10.14639/0392-100X-N1162
Objectives. We have clarified the role of Universal Neonatal Hearing Screening (UNHS) for both early diagnosis and rapid treatment in order to improve the prognosis of the deaf child and reduce patient management costs. Although in Sicily UNHS has been progres- sively implemented, there is scarce data in the literature on this matter. Therefore, the main objective was to collect in the year 2018 the following data: number of newborns screened for hearing loss, number of infants “referred” to transiently evoked otoacoustic emissions (TEOAE), number of infants with pathologic auditory brainstem response (ABR) and num- ber of infants affected by permanent hearing loss.Methods. UNHS monitoring was conducted through the collection of data through a ques- tionnaire, which was analysed evaluating the effectiveness and adherence to the screening program prepared by the Department for Health Activities and the Epidemiological Obser- vatory (DASOE).Results. In 2018, there were 40,243 newborns in Sicily. A total of 37,562 newborns were screened (93.3%). There were 1,328 “referred” infants with TEOAE (3.5%). On the 2nd lev- el, “referred” newborns examined were 1,080 of 1,328 expected (missing 248 “refer” new- borns, equal to 18.6%). The number of “referred” infants confirmed with TEOAE was 113 of 1,080, while “referred” infants confirmed with ABR were 71. On the 3rd level, 67 of 71 were infants examined: 28 infants were suffering from monolateral hearing loss (13 slight/ mild, 13 moderate, 1 severe and 1 profound) and 39 from bilateral hearing loss (1slight/ mild, 19 moderate, 13 severe and 7 profound). Excluding 7 infants from the NICU, 60 of&nbsp;37,562 infants had hearing loss (1.5%).Conclusions. The monitoring of the UNHS in Sicily has allowed obtaining the data of in- dividual centres, absent in the literature to date, to verify the effectiveness of the screening, according to JCIH criteria, to highlight some criticalities and, finally, to propose possible&nbsp;solutions.
text/xml
1827-675X
1162
en
Luciano Bubbico
http://creativecommons.org/licenses/by-nc-nd/4.0
ACTA Otorhinolaryngologica Italica
congenital deafness
Universal newborn hearing screening in the Italian Region of Sicily in 2018
Text.Serial.Journal
https://www.actaitalica.it/
41
Audiology
2021-09-14
2020-10-05
2021-09-15
2021-09-15
356-363
1
Luciano Bubbico
2021/09/14
10.14639/0392-100X-N1162
356
Neurosensorial Disability Research, INAPP/ Italian Institute of Social Medicine, Rome, Italy
1827-675X
congenital deafness
en
363
Universal newborn hearing screening in the Italian Region of Sicily in 2018
https://www.actaitalica.it/article/download/1162/655
41
ACTA OTORHINOLARYNGOLOGICA ITALICA 2021;41:356-363; doi: 10.14639/0392-100X-N1162




Audiology


Universal newborn hearing screening in the Italian
Region of Sicily in 2018
Screening uditivo neonatale universale nella regione Sicilia nel 2018
Salvatore Ferlito1, Antonino Maniaci1, Salvatore Cocuzza1, Ignazio La Mantia1, Paola Di Mauro1, Graziella Poli1,
Luigi Maiolino1, Salvatore Coco1, Federico Merlino1, Marina Maltese1, Margherita Ragliani1, Michele Russo1,
Alessandro Gulino1, Carmen Azieli2, Francesco Martines3, Francesco Galletti2, Luciano Bubbico4
1
  Department of Medical and Surgical Sciences and Advanced Technologies “GF Ingrassia,” ENT Section, University of Catania,
Catania, Italy; 2 Department of Adult and Development Age Human Pathology “Gaetano Barresi”, ENT Section, University of
Messina, Messina, Italy; 3 Department of Experimental Biomedicine and Clinical Neurosciences, University of Palermo, Italy;
4
  Neurosensorial Disability Research, INAPP/ Italian Institute of Social Medicine, Rome, Italy


