Neonatal Screening for Hearing Disorders in Infants at Risk:
Incidence, Risk Factors, and Follow-up
Christiane Meyer, MD*; Jan Witte, MD‡; Agnes Hildmann, MD§; Karl-Heinz Hennecke, MD§;
Karl-Ulrich Schunck, MD\; Kerstin Maul, MD\; Ute Franke, MD\; Hubert Fahnenstich, MD¶;
Heike Rabe, MD#; Rainer Rossi, MD#; Sabine Hartmann, MD**; and Ludwig Gortner, MD*
ABSTRACT. Objective. To determine the incidence
and risk factors for hearing disorders in a selected group
of neonates and the feasibility of selective hearing
screening.
Settings. Multicenter prospective trial at five centers
in Germany.
Methods. Enrollment criteria: in addition to previ-
ously defined risk factors by the Joint Committee on
Infant Hearing (family history of hearing loss, in utero
infections, craniofacial anomalies, birth weight <1500 g,
critical hyperbilirubinemia, ototoxic medications, bacte-
rial meningitis, postnatal asphyxia, mechanical ventila-
tion >5 days, stigmata, or syndromes associated with
hearing loss), the impact of maternal drug abuse, birth
weight <10th percentile, persistent pulmonary hyperten-
sion, and intracranial hemorrhage more than or equal to
grade III or periventricular leukomalacia on infant hear-
ing were evaluated. The screening procedure was per-
formed by automated auditory brainstem response
(A-ABR; ALGO 1-plus; Natus Med Inc, San Carlos, CA).
Statistics: univariate analyses of risk factors versus A-
ABR results and a multivariate regression analysis were
used; additionally, the total test time was recorded.
Results. Seven hundred seventy recordings from 777
infants enrolled consecutively constitute the basis of this
analysis. Mean gestational age was 33.8 6 4.3 weeks,
birth weight 2141 6 968 g; 431 infants being male and 339
female; 41 (5.3%) infants exhibited pathologic A-ABR
results (16 bilateral and 25 unilateral). Meningitis or sep-
sis, craniofacial malformations, and familial hearing loss
were independent significant risk factors. Median total
test time was 25 minutes. Follow-up examinations in 31
infants revealed persistent hearing loss in 18 infants (13
infants sensorineural, 5 from mixed disorders), 7 requir-
ing amplification.
Conclusion. Hearing screening in high-risk neonates
revealed a total of 5% of infants with pathologic A-ABR
(bilateral 2%). Significant risk factors were familial hear-
ing loss, bacterial infections, and craniofacial abnormal-
ities. Other perinatal complications did not significantly
influence screening results indicating improved perina-
tal handling in a neonatal population at risk for hearing
disorders. Pediatrics 1999;104:900 –904; hearing screening,
From the *Children’s Hospital and ‡Department of Otolaryngology, Med-
ical University, Lübeck, Germany; the §Vestische Kinderklinik Datteln,
University Witten, Herdecke, Germany; the \Children’s Hospital Friedrichs-
hain, Berlin, Germany; the ¶Children’s Hospital University, Bonn, Ger-
many; the #Children’s Hospital and the **Department of Phoniatrics and
Pedaudiology, University Münster, Münster, Germany.
Received for publication Sep 14, 1998; accepted Apr 8, 1999.
Address correspondence to Ludwig Gortner, MD, Justus-Liebig-University,
Pediatric Hospital, Feulgenstrabe 12, D-35392, Giessen, Germany.
PEDIATRICS (ISSN 0031 4005). Copyright © 1999 by the American Acad-
emy of Pediatrics.
900 PEDIATRICS Vol. 104 No. 4 October 1999
Downloaded from http://pediatrics.aappublications.org/ by guest on December 11, 2017
neonates, craniofacial malformations, sepsis, familial
hearing loss; follow-up.
ABBREVIATIONS. ABR, auditory brainstem response; OAE, oto-
acoustic emissions; A-ABR, automated auditory brainstem re-
sponse; PPHN, persistent pulmonary hypertension of the new-
born.
