![Schematic view of the Auditory periphery, which is assessed in neonatal hearing screening. Hearing deficits can occur in all 3 compartments (i.e., external, middle, inner) as well at the level of the auditory nerve fibers, modified from [14]. OAEs can identify hearing loss issues up to the level of outer hair cells (the left in green, an example of a hair cell, not to scale with the rest of the illustration); AABR/ABR extend the hearing loss identification at the level of the auditory nerve fibers and the brainstem. Figure created with BioRender.](https://www.researchgate.net/profile/Stavros-Hatzopoulos/publication/385167143/figure/fig1/AS:11431281378482141@1744741976879/Schematic-view-of-the-Auditory-periphery-which-is-assessed-in-neonatal-hearing_Q320.jpg)
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Citation: Hatzopoulos, S.; Cardinali,
L.; Skar˙
zy´nski, P.H.; Zimatore, G. The
Otoacoustic Emissions in the
Universal Neonatal Hearing
Screening: An Update on the
European Data (2004 to 2024).
Children 2024,11, 1276. https://
doi.org/10.3390/children11111276
Academic Editor: Daniele Monzani
Received: 6 October 2024
Revised: 20 October 2024
Accepted: 22 October 2024
Published: 23 October 2024
Copyright: © 2024 by the authors.
Licensee MDPI, Basel, Switzerland.
This article is an open access article
distributed under the terms and
conditions of the Creative Commons
Attribution (CC BY) license (https://
creativecommons.org/licenses/by/
4.0/).
children
Review
The Otoacoustic Emissions in the Universal Neonatal Hearing
Screening: An Update on the European Data (2004 to 2024)
Stavros Hatzopoulos 1, Ludovica Cardinali 2, Piotr Henryk Skar˙
zy ´nski 3,4,5 and Giovanna Zimatore 6, *
1Clinic of Audiology & ENT, University of Ferrara, 44100 Ferrara, Italy; sdh1@unife.it
2Department of Life Science, Health, and Health Professions, Link Campus University, 00165 Rome, Italy;
l.cardinali@unilink.it
3
Heart Failure and Cardiac Rehabilitation Department, Faculty of Medicine and Dentistry, Medical University
of Warsaw, 02-091 Warsaw, Poland
4Institute of Sensory Organs, 05-830 Nadarzyn/Kajetany, Poland
5
World Hearing Center, Department of Teleaudiology and Screening, Institute of Physiology and Pathology of
Hearing, 02-042 Warsaw, Poland
6Department of Theoretical and Applied Sciences Applied Physics, eCampus University, 00182 Rome, Italy
*Correspondence: giovanna.zimatore@uniecampus.it
Abstract: Background: The reported data on European universal neonatal hearing screening (UNHS)
practices tend to be scarce, despite the fact that the European Union project, EUScreen, collected
unofficial data from 38 collaborating European institutions. The objectives of this systematic review
were as follows: (a) to identify the most recent (in a 20-year span) literature information about UNHS
programs in Europe and (b) to provide data on the procedures used to assess the population, the
intervention policies, and on the estimated prevalence of congenital hearing loss with emphasis on the
bilateral hearing loss cases. Methods: Queries were conducted via the Pubmed, Scopus and Google
Scholar databases for the time period of 2004–2024. The Mesh terms used were “OAE”, “Universal
Neonatal Hearing Screening”, “congenital hearing loss” and “well babies”. Only research articles and
review papers of European origin were considered good candidates. The standard English language
filter was not used, in order to identify information from non-English-speaking scientific communities
and groups. Results: Very few data and reports were identified in the literature search. Eleven
manuscripts were identified corresponding to eight UNHS programs. Except in Poland, most of the
data refer to regional and not national programs. The screening coverage estimates of all programs
exceed 90%; infants were mostly assessed by a three-stage protocol (TEOAE + TEOAE + AABR),
followed by a clinical ABR test. The average prevalence (i.e., from well babies AND NICU infants) of
bilateral hearing loss ranged from 0.5 to 20.94 per 1000 (Zurich sample). Infants presenting unilateral
or bilateral hearing losses were first rehabilitated by hearing aids and consequently (>15 mo) by
cochlear implants. Conclusions: Even though UNHS programs are well-established clinical practices
in the European States, the amount of information in the literature about these programs is surprising
low. The existing data in the timespan 2004–2024 corroborate the international UNHS data in terms of
coverage and bilateral hearing loss prevalence, but there is a strong need to supplement the existing
information with the latest developments, especially in the area of hearing loss rehabilitation.
Keywords: congenital hearing loss; newborn hearing screening; otoacoustic emissions; well babies;
NICU; bilateral hearing loss
1. Introduction
Hearing loss (HL) is one of the most common sensory impairments worldwide and
represents a critical medical and public health issue [
1
]. Etiologies vary according to the
age at onset of HL and include genetic, infective, toxic and environmental factors. Overall,
genetic factors account for at least 40% of the cases, and a relevant portion of affected
patients can have a definite molecular diagnosis thanks to Next Generation Sequencing
Children 2024,11, 1276. https://doi.org/10.3390/children11111276 https://www.mdpi.com/journal/children
Children 2024,11, 1276 2 of 14
(NGS) technologies [
2
]. Inner ear malformations (IEMs) are known to be detectable in a
proportion ranging from 30 up to 50% of children with congenital sensorineural hearing
loss [
3
,
4
]. Furthermore, although newborns with hearing deficits can use auditory temporal
information during the development in the first months of life [
5
], it takes them longer
to mature and process this information efficiently along the entire auditory pathway.
If hearing loss is confirmed during clinical tests after the screening, hearing aids and
eventually cochlear implants are effective in auditory rehabilitation; this is also true in the
case of inner ear malformations [6].
