ORIGINAL CONTRIBUTION

Newborn Hearing Screening vs Later Hearing

Screening and Developmental Outcomes
in Children With Permanent
Childhood Hearing Impairment
Anna M. H. Korver, MD, PhD Context Newborn hearing screening programs have been implemented in many coun-
Saskia Konings, MD tries because it was thought that the earlier permanent childhood hearing impairment is
Friedo W. Dekker, PhD detected, the less developmentally disadvantaged children would become. To date, how-
ever, no strong evidence exists for universal introduction of newborn hearing screening.
Mieke Beers, PhD
Objective To study the effect of newborn hearing screening vs distraction hearing
Capi C. Wever, MD, PhD screening, conducted at 9 months of age, on development, spoken communication,
Johan H. M. Frijns, MD, PhD and quality of life.
Anne M. Oudesluys-Murphy, MB, PhD Design, Setting, and Participants Between 2002 and 2006, all 65 regions in the
Netherlands replaced distraction hearing screening with newborn hearing screening.
for the DECIBEL Collaborative Study Consequently, the type of hearing screening offered was based on availability at the
Group
place and date of birth and was independent of developmental prognoses of indi-

P
ERMANENT CHILDHOOD HEAR- vidual children. All children born in the Netherlands between 2003 and 2005 were
ing impairment is a serious, included. At the age of 3 to 5 years, all children with permanent childhood hearing
relatively common condi- impairment were identified. Evaluation ended December 2009.
tion.1-3 Auditory input is essen- Main Outcome Measures Performance (education and spoken and signed com-
tial for development and social func- munication), development (general and language), and quality of life.
tioning, so early awareness of a child’s Results During the study period, 335 560 children were born in a newborn hearing screen-
hearing ability is important in creat- ing region and 234 826 children in a distraction hearing screening region. At follow-up,
ing opportunities for early amplifica- 263 children in newborn hearing screening regions (0.78 per 1000 children) and 171 chil-
tion and habilitation when necessary. dren in distraction hearing screening regions (0.73 per 1000 children) had been diagnosed
Until some years ago, distraction with permanent childhood hearing impairment. Three hundred one children (69.4%) par-
ticipated in analysis of general performance measures. There was no difference between
hearing screening (behavioral testing)
groups in the primary mode of communication or type of education. Analysis of extensive
was used for hearing screening around developmental outcomes included 80 children born in newborn hearing screening regions
the age of 9 months. Newborn hearing and 70 in distraction hearing screening regions. Multivariate analysis of variance showed
screening (within 2 weeks of birth) was that overall, children in newborn hearing screening regions had higher developmental out-
introduced in many developed coun- come scores compared with children in distraction hearing screening regions (Wilks ␭=0.79;
tries because it was thought that the ear- F12 =2.705; P=.003). For social development, the mean between-group difference in quo-
lier permanent childhood hearing im- tient points was 8.8 (95% CI, 0.8 to 16.7) and for gross motor development, 9.1 (95% CI,
pairment was diagnosed, the less 1.1 to 17.1). For quality of life, the mean between-group difference was 5.3 (95% CI, 1.7
developmentally disadvantaged chil- to 8.9), also in favor of children in newborn hearing screening regions.
dren would become.4,5 Conclusion Compared with distraction hearing screening, a newborn hearing screen-
However, to date no strong evi- ing program was associated with better developmental outcomes at age 3 to 5 years
dence exists for universal implemen- among children with permanent childhood hearing impairment.
JAMA. 2010;304(15):1701-1708 www.jama.com
tation of newborn hearing screening.
Studies on developmental effects of
newborn hearing screening have been ethically infeasible.9,11 As an alterna- Author Affiliations and members of the DECIBEL Col-
descriptive in nature and based on con- tive, we used the regional differences laborative Study Group are listed at the end of this
article.
venience samples.6-12 Conducting a ran- in allocation of hearing screening type Corresponding Author: Anna M. H. Korver, MD, PhD,
domized trial, apart from presenting created by national policy in the Neth- Willem-Alexander Children and Youth Center, De-
partment of Social Pediatrics, Leiden University Medi-
practical difficulties (including ex- erlands. Newborn hearing screening cal Center, Albinusdreef 2, 2333 ZA Leiden, the Neth-
pense and duration), was considered was gradually introduced by region in erlands ([email protected]).

