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A-ABR in Rome
Francesca Cianfrone, Fulvio Mammarella, Valerija Evetovic, Claudio Maria Pianura,
and Gianluca Bellocchi
ORL Department , San Camillo Forlanini Hospital
Rome Italy
Abstract
Objectives. Universal Newborn Hearing Screening (UNHS) is the major procedure to
identify hearing loss in the early postnatal period. Early detection is very important for early
intervention. This retrospective study confirms the importance of UNHS to identify babies
with potential hearing loss.
Patiens. All children identified in San Camillo Forlanini Hospital (SCFH) by UNHS program
between May 2011 and May 2013 .
Materials and methods. The screening procedure : first level was performed with
Automatic Transient Otoacustic Emission (A-TEOEs) ; second level , for babies with a
bilateral pass response but with a positive history for hearing loss factors and for babies
with a referred response at first level they underwent to automatic ABR (A-ABR),
tympanometry and acustic reflex.
Results. Seven babies with hearing loss were identified from a screened population of
4719 . Among those five had unilateral hearing loss ( three right ear , two left ear ) and two
had bilateral hearing loss .
Conclusions. The UNHS is effective, non- invasive, quick and easy to perform .
INTRODUCTION
The estimated incidence of profound bilateral hearing impairment (SNHI) ranges from 1 to
3 per 1000 newborns in the United States and it is very high compared other congenital
anomalies (1, 2).
There are very few studies on the prevalence of congenital deafness in Italy (3), a
condition that decreased over the years from a 1.01/1000 ratio observed between 1989
and 2003.
Many studies demonstrated that children with congenital hearing loss who received early
intervention had significantly better outcomes than children who did not received an early
hearing screening (4). Newborns diagnosed with bilateral hearing loss (HL) could begin
rehabilitation starting from 6 months of age. Currently, rehabilitation of hearing loss is
mainly based on the use of hearing aids and the application of cochlear implants; new
perspectives include regeneration of the neural epithelium and ganglion neurons by gene
therapy and implantation of stem cells (5).
It has been demonstrated that an universal newborn hearing screening (UNHS) program,
based on automated hearing screening with the use of otoacoustic emissions (A-OAE) and
auditory brainstem responses (AABR), reduces the negative impact of HL (6, 7). Both
procedures are non- invasive, quick and easy to perform.
Therefore, independently from the screening protocol in use, infants who were born in a
hospital with an established screening program had significantly better outcomes than
children who were born in hospitals that did not screen (4), as newborns with HL in UNHS
programs can be identified earlier and receive earlier intervention. Nevertheless, there are
several open discussion topics such as the technologies used in the screening protocol,
the personnel that should performing the examinations and the cost effectiveness ratio
(8).
The most recent guidelines of the Joint Committee on Infant Hearing (JCIH), outlined in
2007, recommend an implementation of the screening programs (9).
San Camillo Forlanini Hospital (SCFH) is the second biggest public hospital in Rome
with an emergency department that serves as an hub for all emergency and complicated
cases referred by the entire southern region of Latium. SCFH birth point manages more
than 3000 births per year. Internal training program directed to nurses and paramedics
who work in the birth point with the aim to correctly understand and manage the basics of
UNHS started in 2011. Two years later, on May 21st, 2013, regional healthcare authorities
approved law 104/2013 that required all birth points in the region to start UNHS through ATEOAE (10,11). Therefore SCFH introduced a UNHS to all newborns before discharge.
The aim of this retrospective study was to report and discuss the results of the first two
years of UNHS in our hospital, and to highlight the major problems that we faced during
the set up and initial phase of the program, as well as the effectiveness of our screening
protocol to identify patients with potential hearing loss .
66) ( figure 1 ) . Study Procedures: UNHS procedures were divided into first and second
level .
figure 1
4345
185
66
123
Indoor Nursery
NICU
NDP
Different
hospital
4719 test
1st level
For the first level we used Automatic Transient Otoacustic Emissions (A-TEOAEs)
(Accuscreen GN resound) : evaluation method : noise weighted averaging counting of
significant signal peaks ; stimulus : non- linear click sequence ; stimulus level : 70 db spl
(45db hl) , self -calibration depending on ear canal volume ; click rate : 60 Hz ; frequency
range : 1,5 to 4,5 kHz ; display : statistical waveform, measurement progress , TEOAE
detection level , noise level .