O
ne to 2 of 1000 newborns suffer from congen-
ital or perinatally acquired hearing disor-
ders.1–3 The prevalence of neonatal hearing
disorders has been reported to be increased 10- to
50-fold in infants at risk.3–9 Moderate to severe bilat-
eral hearing loss (ie, .40 dB) distorts the developing
child’s perception of his or her attempt at speech
production. If this type of hearing disorder remains
undetected through the critical period of language
acquisition within the first year of life, a profound
impairment of receptive and expressive speech and
language development will result. This in turn will
lead to a decreased acquisition of expected mile-
stones.10 For acquisition of hearing, a sensitive period
up to 6 months of age has been demonstrated.11
Accordingly, introduction of hearing aids within this
period will improve subsequent hearing develop-
ment among hearing-impaired children.12 To ensure
timely therapy, a reasonable goal is to establish the
diagnosis of severe neonatal hearing impairment be-
fore the age of 6 months.
In addition to hereditary hearing loss, a number of
in utero and neonatal complications (eg, infections,
immaturity, asphyxia, ototoxic medications, and hy-
perbilirubinemia) have been described as risk factors
of neonatal hearing disorders.11 This resulted in the
recommendations of the Joint Committee on Infant
Hearing to screen all infants with risk factors within
the neonatal period. This selective screening was
considered a first step toward the introduction of
universal hearing screening.13 Two methods for neo-
natal hearing screening have been developed during
the past 2 decades: the registration of a modified
auditory brainstem response (ABR) first described by
Davis14 in 1976 and the registration of otoacoustic
emissions (OAE) introduced by Kemp15 in 1978. In
contrast to ABR, the use of OAE does not identify
postcochlear hearing disorders. Both methods fur-
ther were adapted for routine screening by an auto-
mated auditory brainstem response (A-ABR) or cor-
responding OAE techniques.
 The objective of the present study was to obtain
actual data on the incidence and risk factors of neo-
natal hearing disorders in light of improved outcome
of high-risk neonates.16,17 We thus performed a hear-
ing screening in all infants with risk factors for hear-
ing disorders,6 – 8,18 –20 in a prospective multicenter
clinical trial, to identify the incidence of moderate to
severe neonatal hearing disorders. We added further
risk factors to those proposed by the Joint Commit-
tee,13 which were demonstrated specifically to affect
the auditory system: intracranial hemorrhage more
than or equal to grade III or periventricular leukoma-
lacia,6,21 maternal drug abuse,22 intrauterine growth
restriction leading to small-for-gestational age in-
fants,23 and persistent pulmonary hypertension of
the newborn (PPHN).24 In addition, the feasibility of
a neonatal screening for hearing disorders and the
time necessary for screening procedures were inves-
tigated.
METHODS
The study was performed from October 1995 through Novem-
ber 1997 at 5 pediatric hospitals in the Federal Republic of Ger-
many (University Children’s Hospitals Bonn, Münster, and Lü-
beck; Vestische Kinderklinik Datteln, University Witten-
Herdecke; and Pediatric Hospital Berlin-Friedrichshain).
Inclusion Criteria
All infants were enrolled in the trial, if informed parental
consent was obtained and at least one of the risk factors according
to the Joint Committee on Infant Hearing was diagnosed:
1. Family history of hereditary childhood sensorineural hearing
loss
2. In utero infections (toxoplasmosis, rubella, cytomegaly, herpes
simplex virus infections, and syphilis)
3. Craniofacial anomalies
4. Birth weight ,1500 g
5. Hyperbilirubinemia at serum levels requiring exchange trans-
fusions (we further extended the criteria: critical hyperbiliru-
binemia 2 mg/dL below serum levels requiring exchange
transfusions according to Maisels25)
6. Ototoxic medications (eg, aminoglycosides alone or in combi-
nation with loop diuretics)
7. Bacterial meningitis (we further extended the criteria: bacteri-
ologic proven sepsis and/or meningitis)
8. Postnatal asphyxia (Apgar #5 at 1 minute or #6 at 5 minutes)
9. Mechanical ventilation lasting 5 days or longer
10. Stigmata or other findings associated with a syndrome known
to include a sensorineural and/or conductional hearing loss13
Definition of additional variables: intracranial hemorrhage
grade III to IV was defined according to Papile et al26 and periven-
tricular leukomalacia according to Hill et al27; small-for-gestational
age infants were classified according to the percentiles worked out
recently by Voigt et al28 for German newborns. PPHN was diag-
nosed according to Fox and Duara.29 Maternal drug abuse was
assumed based on prenatal history and confirmed by analyses of
urine and/or meconium postnatally including alcohol, cocaine,
opiates, and derivatives. Infants who required neonatal intensive
care were tested before discharge from hospital, with all other
newborns tested between days 2 to 7 after birth.
