Results of auditory brainstem response (ABR) evaluation of 145 infants, ages 6 months and younger with craniofacial anomalies (CFA), were examined to determined predicted degree and nature of hearing loss. Approximately 50% of infants demonstrated at least mild bilateral hearing loss. Presence and degree of hearing loss varied by CFA. All infants with bilateral aural atresia exhibited at least a moderate bilateral hearing loss; less than 20% of infants with isolated external ear anomalies (ear tags, isolated microtia) exhibited any degree of hearing loss. In 92% of infants with hearing loss, results of ABR were consistent with primarily conductive dysfunction. Implications for early identification and audiologic management of infants with CFA and hearing loss are discussed.
Get full access to this article
View all access options for this article.
References
1.
JonesRL. Smith's recognizable patterns of human malformation. 4th ed.Philadelphia: WB Saunders Company, 1988: 709.
2.
DownsMPSilverHK. The “ABCD's” to HEAR: early identification in nursery, office, and clinic of the infant who is deaf. Clin Pediatr1972;11:563–5.
3.
FeinmesserM.TellL.Neonatal screening for detection of deafness. Arch Otolaryngol1976;102:297–9.
4.
Joint Committee on Infant Hearing. 1990 position statement. ASHA1991;33:3–6.
5.
HydeMLRikoK.CorbinH.MorosoM.AlbertiPW. A neonatal hearing screening research program using brainstem electric response audiometry. J Otolaryngol1984; 31: 49–54.
6.
HalpernJ.DunnHosford H.MalachowskiN.Four factors that accurately predict hearing loss in “high risk” neonates. Ear Hear1987;8:21–5.
7.
SmithRJHZimmermanB.ConnollyPKJergerSWYelichA.Screening audiometry using the high-risk register in a level III nursery. Arch Otolaryngol Head Neck Surg1992; 118: 1306–11.
8.
FriaTJParadiseJLSaboDLElsterBA. Conductive hearing loss in infants and young children with cleft palate. J Pediatr1987;111:84–7.
9.
HeliasJ.ChobautJ. C.MourotM.LafonJ. C.Early detection of hearing loss in children with cleft palates by brain-stem auditory response. Arch Otolaryngol Head Neck Surg1988; 114:154–6.
10.
CoplanJ.Deafness: ever heard of it? Delayed recognition of permanent hearing loss. Pediatrics1987;79:206–13.
11.
StewartJMDownsMP. Congenital conductive hearing loss: the need for early identification and intervention. Pediatrics1993;91:355–9.
LaryS.BriassoulisG.De VriesL.DubowitzLMSDubowitzV.Hearing threshold in preterm and term infants by auditory brainstem response. J Pediatr1985;107:593–9.
14.
StarrA.AmlieRNMartinWHSandersS.Development of auditory function in newborn infants revealed by auditory brainstem potentials. Pediatrics1977;60:831–9.
15.
CoatsACMartinJL. Human auditory nerve action potentials and brain stem evoked responses: effects of audiogram shape and lesion location. Arch Otolaryngol1977;103:605–22.
16.
JergerJ.MauldinL.Prediction of sensorineural hearing level from the brain stem evoked response. Arch Otolaryngol1978;104:456–61.
17.
MauldinL.JergerJ.Auditory brain stem evoked responses to bone-conducted signals. Arch Otolaryngol1979;105:656–61.
18.
FriaTJSaboDL. Brainstem auditory responses in children with otitis media with effusion. Ann Otol Rhinol Laryngol (Suppl)1980;89:200–26.
19.
KilenyP.MagathanM.Predictive value of ABR in infants and children with moderate to profound hearing impairment. Ear Hear1987;8:217–21.
20.
HydeMLRikoK.MaliziaK.Audiometric accuracy of the click ABR in infants at risk for hearing loss. J Am Acad Audiol1990;1:59–66.
21.
JergerJ.HayesD.JordanC.Clinical experience with auditory brainstem response audiometry in pediatric assessment. Ear Hear1980;1:19–25.
22.
HayesD.Auditory brainstem response (ABR) in infants: screening and diagnostic applications. In: BessFHHallJW, eds. Screening children for auditory function. Nashville, Tenn.: Bill Wilkerson Center Press, 1992; 127–43.
23.
SmithDurieux A.PictonTWEdwardsCGMacMurrayB.GoodmanJT. Brainstem electric-response audiometry in infants of a neonatal intensive care unit. Audiology1987; 26: 284–97.
24.
ParadiseJLBluestoneCDFelderH.The universality of otitis media in 50 infants with cleft palate. Pediatrics1969; 44:35–42.
25.
ParadiseJLBluestoneCD. Early treatment of the universal otitis media of infants with cleft palate. Pediatrics1974; 53: 48–54.
26.
FriaTJCantekinEIEichlerJA. Hearing acuity of children with otitis media with effusion. Arch Otolaryngol1985; 111: 10–6.
27.
ElssmannSFMatkinNDSaboMP. Early identification of congenital sensori-neural hearing impairment. Hear J1987; 9:13–7.
28.
MaceALWallaceKLWhanMQStelmachowiczPG. Relevant factors in the identification of hearing loss. Ear Hear1991;12:287–93.
29.
MaukGWWhiteKRMortensenLBBehrensTR. The effectiveness of screening programs based on high-risk characteristics in early identification of hearing impairment. Ear Hear1991;12:312–9.
30.
SteinLKJabaleyT.SpitzR.StoakleyD.McGeeT.The hearing-impaired infant: patterns of identification and habilitation revisited. Ear Hear1990;11:201–5.
31.
PattiFriel S.HieberFinitzo T.ContiG.BrownKC. Language delay in infants associated with middle ear disease and mild, fluctuating hearing impairment. Pediatr Infect Dis J1982;1:104–9.
32.
KleinJO. Otitis media and the development of speech and language. Pediatr Infect Dis J1984;3:389–91.
33.
PearcePSSaundersMACreightonDESauveRS. Hearing and verbal-cognitive abilities in high-risk preterm infants prone to otitis media with effusion. J Dev Behav Pediatr1988;9:346–51.
34.
WallaceIFGravelJSMcCartonCMRubenRJ. Otitis media and language development at 1 year of age. J Speech Hear Disord1988;53:245–51.
35.
PattiFriel S.FinitzoT.Language learning in a prospective study of otitis media with effusion in the first two years of life. J Speech Hear Res1990;33:188–94.
36.
KuhlPKWilliamsKALacerdaF.StevensKNLindblomB.Linguistic experience alters phonetic perception in infants by 6 months of age. Science1992;255:606–8.