This level was divided in two stages : in the first stage, Automatic Transient Otoacoustic
Emissions (A-TEOAEs) were performed to all newborns 2-3 days after birth. Exam was
performed placing the ear plugs in both ears, one ear at a time, in the nursery, NICU or
NDP by a well-trained nurse during sleep or at the end of feeding. Personal identification
code, birth conditions and history for hearing loss were tracked for each patient and the
record was added to an internal database. After testing, based on A-TEOAEs results,
newborns were divided into two categories:
Babies without hearing risk factors with a bilateral PASS response were considered
to have normal hearing; information on progressive genetic hearing loss or auditory
neuropathy were provided to the family.
Babies without risk factor for hearing loss , with a unilateral or bilateral REFER
response were addressed to 1st level 2nd stage with a new A-TEOAE test in the
pediatric department one week after the discharge (second stage).
2nd level
In the second level we use Automatic Auditory Brainstem Response (A-ABR)
(Accuscreen GN resound) : evaluation method : noise weighted averaging and
semplase masking ; stimulous : 40 db nHL click ; click rate : approx. 80 Hz ; impedance
sense signal : 1 kHz square wave ; impedance test range : 1 to 99 k ; impedance
accepted for test < 12 k ; impedance control : before test , periodically during test
stimulous continues during impedance control ; display : statistical graph , test progress ,
EEG level , ABR detection probability .
Babies with a bilateral PASS response but with a positive history for hearing loss, risk
infection (appendix 1) or risk factors for auditory neuropathy were suggested to monitor
hearing function through A-TEOEAs, automatic ABR (A-ABR) , tympanometry and acustic
reflex (impedance Amplaid A766) at regular intervals every six month for the first three
years then every twelve months for the following three years. This exam reduces the false
positive cases and individuates the late or progressive hearing loss . Babies with a REFER
response at first level second stage testing were addressed to 2nd level to undergo
additional hearing exams. All babies were tested into the third months of life . Based on
the results of these exams, subjects were divided into:
Babies with normal A-ABR at 40 dB nHL with the presence of a normal wave
morphology and latency were considered to have normal hearing ( without risk
factors).
Babies with abnormal A-ABR (unilateral or bilateral) underwent to clinic ABR test
after one month to exclude incomplete neuronal development that could be
responsible for abnormal A-ABR waves.
Diagnostic level
Hearing in patients that showed as REFER in 2nd level was further investigated with
auditory pathway electrophysiological threshold and integrity research (ABR, Epic plus,
Labat). Tests were performed cleaning skin with abrasive paste and applying silver
plated surface electrodes in conventional positions using adhesive tape (black left or
right earlobe , red Fz , green breastbone ). A non- invasive stimulus (click) was
delivered using soft ear drops; impedance less than 3 Kohms was required. In
accordance to actual ABR criteria we analyzed the absolute latency of waves ( I III V
) , interpeaks latency ( I-III , III-V , I-V ) , interneural difference interpeaks I - V or for V
waves. The electrophysiological threshold was found decreasing the intensity of the
stimulus until the lowest intensity could generate wave V; it should be considered that
this method can identify threshold foot pitches between 2000 and 400 Hz. It is also
necessary to confirm results repeating wave tracking at least twice during the same
session. A baby was considered to have a PASS response for the threshold ABR if a
wave V could be found with at least two repetitions at 60 dB SPL (35 dB nHL) with
normal latency and interpeak values for patient's gestational age. The possible presence
of conductive hearing loss was investigated in all patients, and was diagnosed when
otoscopy showed middle ear disorders such acute otitis media, tympanometry showed
type B or C tympanogram with absent acoustic reflexes, and ABR showed increased
values of waves I , III and V absolute latency with normal I III , III V and I V
interpeaks. The aim of the diagnostic level was to confirm the presence (or the
absence) of hearing loss before the sixth month of age.
Behavioral audiometry ( BA )
BA was also used in our center, but not as a screening tool. In the past, this was the first
method to evaluate the presence of hearing loss; however a major limitation of this
technique is the lack of reliability to evaluate hearing threshold during the first months of
life ( 0-5 ), when objective methods are necessary. From 6 to 36 months of age BA can
be a reliable method to study hearing threshold and hearing ability; it is also fundamental
to quantify the effectiveness of hearing aids and identify lower tone threshold.