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 HEARING SCREENING AND DEVELOPMENTAL OUTCOMES

all 65 regions beginning in 2002 and unbiased with respect to type or result during a home visit, together with new-
totally replaced distraction hearing of hearing screening and type or de- born blood spot screening, or at a well
screening by June 2006. This policy gree of hearing impairment. baby visit.
meant that the type of hearing screen- Excluded were children who had We verified whether the hearing
ing offered to children was based solely been in neonatal intensive care units, screening the child was offered on the ba-
on location and date of birth. Because because they are not a target popula- sis of the regional hearing screening pro-
hearing screening type was indepen- tion for universal hearing screening; gram at the time of birth corresponded
dent of the prognosis of the individual children who acquired their hearing im- with the hearing screening the child had
child, the region of birth can be con- pairment after birth; and those who actually received, as reported by par-
sidered an instrumental variable.13-16 were severely cognitively and physi- ents and noted in the audiology rec-
Using the regional differences in hear- cally disabled. Children already par- ords. Parents were invited to partici-
ing screening, we studied the develop- ticipating in other research projects at pate in the study by mail. Medical records
mental effects of newborn hearing 1 center or from a center that joined the of all children identified were reviewed
screening compared with distraction study at a late stage and children whose for available information on character-
hearing screening in 3- to 5-year-old parents were not competent in the istics and performance measures. Char-
children with permanent childhood Dutch language were also excluded acteristics included maternal education
hearing impairment. We hypothesized from participation. level (representing socioeconomic sta-
that newborn hearing screening would tus), parental hearing status, type and re-
be associated with better general devel- Hearing Screening Programs sult of hearing screening, age at start of
opment and improved spoken commu- and Study Design amplification (hearing aid, bone-
nication and quality of life compared The place and date of birth of the child anchored hearing aid, or cochlear im-
with distraction hearing screening. determined the type of hearing screen- plant), degree of hearing impairment,
ing offered: distraction hearing screen- and etiology (if available).
METHODS ing or newborn hearing screening After parents provided written in-
Study Population (eFigure, available at http://www.jama formed consent, they were sent (by mail
The DECIBEL (Developmental Evalu- .com). The 2 programs differed in the or e-mail) 3 standardized instruments
ation of Children: Impacts and Ben- age at screening and the method used; measuring developmental outcome and
efits of Early hearing screening, Leiden) both followed uniform protocols. Dis- a questionnaire to complete the char-
study included children born in the traction hearing screening, offered at the acteristics and performance measures.
Netherlands between January 1, 2003, age of 9 months, is a 3-stage hearing Parents were asked for their country of
and December 31, 2005. With the as- screening using sounds to provoke a be- birth and the race/ethnicity of their
sistance of 2 researchers (A.M.H.K. and havioral reaction. If a child does not re- child to determine any cultural differ-
S.K.), professionals at every audiology act as expected at the first screening, a ences between the groups. Evaluation
center in the Netherlands (n=22) iden- repeat screening is planned, and this is ended December 2009.
tified all children born in 2003 through repeated once more if necessary. A This study was approved by the
2005 with permanent hearing impair- third-stage result positive for hearing medical ethics committee of the Leiden
ment at age 3 to 5 years. The audiol- impairment is followed by referral to an University Medical Center. The pri-
ogy center is the designated and only audiology center for diagnostic inves- vacy committee of the neonatal inten-
organization for diagnostic evaluation tigation and confirmation of hearing im- sive care unit hearing screening pro-
and amplification for children with per- pairment. The distraction hearing gram gave permission for anonymous
manent hearing impairment. screening test is subjective and has been verification of its patients.
Permanent childhood hearing im- shown to be unreliable in children with
pairment was defined as bilateral per- cognitive and physical handicaps.17 Assessment of Development
manent conductive or sensorineural Newborn hearing screening for well Performance measures included the pri-
hearing loss of 40 dB or greater in the infants, offered before the age of 2 weeks, mary mode of communication (oral lan-
better ear and was classified on the ba- is also a 3-stage screening program, but guage only or oral and sign language)
sis of the most recent hearing test (mea- it uses transient evoked oto-acoustic and the type of education (regular edu-
sured unaided and computed using 500, emissions for the first 2 stages and au- cation, education for hearing im-
1000, and 2000 Hz). Hearing loss was tomated auditory brainstem responses paired children, or education for chil-
categorized as moderate (40-60 dB), se- in the third stage. A unilateral or bilat- dren with developmental disabilities)
vere (61-90 dB), or profound (⬎90 dB). eral positive result for hearing impair- as reported by parents or audiology rec-
Because the identification of children ment is followed by repeat screening, ords. General and language develop-
at audiology centers was performed and a positive result in the third stage mental outcomes were measured using
cross-sectionally, independent of is followed by referral to an audiology the Child Development Inventory, ex-
screening type, the identification was center. Screening is performed either pressive language development using
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 HEARING SCREENING AND DEVELOPMENTAL OUTCOMES