Neonatal hearing screening is an early hearing-detection and intervention strategy
which aims to identify infants with potential conductive and sensorineural hearing deficits.
The data in the literature strongly suggest that the early detection and rehabilitation of
a hearing impairment are essential factors for the development of language and relative
social and cognitive skills [7,8].
At present, two clinical methodologies are available to conduct neonatal hearing screen-
ing; these protocols are based on otoacoustic emissions (OAEs) or on automated auditory
brainstem responses (AABRs) [
9
,
10
]. In terms of screening, the OAEs access the functionality
of the inner ear by examining the functional status of the outer hair cells; thus, they provide
an assessment of the auditory periphery. The AABR provides an indication of the auditory
brainstem functionality; thus, the hearing assessment is peripheral and retro-cochlear.
OAEs represent a fast, non-invasive and cost-effective method; a small probe is placed
in the neonatal ear canal, the microphones of the probe emit specific transient stimuli (i.e.,
clicks, chirps, tone busts) and after a short time, in ms, the same microphones record the
acoustical echoes produced by a reflection of the stimulus energy within the inner ear.
These echoes are generated by the nonlinear behavior of the outer hair cells on the organ of
Corti [
9
,
11
]. Since these responses are low-level acoustical signals, there is a need to record
them in silent clinical setups with very low ambient noise.
The AABR is an electrical response; it is generally more accurate than the OAEs
in detecting hearing deficits and does not require a quiet environment, although some
electromagnetic shielding is always recommended in order to avoid signal artifacts and
long acquisition times [
10
]. The AABR is recorded by three electrodes placed on the infant’s
head, and specific algorithms detect the presence of wave V in the acquired response,
providing a positive outcome of the test (PASS).
Overall, the cost of the AABR devices and the relative consumables are more expensive
compared to OAEs. Usually, OAE screeners are used in the first phase of screening and
subsequent evaluation by AABR follows in cases of technical OAE problems or REFER
OAE results. This protocol combination has become the standard in audiological clinical
practice [12,13]. The auditory periphery is depicted in Figure 1.
Children 2024, 11, x FOR PEER REVIEW 3 of 16
Figure 1. Schematic view of the Auditory periphery, which is assessed in neonatal hearing screening.
Hearing deficits can occur in all 3 compartments (i.e. external, middle, inner) as well at the level of
the auditory nerve fibers, modified from [14]. OAEs can identify hearing loss issues up to the level
of outer hair cells (the left in green, an example of a hair cell, not to scale with the rest of the illus-
tration); AABR /ABR extend the hearing loss identification at the level of the auditory nerve fibers
and the brainstem. Figure created with BioRender.
The technologies involved in neonatal hearing screening have not evolved particu-
larly in the last 30 years. In terms of OAEs, two different protocols are still being used, as
in the 90s. These refer to TEOAEs evoked by a series of 80 clicks and DPOAEs evoked by
asymmetrical pure tone stimuli, such as 65- and 55-dB SPL, having a frequency ratio of
1.2. The usual TEOAE or DPOAE response evaluation is still based on a signal-to-noise
relationship, usually in the bands of 2.0, 3.0 and 4.0 kHz. Only the Accuscreen screener
(Natus) uses a stochastic model for the TEOAE response evaluation and a spectral coher-
ence model for the DPOAE evaluation [15–18]. The algorithms of AABRs are the same as
those developed 25 years ago; the AABR algorithm seeks the presence of wave III or wave
IV in a predetermined latency range, until a statistical criterion is satisfied [19].
In terms of terminology, universal neonatal hearing screening (UNHS) is part of an
Early Hearing Detection and Intervention (EHDI) program, since hearing intervention
and rehabilitation are the top priorities of UNHS [20,21]. Different countries have varying
approaches and strategies to neonatal hearing screening. In many developed countries,
universal newborn hearing screening (UNHS) programs are in place and are considered
standard practice [22–25]. However, in some low-income countries (Albania, Romania,
Bulgaria, etc.), these programs are not yet fully implemented due to limited resources and
infrastructure [26,27].
Other reports on national neonatal hearing screening programs raise issues around
implementation, test procedures, type of tests, coverage, detected cases of hearing loss
and costs [28]. Ensuring high-quality neonatal hearing screening involves maintaining
comprehensive coverage (selecting appropriate and accurate screening methods, manag-
ing referral rates (rate of failed tests at discharge) effectively and diagnosing hearing dis-
orders as early as possible.
Figure 1. Schematic view of the Auditory periphery, which is assessed in neonatal hearing screening.
Hearing deficits can occur in all 3 compartments (i.e., external, middle, inner) as well at the level of
the auditory nerve fibers, modified from [
14
]. OAEs can identify hearing loss issues up to the level of
outer hair cells (the left in green, an example of a hair cell, not to scale with the rest of the illustration);
AABR/ABR extend the hearing loss identification at the level of the auditory nerve fibers and the
brainstem. Figure created with BioRender.
Children 2024,11, 1276 3 of 14
The technologies involved in neonatal hearing screening have not evolved particularly
in the last 30 years. In terms of OAEs, two different protocols are still being used, as in
the 90s. These refer to TEOAEs evoked by a series of 80 clicks and DPOAEs evoked by
asymmetrical pure tone stimuli, such as 65- and 55-dB SPL, having a frequency ratio of
1.2. The usual TEOAE or DPOAE response evaluation is still based on a signal-to-noise
relationship, usually in the bands of 2.0, 3.0 and 4.0 kHz. Only the Accuscreen screener
(Natus) uses a stochastic model for the TEOAE response evaluation and a spectral coherence
model for the DPOAE evaluation [
15
–
18
]. The algorithms of AABRs are the same as those
developed 25 years ago; the AABR algorithm seeks the presence of wave III or wave IV in
a predetermined latency range, until a statistical criterion is satisfied [19].
