Abstract
Background/Aim: Hearing impairment affects a small but significant percentage of newborns (0.1-0.4%). Newborn hearing screening (NHS) is recommended for early detection and treatment. The implementation of NHS can vary among countries. In this study, we present the methodology, organization, and technical requirements of NHS. This study analyzed results from a tertiary hospital, identified issues, and proposed solutions. Patients and Methods: In the studied region, there are five maternity hospitals and a perinatal intensive care center and in 2020, there were 5,864 live births. Screening is performed at three levels. The first screening is conducted on the 2nd-3rd day of a newborn’s life in a maternity hospital, the first rescreening on the 3rd-6th week at a relevant ENT department, and the second rescreening on the 3rd-6th month of life at the regional screening center where the central database is also held. Results: In the studied region, 5,793 out of 5,864 (98.79%) newborns received NHS in 2020. Of these, 120 (2.07%) were tested positive on their first screening. Ninety-four patients (78.3%) of those attended the ENT department for a first rescreening. Thirty-four patients (0.59% of total) were tested positive again and referred to the regional screening center. Out of the 27 patients who attended the second rescreening, four (0.07% of the total) were ultimately diagnosed with hearing impairment. Conclusion: Our study found that newborn hearing screening (NHS) in our region achieved a high compliance rate of 98.8% for initial screenings in 2020. However, challenges remain in the rescreening process due to data management issues, inter-regional cooperation, and public awareness. The recent implementation of mandatory screenings, updated guidelines, and a centralized database is expected to enhance the effectiveness of NHS. Further research is needed to evaluate these improvements.
Hearing is essential for a child’s development of speech, communication, mental, and social skills, particularly during their early years (1, 2). Hearing impairment affects approximately 0.1-0.4% of healthy newborns, with a higher incidence of 2-5% in at-risk newborns (3, 4). While universal newborn hearing screening (NHS) is recommended by international committees, the organizational design can vary among countries (5, 6).
The objective of newborn hearing screening is to identify hearing impairment at an early stage and initiate prompt treatment. The World Health Organization, European consensus, and other professional societies advocate adhering to the fundamental guideline of “1-3-6” for screening management (7-10). To ensure early detection of permanent hearing damage in children, it is recommended to conduct first-line screening within the first month of life, followed by a diagnosis by three months of age and auditory rehabilitation as soon as possible, but no later than six months (8, 9).
In the Czech Republic, 100-200 children are born with severe hearing impairment every year (11). Recent efforts by the Czech Society of Otorhinolaryngology and Head and Neck Surgery CLS JEP have significantly improved the management of hearing screening. The Moravian-Silesian region established the first systematic regional screening in 2010 (12), and since 2018, nationwide statistical data have been collected for the country’s 10.5 million inhabitants (13). As of 2021, Decree No. 45/2021 Coll. has legally enshrined the screening process (14), and starting in 2022, the updated methodological instruction of the Ministry of Health Czech Republic for newborn hearing screening has come into effect. The instruction provides recommendations and guidelines for three screening levels, including neonatology, ENT rescreening, and ped-audiology. Additionally, a nationwide database was established in 2023 with support from the Institute of Health Information and Statistics of the Czech Republic.
In accordance with international guidelines, the maternity ward is responsible for conducting the first round of newborn screening (9, 15). If the baby is born outside of the hospital, its pediatrician will arrange for hearing screening at a designated rescreening center during the third to sixth week of the baby’s life (Figure 1). Initial screening is provided by skilled nurses at the hospital, while the first and second rescreenings are conducted by designated regional ENT doctors, including specialists at the regional hearing center (RHS) or the pediatric audiology center.
Flowchart of newborn audiology screening management.