This procedure resulted in a final sample size of 777 infants.
Materials and Methods
An A-ABR, the ALGO-1-Plus (Natus Med Inc, San Carlos, CA)
with a click stimulus of 100-ms duration, an intensity of 35 dB
nHL, and an alternating polarity with an acoustic frequency spec-
trum of 700 to 5000 Hz (65 dB), was used. A built-in artifact
rejection for either myogenic, electric, or environmental noise
interference ensured that data collection was halted given unfa-
vorable testing conditions. The automated screener provided a
pass-fail report.30 All infants with initially abnormal A-ABR
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screening results were reexamined in the hospitals with A-ABR,
ie, a two-stage screening was scheduled in the study protocol.
In 3 out of 5 hospitals additional investigations using OAE (ILO
88, Hatfield, UK31) were performed after having obtained A-ABR.
The transient click-evoked OAE are produced by an active process
related to outer hear-cell activity in the cochlea. These OAE were
rated by an experienced audiologist in consideration of the stabil-
ity of the stimulus and its reproducibility. When the whole re-
sponse reproducibility was .60%, a pass was obtained, and test-
ing was halted. All infants with pathologic A-ABR on two
occasions further were scheduled for a detailed pediatric audio-
logic work-up and therapy if indicated.
All data records were reviewed by the study coordinator (L.G.).
In case of unclear or incomplete reports, they were sent back to the
respective hospital for correction or completion.
Statistics
We analyzed the variables, assumed to be risk factors for neo-
natal hearing loss, according to the criteria of the American Acad-
emy of Pediatrics13 and additionally the above outlined variables:
intracranial hemorrhage grade III to IV and/or periventricular
leukomalacia, birth weight ,10th percentile, PPHN, and maternal
drug abuse. Neonatal characteristics given in Table 1 were not
included in the analysis. In a first step univariate analyses with
appropriate methods, the Mann-Whitney U test and x2 test, SPSS-
program32 were performed to identify significant risk factors. The
validity of single risk factors was further assessed by multivariate
logistic regression analysis, with inclusion of all variables with a
significance level of P # .20 in the univariate analyses.
RESULTS
During the study period, a total of 777 consecutive
newborns were enrolled with 770 valid records avail-
able. Three hundred and fifty-eight (42%) had a ges-
tational age ,32 completed weeks, 241 (32.1%) had a
birth weight ,1500 g, and 74 (9.6%) had a birth
weight #1000 g. Neonatal characteristics of study
infants are given in Table 1. The median number of
risk factors per infant was 1 (range, 1– 8). Twenty-
three infants had $5 risk factors with mechanical
ventilation $5 days (n 5 23), birth weight ,1500 g
(n 5 22), ototoxic medications (n 5 22), and intracra-
nial hemorrhage more than or equal to grade III or
periventricular leukomalacia (n 5 12) being most
prevalent. Table 2 gives an overview on prenatal and
neonatal risk factors in study infants. The first A-
ABR testing gave pathologic results in 62 infants,
which could be confirmed at rescreening in 41 in-
fants (5.3%). Sixteen of these infants (2%) had bilat-
eral pathologic A-ABR, whereas unilateral patho-
logic A-ABR was observed in 25 infants (3.3%).
On univariate analysis sepsis and/or meningitis,
stigmata and/or syndromal disorders, and craniofa-
cial malformations were identified as significant risk
factors of A-ABR testing (Table 3). Cleft palate was
registered in 6 infants as the most common disorder
in the latter group. Meningitis or sepsis, observed in
7 infants with pathologic A-ABR, was caused by five
Gram-positive and two Gram-negative germs. Four
out of 5 infants with stigmata or syndromes were
TABLE 1. Neonatal Characteristics of Study Infants (n 5 770)
Gestational age, wk* 33.8 6 4.3
Birth weight, g* 2141 6 968
Cesarean section, n (%) 503 (65.3%)
Gender, male/female 431/339
Umbilical artery pH* 7.26 6 0.12
* Mean 6 SD.
