMargiesCPentchewA. Pathology of kernicterus and posticteric encephalopathy. In: SwinyardCA, ed. Kernicterus and its importance in cerebral palsy. Springfield, Ill: Charles C Thomas, 1961: 21–228.
20.
KelemenG. Erythroblastosis fetalis. Pathologic report on the hearing organs of a newborn infant. AMA Arch Otolaryngol1956; 63: 392–8.
ByersRKPaineRSCrothersB. Extrapyramidal cerebral palsy with hearing loss following erythroblastosis. Pediatrics1955; 15: 248–54.
25.
PerlsteinMA. The late clinical syndrome of posticteric encephalopathy. Pediatr Clin North Am1960; 7: 665–87.
26.
RosenJ. Rh child: Deaf or “aphasic”? 4. Variations in the auditory disorders of the Rh child. J Speech Hear Disord1956; 21: 418–22.
27.
RubenRLiebermanABordleyJ. Some observations on cochlear potentials and nerve action potentials in children. Laryngoscope1962; 72: 545–53.
28.
NakamuraHTakadaSShimabukuRMatsuoMMatsuoTNagishiH. Auditory nerve and brainstem responses in newborn infants with hyperbilirubinemia. Pediatrics1985; 75: 703–8.
ChinKCTaylorMJPerlmanM. Improvement in auditory and visual evoked potentials in jaundiced preterm infants after exchange transfusion. Arch Dis Child1985; 60: 714–7.
33.
NwaeseiCGVan AerdeJBoydenMPerlmanM. Changes in auditory brainstem responses in hyperbilirubinemic infants before and after exchange transfusion. Pediatrics1984; 74: 800–3.
34.
WennbergRPAhlforsCEBickersRMcMurtyCAShetterJL. Abnormal auditory brainstem response in a newborn infant with hyperbilirubinemia: Improvement with exchange transfusion. J Pediatr1982; 100: 624–6.
35.
GunnCH. Hereditary acholuric jaundice in a new mutant strain of rats. J Heredity1938; 29: 137–9.
36.
StrebelLOdellGB. Bilirubin uridine diphosphoglucuronyltransferase in rat liver microsomes: Genetic variation and maturation. Pediatr Res1971; 5: 548–59.
37.
JohnsonISarmientoFBlancWADayR. Kernicterus in rats with an inherited deficiency of glucuronyl transferase. Am J Dis Child1959; 97: 591–608.
38.
SchuttaHSJohnsonL. Clinical signs and morphologic abnormalities in Gunn rats treated with sulfadimethoxine. J Pediatr1969; 75: 1070–9.
39.
SchmidRAxelrodJHammakerLSwarmR. Congenital jaundice in rats due to a defect in glucuronide formation. J Clin Invest1958; 37: 1123–30.
40.
BlancWAJohnsonL. Studies on kernicterus. Relationship with sulfonamide intoxication, report on kernicterus in rats with glucuronyl transferase deficiency and review of pathogenesis. J Neuropathol Exp Neurol1959; 18: 165–89.
41.
RoseALWisniewskiH. Acute bilirubin encephalopathy induced with sulfadimethoxine in Gunn rats. J Neuropathol Exp Neurol1979; 38: 152–64.
42.
JewJYWilliamsTH. Ultrastructural aspects of bilirubin encephalopathy in cochlear nuclei of the Gunn rat. J Anat1977; 124: 599–614.
BelalAJr.. Effect of hyperbilirubinemia on the inner ear in Gunn rats. J Laryngol Otol1975; 89: 259–65.
45.
UzielAMarotMPujolR. The Gunn rat: An experimental model for central deafness. Acta Otolaryngol (Stockh)1983; 95: 651–6.
46.
BattyHKMillhouseOE. Ultrastructure of the Gunn rat substantia nigra. I. Cytoplasmic changes. Acta Neuropathol (Berl)1976b; 35: 93–107.
47.
BattyHKMillhouseOE. Ultrastructure of the Gunn rat substantia nigra. II. Mitochondrial changes. Acta Neuropathol (Berl)1976a; 34: 7–19.
48.
LeviGSohmerHKapitulnikJ. Auditory nerve and brainstem responses in homozygous jaundiced Gunn rats. Arch Otolaryngol1981; 232: 139–43.
49.
BurkardR. Sound pressure level measurement and spectral analysis of brief acoustic transients. Electroencephalogr Clin Neurophysiol1984; 57: 83–91.
50.
BrownBWHollanderM. Statistics: A biomedical introduction. New York: John Wiley & Sons, 1977.
51.
OdellGBSchuttaHS. Bilirubin encephalopathy. In: McCandlessDW, ed. Cerebral energy metabolism and metabolic encephalopathy. New York: Plenum Publishing, 1985: 229–61.
52.
MøllerAR. On the origin of the compound action potentials (N1, N2) of the cochlea of the rat. Exp Neurol1983; 80: 633–44.
53.
KarpWBMoorePJSubramanyamSBBrownDB. Relationship of plasma total bilirubin, apparent unbound bilirubin and total albumin with cerebellar glycogen and abnormal Purkinje cells in the Gunn rat. Biol Neonate1982; 41: 294–304.