International Journal of Pediatric Otorhinolaryngology 79 (2015) 635–637
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Neonatal hearing screening: What we have achieved and what needs to be improved A B S T R A C T
Keywords: Neonatal Hearing Screening Results Weakness Cost-effective
Objective: To review the literature on neonatal hearing screening and its weaknesses with regard to missed follow-ups and delayed diagnosis and management. Results: The implementation of newborn hearing screening programs has indeed lowered the mean age of hearing loss identification and many deaf children are now diagnosed at an early age of some months. However, the present collection of 32 studies published in the International Journal of Pediatric Otorhinolaryngology revealed that late-onset deafness, auditory neuropathy, and the alarming percentage of newborns who fail the initial testing and then are lost to follow-up are major weaknesses of neonatal hearing screening programs. It seems that parents may be satisfied of hearing screening but in a significant percentage of them do not bring their children for follow-up or further testing due to phycho-social or other reasons. In addition, the same collection revealed that socioeconomic factors may have a significant influence on the effectiveness of hearing screening programs in the developed and developing countries, where improvements in health care politics, tracking system and public awareness is crucial for successful program implementation. Conclusions: Neonatal hearing screening programs have changed the whole picture of congenital deafness as age identification has significantly fallen with a very positive effect on timely management. However, the selected and proposed 32 related articles published in the International Journal of Pediatric Otorhinolaryngology show that there are still serious weakness in the neonatal hearing screening that need improvements in order to achieve an efficient and cost-effective system of deafness identification. ß 2015 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Neonatal hearing screening for congenital sensorineural hearing loss fulfills all the criteria for universal screening. First, it is very frequent; the prevalence of congenital bilateral permanent hearing loss is approximately 1 to 5 per 1000 live births. It is very interesting to note that the incidence of congenital sensorineural hearing loss in the newborn population is greater than the combined incidence of all the metabolic conditions that we currently screen for with blood tests. Second, deafness without early and appropriate management has very serious consequences for the affected child and its family, as hearing during the critical periods of infancy and early childhood is necessary to develop spoken language. Finally, an overwhelming body of evidence has shown that the available technologies (otoacoustic emission and automated auditory brainstem response testing) are accurate, reliable, objective, and cost-effective tests for detecting congenital hearing loss. Taking into account all the above facts and bypassing the common related misconceptions (Table 1), most of the developed countries have started screening programs worldwide. However, http://dx.doi.org/10.1016/j.ijporl.2015.02.010 0165-5876/ß 2015 Elsevier Ireland Ltd. All rights reserved.
several difficulties and weaknesses of the related programs have emerged. Therefore, the aim of the present collection was to review the literature on neonatal hearing screening and its weaknesses with regard to missed follow-ups and delayed diagnosis and management. 2. Results Before the implementation of newborn hearing screening programs, children with profound bilateral sensorineural hearing loss were usually identified around 2 years of age or even older. When universal hearing screening was initially conceived, it was presumed that most hearing-impaired children, especially those without risk factors for progressive hearing loss, would fail the objective screening performed during the newborn period, thus making early diagnosis and intervention possible on a widespread basis. These expectations were met, at least partly, by the reality. Gradually, the health systems that have implemented universal hearing screening programs noticed that indeed the mean age of hearing loss identification became lower and many deaf children
636
T.P. Nikolopoulos / International Journal of Pediatric Otorhinolaryngology 79 (2015) 635–637
Table 1 Common misconceptions held by the public and the clinical facts http://emedicine.medscape.com/article/836646—overview. Misconception Parents will know if their child has a hearing loss by the time their child is 2–3 months of age Parents can identify a hearing loss by clapping their hands behind the child’s head The high risk children screening is all that is needed to identify children with hearing loss Hearing loss does not occur often enough to justify the use of universal screening programs Tests are not reliable and cause too many referrals There is no rush to identify a hearing loss. The loss does not need to be identified until a child is aged 2–3 years