SUMMARY
Objectives. We have clarified the role of Universal Neonatal Hearing Screening (UNHS)
for both early diagnosis and rapid treatment in order to improve the prognosis of the deaf
child and reduce patient management costs. Although in Sicily UNHS has been progres-
sively implemented, there is scarce data in the literature on this matter. Therefore, the main
objective was to collect in the year 2018 the following data: number of newborns screened
for hearing loss, number of infants “referred” to transiently evoked otoacoustic emissions
(TEOAE), number of infants with pathologic auditory brainstem response (ABR) and num-               Received: October 5, 2020
ber of infants affected by permanent hearing loss.                                                  Accepted: January 4, 2021
Methods. UNHS monitoring was conducted through the collection of data through a ques-
tionnaire, which was analysed evaluating the effectiveness and adherence to the screening           Correspondence
program prepared by the Department for Health Activities and the Epidemiological Obser-             Paola Di Mauro
vatory (DASOE).                                                                                     University of Catania. ENT Clinic, Department
Results. In 2018, there were 40,243 newborns in Sicily. A total of 37,562 newborns were             of Medical and Surgical Sciences and Advanced
screened (93.3%). There were 1,328 “referred” infants with TEOAE (3.5%). On the 2nd lev-            Technologies “G.F. Ingrassia”. Azienda Ospedalie-
el, “referred” newborns examined were 1,080 of 1,328 expected (missing 248 “refer” new-             ra Universitaria “Policlinico Vittorio Emanuele”,
borns, equal to 18.6%). The number of “referred” infants confirmed with TEOAE was 113               University of Catania
                                                                                                    Santa Sofia street n. 78, 95123 Catania, Italy
of 1,080, while “referred” infants confirmed with ABR were 71. On the 3rd level, 67 of 71
                                                                                                    E-mail: paola_mp86@hotmail.it
were infants examined: 28 infants were suffering from monolateral hearing loss (13 slight/
mild, 13 moderate, 1 severe and 1 profound) and 39 from bilateral hearing loss (1slight/
                                                                                                    Funding
mild, 19 moderate, 13 severe and 7 profound). Excluding 7 infants from the NICU, 60 of              None.
37,562 infants had hearing loss (1.5%).
Conclusions. The monitoring of the UNHS in Sicily has allowed obtaining the data of in-             Conflict of interest
dividual centres, absent in the literature to date, to verify the effectiveness of the screening,   The Authors declare no conflict of interest.
according to JCIH criteria, to highlight some criticalities and, finally, to propose possible
solutions.
                                                                                                      How to cite this article: Ferlito S, Maniaci A,
KEY WORDS: neonatal hearing loss, universal newborn hearing screening, congenital                     Cocuzza S, et al. Universal newborn hearing
deafness                                                                                              screening in the Italian Region of Sicily in 2018.
                                                                                                      Acta Otorhinolaryngol Ital 2021;41:356-363. htt-
                                                                                                      ps://doi.org/10.14639/0392-100X-N1162
RIASSUNTO
Obiettivi. Abbiamo chiarito il ruolo dello screening uditivo neonatale universale (UNHS)            © Società Italiana di Otorinolaringoiatria
sia come diagnosi precoce che come trattamento rapido al fine di migliorare la prognosi             e Chirurgia Cervico-Facciale
del bambino sordo e ridurre i costi di gestione del paziente. Anche se in Sicilia l’UNHS è
stato progressivamente attuato, vi sono scarsi dati nella letteratura in materia. Pertanto,                        OPEN ACCESS
l’obiettivo principale è stato quello di raccogliere nell’anno 2018 i seguenti dati: numero di      This is an open access article distributed in accordance with
neonati sottoposti a screening per la perdita dell’udito, numero di neonati risultati “refer”       the CC-BY-NC-ND (Creative Commons Attribution-Non-
alle TEOAE, numero di lattanti con ABR patologico e numero di lattanti affetti da perdita           Commercial-NoDerivatives 4.0 International) license. The
                                                                                                    article can be used by giving appropriate credit and mentio-
permanente dell’udito.
                                                                                                    ning the license, but only for non-commercial purposes and
Metodi. Il monitoraggio del UNHS è stato condotto attraverso la raccolta dei dati forniti           only in the original version. For further information: https://
dal questionario, che valuta l’efficacia dello screening e l’aderenza alle fasi successive.         creativecommons.org/licenses/by-nc-nd/4.0/deed.en




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Risultati. Nel 2018, i nati in Sicilia sono stati 40.243. Sono stati sottoposti a screening 37.562 neonati, pari al 93,3%. Sono risultati “refer”
con le TEOAE 1.328 neonati (pari al 3,5%). Al 2° livello, i neonati “refer” esaminati sono stati 1.080 su 1.328 attesi (dispersi 248 neonati refer,
pari al 18,6%). I neonati “refer” confermati con le TEOAE sono stati 113 su 1.080, mentre i neonati “refer” confermati con ABR sono stati 71.
Al 3° livello, i bambini esaminati sono stati 67 su 71 previsti: ventotto bambini soffrivano di perdita monolaterale dell’udito (13 lieve, 13 mo-
derata, 1 grave e 1 profonda) e trentanove soffrivano di perdita bilaterale dell’udito (1 lieve, 19 moderata, 13 grave e 7 profonda). Escludendo
7 bambini provenienti dalla NICU, i bambini con perdita dell’udito sono stati 60 su 37.562 (pari all’1,5‰).
Conclusioni. Il monitoraggio dell’UNHS in Sicilia ha permesso di ottenere i dati dei singoli centri, dati assenti nella letteratura, di verificare
l’efficacia dello screening, secondo criteri JCIH, di evidenziare alcune criticità e, infine, di proporre possibili soluzioni.
PAROLE CHIAVE: ipoacusia neonatale, screening universale uditivo neonatale, sordità congenita