In terms of terminology, universal neonatal hearing screening (UNHS) is part of an Early
Hearing Detection and Intervention (EHDI) program, since hearing intervention and rehabili-
tation are the top priorities of UNHS [
20
,
21
]. Different countries have varying approaches and
strategies to neonatal hearing screening. In many developed countries, universal newborn
hearing screening (UNHS) programs are in place and are considered standard practice [
22
–
25
].
However, in some low-income countries (Albania, Romania, Bulgaria, etc.), these programs
are not yet fully implemented due to limited resources and infrastructure [26,27].
Other reports on national neonatal hearing screening programs raise issues around imple-
mentation, test procedures, type of tests, coverage, detected cases of hearing loss and costs [
28
].
Ensuring high-quality neonatal hearing screening involves maintaining comprehensive cover-
age (selecting appropriate and accurate screening methods, managing referral rates (rate of
failed tests at discharge) effectively and diagnosing hearing disorders as early as possible.
In 2015, Sloot et al. [
29
] reported initial data from the European Union project EUScreen
on the pediatric vision and hearing screening programs. Regarding the hearing screening,
38 programs participated, and data circulated in EUScreen without being officially pub-
lished by the corresponding national contributors. The screening information was obtained
via the collaboration of professionals from various clinical or university environments. The
overall screening paradigm followed three assessment stages, the first two via OAEs and
the last one via AABRs or ABRs.
From 2014 to 2019, the International Newborn and Infant Hearing Screening group [
30
]
asked, via questionnaire, the status of hearing screening practices in 196 countries world-
wide; data from 158 countries were obtained and the surprising results show that in
64 countries
, there is no organized hearing screening (38% of the world population) and
that in 41 countries more than 85% of the babies are screened. For the latter group, the
mean living standard was 10 times higher than in countries without screening. In terms of
identification times, it was found that the average age at diagnosis of hearing disorders
was 4.6 months for screened children and 34.9 months for non-screened children.
While this sort of information (i.e., surveys) is quite valuable for the EDHI national
strategies, unfortunately surveys acquiring information directly from individual profession-
als (as in the case of [
29
,
30
]) cannot substitute the necessary publications of various national
scientific groups on the topic of neonatal hearing screening. In this context, the literature
for recent UNHS information suggests a considerable lack of data on EDHI performance
and rehabilitation results.
The present paper aims to lessen this informational gap, presenting data on the in-
volvement of OAE-based protocols in the European hearing screening practices as of 2024.
The main reason for focusing on the EU data is two-fold: (1) there is already an informa-
tional background from the EUScreen project, which needs to be updated;
(2) extending
the literature update to a world-like scale would exceed the scope of a review paper.
In addition, the paper has sought suitable responses (when information was attainable
from the literature) to the following five issues which are very fundamental for EDHI
strategies [
29
,
31
]: (i) Which countries still perform UNHS? (ii) What percentage of newborns
are involved? (iii) How many present congenital deafness and more precisely bilateral
deafness? (iv) What is the follow-up rate? (v) Which are the most common protocols and
OAE technologies used to assess hearing?
Children 2024,11, 1276 4 of 14
2. Materials and Methods
We sourced scientific articles and reviews using the PubMed, Scopus and Google
Scholar search engines. Considering that the first papers on UNHS and EDHI started
appearing in the literature in the late 90s/early 2000s and since we were interested in the
latest advances in hearing screening, we selected a search time-span window of 20 years.
The literature search, conducted on July 2024, followed the PRISMA 2020 guidelines
(the PRISMA website was visited in July 2024) and utilized the following keywords (mesh
terms): “OAE”, “Universal Neonatal Hearing Screening”, “congenital hearing loss” and
“well babies”. Only research articles and review papers were considered good candidates.
The standard English language filter was not used, in order to identify information from
non-English-speaking, scientific communities and groups. Papers related to UNHS prac-
tices outside the EU were not considered. The quality of the material was dictated by
several factors, with the publication being in a peer-reviewed journal being the cardinal one.
Additionally, clearly stated methodologies and a rigorous application of the established
screening protocols were also taken into consideration (i.e., all infants had to be screened
for any hearing deficits). The most prominent inquiry results are shown in Table 1.
The selection criteria for the candidates of the search were based on the following:
(i) the
origin of the paper (European group or not); (ii) the number of infants screened
(large sample studies were preferred); and (iii) the most recent data per country (the most
recent study/studies per country were selected).
Exceptions to these rules include the following: (i) cases from similar studies (in terms
of sample size) where both manuscripts were included; and (ii) cases where national data
and regional data were reported (again both manuscripts were included).
Two independent reviewers went over the papers from Outcome No. 2 (universal
newborn hearing screening). There was concordance in the number of the manuscripts
selected except one case (for an early Italian screening report of 2006). The final number
of eligible papers was 11 (related to 8 UNHS national programs), and they are reported
analytically in Table 2. The PRISMA flowchart process is reported in Figure 2.
Children 2024, 11, x FOR PEER REVIEW 5 of 16
The selection criteria for the candidates of the search were based on the following: (i)
the origin of the paper (European group or not); (ii) the number of infants screened (large
sample studies were preferred); and (iii) the most recent data per country (the most recent
study/studies per country were selected).
Exceptions to these rules include the following: (i) cases from similar studies (in terms
of sample size) where both manuscripts were included; and (ii) cases where national data
and regional data were reported (again both manuscripts were included).
Two independent reviewers went over the papers from Outcome No. 2 (universal
newborn hearing screening). There was concordance in the number of the manuscripts
selected except one case (for an early Italian screening report of 2006). The final number
of eligible papers was 11 (related to 8 UNHS national programs), and they are reported
analytically in Table 2. The PRISMA flowchart process is reported in Figure 2.