Two non-invasive techniques are used to evaluate hearing abilities as part of the NHS (16, 17). The first method is Transient-Evoked Otoacoustic Emissions (TEOAE), which gauges the response of the outer hair cells in the inner ear to an acoustic signal emitted by a device. A microphone located in the outer auditory canal measures the reaction (18). The second method is Automated Auditory Brainstem Response Audiometry (AABR), which assesses the electrophysiological response to sound in the auditory nerve and brainstem (19). TEOAE is used to assess newborns at the first NHS level or initial rescreening level. AABR is recommended for newborns at higher risk. Both methods are quick and safe, with sensitivity rates approaching 100%. They can also be performed on children who are asleep or quiet (20).
The purpose of this study was to delve into the approach, rules and structure, and technological necessities surrounding newborn hearing screening in our region, which comprises a population of 600,000. Our work includes an analysis of the findings from the tertiary healthcare facility for the calendar year 2020, highlight any challenges, and propose enhancements to the screening protocol.
Patients and Methods
The study was conducted according to the guidelines of the Declaration of Helsinki, and approved by the Ethical Committee of the University Hospital in Pilsen and Faculty of Medicine in Pilsen, Charles University, on 5th of October 2023. Protocol code 376/23.
In the region under study, 5,864 live births occurred in the year 2020. These data were used to conduct a retrospective analysis of newborn hearing screenings in the area. The region adheres to the recommended NHS procedure at three levels. There are four regional maternity wards, as well as a fifth maternity ward and a perinatal intensive care center (PICC) situated at the tertiary hospital. All newborns in these facilities receive hearing tests using the otoacoustic emissions (TEOAE) method.
Physiological newborns with positive screening results (unilaterally or bilaterally absent TEOAE, or screening not performed) undergo rescreening using TEOAE at the relevant ENT department, either in the hospital or outpatient department. This is the second level of screening (first rescreening). Children with a positive first rescreening from the outpatient ENT department undergo a second rescreening at a regional screening center (RSC), which is an ENT department at the faculty hospital (the third level of screening). Newborns at risk with a positive NHS (discharged and referred from the PICC) are recommended to be screened using AABR at the tertiary hospital. As part of the third level of screening, a comprehensive audiological examination is conducted at the RSC, and further treatment is provided if a hearing defect is detected.
The RSC stores a database containing the results of all levels of screening for the entire region. Every month, authorized workers from individual maternity hospitals and ENT departments send data on the number of births, screening results, and identification of children with a positive screening, which is then entered into the RSC database (Figure 1).
The machines (TEOAE or BERA) used in our region are suitably designed for NHS. These machines can detect hearing thresholds of 35 dB HL, or worse, in each ear separately. The first screening is conducted on the 2nd-3rd day of a newborn’s life, followed by the first rescreening on the 3rd-6th week and the second rescreening on the 3rd-6th month of life. The parents of the patients are informed about the goals and procedures of NHS, and they may refuse to participate. The initial screening is carried out by nurses or midwives in hospital maternity wards, while the first and second rescreenings are conducted by a doctor-specialist.
The retrospective study was created with subsequent statistical evaluation. Relative frequencies are stated in percentages. Frequencies were calculated with respect to the total of 5,864 patients included in the study. The patients’ personal data were accessed only by authorized medical staff and were completely anonymized before evaluation.
Results
Out of 5,864 children born in the studied region, 5,793 (98.79%) underwent NHS in 2020. During the screenings, 120 children (2.07%) tested positive in the first round and were asked to return for a rescreening. Out of those, ninety-four attended the appointment (compliance of 78.33%) and thirty-four (0.59% of the total) had a positive finding in the second screening. These children were referred to the RSC for a third screening. Table I provides an overview of the NHS.
Newborn hearing screening (NHS) in the region in 2020 according to the place of birth.
As expected, infants who were considered at risk and were admitted to the PICC unit display different outcomes in the initial and subsequent levels of NHS screening. The first screening indicated a higher positivity rate of 5.92% and positivity in a first rescreening in 1.66%.