Introduction                                                               In 2016, 2,160 (5.7%) were registered as “referred” out of
                                                                           a population of 37,250 newborns, of whom 95 were diag-
Significant permanent hearing impairment (more than 30-
                                                                           nosed with deafness (2.5 per 1,000) 19.
40 dB HL in both ears) is the most common sensory dis-                     From these reported rates it follows that, in order to evalu-
ability, and represents a relevant problem not only for the                ate the efficiency of the UNHS correctly, it is necessary
health of the child, but also for the economic and social                  not only to evaluate the coverage rate, but equally essential
costs that follow 1-3.                                                     to know information relating to the rate of “referred” and
Significant bilateral hearing impairment, if undetected, can               newborns diagnosed with hearing loss.
cause profound effects on speech, language and cognitive                   Through these assumptions, we have developed and pro-
development, and can thus hamper emotional and social                      posed a questionnaire sent to birth centers and Otolaryngol-
well-being 4-6.                                                            ogy Units in Sicily in the year 2018 to collect the following
In Italy and Western countries, it is estimated between 0.5                data: number of newborns screened for hearing loss, num-
and 2 cases per 1,000 live births have profound hearing                    ber of infants resulted refer to TEOAE, number of infants
loss 5-7. Nevertheless, in some paediatric subpopulations,                 with pathologic ABR and number of infants affected by
the rates are significantly higher (about 4%), especially in               permanent hearing loss.
the presence of risk factors or in neonates hospitalised in
the Neonatal Intensive Care Unit (NICU) 6-12.
The latest universal screening diagnostic protocols using                  Materials and methods
TEOAE and automated auditory brainstem response (A-                        The search covered the 9 Sicilian provinces: Palermo, Cat-
ABR) have shown a sensitivity (percentage of children                      ania, Messina, Agrigento, Trapani, Ragusa, Caltanissetta,
with abnormal hearing who fail the test) close to 100% and                 Siracusa and Enna. The screening was performed following
specificity above 90% 13-16.                                               the protocol designed by DASOE 19. The UNHS program is
To be successful, a neonatal hearing screening programme                   structured in three levels, each performed in an adequately
should be universal (i.e., include all neonates), because                  equipped centre.
selective screening, based on high-risk criteria, detects at               The first level is represented by public and private birth
most half of all infants with congenital hearing loss 17.                  points. The second level is represented by the Audiology
A further control process is the one modified in 2019 by                   Services, autonomous or aggregate to ENT-Unit. The Re-
JCHI which, noting promising results from the respect of                   gional Reference Centres represent the third level.
benchmarks 1-3-6 (complete screening within 1-month,                       According to the screening protocol designed by DASOE,
audiological diagnosis within 3 months, early intervention                 all birth points must screen all newborns by searching for
within 6 months), invited the audiological reference centres               (TEOAE), during spontaneous sleep and before hospital
to perform a reduced temporal sequence 1-2-3 18.                           discharge (generally, within 48-72 hours).
Although in Sicily the UNHS has been progressively im-                     “Pass” test infants leave the screening process, while “re-
plemented since 2011 and currently the coordination is en-                 ferred” infants (mono or bilaterally) must be re-examined,
trusted to the Department for Health Activities and the Epi-               within the first month of life, with TEOAE. If the test result
demiological Observatory (DASOE), there is scarce data                     is “referred”, it is necessary to perform the ABR at the Au-
in the literature on this matter. Interestingly, from 2011 to              diology Services (Level 2). In case of “referred” confirma-
2017, the percentage of children screened increased every                  tion, the newborn is sent to the Regional Reference Centre
year as follows: 6%, 18%, 32%, 57%, 87%, 92%, 98%.                         (Level 3) within the third life month for definitive diagno-
However, in 2018 a lower coverage of 37,303 (91.6%) out                    sis, and starting prosthetic rehabilitation no later than the
of 40,645 screened newborns was reported 19.                               sixth month of life.



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S. Ferlito et al.