Table 2. The 11 eligible papers after the filtering process; the national data are presented in
alphabetical order. For comparison purposes, the EUscreen data, published by Sloot et al. in 2015
[29], are also inserted (5th column); NA = Not Available data.
n Country Sample Sie (n)
Yea
r
the Study Was Carried
Out and Population %
Coverage
Coverage Data from
Sloot 2015 Author Publication Year
1 Albania
*
1561 2006 (23%) >10% Hatzopoulos 2007
2 22,818 2018–2019 (96.6%) >10% Busseè 2023
3 France 160,196 2012 (99%) >50% Chays 2014
4 Germany 296,700 2009 (50%) >95% Thangavelu 2023
5 Italy ^ 7775 2000–2006 (94%) 70% Ciorba 2008
6 20,841 2012–2014 (93.8%) Molini 2016
7 Poland 7,149,809 2002–2022 (96%) >95% Greczka 2022
8 Russian Fed. 1,232,137 2020 (87.8%) NA Tavartkeladge 2023
9 NA 2008–2021 (NA) NA Chibisova 2024
10 Switzerland 79,721 2012 (97.9%) >95% Metzger 2013
11 UK 4,645,823 2015 (97.5%) >95% Wood 2015
* pilot study; ^ regions: Emilia Romagna and Umbria.
Figure 2. Flow diagram of the literature search, according to PRISMA criteria (hp://www.prisma-
statement.org/, accessed on 31 July 2024), with the steps followed in the manuscript selection
procedure. After the application of the selection criteria, the initial 244 manuscripts were reduced to
11 (see Table 1 “Universal newborn hearing screening” Pubmed + Scopus).
Figure 2. Flow diagram of the literature search, according to PRISMA criteria (http://www.prisma-
statement.org/, accessed on 31 July 2024), with the steps followed in the manuscript selection
procedure. After the application of the selection criteria, the initial 244 manuscripts were reduced to
11 (see Table 1“Universal newborn hearing screening” Pubmed + Scopus).
Children 2024,11, 1276 5 of 14
Table 1. Query outcomes from the 3 search engines (articles or reviews). The star symbol “*” indicates
that for the Google Scholar database, the term “Scientific” does not distinguish between articles and
reviews. Shaded cells refer to the total number of manuscripts in 2024, per database.
2004–2024 OAE and “Universal Screening” “Universal Newborn Hearing
Screening” “Well Babies” and “OAE”
Article Review In 2024 Article Review In 2024 Article Review In 2024
PubMed 10 1 0 111 10 1 3 0 0
Scopus 11 1 0 111 12 3 12 1 0
Google scholar 123 * 7 897 * 37 42 * 0
TOTAL 144 1119 57
Table 2. The 11 eligible papers after the filtering process; the national data are presented in alphabeti-
cal order. For comparison purposes, the EUscreen data, published by Sloot et al. in 2015 [
29
], are also
inserted (5th column); NA = Not Available data.
n Country
Sample Size (n) Year the Study Was Carried Out
and Population % Coverage
Coverage Data
from Sloot 2015
Author Publication
Year
1Albania * 1561 2006 (23%) >10% Hatzopoulos 2007
2 22,818 2018–2019 (96.6%) >10% Busseè2023
3 France 160,196 2012 (99%) >50% Chays 2014
4 Germany 296,700 2009 (50%) >95% Thangavelu 2023
5Italy ˆ 7775 2000–2006 (94%) 70% Ciorba 2008
6 20,841 2012–2014 (93.8%) Molini 2016
7 Poland 7,149,809 2002–2022 (96%) >95% Greczka 2022
8Russian Fed. 1,232,137 2020 (87.8%) NA Tavartkeladge 2023
9 NA 2008–2021 (NA) NA Chibisova 2024
10 Switzerland 79,721 2012 (97.9%) >95% Metzger 2013
11 UK 4,645,823 2015 (97.5%) >95% Wood 2015
* pilot study; ˆ regions: Emilia Romagna and Umbria.
3. Results
The data were classified alphabetically according to the country of origin into two
tables. Table 2shows the identified programs from all of the eligible papers used in the
study. For the eight referenced screening realities, the published UNHS data refer to the
year 2006 onwards. Reported data regarding the etiological diagnoses and treatment were
found only for one Italian dataset (Umbria).
Table 3shows data related to the EDHI questions expressed at the end of the Introduc-
tion section, including the prevalence of congenital hearing loss.
Table 3. Data related to the EDHI-related questions posted in the Introduction. The national data are
presented in alphabetical order; in bold the prevalence index by Bilateral Deafness; BD = bilateral
deafness; WB = well babies; NICU = infants from the intensive care unit; NA = not available data.
The gray shaded estimates indicate high prevalence values.
Country
Participants
(n)
Screening
Protocol
Bilater. Deafness
Cases and Prevalence Causes Intervention
Policies
Albania * 22,818 TEOAE, AABR 16/22,818 = 0.0007 1
0.7 per 1000 NA NA
France 160,196 TEOAE, AABR 116/160,196 = 0.00072
0.7 per 1000 Hearing Aids
Germany 296,700 TEOAE, AABR 2368/296,700 = 0.007
7 per 1000 NA NA
Children 2024,11, 1276 6 of 14
Table 3. Cont.
Country
Participants
(n)
Screening
Protocol
Bilater. Deafness
Cases and Prevalence Causes Intervention
Policies
Poland 7,149,809 TEOAE, AABR 10,367/7,149,809 = 0.00145
1.44 per 1000 NA Cochlear Implants
Italy: Emilia Rom.