the MacArthur Communicative Devel- cabulary inventory were currently spo- was used for continuous variables and
opment Inventory, and quality of life ken, signed, or both. In children not ␹2 test for categorical variables.
using the Pediatric Quality of Life In- speaking in sentences, sentence com- Because of multiple testing with cor-
ventory 4.0, all completed by parents. plexity and mean length of longest ut- related subscales, a multivariate analy-
The Child Development Inventory is terance were not applicable and were sis of variance (MANOVA) was used to
a standardized instrument designed to classified as missing. Crude scores were determine the overall difference in out-
assess the development of children from used in the analysis, eliminating any ceil- come between groups. If the MANOVA
age 15 months to 6 years and is often ing or floor effects caused by including was significant, linear regression was
used in research on this topic.18 The children who were chronologically or used to compare developmental out-
1992 version of the Child Develop- developmentally older than the popu- come per subscale. The assumptions for
ment Inventory was translated into lation for whom the scale was origi- this type of analysis were tested and met
Dutch according to rules formulated by nally designed. (independence, normality, and homo-
Guillemin et al 19 and was also ad- The Pediatric Quality of Life Inven- geneity of variance of the residuals). Ad-
justed for use in children whose pri- tory21,22 questionnaire encompasses justment for residual confounding was
mary language is sign language. Par- both physical functioning and psycho- done for maternal education and
ents completed the questionnaire by social functioning. Each item is scored chronological age at developmental
indicating which of the listed 270 be- on a 5-point Likert scale. To create scale evaluation (when applicable). The dif-
havioral items they observed in their scores, the mean crude score was com- ference in chronological age at devel-
child. The items are grouped to form puted as the sum of the items divided opmental evaluation between the 2
scales, including social development, by the number of items answered groups was considered a consequence
motor development, and expressive lan- (which corrects for missing items). The of the gradual introduction of new-
guage and language comprehension total quality-of-life score is the sum of born hearing screening, with more chil-
(combined in a total language scale). the mean crude score on all scales. dren in newborn hearing screening
The general development score is a Higher scores indicate better quality of being younger.
summary score that provides an over- life (maximum score, 100). Two age- It is known that hearing impair-
all index of development by including specific versions were used: for chil- ment in children with congenital cyto-
10 of the most age-discriminating items dren aged 2 to 4 years and those aged megalovirus infection may be progres-
from each of the scales. The scores were 5 to 7 years, both with comparable con- sive over time and not yet detectable by
recalculated by the use of the original structs and scoring. newborn hearing screening. The pres-
norm data into developmental ages, and ence of this infection can be con-
these generated developmental quo- Statistical Analysis firmed prior to hearing screening when
tients when divided by chronological First, the proportion of children with appropriate methods are used.23 For
age and multiplied by 100. Higher permanent childhood hearing impair- these reasons, a sensitivity analysis was
scores indicate better development. A ment in both hearing screening pro- performed excluding the children with
developmental quotient of 80 or more grams was compared, along with base- known congenital cytomegalovirus
represents normal development. A quo- line characteristics. A main analysis infection.
tient between 70 and 80 is regarded as comparing performance measures and An analysis based on the type of hear-
borderline development. A difference developmental outcome was per- ing screening children actually re-
of 4 points is generally interpreted as formed according to the type of hear- ceived was also performed. Differences
clinically relevant.18 ing screening children were offered (de- between this sensitivity analysis and the
The short-form version of the termined by location and date of birth). main analysis are reported. The signifi-
MacArthur Communicative Develop- We also investigated whether chil- cance level was set at P⬍.05 and 2-sided
ment Inventory (hereafter referred to as dren who agreed to participate in ex- testing performed. All statistical tests
MacArthur) was used to assess 3 as- tensive developmental outcome mea- were carried out using SPSS version 17.0
pects of expressive language. Active vo- sures were comparable with those not (SPSS Inc, Chicago, Illinois).
cabulary (number of words spoken and participating. For those participating in
signed; maximum score, 100), sen- the extensive outcome study, vari- RESULTS
tence complexity (ranging from 1 for ables believed to affect the outcome in In 2003-2005, the number of children
least complex to 3 for most complex for children with permanent childhood born in the Netherlands was 582 214.