Children younger than 12 months cannot be fitted with hearing aids
are now diagnosed at an early age of some months. However, it is still not uncommon for parents of cochlear implant candidates to report that newborn hearing screening did not identify hearing loss in their child (i.e., the child passed screening). Children with lateonset deafness and negative initial hearing screening are in a worse situation that those with no hearing screening at all, as diagnosis may further delay due to false reassurance by the negative testing when these children were born. Therefore, hearing loss in this subpopulation of children (mainly the late-onset hearing loss children) may not be diagnosed, and these children may not undergo implantation at as young an age as those who fail screening. Risk factors of late-onset hearing loss should always be taken into account (e.g., cytomegalovirus (CMV) infection, syndromes associated with progressive hearing loss, neurodegenerative disorders, trauma, or culture-positive postnatal infections associated with sensorineural hearing loss; for children who have received extracorporeal membrane oxygenation (ECMO) or chemotherapy, when there is caregiver concern or a family history of hearing loss. etc.). Another reason is the so called ‘auditory neuropathy’, a disease associated with ‘normal’ pass otoacoustic emmissions and abnormal ABR. Therefore, a child with auditory neuropathy who has a hearing loss may pass the otoacoustic emmissions’ screening. Moreover, a significant weakness of universal hearing screening programs is the alarming percentage of newborns who failed the initial testing and then did not have any further testing or they were lost to follow-up. Studies in the present collection of papers published in the International Journal of Pediatric Otorhinolaryngology show that this percentage may pass 50%, in other words one every two newborns who failed hearing screening may be missed to follow-up. Moreover, it was also published that while cochlear implant team members identified delayed insurance approval and medical comorbidities as reasons for delayed implantation, the most significant factor identified was parental, with delayed/missed appointments or reluctance for evaluations or surgery. This is in high contrast with studies, also published in the present collection, that have showed overall parental satisfaction of 90% with regard to neonatal hearing screening. It seems that parents may be satisfied of hearing screening but in a significant percentage of them do not bring their children for follow-up or further testing due to phycho-social or other reasons. Therefore it is not surprising that in another study of this collection, the authors found that while cochlear implant team members identified delayed insurance approval and medical comorbidities as reasons for delayed implantation, the most significant factor identified was parental, with delayed/missed appointments or reluctance for evaluations or surgery. It seems that a major factor in the whole process of identification of
Clinical fact Prior to the universal screening, the average age at which children were found to have a hearing loss is 2–3 years. Children with mild-tomoderate hearing loss were often not identified until 4 years of age Children can compensate for a hearing loss. They use visual cues, such as shadows or parental expressions and reactions, or they may feel the breeze caused by the motion of the hands It misses approximately 50% of all children with hearing loss Hearing loss affects approximately 2–4 per 1000 live births, and it has been estimated to be one of the most common congenital anomalies Referral rates are as low as 5–7% Children identified when they are older than 6 months can have speech and language delays. Children identified when they are younger than 6 months may not have these delays and be equal to their hearing peers in terms of speech and language Children as young as 1 month of age can be fit with and benefit from hearing aids
deafness is parental attitude and consensus. In addition, socioeconomic factors may have a significant influence on the effectiveness of hearing screening programs in the developed and developing countries, where improvements in health care politics, tracking system and public awareness is crucial for successful program implementation.
3. Conclusions Neonatal hearing screening programs have changed the whole picture of congenital deafness as age identification has significantly decreased with a very positive effect on timely management. However, the selected and proposed 32 related articles published in the International Journal of Pediatric Otorhinolaryngology show that there are still serious weakness in the neonatal hearing screening that need improvements in order to achieve an efficient and cost-effective system of deafness identification.