Italy: Umbria
7775
20,841
TEOAE, AABR
TEOAE, AABR
WB: 4/6759 = 0.0005
0.5 per 1000
NICU: 8/1016 = 0.0078
7.8 per 1000
WB: 40/20,051 = 0.00199
1.99 per 1000
NICU: 33/790 = 0.041
41 per 1000
NA
Non-syndromic genetic
mutations without
familiarity (10)
Unknown: (22)
Cochlear Implants
WB: Hearing Aids (28)
Cochlear Implants (2)
NICU: Hearing Aids (28)
Cochlear Implants (3)
Russian Fed
Second sample
(Chibisova 2024)
1,232,137
NA
TEOAE, ABR
NA
3002/1,232,137 = 0.0024
2.43 per 1000
1292/NA = NA
NA
NA
GJB2 genotype profile
Cochlear Implants
NA
Switzerland # 12,080 TEOAE, ABR 253/12,080 = 0.02094
20.94 per 1000 NA Cochlear Implant (1)
Hearing Aids (14)
UK 4,645,823 TEOAE, AABR
Exact numbers not available
<1 per 1000 (well babies)
5–7 per 1000 (NICU)
NA NA
1See comments in the Section 3.1.1.; * pilot study; # sample only from Zurich.
3.1. Analytical UNHS Data
3.1.1. Albania
The first UNHS data from Albania were reported by Hatzopoulos et al. [
31
] assessing
463 well babies and 1098 NICU residents in the main maternity clinic of Tirana. The
standard three-stage protocol was used (TEOAE + TEOAE + AABR) + a clinical ABR test.
The paper reported bilateral hearing loss (>60 dB HL) in two NICU infants and a prevalence
of 1.8 per 1000.
In 2023, Busseèet al. [
32
] reported the data from the established UNHS Albanian
program implemented in four maternity clinics and included in the EUScreen European
program. The program used the standard three-stage protocol (TEOAE + TEOAE + AABR)
and an ABR test to assess 22,818 well babies and NICU residents with an initial coverage of
96.6%. One of the main objectives of the study was to identify the causes of loss-to-follow-
up (LTFU) which was reported as 33.6%, 40.4% and 35.8% for the second (TEOAE), third
(AABR) and clinical evaluation (ABR) stages. The authors reported that “of the 81 infants
who were referred for a diagnostic assessment, 52 (64.2%) attended. Twenty-two infants
(0.1% of 51 infants who were screened at least once) were diagnosed with an HL of 40 dB
or greater, of which 6 had a unilateral hearing loss”.
The estimated bilateral hearing loss prevalence was 0.7 per 1000, but this figure does
not include 29 AABR-referred infants who did not attend the clinical ABR evaluation.
3.1.2. France
In France, universal newborn hearing screening has been mandatory (i.e., all infants
had to be screened for any hearing deficits) since April 2012. The pilot project began in the
Champagne-Ardenne region in 2004.
The details of the program are reported in an earlier paper by the same group [
33
],
consisting of the following: First, TEOAEs were performed before discharge. If TEOAEs
were absent in both ears (positive screening test), the infant was referred for a second
assessment 15 days after discharge, which could be either a TEOAE test or an automated
auditory brainstem response (AABR) test. The second test was conducted by a physician in
an outpatient clinic. If the retest was found positive in both ears, the infant was referred
to diagnostic tests in a reference center. The same procedure was used for infants in the
neonatal intensive care unit (NICU), but, in those cases, the first test procedure was an
AABR because of the higher incidence of auditory neuropathies in those units.
Children 2024,11, 1276 7 of 14
The most recent report from Chays et al. [
34
] states that more than 99% of
160,196 newborns
in the region of Champagne-Ardenne have been screened. Bilateral hearing impairment
was identified in 116 infants when they were around 3.5 months old. The outcome of
deafness, without screening, is only diagnosed around an age of 20 months.
The reported bilateral deafness prevalence was estimated at 0.7 infants per 1000. The
reported data do not discriminate between well babies and NICU infants.
3.1.3. Germany
Since January 2009, UNHS has been obligatory in Germany [
35
]. In 2023, Thangavelu
et al. [
36
] reported data on a population-based newborn hearing screening program in
North Rhine, Germany, and a hospital-based screening at a university hospital for the
years 2007–2016. Newborns were enrolled in the two-stage “screening” and “follow-up”
program, which involved TEOAE and AABR tests, through participating birth centers. The
paper provides only screening data (no information is available on the follow-up and the
relative intervention policies) for 296,700 infants. The average referral rate was below 4%
and hearing screening was completed in 28.2 ±51.6 days.
The reported bilateral deafness prevalence was estimated at 7 per 1000. This estimate
is approaching the prevalence of NICU babies, but the reported data do not discriminate
between the two classes of infants.
3.1.4. Italy
Independent UNHS programs run in most Italian regions, without any central coor-
dination. The first UNHS data were reported in 2006 by Bubbico et al. [
37
], who used a
questionnaire survey to assess clinical data from various hospitals. The authors reported
“that in Italy the UNHS coverage had undergone a steep increase from 29.3% in 2003
(156,048 newborns screened) to 48.4% in 2006 (262,103 screened). The majority of UNHS
programs were implemented in the two most economically developed areas, i.e., in the
north-west area (79.5%, 108,200 of 136,109 births), and in the north-east area (57.2%, 52,727
of 92,133 births), while a limited diffusion remains in some areas, typically in the islands
(11.3%, 7158 of 63,460 births)”. The paper did not report the methods of screening or any
bilateral deafness estimate.
A 2007 [
38
] and a 2008 [
39
] paper by our group reported the status and efficiency
of an Italian non-national UNHS program (for clarity reasons, in Table 3, only the 2008
paper referring to the highest number of tested infants is shown). The data refer to a 6-year
regional program in Emilia-Romagna called Cheap & Cheaper, with a local coverage of 94%.