RESULTS
In our study we analyzed a period of 24 months (September 2011 September 2013 ) of
UNHS performed in the SCFH . During this timeframe, we screened 4.719 neonates born
in the SCFH (4596 - 97,39 % ) and in other smaller birth centers of our region (123 2,61%). At the 1st level 1st stage 4465 newborns had a PASS response at TOAEs
(94,61%), while 254 (5,39%) had a REFER response. Among those,108 (2,29%) in both
ears and 146 (3,10% ) in 1 ear (56 right ear and 90 left ear) (figure 2) .
Figure 2
4465 Pass
254 Refer
56 R
90 L
108 B
Of those babies that were addressed to the 2nd stage, 130 (51,1 % ) had a PASS TOAEs
response and 48 (18,8 %) had a new REFER response: 27 (10,6 %) in both ears and 21
(8,2 %) in 1 ear (7 right ear and 14 left ear). 76 babies (29,9 %) dropped out of the
screening protocol for unreported reasons. Surprisingly, only 30 parents reported the
presence of familiar genetic risk factors for hearing loss (figure 3).
Figure 3
76 drop- out
178 test
130 pass
48 refer
7R
14 L
27 B
146 babies (3,09 %) were addressed to 2nd level testing using A-ABR : 48 for a REFER
response during 1st level 2nd stage testing while 98 for a PASS response but potential
hearing loss due to pregnancy risk factors. Among those, 122 babies were studied with AABR while 24 dropped out (16,43 %). Out of 98 pass newborns studied (83,5 %) (all
confirmed pass), 7 (5,73%) had a PASS A-ABR response (80,32 % including all the 122
babies tested) and 17 (13,9 %) were REFER (10 in both ears and 7 in one ear - 3 right ear
and 4 left ear). It should be noted that 100% of A-ABR REFER patients were from the 1st
level 2nd stage REFER group (figure 4).
Figure 4
24 drop- out
24 test
7 pass
17 refer
3R
4L
10 B
The 17 babies that failed 2nd level were further studied in diagnostic stage with ABR to
find hearing threshold. Among those, 8 babies had normal hearing threshold, while in 5
threshold could not be found in one ear (3 right ear and 2 left ear) and in 2 in both ears.
For these baby with bilateral hearing loss, anamnestic investigation showed that no one
had risk factors for hearing loss. 2 babies dropped out and parents could not be contacted
(figure 5) .
Figure 5
2 drop -out
15 test
8 pass
7 refer
3R
2L
2B
Work in progress : From May 2013 to May 2014 thanks to a better formation of the nurse ,
a major collaboration with neonatology and a new regional law that improve the UNHS ,
the percentage of babies that underwent UNHS is about 95% like the international
organism guidelines report (NIH, AAP, JCIH ).
DISCUSSION
It is universally recognised that the most important indicators of success of an Universal
Hearing Screening Program are the numbers of cases of hearing loss that are diagnosed
and treated early (12,13). Literature reports an estimated prevalence of moderate, severe
and profound Sensorineural Hearing Loss (SNHL) among newborns between 1/900 and
1/2500. Congenital SNHL is associated with delayed language, learning and speech
development (14, 15). However, without a screening program, hearing impairment in
children is not easily detectable at an early stage and could therefore result in a delayed
diagnosis. Instead, the early use of available therapies, such as speech and language
therapy in association with an adequate amplification, could reduce the gap in language
skills between deaf and hearing children (16, 17). The main well known problem is the
heterogeneity in criteria for referring to further examinations during the screenings phase
and in identifying high-risk neonates, protocol and tests (18) . About protocol it exists
numerous different protocols : 2 teoae then abr (19) , 2 or 3 teoae then abr (20) , 3 teoae
then aabr (21) , 1 teoae then abr (22) or 2 teoae then aabr (23,24) (the one we use) and
more .The reason we decided to use teoae like first step is because it's accurate,
economic , simple in execution (25) and appropriate to test in babies without risk factors
like demonstrate over 100,000 screening tests (26). Also a initiating a 2-stage aabr
screening protocol with an ear insert technique may be impracticable in newborn nurseries
given the greater number of false positive cases (27) . For this reason we agree to
reserve aabr not only for infants who fail the initial screening but also for the ones with risk
factors of hearing impairment (28) .