ARTICLES 901
TABLE 2. Prenatal and Neonatal Risk Factors of Study In- TABLE 4. Multivariate Logistic Regression Analysis of Risk
fants (n 5 770) Factors for Pathologic A-ABR Testing
n % Variable Coefficient P
Prenatal risk factors Craniofacial anomalies 1.69 ,.001
Small-for-gestational age 163 21.2 Meningitis and/or sepsis 1.37 ,.01
Familial history of hearing loss 25 3.2 Familial hearing loss 1.40 .03
Maternal drug abuse 14 1.8 Maternal drug abuse 1.40 .07
In utero infections 8 1 Stigmata and/or syndromes 1.02 .10
Neonatal risk factors Birth weight ,1500 g 0.27 .49
Ototoxic medications 464 60.3
Birth weight ,1500 g 241 32.1 Abbreviation: A-ABR, automated audiologic brainstem response.
Mechanical ventilation .5 days 133 17.2
Craniofacial anomalies 69 9
Postnatal asphyxia 75 9.7
Sepsis or meningitis 61 7.9
OAE predicted pathologic A-ABR with a sensitivity
Intraventricular hemorrhage/PVL 27 3.5 of 71% and a specificity of 73%.
Hyperbilirubinemia 26 3.4 Follow-up in 31 infants with pathologic A-ABR
Stigmata and/or syndromes 20 2.6 revealed persisting severe hearing loss in 18 infants
Persistent pulmonary hypertension 6 0.8 (13 infants suffered from sensorineural, 5 from mixed
Abbreviation: PVL, periventricular leukomalacia. hearing disorders). Underlying disorders included
craniofacial anomalies (n 5 7), familial hearing loss
(n 5 3), sepsis and/or meningitis (n 5 3), very low
diagnosed as Down-Syndrome and 1 with fragile weight at birth and mechanical ventilation .5 days
X-syndrome by karyotyping. A weight at birth of (n 5 2), congenital rubella (n 5 1), postnatal asphyxia
,1500 g was no specific independent risk factor for (n 5 1), and Down Syndrome (n 5 1). Seven infants
pathologic A-ABR in the present study population. required early amplification at 3 to 6 months of age.
Breaking down birth weight categories for infants A transient hearing loss was diagnosed in 10 infants,
with 1000 to 1500 g and those with ,1000 g also as follow-up findings were normal. In 3 infants with
exhibited no association with pathologic A-ABR test- moderate conductional hearing disorders, no ampli-
ing in both subgroups. Chronic lung disease, defined fication was necessary. Two infants died after dis-
as oxygen dependency (fraction of inspired oxygen charge from the hospital, 8 infants were lost to fol-
.0.21 on day 28), was not found to be associated low-up because reexaminations were refused by the
with pathologic A-ABR testing. Mean (6SD) serum parents.
bilirubin concentrations in infants with critical hy- The median time for the A-ABR screening was 25
perbilirubinemia were 14.6 (61.7) mg/dL in infants minutes including 15 minutes for preparation and 10
,1500 g birth weight, 16.0 (62.7) mg/dL in infants of
1500 to 2500 g birth weight, and 23.7 (61.9) mg/dL
in infants .2500 g birth weight. TABLE 5. Comparison of A-ABR versus OAE in 464 Infants*
The result of the multivariate regression analysis is Pathologic Normal
given in Table 4. A-ABR A-ABR
In addition to A-ABR testing, OAE were measured Pathologic OAE 17 120
in 464 infants (only possible in three hospitals). Normal OAE 7 320
Pathologic OAE results were observed in 137 infants
Abbreviations: A-ABR, automated audiologic brainstem response;
(29.5%; unilateral in 61 and bilateral in 76 infants). OAE, otoacoustic emissions.
Comparison of OAE and A-ABR testing is given in * Not all infants tested by the A-ABR could also be investigated by
Table 5. Assuming A-ABR to be the gold standard, the OAE method.