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versus distortion product otoacoustic emissions in hearing screening of high risk neonates, Int. J. Pediatr. Otorhinolaryngol. 75 (9) (2011) 1109–1116, http:// dx.doi.org/10.1016/j.ijporl.2011.05.026 (Epub 2011 Jun 29. PubMed PMID: 21719120). J. Lu¨, Z. Huang, T. Yang, Y. Li, L. Mei, M. Xiang, et al., Screening for delayed-onset hearing loss in preschool children who previously passed the newborn hearing screening, Int. J. Pediatr. Otorhinolaryngol. 75 (8) (2011) 1045–1049, http://dx.doi.org/10.1016/ j.ijporl.2011.05.022 (Epub [2011] Jun 25. PubMed PMID: 21705096). E.M. Fitzpatrick, E. Johnson, A. Durieux-Smith, Exploring factors that affect the age of cochlear implantation in children, Int. J. Pediatr. Otorhinolaryngol. 75 (9) (2011) 1082– 1087, http://dx.doi.org/10.1016/j.ijporl.2011.05.018 (Epub 2011 Jun 23. PubMed PMID: 21703694). M. Maris, C. Venstermans, A.N. Boudewyns, Auditory neuropathy/dyssynchrony as a cause of failed neonatal hearing screening, Int. J. Pediatr. Otorhinolaryngol. 75 (7) (2011) 973–975, http://dx.doi.org/10.1016/j.ijporl.2011.04.012 (Epub 2011 May 19. PubMed PMID: 21596446). I. Bielecki, A. Horbulewicz, T. Wolan, Risk factors associated with hearing loss in infants: an analysis of 5282 referred neonates, Int. J. Pediatr. Otorhinolaryngol. 75 (7) (2011) 925–930, http://dx.doi.org/10.1016/j.ijporl.2011.04.007 (Epub [2011] May 14. PubMed PMID: 21571377). M.D. Mohd Khairi, K.N. Rafidah, A. Affizal, A.R. Normastura, M. Suzana, Z.M. Normani, Anxiety of the mothers with referred baby during Universal Newborn Hearing Screening, Int. J. Pediatr. Otorhinolaryngol. 75 (4) (2011) 513–517, http://dx.doi.org/10.1016/ j.ijporl.2011.01.009 (Epub 2011 Feb 2. PubMed PMID: 21292333). B.O. Olusanya, Making targeted screening for infant hearing loss an effective option in less developed countries, Int. J. Pediatr. Otorhinolaryngol. 75 (3) (2011) 6–21, http:// dx.doi.org/10.1016/j.ijporl.2010.12.002 (Epub 2011 Jan 5. Review. PubMed PMID: 21211856). S. Coenraad, A. Goedegebure, L.J. Hoeve, An initial overestimation of sensorineural hearing loss in NICU infants after failure on neonatal hearing screening, Int. J. Pediatr. Otorhinolaryngol. 75 (2) (2011) 159–162, http://dx.doi.org/10.1016/j.ijporl.2010.10.026 (Epub 2010 Nov 12. PubMed PMID: 21074864). S. Kumar, B. Mohapatra, Status of newborn hearing screening program in India, Int. J. Pediatr. Otorhinolaryngol. 75 (1) (2011) 20–26, http://dx.doi.org/10.1016/ j.ijporl.2010.09.025 (Epub 2010 Nov 10. PubMed PMID: 21071101). M. Geal-Dor, C. Adelman, H. Levi, G. Zentner, C. Stein-Zamir, Comparison of two hearing screening programs in the same population: oto-acoustic emissions (OAE) screening in newborns and behavioral screening when infants, Int. J. Pediatr. Otorhinolaryngol. 74 (12) (2010) 1351–1355, http://dx.doi.org/10.1016/j.ijporl.2010. 08.023 (Epub [2010] Sep 24. PubMed PMID: 20869779).