The infants hospitalised at the NICU, or the ones who bi-                       tem. The collected data cannot be traced back to a specific
laterally “pass” both screening tests (TEOAE and ABR),                          patient according to the privacy rights of the children and
but who have risk factors for late-onset hearing loss, are                      their families.
sent to level II centres for an audiological surveillance pro-                  In the absence of regional indicators of screening efficien-
gramme that includes assessment every 6-12 months in the                        cy, the data collected in the questionnaire were analysed
first three years of life (Tab. I).                                             considering the indicators recommended by JCIH, which
UNHS monitoring was conducted through the collection of                         allow evaluating the effectiveness of the screening and ad-
data from a questionnaire sent to and filled in by the UNHS                     herence to the subsequent phases.
program’s coordinator of each birth point of the National                       More specifically, the quality indicators for screening and
Health System (NHS) in Sicily in the year 2018. All coor-                       confirmation of deafness are the following:
dinators invited to participate in the surveys returned the                     • the percentage of infants who completed the screening
questionnaires filled in. For each birth point participating in                    should be more than 95% of the neonatal population
the survey, the following figures were collected:                                  during the first month of life;
1. total births in the year;                                                    • the percentage of infants, without risk indicators, who
2. number of newborns screened for hearing loss;                                   passed neither the initial testing nor the subsequent
3. number of infants “referred” to TEOAE;                                          ones - “referred” confirmed should be below 4% of eval-
4. number of infants transferred to NICU, premature, de-                           uated infants;
   ceased.                                                                      • the audiological evaluation must be completed in at least
For each ENT-Unit (level 2), participating in the survey, the                      90% of children who do not pass the screening.
following figures were collected:                                               Hearing loss can be classified or defined in many ways
1. number of expected infants resulting “refer” and their                       and categories. This study has used the classification of the
   birth point;                                                                 WHO: slight/mild (26-40 B), moderate (41-60 dB, severe
2. number of confirmed infants “referred” to TEOAE;                             (61-80 dB) and profound (over 81 dB). In the case of mod-
3. number of infants subjected to ABR;                                          erate hearing loss, the range for children is 31-60 dB.
4. number of infants with pathologic ABR.
For each Regional Reference Centre (level 3) participating                      Results
in the survey, the following figures were collected:
1. number of infants affected by monolateral hypoacusis                         This report shows the data relating to UNHS of all birth
   (mild, moderate, severe and profound);                                       points of Sicily in the year 2018.
2. number of infants affected by bilateral hypoacusis (mild,                    The data relating to the activity of the UNHS included 46
   moderate, severe and profound).                                              of 47 birth points active in 2018, of which 36 public hospi-
The data collected by this survey, expressed in numbers,                        tals, 3 University-Hospitals (Palermo, Catania and Messi-
concern procedural aspects and are used for the generation                      na) and 7 private structures affiliated with the NHS.
of reports useful for the strategic planning of the health sys-                 The birth points of Bronte and Biancavilla (province of
                                                                                Catania) were unified in the chart (Tab. II), while the data
                                                                                of the birth point of Pantelleria were not received (number
Table I. Risk indicators associated with permanent congenital hearing loss in   of newborns < 50).
childhood (DASOE, 2016) 19.                                                     Table II shows the data for each birth point: in total there
 Family history of permanent childhood hearing loss                             were 40,243 newborns, of which 37,562 were screened
 Birth weight less than 1500 g                                                  (equal to 93.3%) and, of these, 1,328 infants resulted “re-
 Neonatal intensive care                                                        ferred” to TEOAE (equal to 3.5%) and were sent to the 2nd
 Hyperbilirubinemia that requires exchange transfusion                          level.
 Exposure to ototoxic medications                                               Only a few coordinators have reported the number of new-
 In utero infections, such as cytomegalovirus, herpes, rubella, syphilis, and   borns transferred to NICU, the premature ones and the de-
 toxoplasmosis                                                                  ceased ones. The territorial screening coverage, a key indi-
 Craniofacial anomalies, including those that involve the pinna, ear canal,     cator to assess the adherence to the program, was 93.3%,
 ear tags, ear pits                                                             above 92% reported by DASOE, but still below the interna-
 Syndromes or physical findings, such as white forelock, that are associated    tional standard (95%).
 with hearing loss
                                                                                In the years 2015, 2016 and 2017, the territorial coverage
 Neurodegenerative disorders or sensory motor neuropathies                      had been, respectively, 87%, 93% and 98% 19.
 Meningitis                                                                     It should be noted that, in the year 2018, the screening cover-