TABLE 3. Distribution of Risk Factors in Infants With Normal and Pathologic A-ABR
Normal A-ABR Pathologic A-ABR P Odds Ratio 95%
(n 5 729) (n 5 41) Confidence Interval
n % n %
Small-for-gestational age 154 21.1 9 22.0 .90 1.05 (0.49–2.25)
Familial hearing loss 22 3.0 3 7.3 .13 2.54 (0.76–8.49)
Maternal drug abuse 12 1.7 2 4.9 .13 3.08 (0.71–13.6)
In utero infections 7 1.0 1 2.4 .36 2.58 (0.33–19.92)
Ototoxic medications 442 60.6 22 53.7 .38 0.75 (0.22–2.41)
Birth weight ,1500 g 232 31.8 9 22.0 .19 0.60 (0.29–1.27)
Mechanical ventilation $5 days 125 17.2 8 19.5 .70 1.17 (0.53–2.60)
Craniofacial anomalies 56 7.7 13 31.7 ,.001 5.58 (2.40–13.0)
Postnatal asphyxia 72 9.9 3 7.3 .60 0.73 (0.22–2.41)
Sepsis or meningitis 54 7.4 7 17.1 .03 2.57 (1.12–5.91)
Intraventricular hemorrhage, PVL 27 3.7 0 0.0 .21
Hyperbilirubinemia 26 3.6 0 0.0 .22
Stigmata and/or syndromes 15 2.1 5 12.2 ,.001 6.61 (2.60–16.80)
Persistent pulmonary hypertension 6 0.8 0 0.0 .56
Abbreviations: PVL, periventricular leukomalacia; A-ABR, automated auditory brainstem response.

902 NEONATAL HEARING SCREENING IN INFANTS AT RISK

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minutes for registration compared with a total of 8
minutes for measuring OAE.
DISCUSSION
Criteria for establishing screening programs for
newborns have been published in the past.33 At
present, screening in Europe and North America
include at least blood tests for congenital hypothy-
roidism, phenylketonuria, and galactosemia during
the first postnatal week (incidence in Europe and in
the United States: congenital hypothyroidism ;30
per 100 000; phenylketonuria ;10 per 100 000, galac-
tosemia ;2 per 100 000 live births).34,35 Currently
considerations are ongoing to include other tests into
routine screening procedures. Some individual states
have already added tests for maple syrup disease,
homocystinuria, biotinidase deficiency, adrenal hy-
perplasia, cystic fibrosis, tyrosinemia, hemoglobi-
nopathies, and toxoplasmosis.34 For example, maple
syrup disease currently is diagnosed in 1 per 250 000
to 400 000 newborns.35 The prevalence of neonatal
hearing disorders is in the range of 1 to 2 per 1000
newborns. As it recently has been demonstrated,
language abilities of early-identified children to be
superior to late-identified children with identical
treatment,36 it seems reasonable to also include hear-
ing screening into routine programs. Thus, screening
in a population at risk as performed in the present
study can only be regarded to be the first step toward
a universal screening.
Hearing disorders in the range of 5 to 60 per 1000
were observed in at-risk study populations enrolling
between 117 and 1401 infants.2–9 Most of the studies
used inclusion criteria similar to the guidelines of the
Joint Committee on Infant Hearing. Our observed
incidence of persisting hearing disorder was 18 out
of 770 infants (2.3%) and therefore is consistent with
the concept of a 10-fold increased risk for neonatal
hearing disorders in high-risk groups.
Familial hearing loss, ie, hereditary factors, sepsis
and/or meningitis, and craniofacial malformations
were identified to be independent significant risk
factors for pathologic A-ABR results in the present
study population. Prematurity ,32 weeks and
weight at birth ,1500 g did not significantly increase
the risk for neonatal hearing disorders. This is some-
what contradictory to the findings of previous stud-
ies.6 – 8,18 –22,37,38 However, this finding may be ex-
plained by improved conditions of perinatal care and
overall reduced incidence of complications of prema-
turity.16 Although being major predictors for im-
paired neurodevelopmental outcome, cerebral com-
plications of prematurity (eg, intraventricular
hemorrhage or periventricular leukomalacia) also
were not significantly associated with pathologic A-
ABR testing. Ototoxic medications and hyperbiliru-
binemia at concentrations observed in our study in-
fants have been demonstrated to be risk factors for
hearing loss by several authors in the past.6,18 –20,37
However, we were unable to confirm those findings.
Strict monitoring of aminoglycoside serum concen-
trations and dose adjustment may have contributed
to the avoidance of toxic side effects in study infants.