Thomas P. Nikolopoulos* Department of Otorhinolaryngology, 125 Anakous Street, 14342 Athens, Greece *Tel.: +30 6973956979; fax: +30 2106994904 E-mail address: [email protected] Received 2 February 2015 Accepted 6 February 2015 Available online 16 February 2015
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Neonatal hearing screening: What we have achieved and what needs to be improved A B S T R A C T
Keywords: Neonatal Hearing Screening Results Weakness Cost-effective
Objective: To review the literature on neonatal hearing screening and its weaknesses with regard to missed follow-ups and delayed diagnosis and management. Results: The implementation of newborn hearing screening programs has indeed lowered the mean age of hearing loss identification and many deaf children are now diagnosed at an early age of some months. However, the present collection of 32 studies published in the International Journal of Pediatric Otorhinolaryngology revealed that late-onset deafness, auditory neuropathy, and the alarming percentage of newborns who fail the initial testing and then are lost to follow-up are major weaknesses of neonatal hearing screening programs. It seems that parents may be satisfied of hearing screening but in a significant percentage of them do not bring their children for follow-up or further testing due to phycho-social or other reasons. In addition, the same collection revealed that socioeconomic factors may have a significant influence on the effectiveness of hearing screening programs in the developed and developing countries, where improvements in health care politics, tracking system and public awareness is crucial for successful program implementation. Conclusions: Neonatal hearing screening programs have changed the whole picture of congenital deafness as age identification has significantly fallen with a very positive effect on timely management. However, the selected and proposed 32 related articles published in the International Journal of Pediatric Otorhinolaryngology show that there are still serious weakness in the neonatal hearing screening that need improvements in order to achieve an efficient and cost-effective system of deafness identification. ß 2015 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Neonatal hearing screening for congenital sensorineural hearing loss fulfills all the criteria for universal screening. First, it is very frequent; the prevalence of congenital bilateral permanent hearing loss is approximately 1 to 5 per 1000 live births. It is very interesting to note that the incidence of congenital sensorineural hearing loss in the newborn population is greater than the combined incidence of all the metabolic conditions that we currently screen for with blood tests. Second, deafness without early and appropriate management has very serious consequences for the affected child and its family, as hearing during the critical periods of infancy and early childhood is necessary to develop spoken language. Finally, an overwhelming body of evidence has shown that the available technologies (otoacoustic emission and automated auditory brainstem response testing) are accurate, reliable, objective, and cost-effective tests for detecting congenital hearing loss. Taking into account all the above facts and bypassing the common related misconceptions (Table 1), most of the developed countries have started screening programs worldwide. However, http://dx.doi.org/10.1016/j.ijporl.2015.02.010 0165-5876/ß 2015 Elsevier Ireland Ltd. All rights reserved.
several difficulties and weaknesses of the related programs have emerged. Therefore, the aim of the present collection was to review the literature on neonatal hearing screening and its weaknesses with regard to missed follow-ups and delayed diagnosis and management. 2. Results Before the implementation of newborn hearing screening programs, children with profound bilateral sensorineural hearing loss were usually identified around 2 years of age or even older. When universal hearing screening was initially conceived, it was presumed that most hearing-impaired children, especially those without risk factors for progressive hearing loss, would fail the objective screening performed during the newborn period, thus making early diagnosis and intervention possible on a widespread basis. These expectations were met, at least partly, by the reality. Gradually, the health systems that have implemented universal hearing screening programs noticed that indeed the mean age of hearing loss identification became lower and many deaf children
636
T.P. Nikolopoulos / International Journal of Pediatric Otorhinolaryngology 79 (2015) 635–637
Table 1 Common misconceptions held by the public and the clinical facts http://emedicine.medscape.com/article/836646—overview. Misconception Parents will know if their child has a hearing loss by the time their child is 2–3 months of age Parents can identify a hearing loss by clapping their hands behind the child’s head The high risk children screening is all that is needed to identify children with hearing loss Hearing loss does not occur often enough to justify the use of universal screening programs Tests are not reliable and cause too many referrals There is no rush to identify a hearing loss. The loss does not need to be identified until a child is aged 2–3 years