9 sentences; maximum score, 27), and hearing impairment (maternal educa- Of these, 11 828 children (2%) were ad-
mean length of 3 longest utterances tion, degree of hearing impairment, mitted to a neonatal intensive care unit
(number of words) were evaluated.20 parental hearing status, mode of com- and therefore excluded. Of all other live
With regard to active vocabulary, par- munication, and age at start of ampli- newborns, 335 560 children were born
ents were asked to indicate which words fication) were compared between in a region where newborn hearing
of the child’s originally only spoken vo- groups. An independent-samples t test screening was offered and 234 826 in
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 HEARING SCREENING AND DEVELOPMENTAL OUTCOMES

a region where distraction hearing ing region (0.73 per 1000 children) (dif- abled, 52 were participating in other
screening was offered. At follow-up, 263 ference, 0.05 per 1000; 95% confi- research projects, and 11 had parents
children in a newborn hearing screen- dence interval [CI], −0.12 to 0.09) who were not competent in the Dutch
ing region had been diagnosed with per- (TABLE 1). Of these 434 children, 133 language (FIGURE and Table 1). The re-
manent childhood hearing impair- children were excluded: 17 had an ac- maining 301 children (69.4%) were in-
ment (0.78 per 1000 children) and 171 quired hearing impairment, 53 were se- cluded in the study on general perfor-
children in a distraction hearing screen- verely cognitively and physically dis- mance measures. Of these, 150 children
(49.8%) agreed to participate in exten-
sive investigations on developmental
Table 1. Number of Children Allocated to Distraction or Newborn Hearing Screening outcome (Figure). The main reason for
Programs and the Prevalence of Permanent Childhood Hearing Impairment refusal to participate was an already ex-
Birth Year tensive schedule of medical evalua-
2003 2004 2005 Overall tions.
Distraction hearing screening In the analysis of general perfor-
Children screened, No. 152 900 76 200 5526 234 626 mance measures, the 2 groups (new-
Children identified with permanent hearing 118 48 5 171 born hearing screening, n = 183; dis-
impairment, No.
traction hearing screening, n = 118)
Prevalence of hearing impairment per 1000 0.77 0.63 0.90 0.73
children screened a
were comparable in degree of hearing
Newborn hearing screening impairment, primary mode of commu-
Children screened, No. 42 166 114 374 179 020 335 560 nication, and type of education
Children identified with permanent hearing 32 94 137 263 (TABLE 2). Children included in analy-
impairment, No. sis of extensive developmental out-
Prevalence of hearing impairment per 1000 0.76 0.82 0.77 0.78
children screened a
comes (n=150) were comparable with
a Calculated by dividing the number of children identified with permanent hearing impairment by the number of children those not participating (n=151) in de-
screened. gree of hearing impairment, sex, and
type of amplification (eTable 1). In the
Figure. DECIBEL Study Flow Diagram analysis of developmental outcome
measures, the 2 groups were compa-
582 214 Children born in the Netherlands rable in all baseline characteristics
in 2003-2005
(newborn hearing screening, n=80; dis-
11 828 Excluded (admitted to neonatal
traction hearing screening, n = 70).
intensive care) Compared with children in distrac-
tion hearing screening, children in new-
570 386 Potentially eligible
born hearing screening were screened
at a younger age, their hearing ampli-
fied 13 months earlier, and their de-
335 560 Born in regions with newborn 234 826 Born in regions with distraction
hearing screening hearing screening velopment evaluated 13 months ear-
lier (47.9 mo vs 60.7 mo) (TABLE 3).
335 297 Excluded (no permanent
childhood hearing
234 655 Excluded (no permanent
childhood hearing
Multivariate analysis of variance
impairment) impairment) (both crude and adjusted for maternal
education) showed that children in
263 Had permanent childhood
hearing impairment at age
171 Had permanent childhood
hearing impairment at age
newborn hearing screening regions had
3-5 years 3-5 years higher developmental outcome scores
overall compared with children in dis-
80 Excluded 53 Excluded traction hearing screening regions
31 Other research projects 21 Other research projects
10 Postnatally acquired 7 Postnatally acquired (Wilks ␭=0.79; F12 =2.705; P=.003). On
hearing loss hearing loss
33 Cognitively and physically 20 Cognitively and physically
the Child Development Inventory, af-
disabled disabled ter adjustment for maternal educa-
6 Neither parent competent 5 Neither parent competent
in Dutch in Dutch tion, we found a statistically signifi-
cant difference in social development
183 Included in analysis of general 118 Included in analysis of general and gross motor development and a
performance measures performance measures
nonsignificant difference in the same di-
80 Available and included in analysis 70 Available and included in analysis rection on all other subscales (TABLE 4).
of extensive developmental of extensive developmental
outcome measures outcome measures Additional adjustment for the degree of
hearing impairment, race/ethnicity, and
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 HEARING SCREENING AND DEVELOPMENTAL OUTCOMES