The program used a standard two-stage testing (TEOAE + TEOAE) + a clinical ABR test for
the diagnostic phase. Ciorba et al. stated “for the Well -baby group (WB) 53 cases (0.78%)
failed the second-stage TEOAE test and were assessed in the third phase with a clinical ABR
and only in selected cases with electrocochleography. From the 53 cases 13 (0.19%) were
identified with a hearing impairment. Four neonates presented bilateral profound hearing
loss and nine unilateral severe deafness. From the bilateral group three infants received a
cochlear implant; the unilateral cases remained in follow-up. The prevalence of bilateral
profound hearing loss in the WB group was estimated as 0.5 per 1000 (4/6759). From the
107 retested NICU infants, 22 cases (2.2%) were found with hearing impairment. Of these,
14 presented unilateral severe deafness, and 8 presented severe bilateral hearing loss. For
the bilateral hearing loss group four infants received a cochlear implant and one is still in
a waiting list. Three of these infants presented concomitant severe psycho-neurological
retardation and were placed in a follow-up program. The prevalence of bilateral profound
hearing loss in the NICU group was estimated as 7.8 per 1000”.
The most recent UNHS report by Molini et al. [
40
] reported data from the Umbria
region in the years 2012–2014. A standard three-phase screening procedure (TEOAE +
TEOAE + AABR) + a diagnostic ABR test assessed a total of 20,841 infants (20,051 WB and
790 NICU residents). The coverage of the project was 93.8%. The prevalence of hearing
loss was 2.44 per 1000 for the WB infants and 41 per 1000 for the NICU residents. The
Children 2024,11, 1276 8 of 14
distribution of the etiological diagnoses and initial treatment for the observed congenital
hearing loss was reported as follows. (1) For the 32 WB infants with bilateral hearing loss,
10 cases were attributed to non-syndromic genetic mutations without familiarity and 22 to
unknown causes. Twenty-eight received binaural hearing aids, two a cochlear implant and
two refused any treatment. (2) For the thirty-three NICU residents with bilateral hearing
losses, seven cases were attributed to non-syndromic genetic mutations with familiarity,
nine to NICU factors (i.e., a prolonged stay in the NICU), three to cytomegalovirus, nine
to syndromic genetic causes, four to craniofacial anomalies and one to neurodegenerative
disorders. Twenty-eight infants received a binaural hearing aid, three received a cochlear
implant and two refused any treatment.
3.1.5. Poland
The Polish universal neonatal hearing screening program has been carried out for
20 years, with 486 participating centers and more than 7 million children registered in the
central database [4,41].
The data from Greczka et al. [
4
] report that during the 20-year Polish hearing screening,
with an average coverage of 96%, a total of 10367 infants with bilateral deafness were
identified. The program used the standard three-stage protocol (TEOAE + TEOAE +
AABR) + a diagnostic ABR test. The number of identified infants with hearing losses were
classified into the following groups: (i) no data on the type of hearing loss (
565 cases
);
(ii) sensorineural
origin (6118 cases); (iii) conductive origin (2101 cases); and (iv) mixed
origin (1205 cases). The sensorineural cases were rehabilitated with cochlear implants
within a timespan from 3 to 6 months, with an average of 15 months. Additional information
about the genetics of the hearing losses or the causes of the impairments is not reported.
The reported bilateral deafness prevalence was estimated at 1.44 per 1000. The reported
data do not discriminate between well babies and NICU residents.
3.1.6. Russia
In 2008, Russia upgraded its high-risk newborn hearing screening program with a
UNHS program. Data from a 2023 paper by Tavartkiladze et al. [
42
] report the following:
“A total of 1,232,137 newborns were audiologically screened in 2020, covering 87.8% of all
live-born neonates. Of those screened at the first TEOAE test, 14,240 (1.2%) had suspected
hearing impairment. At stage 2 (second TEOAE testing), 13,244 (93%) newborns were
examined, and hearing impairment was identified (by a clinical ABR) in 3002 (23%), or
2.1 per 1000 newborns”. The study verified previously published Russian data [
43
,
44
]
that suggest that frequently, cases where congenital loss is present (and expressed by an
abnormal genotype) are not identified by the hearing screening protocols. The authors
conclude that it might be advantageous to conduct hearing and genetic screening in parallel,
at least for the most frequent mutations.
In 2024, Chibisova et al. [
45
] presented a more in-depth analysis of the data collected
by the Russian national UNHS. This is the only paper where additional data on the genetic
aspects of the bilateral deafness were reported. The authors analyzed genetic, audiological
and NHS data of 1292 pediatric patients with bilateral sensorineural hearing loss born from
2008 to 2021. GJB2 sequencing was conducted on all subjects, resulting in 644 patients with
a pathological GJB2 genotype profile. Four hundred and six (406) of these patients were
found homozygous for the c.35delG variant. In addition, a group of 155 GJB2-negative
patients were tested for other SNHL genes, whose genotypes were identified in 87 patients.
The authors reported that “the most frequent genes were STRC (21.8%), USH2A (16.1%),
OTOF (8%) and SLC26A4 (6.9%). Children with confirmed genetic etiology passed NHS
only in 21% of cases”.
The reported bilateral deafness prevalence (from the Tavartkiladze reported sample)
was estimated at 2.43 infants per 1000. The reported data do not discriminate between well
babies and NICU residents.
Children 2024,11, 1276 9 of 14
3.1.7. Switzerland
UNHS was introduced in Switzerland in 1999. In 2013, Metzger et al. [
46
] reported
some estimates about the total number of 79,721 screened infants (a coverage of 97.9%) for
the year 2012 and, more precisely, about a sample of 12,080 infants born at the University
Hospital of Zurich.