Out of the thirty-four children who were eligible for the RSC examination, a majority of twenty-seven attended (yielding a compliance rate of 79.41%). Among them, twenty-three were found to have no hearing impairment, while four necessitated further dispensary care (representing 0.07% of the total). Of these four children, two had a cleft palate issue and were presumed to have improved hearing after undergoing surgical correction, while the other two had a borderline hearing impairment that could be rectified with hearing aids, pending the results of future examinations. Cochlear implantation or hearing correction with a bone hearing aid was not recommended for any of the children from this sample (Figure 2).
Newborn hearing screening (NHS) results in regional screening center (RSC) in 2020.
Discussion
Newborn hearing screening is commonly performed in Europe (5) and there are numerous scientific papers on the subject from different countries, such as Brazil (21), USA (22), Saudi Arabia (23), as well as from Asian countries (24, 25). These studies, conducted by various organizations, differ in their effectiveness levels, but all aim to ensure effective medical management (9). In recent years, the Czech Republic has seen a steady increase in the number of screening exams and system effectiveness (13, 26, 27), but there are variations in NHS implementation success rates among different regions. Based on our findings, our region has performed first-level screening on 98.79% of live births in 2020, which is comparable to statistical data from foreign countries (28, 29) and other regions (Table II). However, only 2.07% of children in our region were sent to a first rescreening, a lower percentage compared to other Czech regions (Table II) (13, 27, 30).
Results of newborn hearing screening (NHS). Comparison of four similar regions (0.5-1 mil. inhabitants in each region).
During the first level of hearing screening, it is anticipated that around 5-10% of individuals will receive a positive result. If this percentage is significantly lower, it may suggest that the examining health professionals lack experience or training (26, 31). In 2020, the PICC equipment used for the NHS’s first level was deemed insufficient, because at-risk newborns are advised to undergo an AABR examination instead. An updated methodological instruction is slated to go into effect in 2022 (32), and the possibility of acquiring AABR equipment is discussed in 2023. Currently, all at-risk newborns who receive a positive result during the NHS’s first level at the PICC are referred for rescreening at the RSC, where brainstem evoked response audiometry (BERA) is employed as part of the second level of screening.
In our study, rescreening resulted in positive results in only 0.59% of children from the total number examined. Our findings reveal a contradiction. The second level of screening in newborns from the faculty hospital has a relatively low compliance rate despite the fact that there is RSC placed. According to data from our RSC database, only fifty-nine out of seventy patients from the standard maternity ward of our tertiary hospital with an initial positive screening underwent a subsequent rescreening (compliance rate of 84.3%), while only 44% from PICC complied. However, we discovered that 21 out of 25 missing children from the faculty hospital had a next follow-up and a rescreening in the ENT outside of our region, which is not reflected in our RSC database version for 2020. Thus, we have no information on only five patients in total (four from the faculty hospital and one from regional hospital III). The same principle explains the low compliance rate for PICC. It is hoped that this issue will be solved by the establishment of a national database in 2023. The nationwide database will provide simpler and better tracking of patients (Figure 2).
During the second round of rescreening, we observed a similar situation. Although it was initially reported that seven children were missing (with a general compliance rate of 79.4%), it was discovered that five of them had follow-up appointments at another screening center. This means that only two patients dropped out of the screening at this level. Most of the children (twenty-three patients, 85.19% of those examined) at the rescreening center did not have permanent hearing impairment. Four children required further dispensary treatment, and the decision to correct their hearing was made after further investigations. Two of these children underwent surgery for a cleft palate, while the other two used hearing aids.