Furthermore comorbidity of infants with hyperbil-
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irubinemia may have influenced the results of the
aforementioned studies. In addition, PPHN was not
associated with an increased risk for neonatal hear-
ing disorders in the present trial. This finding might
be explained by the fact, that hyperventilation was
performed less aggressively; ie, targeted hypocarbia
was less pronounced during the study period com-
pared with guidelines used in the past.29,39 Our data
are further supported by two recently published tri-
als indicating that PPHN and its treatment are not
risk factors for hearing disorders.40,41
The high number of infants with either familial
hearing disorders, craniofacial malformations, and
syndromal disorders, who per se did not require
intensive care after birth emphasizes the need, to
screen all newborns, not just those treated in neona-
tal intensive care units. Recently published con-
trolled multicenter trials on universal newborn hear-
ing screening do support this view.42,43
Limitations of the conclusion drawn from the mul-
tivariate regression model potentially could result
from the preselection of risk factors being the basis of
enrollment criteria,13 and their uneven distribution
within our study population. The addition of 4 fur-
ther potential risk factors did not contribute signifi-
cant associations.
Our study population was primarily screened us-
ing A-ABR because this method had been shown to
have a sensitivity of #98% and a specificity of
#96%.44 – 47 Although not the primary goal of the
present study, our data indicate a superior sensitivity
and specificity of the A-ABR screening in compari-
son to OAE. The high number of false-positive re-
sults47 and the problem of missing single infants with
severe hearing loss48 further add some concern about
OAE testing in at-risk groups. Psychologic problems
resulting from the high false-positive rate of screen-
ing using OAE have further been discussed.49 At
present, OAE measurements should probably not be
used in hearing screening for high-risk neonates.
CONCLUSION
In summary, our data indicate a change in risk
factors for neonatal hearing disorders. Craniofacial
malformations, familial hearing disorders, and neo-
natal bacterial infections were significant factors as-
sociated with pathologic A-ABR testing. In contrast,
very low birth weight and complications of prema-
turity were not independent risk factors for patho-
logic screening results in our study population. This
change in the risk profile for neonatal hearing im-
pairment in a high-risk population is speculated to
be related to changes in perinatal and neonatal care.
ACKNOWLEDGMENTS
This study was supported by the “Stiftung für das behinderte
Kind,” Frankfurt am Main, Germany.
We thank I. Dickau for careful preparation of the manuscript,
B. Roenspiess for skilled technical assistance, and H.-J. Friedrich,
PhD, for statistical advice.
REFERENCES
1. Mason JA, Herrmann KR. Universal infant hearing screening by auto-
mated auditory brainstem response measurement. Pediatrics. 1998;101:
221–228
ARTICLES 903
 2. Mehl AL, Thomson V. Newborn hearing screening: the great omission.
Pediatrics. 1998;101(1). URL: http://www.pediatrics.org/cgi/ content/
full/101/1/e4
3. Watkin PM. Neonatal otoacoustic emission screening and the identifi-
cation of deafness. Arch Dis Child. 1996;74:F16 –F25
4. Bradford BC, Baudin J, Conway MJ, Hazell JW, Stewart AL, Reynolds
EO. Identification of sensory neural hearing loss in very preterm infants
by brainstem auditory evoked potentials. Arch Dis Child. 1985;60:
105–109
5. Durieux-Smith A, Picton TW, Edwards CG, McMurray B, Goodman JT.
Brainstem electric-response audiometry of infants in a neonatal inten-
sive care unit. Audiology. 1987;26:284 –297
6. Swigonski N, Shallop J, Bull MJ, Lemons JA. Hearing screening of high
risk newborns. Ear Hear. 1987;8:26 –30
7. Watkin PM, Baldwin M, McEnery G. Neonatal at risk screening and the
identification of deafness. Arch Dis Child. 1991;66:1130 –1135
8. McClelland RJ, Watson DR, Lawless V, Houston HG, Adams D. Reli-
ability and effectiveness of screening for hearing loss in high risk
neonates. Br Med J. 1992;304:806 – 809
9. White KR, Vohr BR, Behrens TR. Universal newborn hearing screening
using transient evoked otoacoustic emissions: results of the Rhode
Island Hearing Assessment Project. Semin Hear. 1993;14:18 –29
10. Peck JE. Development of hearing. Part III. Postnatal development. J Am
Acad Audiol. 1995;6:113–123
11. Brookhouser PE. Sensoneurinal hearing loss in children. Pediatr Clin
North Am. 1996;43:1195–1216
12. Markides A. Age at fitting of hearing aids and speech intelligibility. Br J
Audiol. 1986;20:165–167
13. American Academy of Pediatrics. Joint Committee on Infant Hearing
1994 position statement. Pediatrics. 1995;95:152–156
14. Davis H. Brainstem and other responses in electric response audiome-
try. Ann Otol. 1976;85:3–13
15. Kemp DT. Stimulated acoustic emission from the human ear. J Acoust
Soc Am. 1978;85:1386 –1391
16. Hack M, Friedman H, Fanaroff AA. Outcomes of extremely low birth
weight infants. Pediatrics. 1996;98:931–937
17. UK Collaborative ECMO Trial Group. UK collaborative randomised
trial of neonatal extracorporeal membrane oxygenation. Lancet. 1996;
348:75– 82
18. Shannon DA, Felix JK, Krumholz A, Goldstein PJ, Harris KC. Hearing
screening of high-risk newborns with brainstem auditory evoked
potentials: a follow-up study. Pediatrics. 1984;73:22–26
19. Bergmann 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
20. Salamy A, Eldredge L, Tooley WH. Neonatal status and hearing loss in
high-risk infants. J Pediatr. 1989;114:847– 852
21. Das VK. Aetiology of bilateral sensorineural hearing impairment in
children: a 10 year study. Arch Dis Child. 1996;74:8 –12
22. Napiorkowski B, Lester BM, Freier MC, et al. Effects of in utero sub-
stance exposure on infant neurobehavior. Pediatrics. 1996;98:71–75
23. Holst K, Andersen E, Philip J, Henningsen I. Antenatal and perinatal
conditions correlated to handicap among 4-year-old children. Am J
Perinatol. 1989;6:258 –267
24. Sell EJ, Gaines JA, Gluckman C, Williams S. Persistent fetal circulation.
Am J Dis Child. 1985;139:25–28
25. Maisels MJ. Jaundice. In: Avery GB, Fletcher MA, McDonald MG, eds.
Neonatology: Pathophysiology and Management of the Newborn. 4th ed.
Philadelphia, PA: JB Lippincott Company; 1994:687–706
26. Papile LA, Burstein J, Burstein R, Koffler H. Incidence and evolution of
904 NEONATAL HEARING SCREENING IN INFANTS AT RISK
Downloaded from http://pediatrics.aappublications.org/ by guest on December 11, 2017