Children younger than 12 months cannot be fitted with hearing aids
are now diagnosed at an early age of some months. However, it is still not uncommon for parents of cochlear implant candidates to report that newborn hearing screening did not identify hearing loss in their child (i.e., the child passed screening). Children with lateonset deafness and negative initial hearing screening are in a worse situation that those with no hearing screening at all, as diagnosis may further delay due to false reassurance by the negative testing when these children were born. Therefore, hearing loss in this subpopulation of children (mainly the late-onset hearing loss children) may not be diagnosed, and these children may not undergo implantation at as young an age as those who fail screening. Risk factors of late-onset hearing loss should always be taken into account (e.g., cytomegalovirus (CMV) infection, syndromes associated with progressive hearing loss, neurodegenerative disorders, trauma, or culture-positive postnatal infections associated with sensorineural hearing loss; for children who have received extracorporeal membrane oxygenation (ECMO) or chemotherapy, when there is caregiver concern or a family history of hearing loss. etc.). Another reason is the so called ‘auditory neuropathy’, a disease associated with ‘normal’ pass otoacoustic emmissions and abnormal ABR. Therefore, a child with auditory neuropathy who has a hearing loss may pass the otoacoustic emmissions’ screening. Moreover, a significant weakness of universal hearing screening programs is the alarming percentage of newborns who failed the initial testing and then did not have any further testing or they were lost to follow-up. Studies in the present collection of papers published in the International Journal of Pediatric Otorhinolaryngology show that this percentage may pass 50%, in other words one every two newborns who failed hearing screening may be missed to follow-up. Moreover, it was also published that while cochlear implant team members identified delayed insurance approval and medical comorbidities as reasons for delayed implantation, the most significant factor identified was parental, with delayed/missed appointments or reluctance for evaluations or surgery. This is in high contrast with studies, also published in the present collection, that have showed overall parental satisfaction of 90% with regard to neonatal hearing screening. It seems that parents may be satisfied of hearing screening but in a significant percentage of them do not bring their children for follow-up or further testing due to phycho-social or other reasons. Therefore it is not surprising that in another study of this collection, the authors found that while cochlear implant team members identified delayed insurance approval and medical comorbidities as reasons for delayed implantation, the most significant factor identified was parental, with delayed/missed appointments or reluctance for evaluations or surgery. It seems that a major factor in the whole process of identification of
Clinical fact Prior to the universal screening, the average age at which children were found to have a hearing loss is 2–3 years. Children with mild-tomoderate hearing loss were often not identified until 4 years of age Children can compensate for a hearing loss. They use visual cues, such as shadows or parental expressions and reactions, or they may feel the breeze caused by the motion of the hands It misses approximately 50% of all children with hearing loss Hearing loss affects approximately 2–4 per 1000 live births, and it has been estimated to be one of the most common congenital anomalies Referral rates are as low as 5–7% Children identified when they are older than 6 months can have speech and language delays. Children identified when they are younger than 6 months may not have these delays and be equal to their hearing peers in terms of speech and language Children as young as 1 month of age can be fit with and benefit from hearing aids
deafness is parental attitude and consensus. In addition, socioeconomic factors may have a significant influence on the effectiveness of hearing screening programs in the developed and developing countries, where improvements in health care politics, tracking system and public awareness is crucial for successful program implementation.
3. Conclusions Neonatal hearing screening programs have changed the whole picture of congenital deafness as age identification has significantly decreased with a very positive effect on timely management. However, the selected and proposed 32 related articles published in the International Journal of Pediatric Otorhinolaryngology show that there are still serious weakness in the neonatal hearing screening that need improvements in order to achieve an efficient and cost-effective system of deafness identification.
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Thomas P. Nikolopoulos* Department of Otorhinolaryngology, 125 Anakous Street, 14342 Athens, Greece *Tel.: +30 6973956979; fax: +30 2106994904 E-mail address: [email protected] Received 2 February 2015 Accepted 6 February 2015 Available online 16 February 2015