parental hearing status did not result

Table 2. General Performance Measures in Children With Permanent Childhood Hearing
in materially different results. Impairment at Age 3 to 5 Years a
Using the MacArthur, children in the No. (%)
newborn hearing screening group were
found to use statistically significantly Newborn Distraction
Hearing Hearing
fewer signed words compared with chil- Screening Screening P
dren in the distraction hearing screen- Characteristic (n = 183) (n = 118) Value
ing group (mean difference, −11.2; 95% Degree of hearing loss (n = 179) (n = 118)
CI, −20.6 to −1.9) when adjustment was Moderate (40-60 dB) 88 (49.2) 58 (49.2)
made for maternal education and age at Severe (61-90 dB) 49 (27.4) 30 (25.4) .90
developmental evaluation. In children in Profound (⬎90 dB) 42 (23.5) 30 (21.7)
the newborn hearing screening region, Primary mode of communication (n = 97) (n = 80)
Oral language only 28 (28.9) 26 (32.5)
a larger (but not statistically signifi- .60
Oral and sign language 69 (71.1) 54 (67.5)
cantly different) spoken vocabulary was
Education type in children aged ⬎48 mo (n = 71) (n = 89)
found (mean difference, 8.7; 95% CI,
Regular education 18 (25.4) 23 (25.8)
−3.9 to 21.2). The number of spoken
Regular education with counseling for hearing impaired 1 (1.4) 5 (5.6)
words was inversely associated with the .13
Education for children with hearing impairment 49 (69.0) 61 (68.5)
number of signed words, resulting in op-
Education for children with developmental disabilities 3 (4.2) 0
posite results, in favor of children in new- a Categorical variables were compared between groups with ␹2 test.
born hearing screening. The level of sen-
tence complexity and the mean length
of longest spoken utterance were com- Table 3. Characteristics at Birth and at Extensive Developmental Evaluation: Main Analysis of
parable in both groups after adjust- Children With Permanent Childhood Hearing Impairment
ment. Distraction
Newborn Hearing Hearing
Quality of life was statistically sig- Screening Screening P
nificantly higher in children in the new- Characteristic (n = 80) (n = 70) Value
born hearing screening group on all At baseline
Male sex, No. (%) 47 (58.8) 40 (57.1) .84
scales, except for the mean crude score
White, No. (%) 65 (81.3) 61 (87.4) .23
on the emotion scale. Adjustment for
Mother with higher education, No. (%) a 35 (44.3) 33 (49.3) .82
maternal education did not influence
Both parents normal hearing, No. (%) 66 (83.5) 60 (87.0) .74
the results (Table 4).
Referral at hearing screening, No. (%) 65 (81.3) 41 (58.6) b .15
In a sensitivity analysis, 10 children
Degree of hearing loss at first evaluation, No. (%)
with congenital cytomegalovirus infec- Lower than moderate (⬍40 dB) 9 (11.3) 7 (10.0)
tion were excluded (5 in each group). Moderate (40-60 dB) 44 (55.0) 32 (45.7)
.57
In addition to social development, gross Severe (61-90 dB) 13 (16.3) 17 (24.3)
motor development, and quality of life, Profound (⬎90 dB) 9 (11.3) 14 (20.0)
the mean differences on language de- At age 3-5 years
velopmental outcome measures (ex- Age at amplification, mean (SD), mo 15.7 (14.0) 29.2 (14.8) ⬍.001
pressive language, total language, and Type of amplification, No. (%)
Hearing aid 57 (71.3) 53 (79.7)
spoken vocabulary) increased, al-
Bone-anchored hearing aid 5 (6.3) 2 (2.9)
though they remained not statistically Cochlear implant 18 (22.5) 14 (20.0)
.52
significant, in favor of children who had Hearing aid previously but not currently 0 1 (1.4)
newborn hearing screening (eTable 2). Mode of communication, No. (%)
Verification of the type of hearing Oral language only 27 (35.1) 28 (41.2)
.42
screening showed that 12 children un- Oral and sign language 53 (66.3) 42 (60.0)
derwent no hearing screening, 9 chil- Additional handicaps, No. (%) 5 (6.3) 8 (12.9) .17
dren underwent direct diagnostic evalu- Age at development evaluation, mean (SD), mo c 47.9 (9.9) 60.7 (6.8) ⬍.001
ation, 10 children were screened by Age at language and QOL evaluation, 45.9 (9.6) 59.1 (7.2) ⬍.001
newborn hearing screening but were in mean (SD), mo d
Abbreviation: QOL, quality of life.
the main analysis in distraction hearing a Mother’s education level is used to represent socioeconomic status. “Higher” education indicates that the mother
screening, and 1 child was screened by either completed 6 years of secondary education, completed university, or completed another higher education institu-
tion.
distraction hearing screening instead of b In the distraction hearing screening group, no result was available for 12 children because they had not been
screened.
newborn hearing screening. This re- c Refers to chronological age of child when evaluated with Child Development Inventory.
d Refers to chronological age of child when evaluated with Pediatric Quality of Life Inventory and MacArthur Communi-
sulted in 129 children who underwent
cative Development Inventory.
1 of the 2 types of hearing screening (sen-
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 HEARING SCREENING AND DEVELOPMENTAL OUTCOMES