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Table II. Overall data for hospitals.
 Birth points                      Borns                       TEOAE                  % screened                   Refer                   % refer             Trasf/Prem/Dec
 Palermo-Poi.                        611                         608                    99.5%                       18                      3.0%                      ND
 PA-Arnas C.                        1374                        1193                    86.8%                       13                      1.1%                      ND
 PA- Buccheri                       2050                        2046                    99.8%                       84                      4.1%                      ND
 PA-CDC Zancla                      1123                        1106                    98.5%                       12                      1.1%                      16
 PA-CDC Serena                      1014                        1014                   100.0%                       28                      2.8%                      ND
 PA-lngrassia                        578                         567                    98.1%                        9                      1.6%                      ND
 PA-Cervello                        1617                        1550                    95.9%                       11                      0.7%                      ND
 PA-CDC Candela                     1176                        1150                    97.8%                       13                      1.1%                   20-0-0
 Corleone                            225                         225                   100.0%                        1                      0.4%                      ND
 Partinico                           452                         296                    65.5%                        2                      0.7%                      ND
 Cefalu’                             400                         385                    96.3%                        1                      0.3%                      ND
 Termini lmerese                     614                         602                    98.0%                        5                      0.8%                      12
 Catania-Po I                       2009                        1692                    84.2%                       267                    15.8%                      ND
 CT-Cannizzaro                      1276                        1101                    86.3%                       23                      2.1%                   27-0-0
 CT-S.Bambino                       1818                        1658                    91.2%                       246                    14.8%                    5-0-6
 CT-Garibaldi Nes                   2082                        2082                   100.0%                       64                      3.1%                      ND
 Acireale                            524                         198                    37.8%                         o                     0.0%                      ND
 Biancavilla-Brante                  689                         603                    87.5%                        2                      0.3%                    4-0-0
 Caltagirone                         587                         438                    74.6%                        7                      1.6%                      ND
 CDC Gretter                         568                         568                   100.0%                        6                      1.1%                      ND
 CDC Falcidia                        808                         795                    98.4%                        16                     2.0%                   13-0-0
 Messina Poi                        1404                        1281                    91.2%                       155                    12.1%                  91-ND-ND
 Me-Papardo                          689                         640                    92.9%                        3                      0.5%                   0-74-2
 Patti                               843                         839                    99.5%                        1                      0.1%                    3-0-1
 Milazzo                             714                         492                    68.9%                        1                      0.2%                    5-0-0
 Sant’Agata M.                       307                         307                   100.0%                        1                      0.3%                      ND
 Taormina                            549                         274                    49.9%                        7                      2.6%                   16-0-0
 Ragusa                             1464                        1443                    98.6%                       14                      1.0%                    9-2-6
 Vittoria                            880                         838                    95.2%                        2                      0.2%                   35-0-5
 Modica                              856                         832                    97.2%                        66                     7.9%                   33-0-0
 Agrigento                          1523                        1484                    97.4%                       128                     8.6%                      ND
 Canicattì                           611                         586                    95.9%                        9                      1.5%                      ND
 Licata                              270                         265                    98.1%                       ND                       ND                       ND
 Sciacca                             607                         595                    98.0%                         8                     1.3%                    8-0-2
 Trapani                             961                         950                    98.9%                        12                     1.3%                   0-12-0
 Marsala                             530                         522                    98.5%                        3                      0.6%                    8-0-0
 Castelvetrano                       376                         332                    88.3%                        21                     6.3%                      ND
 Mazzara del Vallo                   463                         431                    93.1%                        26                     6.0%                      ND
 CDC-Sant’Anna                       510                         506                    99.2%                        16                     3.2%                    4-0-0
 Siracusa                           1473                        1473                   100.0%                       ND                       ND                       ND
 Lentini                            1113                        1113                   100.0%                       ND                       ND                       ND
 Caltanissetta                       582                         567                    97.4%                         3                     0.5%                   15-0-0
 Gela                                757                         751                    99.2%                         7                     0.9%                    6-0-0
 Enna                                975                         975                   100.0%                        17                     1.7%                      ND
 Nicosia                             191                         189                    99.0%                         o                     0.0%                    2-0-0
 TOTAL                            40,243                       37,562                   93.3%                      1,328                    3.5%
Borns: Total number of births in the year; TEOAE: Transient Evoked Otoacoustic Emissions screened infants; % Screened: Relation between number of screened infants and borns;
Refer: Infants number resulted Refer to TEOAE and sent to the ENT Unit 2nd level; % Refer: Relation between number of infants resulted Refer and screened; Trasf/Prem/Dec: Number
of infants transferred to NICU, premature, deceased; Palermo-Pol: Policlinic of Palermo; PA: Palermo; CdC: Nursing home; Catania-Pol: Policlinic of Catania; CT: Catania; ME: Messina;
PA-Arnas C: ARNAS Civic Hospital of Palermo; CT/ S. Bambino: S. Bambino Hospital of Catania; Garibaldi Nes.: Garibaldi Hospital of Catania; Messina-Pol.: Policlinic of Messina; S.
Agata M.: Sant’Agata di Militello (ME) Hospital; ND: Data not available.