The hearing screening protocols at the Zurich hospital used a two-stage TEOAE test
with a third stage clinical ABR evaluation. Metzger et al. [
46
] reported “A total of 253/12,080
(2.1%) infants failed the UNHS and were followed up in our pediatric audiology unit. The
mean age at follow-up was 2.4 months (range 0.2–6.2 months). A relevant bilateral hearing
loss (of 40 dB or more) was found in 15/253 (5.9%). One of those received a cochlear
implant, the others hearing aids”.
The incidence of bilateral hearing loss was shown to be 20.94 per 1000, one of the
highest estimates in the countries of the European Union. The data do not discriminate
between well babies and NICU residents.
3.1.8. UK
The UNHS practices in the UK were one of the earliest ones in Europe, starting in
2002 [
47
] and becoming fully implemented in 2006 [
48
]. Annual hearing screening data can
be found at the government site of the National Hearing Screening Program (NHSP) [49].
Wood et al. [
48
] reported some official screening numbers from the screening program
in England, summarized in the following: “A total of 4,645,823 children were assessed born
between April 2004 to March 2013. In terms of coverage, 97.5% of the eligible population
completed screening by 4/5 weeks of age and 98.9% completed screening by three months
of age. The refer rate was estimated is 2.6%. The percentage of screen positive (i.e., referred)
babies commencing follow up by four weeks of age and six months of age is 82.5% and
95.8% respectively. The yield of bilateral hearing loss for the well babies was estimated to
be around 1 per 1000, while the corresponding NICU estimate was 5–7 per 1000”.
Assessing the NHSP portal for information related to more recent data (for the year
2021), the following updated indices were observed: (i) the coverage was between 98 to
99.5%; (ii) the referral rate has dropped to 1.6%.
4. Discussion
Deafness can be caused by a variety of factors, which can be broadly categorized into
genetic and environmental causes. In fact, deafness can be inherited, often as a result of
mutations in specific genes [
50
,
51
]. These genetic conditions can be autosomal dominant,
autosomal recessive or linked to the X chromosome. When hearing loss is associated with
other symptoms as part of a syndrome (e.g., Usher syndrome, Waardenburg syndrome)
it is called “syndromic deafness”; the most common form of inherited deafness is non-
syndromic deafness [
52
]. The second causes are environmental ones, such as infections
during pregnancy, such as rubella, cytomegalovirus, syphilis and toxoplasmosis. Even
factors such as prematurity, low birth weight, lack of oxygen (anoxia) or severe jaundice
(hyperbilirubinemia) can lead to hearing loss in newborns. Furthermore, certain medi-
cations, particularly some antibiotics (e.g., aminoglycosides) and chemotherapy drugs,
postnatal infections and prolonged exposure to loud noise or a sudden loud sound (like an
explosion) are environmental causes that can occur after newborn screening. In some cases,
the cause of deafness remains unknown (idiopathic), despite thorough investigation.
The first objective of the paper was to provide updates on the European UNHS
practices, from reports found in the literature in the period 2004–2024. The European
project EUScreen (2015–2019) provided information on 38 UNHS European realities, but
the majority of the data were never published analytically in the scientific literature. As
such, the EUScreen data are of limited use.
The second objective of the paper was to extract information from the identified on-
going UNHS programs regarding the coverage of the project, the congenital hearing
impairment estimates (with emphasis into the bilateral hearing loss), the description
Children 2024,11, 1276 10 of 14
of causes leading to hearing loss and the relative intervention strategies and lastly the
technologies and protocols used.
4.1. The Number of UNHS Programs
The first impression from the database queries was that the number of papers related
to the topic of otoacoustic emissions and hearing screening was relatively low. The data in
Table 1(see the shaded columns) suggest that in 2024, the topic of hearing screening is not
highly considered, despite the fact that considerable amounts of information are missing
from the literature.
The data from Sloot et al. [
29
] have indicated that the number of collaborating UNHS
programs in the EU was 38. Analytical reports in the literature (2004–2024) were found from
only eight screening realities. In the initial search, a number of papers describing small
(local or pilot programs) were found, but they were not included in the review, since the
data were referring to small samples. It is not clear at this point if (1) the unofficial reports
of the EUScreen project (found at www.euroscreen.org/reports (accessed on 25 September
2024)) constitute some sort of official record in the minds of the European Audiologists and
researchers; or (2) some data have been published in local European journals outside the
indexing of the Pubmed and Scopus databases.
4.2. Reported UNHS Coverage
The reported coverage estimates are quite high (i.e., >90%), but the data primarily
report specific years and specific regions. Only the Polish data correspond to a wide-spread
national hearing screening program.
There are some conflicts between the coverage values reported by Sloot et al. [
29
]
(see Table 2, column 5) and the ones from the reported data in the literature. Important
differences can be seen in the data from Albania (10% vs. 23%, or 96.6%), France (50% vs.
99%), Germany (95% vs. 50%) and Italy (70% vs. 94% or 93.8%). It is quite possible that
these differences are generated by the fact that none of the reported data refer to a national
estimate, but instead reflect data from different local areas and possibly from different
times (the Sloot et al. EUScreen data were published in 2015, which means that for the
majority of programs, the coverage estimates were from 2014 or much earlier).
4.3. Estimates of Bilateral Hearing Loss Prevalence
For the majority of programs, the estimates of bilateral hearing loss refer to the average
number, of well babies and intensive care unit infants with hearing loss, without considering
the discrete diversities between well babies and NICU residents.
These estimates concord with the data trends reported in the literature for the UNHS
realities outside Europe [24,25,53–56].
The prevalence in Switzerland [
46
] and in the Italian Umbria NICU sample [
40
] present
the highest values, with 20.94 and 41 infants per 1000. For the first group, the prevalence
refers to a small sample from the area of Zurich, and as such, the national estimate should
be different. The Metzger et al. paper [
46
] does not include detailed information on the loss-
to-follow-up estimate, nor any data on possible demographic factors which might influence
the final prevalence. The same rationale can be applied to the Molini et al. paper [40].