sitivity analysis based on screening ac- This study was not a randomized trial. cern because it is unknown whether
tually received: 85 children in newborn Due to national policy, all regions re- parents of children with abnormal de-
hearing screening and 45 in distraction placed distraction hearing screening by velopment were more eager to partici-
hearing screening). The results in the newborn hearing screening some time pate. However, it is not likely that this
sensitivity analysis were largely compa- during the period of study, which meant bias affected children unequally in the
rable with the results found in the main that allocation of newborns to one or the 2 hearing screening groups. Second,
analysis (eTable 3). other type of hearing screening was based possible identification bias is impor-
solely on location and date of birth and tant to address. Because allotment to 1
COMMENT not on prognosis of developmental out- of the 2 hearing screening regions was
We found that newborn hearing come. Therefore, we were able to study established earlier (when the child was
screening, compared with distraction the effect of regional differences in hear- born), and was independent of the
hearing screening, was associated with ing screening type on developmental out- prognoses of hearing and develop-
statistically significantly fewer words come, rather than the effect of hearing ment for individual children, we be-
signed and better overall, social, and screening offered to the individual child, lieve no bias was introduced during
gross motor development and quality which might be based on a specific rea- identification procedures at the audi-
of life at 3 to 5 years of age among son.16 ology centers. It is not impossible that
children with permanent hearing Limitations of the study should be after closure of data collection, very
impairment. considered. First, response bias is a con- late–onset hearing loss was missed, es-
pecially in children born in later years
Table 4. Developmental Outcome in Newborn Hearing Screening and Distraction Hearing
of the study. However, the proportion
Screening: Main Analysis of Children With Permanent Childhood Hearing Impairment of children with permanent hearing im-
Mean (SD) pairment of congenital cause who were
unidentified and presented later than
Newborn Distraction
No. of Hearing Hearing Adjusted school age is most likely small. The ex-
Assessed Screening Screening Between-Group tensive network of youth health care or-
Cases (n = 80) a (n = 70) a Difference (95% CI) b ganizations monitoring development
Child Development Inventory quotient c (n = 70) (n = 60)
probably would have detected and re-
General development 130 81.4 (17.2) 79.3 (16.3) 3.0 (−2.5 to 8.6)
ferred such children. There is also no
Self help 130 87.1 (24.3) 81.7 (20.7) 5.9 (−1.8 to 13.6)
reason to believe that an identification
Fine motor development 130 89.2 (20.0) 85.4 (15.9) 4.6 (−1.3 to 10.4)
bias affected the 2 hearing screening
Gross motor development 130 86.1 (24.3) 77.6 (21.0) 9.1 (1.1 to 17.1) d
groups unequally.
Social development 130 79.9 (25.1) 71.5 (22.0) 8.8 (0.8 to 16.7) d
Third, the informant perspective (pa-
Expressive language 130 82.4 (25.7) 76.0 (25.0) 7.2 (−1.3 to 15.8)
rental reporting) could have caused in-
Language comprehension 130 75.4 (19.9) 72.7 (19.2) 3.6 (−2.8 to 10.1)
formation bias. Again, it is not likely that
Total language 130 78.9 (21.9) 74.4 (20.6) 5.4 (−1.6 to 12.5)
such bias affected children unequally in
MacArthur crude score e
Total words spoken 136 54.0 (34.3) 66.0 (32.5) 8.7 (−3.9 to 21.2) the 2 hearing screening groups. Fourth,
Total words signed 136 11.8 (17.7) 18.9 (25.1) −11.2 (−20.6 to −1.9) d it is important to acknowledge the dif-
Spoken sentence structure 126 16.5 (7.9) 20.5 (6.9) 0.6 (−2.4 to 3.7) ference in age at developmental evalua-
Mean length of longest utterance 115 5.4 (2.4) 6.7 (3.1) 0.3 (−1.5 to 0.9) tion between the 2 screening groups. Be-
Pediatric Quality of Life Inventory score f (n = 77) (n = 66) cause age-referenced norm data and age-
Total 143 85.8 (8.9) 80.5 (12.0) 5.3 (1.7 to 8.9) d specific questionnaire constructs were
Physical 143 91.5 (10.7) 86.2 (17.4) 5.4 (0.6 to 10.2) d used and (when applicable) adjust-
Emotional 143 76.2 (15.3) 72.2 (14.8) 3.6 (−1.5 to 8.7) ment for age was made in the analysis,
Social 143 85.6 (15.1) 77.7 (15.4) 8.1 (2.9 to 13.3) d it is not likely that bias caused by differ-
Psychosocial 143 82.2 (11.4) 77.1 (11.4) 5.2 (1.3 to 9.0) d ences in age at developmental assess-
Abbreviations: CI, confidence interval; DHS, distraction hearing screening; NHS, newborn hearing screening.
a Multivariate analysis of variance showed that children in NHS regions had higher scores overall on developmental out-
ment influenced the results. Selection
comes compared with children in DHS regions (Wilks ␭=0.79; F12 =2.705; P=.003). bias, possibly introduced by 1 center that
b Differences in Child Development Inventory and Pediatric Quality of Life Inventory scores were adjusted for maternal edu-
cation; differences in MacArthur score were adjusted for maternal education and age at developmental evaluation.
excluded some children already partici-
c A developmental quotient ⱖ80 represents normal development. A quotient between 70 and 80 is regarded as borderline pating in research, was ruled out in a sub-
development.
d P⬍.05. set analysis excluding this center. The re-
e The category “words signed” was added to the original MacArthur Communicative Development Inventory with permis-
sion from the Dutch authors. For active vocabulary (number of words spoken and signed), the maximum score was 100;
sults were unchanged. Fifth, as a result
for spoken sentence structure (ranging from 1 for least complex to 3 for most complex for 9 sentences), the maximum of the relatively small sample size, we
score was 27; and the mean length of 3 longest utterances was measured in number of words. The numbers of children
assessed for each MacArthur subscore were as follows: total words spoken and total words signed, 74 NHS, 62 DHS; were only able to detect relatively large
spoken sentence structure, 68 NHS, 58 DHS; and mean length of longest utterance, 62 NHS, 53 DHS.
f Higher scores indicate better quality of life (maximum, 100). differences between the 2 groups. Nev-
ertheless, we did find differences be-
1706 JAMA, October 20, 2010—Vol 304, No. 15 (Reprinted) ©2010 American Medical Association. All rights reserved.