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S. Ferlito et al.




age was more than 95% in 7 of 9 provinces, while it was lower          The Neonatology Unit of Santo Bambino Hospital of Cata-
in the provinces of Catania and Messina; the percentage of             nia screened 1,658 infants out of 1,818 (91.1%): 1,405
screening in the provinces of Catania and Messina was 88.2%            (84.7%) at the Nursery and 253 (15.3%) at NICU. New-
and 85.1% respectively. The reasons for these low percentages          borns resulted “referred” were 246 (14.8%) out of 1,658: of
reported by the persons in charge of the birth points were de-         which 211 (85.8%) at the Nursery and 35 (14.2%) at NICU.
vice breakage and temporary absence of trained personnel.              Fifty-seven infants did not show up for the test, which had
In 2018, the percentage of infants “referred” to the test and          been postponed due to the temporary absence of staff.
retest was 3.5%, equal to 1,328 children. This data, which is          The neonatology data of the Policlinic of Messina were re-
not present in literature, satisfies the quality indicator (4%);       covered thanks to the ENT Unit: 1.281 infants out of 1,404
however, in 7 birth points, it exceeds the threshold value.            have been screened (91.2%). The infants resulted “referred”
The Neonatology Unit of the Catania Policlinic screened                were 155 (12.1%). Newborns transferred to the NICU were
1,692 infants out of 2,009 (84.2%): 1,482 (73.7%) at the               91. Moreover, it was not possible to trace the causes that
Nursery and 210 (10.4%) at the NICU. Newborns result-                  led to the dispersion of the “referred” babies due to the lack
ed “referred” were 267 (15.8%) of 1,692: of which 262                  of a paper or computer tracking system.
(15.5%) at the Nursery and 5 (0.3%) at NICU.                           On the 2nd level (Tab. III), “refer” newborns examined


Table III. Overall data on the 2nd and 3rd level.
                                        Level 2                                                      Level 3
         ENT             Newborn examined           Refer   ABR      ABR            Monolateral                    Bilateral
         Unit                   and                 TEOAE   perf.   Pathol.         Hearing loss                 Hearing loss
                            Birth points                                       L     M       S      P      L      M       S     P
 Palermo                 15 - A.O.U. Policlinico     6       11       3                                           2             1
 A.O.U. Policlinico        18 - ARNAS Civico         7       17       2                                                   2
 P. Giaccone
                         21 - Buccheri/LaFerla       6       21       6               3                           2       1
                              9 - Ingrassia          4        9       3       ND
                              31 - Cervello          8       16       6               2                           4
                             6 - CdC Zancla          4        6       2                                           1       1
                             8 - CdC Serena          2        8       2       1                                   1
                            5 - CdC Candela          1        5       1                                                   1
                        1 - CdC Sant’Anna Erice      0        1       0
                          1 - Mazara del Vallo       1        1       1                                                   1
                                1 - Licata           1        1       1                             1
                          8 – Civile Termini Im.     1        3       1                                           1
                          4 - Osp di Agrigento       2        4       1                                                   1
                           3 - Giglio di Cefalù      1        3       1                                           1
                            1 - Castelvetrano        1        1       1                                                   1
                           1 - Osp di Trapani        0        1       0
                         1 - Ospedale di Enna        1        1       1                                           1
 Termini Im.              5 - Cimino Term. Im.       5        5       0
 A.O. Cimino             9 - Ingrassia Palermo       9       12       3                                           1       2
                         1 - Civico di Corleone      1
                         2 - Civico di Partinico     2
 Catania                    267 - Policlinico        1        1       1                                                         1
 A.O.U. Policlinico      210 - NICU Policlinico      5        3       1               1
                           170 - S. Bambino          3        3       3       1       1                    1
                         11 - NICU S. Bambino        0       11       0
                          2 - Garibaldi Catania      2       2        1                                                         1