4.4. Causes Leading to Hearing Loss and Intervention Strategies
Very few papers have examined the causes of congenital hearing loss presented by
the screened WB and NICU infants. The only project which provided etiological data is
the Umbria UNHS project, where factors such as non-syndromic genetic mutations with
familiarity, neonatal intensive care stay, cytomegalovirus in utero infection, genetic and
syndromic factors, craniofacial anomalies and neurodegenerative disorders were reported.
The intervention policies included unilateral and binaural hearing aids and cochlear
implants. The exact times of the cochlear implant surgery are not always reported; the
time-estimates reported refer to times > 15 months of age (Polish UNHS program).
Children 2024,11, 1276 11 of 14
The data from the Russian UNHS program are quite interesting in the fact that they
show that NHS practices do not always capture infants presenting genetic
complications [43,44]
. This can be explained by the fact that these complications have
a relatively slow onset (and impact on cochlear functionality), which does not always
coincide with the first days of life. The Russian data suggest that for a completer and more
accurate UNHS program, a genetic screening of the most frequent mutations should be
conducted as well, at least in the NICU population.
4.5. Technological Issues
All of the identified UNHS programs used a two-stage protocol (TEOAE + TEOAE)
where the third stage was an ABBR or directly a diagnostic ABR evaluation. Distortion
product otoacoustic emissions were not utilized. There is a lack of information on the actual
TEOAE hardware used, but the descriptive information of these projects refers primarily to
portable TEOAE screeners, which have the option to carry out an AABR assessment on the
tested infant.
Previous data in the literature [
57
,
58
] have identified and recommended the need to
have a consensus on the characteristics of the TEOAE probes across the various manufacture
models, which might alter the screening outcomes, especially in borderline screening
scenarios. Unfortunately, this aspect was missed completely in all of the UNHS reports
presented in this review.
4.6. Some Comments About the Search Engines Using Artificial Intelligence (AI) Interfaces
With the introduction of artificial intelligence tools, the Scopus database offers new possi-
bilities of research, exceeding the standard paradigms of Mesh terms and Boolean operators.
Unfortunately, this approach seems to have a number of limitations. In fact, although
useful for many general questions, AI cannot replace the human operator and cannot be
used as the sole source of information, as it does not really understand the meaning of
words or phrases but relies on statistical models to generate the answers. As an example, we
report the answer to a specific question about the aim of this review, where the references
quoted are analyzed but the data included are only partial. The question “Which are
the European countries where a Universal Neonatal Hearing Screening is active?” was
submitted to the SCOPUS AI. The AI tool responded: “Based on the abstracts reported, the
countries where Universal Neonatal Hearing Screening (UNHS) is carried out include Poland,
France and Switzerland. However, it’s important to note that the abstracts do not provide a
comprehensive list of all countries where UNHS is carried out. The information is limited to the
countries mentioned in the abstracts, and there may be other countries implementing UNHS that
are not covered in the provided abstracts”.
The Scopus AI tool identified 3 out of the 11 papers used in this review. Such a tool can
be used as an initial guideline, but obviously needs to become more precise and examine
the total lexical content of a manuscript. It should be also mentioned that numerous
publications in the literature refer to a specific condition where Artificial Intelligence bots
produce fictitious and erroneous information, which is called AI hallucinations [
59
–
61
]. In
this context, the use of AI bots to extract information needs to be constantly monitored till
the produced errors are minimal.
4.7. Limitations of the Study
The results obtained from the database queries refer to regional (not national) studies
with large datasets (i.e., >1000 subjects) in order to attain a certain solidity of the reported
results. A number of studies with significantly smaller sample sizes were also identified
but were not included in the body of manuscripts of this review. It might have been more
informative to include this sort of study, but all of the contributors to this review were
against it.
Another negative aspect of systematic reviews is the fact that they depend on the
proper indexing of the databases used. In this context, we might have lost a num-
Children 2024,11, 1276 12 of 14
ber of recent papers because they were not properly indexed in Pubmed, Scopus and
Google Scholar.
We are also including a remark from an anonymous reviewer who suggested that
many European clinical realities might use dedicated AABR/ABR protocols to assess the
neonatal population at hand. In this context, the PRISMA review conducted would miss
a number of publications where the terms OAE or UNHS were not included. On the
other hand, according to the EUScreen practices ALL regional or national programs use a
three-phase UNHS protocol, involving OAEs in the first screening stages; therefore, the
information not included in this review would reflect the screening outcomes only from
small clinical realities.
5. Conclusions
Even though UNHS programs are well-established clinical practices in the European
states, the amount of information in the literature about these programs is surprisingly low.
The existing data in the timespan of 2004–2024 corroborate the international UNHS data
in terms of coverage and bilateral hearing loss prevalence, but there is a strong need to
supplement the existing information with the latest developments, especially in the area of
hearing loss rehabilitation.
Author Contributions: Bibliographic search, S.H., G.Z. and L.C.; investigation and data curation:
S.H., G.Z. and L.C.; writing—original draft preparation, S.H. and G.Z.; writing—review and editing,
S.H., G.Z. and P.H.S. All authors have read and agreed to the published version of the manuscript.
Funding: This research received no external funding.
Data Availability Statement: Data are available upon request.
Conflicts of Interest: The authors declare no conflicts of interest.
Abbreviations
AABR automated auditory brainstem response
ABR auditory brainstem response
BD bilateral deafness
EDHI Early Hearing Detection and Intervention
EU European Union
IEMs inner ear malformations
NICU neonatal intensive care unit
OAE otoacoustic emissions
UNHS universal neonatal hearing screening
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