subependymal and intraventricular hemorrhage: a study of infants with
birth weights less than 1,500 gm. J Pediatr. 1978;92:529 –534
27. Hill A, Melson L, Clark B, Volpe JJ. Hemorrhagic periventricular
leukomalacia: diagnosis by real time ultrasound and correlation with
autopsy findings. Pediatrics. 1982;69:282–284
28. Voigt M, Schneider KT, Jahrig K. Analysis of a 1992 birth sample in
Germany. 1: New percentile values of the body weight of newborn
infants. Geburtshilfe Frauenheilkd. 1996;56:550 –558
29. Fox WW, Duara S. Persistent pulmonary hypertension in the neonate:
diagnosis and management. J Pediatr. 1983;103:505–514
30. Natus Medical, Inc. ALGO-1-Plus Infant Hearing Screener, User Manual.
Foster City, CA: Natus Medical, Inc; 1990
31. Kemp DT, Ryan S, Bray P. A guide to the effective use of otoacoustic
emissions. Ear Hear. 1990;11:93–105
32. SSPS-Version 6.12 for Windows. Chicago, IL: SPSS Inc
33. Frankenburg WK. Selection of diseases and tests in pediatric screening.
Pediatrics. 1974;54:612– 616
34. Committee on Genetics. Newborn screening fact sheets. Pediatrics. 1996;
98:473– 475
35. Hoffmann GF. Selective screening for inborn errors of metabolism—
past, present and future. Eur J Pediatr. 1994;153:S2–S8
36. Yoshinaga-Itano C, Sedey AL, Coulter DK, Mehl AL. Language of early-
and later-identified children with hearing loss. Pediatrics. 1998;102:
1161–1171
37. Davis A, Wood S. The epidemiology of childhood hearing impairment:
factors relevant to planning of services. Br J Audiol. 1992;26:77–90
38. Sutton GJ, Rowe SJ. Risk factors for childhood sensorineural hearing
loss in the Oxford region. Br J Audiol. 1997;31:39 –54
39. Roberts JD Jr, Shaul PW. Advances in the treatment of persistent pul-
monary hypertension of the newborn. Pediatr Clin North Am. 1993;40:
983–1004
40. Marron MJ, Crisafi MA, Driscoll JM, et al. Hearing and neurodevelop-
mental outcome in survivors of persistent pulmonary hypertension of
the newborn. Pediatrics. 1992;90:392–396
41. Fujikawa S, Yang L, Waffarn F, Lerner M. Persistent pulmonary hyper-
tension of the newborn (PPHN) treated with inhaled nitric oxide: pre-
liminary hearing outcomes. J Am Acad Audiol. 1997;4:263–268
42. Finitzo T, Albright K, O’Neal J. The newborn with hearing loss: detec-
tion in the nursery. Pediatrics. 1998;102:1452–1460
43. Wessex Universal Neonatal Hearing Screening Trial Group. Controlled
trial of universal neonatal screening for early identification of perma-
nent childhood hearing impairment. Lancet. 1998;352:1957–1964
44. Jacobson JT, Jacobson CA, Sparh RC. Automated and conventional ABR
screening techniques in high-risk infants. J Am Acad Audiol. 1990;1:
187–195
45. White KR, Vohr BR, Maxon AB, Behrens TR, McPherson MG, Mauk
GW. Screening all newborns for hearing loss using transient evoked
otoacoustic emissions. Int J Pediatr Otorhinolaryngol. 1994;29:203–217
46. Van Straaten HL, Groote ME, Oudesluys-Murphy AM. Evaluation of an
automated auditory brainstem response infant hearing screening
method in at risk neonates. Eur J Pediatr. 1996;155:702–705
47. Doyle KJ, Burggraaff B, Fujikawa S, Kim J. Newborn hearing screening
by otoacoustic emissions and automated auditory brainstem response.
Int J Pediatr Otorhinolaryngol. 1997;41:111–119
48. Psarommatis IM, Tsakanikos MD, Kontorgianni AD, Ntouniadakis DE,
Apostolopoulos NK. Profound hearing loss and presence of click-
evoked otoacoustic emissions in the neonate: a report of two cases. Int
J Pediatr Otorhinolaryngol. 1997;39:237–243
49. Lang DV. Neonatal otoacoustic emission screening (OAE) for deafness:
psychological costs. Arch Dis Child Fetal Neonatal Ed. 1996;75:F143
 Neonatal Screening for Hearing Disorders in Infants at Risk: Incidence, Risk
Factors, and Follow-up
Christiane Meyer, Jan Witte, Agnes Hildmann, Karl-Heinz Hennecke, Karl-Ulrich
Schunck, Kerstin Maul, Ute Franke, Hubert Fahnenstich, Heike Rabe, Rainer Rossi,
Sabine Hartmann and Ludwig Gortner
Pediatrics 1999;104;900
DOI: 10.1542/peds.104.4.900

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Pediatrics is the official journal of the American Academy of Pediatrics. A monthly publication, it
has been published continuously since . Pediatrics is owned, published, and trademarked by the
American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village, Illinois,
60007. Copyright © 1999 by the American Academy of Pediatrics. All rights reserved. Print ISSN:
.

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 Neonatal Screening for Hearing Disorders in Infants at Risk: Incidence, Risk
Factors, and Follow-up
Christiane Meyer, Jan Witte, Agnes Hildmann, Karl-Heinz Hennecke, Karl-Ulrich
Schunck, Kerstin Maul, Ute Franke, Hubert Fahnenstich, Heike Rabe, Rainer Rossi,
Sabine Hartmann and Ludwig Gortner
Pediatrics 1999;104;900
DOI: 10.1542/peds.104.4.900

The online version of this article, along with updated information and services, is
located on the World Wide Web at:
http://pediatrics.aappublications.org/content/104/4/900

Pediatrics is the official journal of the American Academy of Pediatrics. A monthly publication, it
has been published continuously since . Pediatrics is owned, published, and trademarked by the
American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village, Illinois,
60007. Copyright © 1999 by the American Academy of Pediatrics. All rights reserved. Print ISSN:
.

Downloaded from http://pediatrics.aappublications.org/ by guest on December 11, 2017