                                                                                                                          continues u



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Table III. Overall data on the 2nd and 3rd level (follows).
                                        Level 2                                                                                       Level 3
         ENT                Newborn examined                Refer          ABR          ABR                    Monolateral                              Bilateral
         Unit                      and                      TEOAE          perf.       Pathol.                 Hearing loss                           Hearing loss
                               Birth points                                                            L        M         S         P         L         M         S         P
 Catania                      3 - Cannizzaro CT                3             3             0
 A.O.U. Policlinico          5 - ASP di Acireale               5             5             0
                            2 - Biancavilla/Bronte             2             2             1                                                                                1
                             0 - ASP Caltagirone               0             0             0
                               1 - CdC Gretter                 1             1             0
                               0 - CdC Falcidia                0             0             0
                            1 - Umberto I - Enna               1             1             0
                             1 - Civile di Ragusa              1             1             1           1
                               2 - Osp. Lentini                2             2             0
                           1 - Umberto-Siracusa                1             1             1           1
 ARNAS Garibaldi                      ND
 Messina                       125 - Policlinico               7             7             3                     2                                      1
 A.O.U. Policlinico         91 - NICU Policlinico             10            10             6                     3                                      2                   1
 Patti                        1 - Barone di Patti              1            1              1           1
                           1 - Sant’Agata Militello            1            1              1          ND
 Taormina                   2 - Osp. di Taormina               2            2              2                     1                                      1
 Ragusa                      1 - Civile di Ragusa              1            1              1                                                            1
 A.O. Civile                  0 - Osp. di Vittoria             0
                              0 - Osp.di Modica                0
 Agrigento                    6 - Osp. Agrigento               6             6             6           6
 Sciacca                     3 - Osp. di Sciacca               3             3             2                              1                                                 1
 Trapani                      3 - Osp. di Trapani              3             3             1                                                                                1
 A.O.                        1 - Osp. di Marsala               1             1             0
 Sant’Antonio
 Abate                       10 - Castelvetrano               10             6             3           2                                                                    1
                             7 - Mazara del Vallo             7              5             0
                          2 - CdC Sant’Anna Erice             2             2              1                                                                                1
 Siracusa                    0 - Osp. di Siracusa                           26             0
 Umberto I
                                                                                                      13        13        1         1         1        19        10         9
 TOTAL                              1,080                     113          235            71                         28                                     39
                                                                                                                                         67
ENT: Otorhinolaryngology; TEOAE: Transient Evoked Otoacoustic; ABR perf.: ABR performed; ABR pathol.: Pathological ABR; L: slight/mild; M: moderate; S: severe; P: profound; NICU:
neonatal intensive care unit; ND: data not available.



were 1,080 out of 1,328 expected (missing 248 new-                                          these, 6 were confirmed “referred” and inserted in the 3rd
borns). The number of “referred” infants confirmed with                                     level of screening.
TEOAE was 113 out of 1,080 and the number of “refer”                                        On the 3rd level, 67 of 71 infants were examined: 28 infants
infants confirmed with ABR was of 71. In ENT Unit of                                        were suffering from monolateral hearing loss (13 slight/
the Policlinic of Messina, the “referred” infants examined                                  mild, 13 moderate, 1 severe and 1 profound) and 39 from
with the combination of TEOAE and A-ABR were 125 of                                         bilateral hearing loss (1 slight/mild, 19 moderate, 13 severe
145 (20 children contacted didn’t show up), of which 3                                      and 7 profound).
children were confirmed “referred” and inserted in the 3rd                                  The percentage of “referred” newborns at 1st level of screen-
level of screening. All infants from the NICU, 91 in total,                                 ing that ended the diagnostic process was 81.0% (1,080 –
were examined at 2nd level by TEOAE and ABR and, of                                         4 = 1,076 of 1,328).



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S. Ferlito et al.