Out of the total 5,793 children who underwent primary screening, only 0.07% had hearing defects. This is a much lower rate than the generally reported prevalence values of 0.1-0.4% or 2-5% for at-risk newborns, as provided by Chrobok in 2015 (3) and Boshuizen in 2001 (33). Compliance problems may have contributed to this low rate. There are several reasons for this, including unreliable systems for transferring information from rescreening ENT workplaces to the RSC database in 2020 and non-cooperation from parents. Metwally (34) and Graham (35) in 2023 have described this behavior, which may also be due to a lack of valid information and an underestimation of the issue. Cooperation is particularly challenging with parents who are “socially less adaptive”, as noted by Ravi (36) and Wang (37). It is crucial to educate parents about the importance of rescreening and following up with the workplaces where their child is examined, as posted by Sung and Ren in 2023 (38, 39).
According to a study conducted by Matulat and Parfitt in 2020 (40), the effectiveness of the NHS in our region was found to be comparable to that of Germany in 2012. The study revealed that while 79.9% of children were screened in Germany, compliance with the first rescreening level was lacking, consistent with a previous NHS study by Mehra et al. in 2009 (41). Following the state of our screening in 2020, the Czech Republic took further steps to improve screening, enacting a decree (No. 45/2021 Coll.) that legally obligates general practitioners to screen children and adolescents. In 2022, the Ministry of Health of the Czech Republic updated its methodological instruction for performing NHS, and by 2023, a nationwide database was established at the Institute of Health Information and Statistics of the Czech Republic, with a national screening coordinator appointed (Figure 3). Tracking situation and problems were described by Matulat in 2017 (42) and Bussé in 2023 (43).
The use of national database data for patient tracking across regions in cooperation with the Institute of Health Information and Statistics of the Czech Republic.
Ensuring that newborns are screened for hearing defects is crucial, and our investigation has confirmed, in accordance with Brockow in 2017 (28) and by Holowell in 2022 (29), that clear procedures and collaboration between obstetricians, neonatology departments, otorhinolaryngologists, general practitioners, and parents are key to its effectiveness. Our initial screening results are promising, with a 98.8% compliance rate. However, achieving 100% compliance is not possible due to births occurring outside of hospitals. Infants born at home are first screened during the ENT rescreening process (Figure 1). To improve compliance, we hope to enhance the tracking of data from the first and second rescreenings through a new patient tracking system (Figure 2).
Study limitations. Insufficient processing of system data and unsatisfactory inter-regional cooperation between workplaces of the first and second rescreenings in 2020.
Conclusion
Our research found that the newborn hearing screening in 2020 had an impressive compliance rate of 98.8% in its first level. However, there is still room for improvement in the rescreening process. This was due to factors, such as the lack of a national database for hearing screening, lack of cooperation between NHS locations, insufficient training and experience of health professionals conducting the screenings, and limited public awareness. We are hopeful that the mandatory screening of children and adolescents by general practitioners implemented in 2021, the updated NHS performance guidelines from the Ministry of Health in 2022, and the establishment of a centralized national database in 2023 will provide significant benefits for patients. Further studies in 2024 or 2025 will be necessary to confirm the effectiveness of NHS in the Czech Republic.
Footnotes
Authors’ Contributions
Conceptualization: D.S., T.K. and P.K.; methodology: D.S. and B.G.; statistics and methodology: D.S. and H.L.; investigation: P.Š. and H.L.; writing - original draft preparation: D.S. and T.K.; writing - review and editing: A.S., V.Š. and R.K. All Authors have read and agreed to the published version of the manuscript.
Conflicts of Interest
All Authors declare no conflicts of interest in relation to this study.
Funding
The work was supported by the Cooperation Program, research area SURG and by the Ministry of Health, Czech Republic—conceptual development of research organization (Faculty Hospital in Pilsen—FNPl, 00669806).
- Received March 20, 2024.
- Revision received April 14, 2024.
- Accepted April 15, 2024.
- Copyright © 2024 The Author(s). Published by the International Institute of Anticancer Research.
This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY-NC-ND) 4.0 international license (https://creativecommons.org/licenses/by-nc-nd/4.0).
References
In this issue
Jump to section
Related Articles
Cited By...
- No citing articles found.