The coverage rate of newborn hearing screening was generally near 100% in occidental
countries : 99% (29) / 98%(30) in USA and 99 % (31) / 98% (32) in France . In our study,
the coverage rate was 76,11 % ( 4719 / 6200) like in Korean (75%) (33) . In our country
UNHS coverage had undergone a steep increasing during years from 29.3% in 2003
(156,048 newborns screened) to 48.4% in 2006 (262,103 screened) (34) . For the majority
implementation set in the two most economically developed areas : north-west (79.5%,
108,200 of 136,109 births) and north-east area (57.2%, 52,727 of 92,133 births) while a
limited diffusion still remains in some areas, typically in the islands (11.3%, 7158 of 63,460
births) (34) . Fortunately this dates are modified in the last ten years like the 80% of
screened babies shown in the Campania region (south of Italy) (34) . In our date the low
percentage is a consequence of a preliminary approach to UNHS before the approval of a
regional guideline (2013) . In fact from May 2013 to May 2014 thanks to a better formation
of the nurse , a major collaboration with neonatology and a new regional law , the
percentage of babies that underwent UNHS is major than 95% like the international
organism guidelines report (NIH, AAP, JCIH ). In some way , even though thirty years later
than the lions club in Mississipi (35) , we try , in small scale, to improve healthy in our
country .
Our data show that REFER tests at 1st level 1st stage were 254 then became 48 2nd
stage . This reduction justifies the existence of 1st stage 2nd level . The AABR REFER tests
during 2nd level was 17 : 5 unilateral hearing loss and 2 bilateral hearing loss without risk
factors . Also aabr investigation is useful for identify patients with auditory neuropathy in
neonates with audiological risk factors and to re-test the babies whose otoacoustic
emissions were referred (13,14,28).
The two UNHS main problem remains :
- Drop out babies
The alarming percentage of newborns who fail the initial testing and then are lost, this is a
common contemporary problem (36) ( especially in country where the screening procedure
is not mandatory , recent beginning or in NICU babies (37) ) . In order to fight this situation
we introduced from 2011 a targeted training to the personnel who was included in the
screening protocol, and also provided an appropriate education about newborn hearing
screening and the risk factors of hearing loss to pediatricians, neonatologists and
gynecologists . The issue of the elevate number of drop-out patients (2,16 %) highlights
the importance of a serious cooperation between audiological centers, maternity units and
families. A correct training of family pediatricians is also crucial for a correct follow-up for
children with potential progressive hearing loss. Similarly to other screening programs, a
key factor in the UNHS is its diffusion to the largest extent of the possible population, to
identify hearing problems at an early stage and to allow diagnosis and intervention within
the first 6 months of age, thus reducing the disability of deafness. Based on our data, the
incidence of hearing loss in this study was 1,48/1.000 newborns, however the relevant
number of drop out patients (n. 102) and the low incidence of bilateral (0,084 %) and
unilateral (0.105 %) hearing loss , compared to other studies in literature ,it could suggest
a higher number .
- Auditory neuropathy
It is a rare disease , jet unknown , that can affect pass teoae babies and compromise their
growing .Much more than a single periferic acoustic disorder, it is a broad of
heterogeneus disorders characterised by integrity of outer hair cells . We recommend at
each one parents of a TEOAE pass babies to take another control, calling our switchboard
, if they fell something strange about the hearing of the son . We think that this is actually
the only system to identify auditory neuropathy's patients without risk factors , at least until
the international community will find a more appropriate and cheaper screening methods .
CONCLUSIONS
The UNHS and hearing pediatric sensibility needs to be improved : no country have 100%
of screened babies. Although we are not able to affirm that our is the best world protocol,
we are convinced that is the most appropriate for our hospital NOW . Although actually
the first teoae level performed after birth and before discharge guarantees the 100% of
first level TEOAE screening , however the drop -out and auditory neuropathy remain a
real diagnostic problems .
Acknowledgment
To Livia Mammarella and Massimo Ralli (MD , Catholic University of Sacred Hearth
Rome) for their help in translation
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