Excluding 7 infants from the NICU, 60 out of 37,562 in-          communication of data to the regional register should be
fants had hearing loss (1.5‰).                                   well defined.
                                                                 The JCIH states that infants with risk factors should per-
Discussion                                                       form both the TEOAE (often present) and the A-ABR
                                                                 before discharge from the NICU, because the A-ABR is
The adverse effects of childhood hearing loss are mitigated      more sensitive to recognising retrocochlear hearing loss 16.
through universal newborn hearing screening and early            Infants “referred” to one or both ears should be sent to a
action. In a recent systematic review and meta-analysis,         3rd level, within the third/fourth month of life corrected for
conducted in highly developed countries, the prevalence of       gestational age and should undergo periodic audits every
childhood hearing loss was 1.1 per 1,000 screened children       6 months during the first 3 years of life. The JCHI 2019
and was 6.9 times higher among those admitted to NICU 20.        Declaration approved, only for infants with failed A-ABR
In our study, from the overall data of the 1st level, some as-   screening, that the review and transition to TEOAE are ac-
pects of inhomogeneity emerged. In 11 of 46 birth points,        ceptable, given the very low incidence of auditory neuropa-
the activity had been interrupted for a few months, due to       thy in this population 18. Recently, Frezza et al. reported
the TEOAE device breakdown, with consequent less pro-            a trend towards improvement of hearing threshold in 47%
gram adherence. The absence of a secondary device and a          of very preterm infants with normalisation of initial mild-
recovery procedure has caused a large dispersion number          moderate hearing losses at final diagnosis 21.
of newborns. However, a significant number of children did       Furthermore, no diagnostic strategy has been identified re-
not show up on the 1st level check-up. It is necessary to is-    garding the infant with a suspicion of hearing loss due to
sue guidelines to reduce dispersion.                             congenital cytomegalovirus (CMV) infection. As strongly
In 7 of 46 birth points a high percentage of “referred” in-      recommended by a review in the literature, in all infants,
fants was present, which overcomes the quality indicator         referring to the 1st level screening, it is necessary to search
mentioned above (< 4%); some coordinators assumed that           for CMV-DNA in urine no later than 15 days after birth by
it is due to the replacement of health professionals assigned    PCR 22,23.
to the screening.                                                Finally, recording of the screening results is desirable to
In order to minimise the number of infants to be addressed       take place in an online database. The questionnaire high-
to audiological follow-up, an outpatient rescreening proto-      lighted that the 1st level screening operators are not in-
col should be provided within the first month of discharge.      formed about the results of the audiological checks fol-
In the literature, it has been shown that the use of A-ABR       lowing screening. The commitment of all the professionals
in addition to TEOAE in neonates without audiological risk       involved could improve adherence to screening. In the or-
factors can reduce the number of false positives 16.             ganization of the screening, it is crucial to know the rate of
The percentage of “referred” newborns to screening and           “referred” infants who complete the diagnostic process; it
concluding the diagnostic procedure was 81.0%, below the         must be over 90% 16.
benchmark of 90% 16.                                             The Region should implement a data management and
It was not possible to trace the causes that led to the dis-     traceability system to monitor the quality of screening, to
persion of the “referred” babies due to the lack of a paper      measure outcomes and to report on the adequacy of the ser-
or computer tracking system. The paediatrician could be          vice.
useful for this; however, the role of the family paediatri-
cian in neonatal audiological screening and audiological
surveillance is not defined in the regional plan of preven-
                                                                 Conclusions
tion 2014-2018.                                                  The study addresses a very important critical issue, es-
The analysis of the data collected at points of birth under-     pecially in a context in which the mandatory application
line that no distinction was made between newborns with          of UNHS is recent and the data published by the regional
or without risk factors for hearing loss. Therefore, in cen-     reference organisation are scarce and partial. Even in the
tres where births are more numerous or where there is in-        absence of a shared protocol, the UNHS in Sicily is carried
tensive care, a close relationship between the birth point       out, and the direct data collection has made it possible to
and the Audiology centre is desirable.                           verify the screening effectiveness at each birth point and,
Another criticality of data communication was found in           above all, the possibility of making known some data ab-
second and third level centres, in which no discrimination       sent in the literature. However, it is evident that too many
between the group of new-borns from neonatology and the          children are lost between not passing the initial screening
one from NICU was made. Therefore, the protocols for             and rescreening and between rescreening and diagnosis.



362
                                                                                                                    Newborn hearing screening in Sicily




The use of both ABR and TEOA tests in the audiological                           editor. Dalla diagnosi all’autonomia comunicativa - XXV Relazione
screening decreases the number of newborns sent for au-                          Ufficiale SIOP 2019. Bagheria-Palermo: Plumelia Editore; 2019. pp
                                                                                 16-20.
diological evaluation with a notable reduction of costs 24.                 9
                                                                                 Martini A, Marchisio P, Bubbico L, et al. Permanent childhood hear-
Lastly, there is a need to establish a shared project, a re-                     ing impairment: universal newborn hearing screening, PCHI manage-
gional network with a reference element and coordination                         ment. Minerva Pediatr 2013;65:231-250.
of information, monitoring and quality controls of regional                 10
                                                                                 Yoshinaga-Itano C, Sedey AL, Coulter DK, et al. Language of
data.                                                                            early- and later-identified children with hearing loss. Pediatrics
                                                                                 1998;102:1161-1171. https://doi.org/10.1542/peds.102.5.1161
                                                                            11
                                                                                 Moeller MP. Early intervention and language development in children
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• Palermo: Cinquegrani MR, Lunetta F, Pellegrino C, Pu-                          fusion otitis media: single center experience and review of litera-
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