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 HEARING SCREENING AND DEVELOPMENTAL OUTCOMES

tween the 2 hearing screening groups in sure reflecting both positive and po- study was performed nationwide,
many outcome domains that are consid- tentially negative effects of screening among all children born in the Neth-
ered clinically relevant and important. A programs. It is not clear how motor de- erlands in 3 subsequent years, we be-
number of differences in developmen- velopment is affected by hearing screen- lieve our results can be generalized to
tal outcomes did not reach statistical sig- ing programs. other countries with universal hear-
nificance but did meet the generally ac- In a previous study, we found that ing screening programs, but the feasi-
cepted level for clinical significance. A the presence of congenital cytomega- bility and effectiveness of newborn
larger sample would be necessary to ex- lovirus infection influences develop- hearing screening programs in other
amine more subtle differences. mental outcome.23 Special attention countries remain to be studied.
Our results confirm those of previ- should be paid to the habilitation of
ous studies, most of which were per- these children. Further insight into the CONCLUSION
formed in convenience samples, that re- developmental consequences of the We found that a newborn hearing
port that newborn hearing screening various causes of hearing impairment screening program, compared with dis-
leads to advantages in language devel- will only be achieved if future studies traction hearing screening, was associ-
opmental outcome for children with per- take etiology into account. ated with better developmental out-
manent hearing impairment, when com- It is unlikely that large developmen- comes at age 3 to 5 years among children
pared with children with no screening tal differences could occur simply by with permanent childhood hearing im-
or only targeted screening of high-risk identifying hearing impairment early. pairment.
infants.7,9,24-27 Our results on the Pedi- Improved outcomes are to be expected
Author Affiliations: Willem-Alexander Children and
atric Quality of Life Inventory replicate only when early identification is fol- Youth Center, Department of Social Pediatrics (Drs Kor-
earlier findings by Moeller24 that chil- lowed by early intervention.9 The Joint ver and Oudesluys-Murphy), Department of Oto-
rhinolaryngology (Drs Konings, Beers, Wever, and
dren identified with permanent hear- Committee on Infant Hearing recom- Frijns), and Department of Clinical Epidemiology (Dr
ing impairment later are at risk in areas mends that intervention (amplifica- Dekker), Leiden University Medical Center, Leiden, the
Netherlands.
such as behavior, emotion, and quality tion, family support and communica- Author Contributions: Dr Korver had full access to all
of life. Moreover, in our study, better tion, language and auditory development of the data in the study and takes responsibility for
outcomes following early hearing screen- support) following positive results for the integrity of the data and the accuracy of the data
analysis.
ing were demonstrated in the strongest hearing impairment and confirmation of Study concept and design: Korver, Dekker, Wever,
design possible to date, with an instru- permanent childhood hearing impair- Frijns, Oudesluys-Murphy.
Acquisition of data: Korver, Konings.
mental variable facilitating the study.16 ment should start no later than age 6 Analysis and interpretation of data: Korver, Dekker,
We found statistically significant dif- months.4 In our study, however, this rec- Beers, Wever, Frijns, Oudesluys-Murphy.
Drafting of the manuscript: Korver, Dekker, Oudesluys-
ferences in overall development, total ommendation was not always achieved. Murphy.
words signed, social development, gross This was due (at least in part) to the fact Critical revision of the manuscript for important in-
motor development, and quality of life. that newborn hearing screening was still tellectual content: Korver, Konings, Dekker, Beers,
Wever, Frijns, Oudesluys-Murphy.
Children in newborn hearing screen- in its implementation phase. If any- Statistical analysis: Korver, Dekker.
ing regions used statistically signifi- thing, the delay between identification Obtained funding: Frijns, Oudesluys-Murphy.
Administrative, technical, or material support: Konings,
cantly fewer signed words than chil- and amplification might have resulted Wever.
dren in distraction hearing screening in a reduction of the developmental dif- Study supervision: Dekker, Wever, Frijns, Oudesluys-
Murphy.
regions. Although not statistically sig- ferences between newborn and distrac- Financial Disclosures: None reported.
nificant, the 8.7-point difference in spo- tion hearing screening groups in this Funding/Support: This study received funding from
the Willem-Alexander Children’s Fund, the Wieger
ken words could be considered clini- study. Wakinoerfund, and the Heinsius Houbolt Fund.
cally significant. In the hearing world, Finally, it is important to realize that Role of the Sponsor: The funding organizations had
a larger spoken vocabulary can assist despite early hearing screening, the de- no role in the design and conduct of the study; in the
collection, analysis, and interpretation of the data; or
effective communication strategies. It velopment of children with perma- in the preparation, review, or approval of the manu-
may well be that more effective com- nent childhood hearing impairment at script.
The DECIBEL Collaborative Study Group: Jutte de
munication strategies caused by bet- age 3 to 5 years following newborn Vries, MD, and Ann Vossen, MD, PhD (Laboratory of
ter language development increased hearing screening is still not compa- Medical Microbiology, Leiden University Medical Cen-
ter [LUMC]); Sarina Kant, MD, PhD (Department of
social development and thereby qual- rable with that of normally develop- Clinical Genetics, LUMC); Elske van den Akker-van
ity of life in children who received ing children with normal hearing. Their Marle, PhD (Department of Health Care Economics,
newborn hearing screening. Quality- mean language comprehension is LUMC); Saskia Le Cessie, PhD (Department of Clini-
cal Epidemiology, LUMC); Carolien Rieffe, PhD (Fac-
of-life outcomes reflect parental aware- within the borderline range. ulty of Social Sciences, University of Leiden); Martina
ness, the effect of possible false-negative The results of the DECIBEL study Ens-Dokkum, MD, PhD (Royal Dutch Kentalis); Irma
van Straaten, MD, PhD (Isala Clinics Zwolle); Noelle
screening results, and the effect of per- add evidence to the presumed impor- Uilenburg, MSc (Dutch Foundation for the Deaf and
manent childhood hearing impair- tance and effectiveness of the imple- Hard of Hearing Child); Bert Elvers; Gerard Loeber, PhD;
and Anneke Meuwese-Jongejeugd, MD, PhD (Na-
ment on daily life. Quality of life is mentation of universal newborn hear- tional Institute for Public Health and Environment). Par-
therefore an important outcome mea- ing screening programs. Because this ticipating Audiology Centers: Marcel Maré, MSc

©2010 American Medical Association. All rights reserved. (Reprinted) JAMA, October 20, 2010—Vol 304, No. 15 1707

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 HEARING SCREENING AND DEVELOPMENTAL OUTCOMES

(Academic Medical Centre); Bert van Zanten, PhD tee on Infant Hearing. Year 2007 position statement: 17. Oudesluys-Murphy AM, van Straaten HL,
(Wilhelmina Children’s Hospital, University Medical principles and guidelines for early hearing detection Bholasingh R, van Zanten GA. Neonatal hearing
Center Utrecht); André Goedegebure, PhD (Sophia and intervention programs. Pediatrics. 2007;120 screening. Eur J Pediatr. 1996;155(6):429-435.
Children’s Hospital, Erasmus Medical Center); Fran- (4):898-921. 18. Ireton H, Glascoe FP. Assessing children’s devel-
cien Coster, PhD, and Pim van Dijk, PhD (University 5. Kennedy C, McCann D, Campbell MJ, Kimm L, opment using parents’ reports: the Child Develop-
Medical Center Groningen); Theo Goverts, PhD (VU Thornton R. Universal newborn screening for perma- ment Inventory. Clin Pediatr (Phila). 1995;34(5):
University Medical Center); Ronald Admiraal, MD, PhD; nent childhood hearing impairment. Lancet. 2005; 248-255.
Cor Cremers, MD, PhD; and Dirk Kunst, MD, PhD 366(9486):660-662. 19. Guillemin F, Bombardier C, Beaton D. Cross-
(Radboud University Nijmegen Medical Centre); 6. Sininger YS, Grimes A, Christensen E. Auditory de- cultural adaptation of health-related quality of life
Marina de Leeuw (Royal Auris Group); Janette velopment in early amplified children. Ear Hear. 2010; measures. J Clin Epidemiol. 1993;46(12):1417-
Dijkhuizen, PhD; Marleen Scharloo, MSc; and Dirk 31(2):166-185. 1432.
Hoeben (Royal Dutch Kentalis); Gerti Rijpma, MSc; 7. Kennedy CR, McCann DC, Campbell MJ, et al. Lan- 20. Fenson L, Marchman VA, Thal D, Dale PS, Bates
Wim Graef, MSc; Dik Linschoten, MSc; Jessica Kuijper, guage ability after early detection of permanent child- E, Reznick JS. The MacArthur-Bates Communicative
MSc; Nanda Hof, MSc; and Reinoud Koldewijn, MSc hood hearing impairment. N Engl J Med. 2006; Development Inventories: User’s Guide and Techni-
(Pento Audiology Centers); Donné Pans, MSc (Hoens- 354(20):2131-2141. cal Manual. 2nd ed. Baltimore, MD: Paul H. Brookes;
broeck Audiology Center); Frank Jorritsma, MSc, and 8. Nelson HD, Bougatsos C, Nygren P; 2001 US Pre- 2007.
Maarten van Beurden, MSc (Audiology Center Til- ventive Services Task Force. Universal newborn 21. Varni JW, Seid M, Kurtin PS. PedsQL 4.0: reliabil-
burg and Breda); Christien ter Huurne, MSc (Audiol- hearing screening. Pediatrics. 2008;122(1):e266- ity and validity of the Pediatric Quality of Life Inven-
ogy Center Eindhoven); Patrick Brienesse, PhD (Au- e276. tory version 4.0 generic core scales in healthy and pa-
diology Center Holland Noord). Lisanne Seekles, Jantine 9. Yoshinaga-Itano C, Sedey AL, Coulter DK, Mehl tient populations. Med Care. 2001;39(8):800-812.
de Jong, Andrea Thijssen, Andrea Lievense, Marina AL. Language of early- and later-identified children 22. Engelen V, Haentjens MM, Detmar SB, Koopman
van Egdom–van der Wind, Stephanie Theunissen, with hearing loss. Pediatrics. 1998;102(5):1161- HM, Grootenhuis MA. Health related quality of life
Sophie Mooij, and Charlotte Schouten (medical stu- 1171. of Dutch children. BMC Pediatr. 2009;9:68.
dents, LUMC). 10. Calderon C, Naidu S. Further support of the ben- 23. Korver AM, de Vries JJ, Konings S, et al; DECIBEL
Online-Only Material: The eFigure and eTables 1 efits of early identification and intervention with chil- collaborative study group. DECIBEL study: congeni-
through 3 are available at http://www.jama.com. dren with hearing loss. Volta Rev Monogr. 2000; tal cytomegalovirus infection in young children with
Additional Contributions: We thank all children and 100(5):53-84. permanent bilateral hearing impairment in the
their parents for participating in the DECIBEL study. 11. Thompson DC, McPhillips H, Davis RL, Lieu TL, Netherlands. J Clin Virol. 2009;46(suppl 4):S27-
The quality-of-life measure used in this study was the Homer CJ, Helfand M. Universal newborn hearing S31.
Pediatric Quality of Life Inventory developed by James screening. JAMA. 2001;286(16):2000-2010. 24. Moeller MP. Current state of knowledge: psy-
W. Varni, PhD. 12. Moeller MP, Tomblin JB, Yoshinaga-Itano C, chosocial development in children with hearing
Connor CM, Jerger S. Current state of knowledge: lan- impairment. Ear Hear. 2007;28(6):729-739.
guage and literacy of children with hearing impairment. 25. Wake M, Poulakis Z, Hughes EK, Carey-Sargeant
REFERENCES Ear Hear. 2007;28(6):740-753. C, Rickards FW. Hearing impairment: a population
13. Martens EP, Pestman WR, de Boer A, Belitser SV, study of age at diagnosis, severity, and language out-
1. Watkin PM, Baldwin M. Confirmation of deafness Klungel OH. Instrumental variables: application and comes at 7-8 years. Arch Dis Child. 2005;90(3):
in infancy. Arch Dis Child. 1999;81(5):380-389. limitations. Epidemiology. 2006;17(3):260-267. 238-244.
2. Fortnum HM, Summerfield AQ, Marshall DH, Davis 14. Hertogh EM, Schuit AJ, Peeters PH, Monninkhof 26. Wolff R, Hommerich J, Riemsma R, Antes G, Lange
AC, Bamford JM. Prevalence of permanent child- EM. Noncompliance in lifestyle intervention studies. S, Kleijnen J. Hearing screening in newborns. Arch Dis
hood hearing impairment in the United Kingdom and J Clin Epidemiol. 2010;63(8):900-906. Child. 2010;95(2):130-135.
implications for universal neonatal hearing screening. 15. Rassen JA, Brookhart MA, Glynn RJ, Mittleman 27. Yoshinaga-Itano C, Coulter D, Thomson V. De-
BMJ. 2001;323(7312):536-540. MA, Schneeweiss S. Instrumental variables I: instru- velopmental outcomes of children with hearing loss
3. Kennedy CR; Wessex Universal Neonatal Screen- mental variables exploit natural variation in nonex- born in Colorado hospitals with and without univer-
ing Trial Group. Controlled trial of universal neonatal perimental data to estimate causal relationships. J Clin sal newborn hearing screening programs. Semin
screening for early identification of permanent child- Epidemiol. 2009;62(12):1226-1232. Neonatol. 2001;6(6):521-529.
hood hearing impairment. Acta Paediatr Suppl. 1999; 16. Vandenbroucke JP. When are observational stud- 28. Fellinger J, Holzinger D, Sattel H, Laucht M. Men-
88(432):73-75. ies as credible as randomised trials? Lancet. 2004; tal health and quality of life in deaf pupils. Eur Child
4. American Academy of Pediatrics, Joint Commit- 363(9422):1728-1731. Adolesc Psychiatry. 2008;17(7):414-423.

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