Universal Neonatal Hearing Screening - Experience in a Tertiary Care Hospital in
Southern India


From the Department of ENT, Christian Medical College, Vellore; *Department of Neonatology,
Christian Medical College, Vellore and **Department of Medical Genetics, Christian Medical College,
Vellore, Tamil Nadu, India.

Correspondence to: Dr Achamma Balraj, Department of ENT Unit IV, Christian Medical College,
Vellore, Tamil Nadu 632 004, India. Email:

Received: June 01, 2013; Initial review: June 26, 2013; Accepted: September 20, 2013.

PII: S097475591300554


1                OCTOBER 5, 2013 [E-PUB AHEAD OF PRINT] 

Neonatal hearing loss has a prevalence that is more than twice that of other screenable newborn disorders such as congenital hypothyroidism and phenylketonuria [1. et al. Key Words: Automated ABR. The estimated prevalence of confirmed hearing loss was comparable to that in literature. Studies done in India using different hearing screening protocols have estimated the prevalence of neonatal hearing loss to vary INDIAN PEDIATRICS 2                OCTOBER 5. prevalence of neonatal hearing loss and risk factors found in association with neonatal hearing loss. Neonatal hearing screening. vital for early identification and rehabilitation of neonatal hearing loss. Overcoming the large numbers of loss to follow-up proves to be a challenge in the implementation of such a program.AUGUSTINE.1 per 1000) and were rehabilitated appropriately. Participants: 9448 babies born in the tertiary care hospital over a period of 11 months. 6 had evidence of TORCH infection and 1 had connexin 26 gene mutation. 39 were confirmed to have hearing loss (4. UNIVERSAL NEONATAL HEARING SCREENING ABSTRACT Objective: To implement a universal neonatal hearing screening program using automated auditory brainstem response audiometry in a tertiary care set-up and assess the prevalence of neonatal hearing loss. Neonates suspected of hearing loss underwent confirmatory testing using auditory steady state response audiometry and serological testing for TORCH infections and connexin 26 gene. Among 45 babies who had confirmatory testing.2]. 2013 [E-PUB AHEAD OF PRINT]  . Main Outcome Measures: Feasibility of the screening program. Congenital. Neonatal hearing loss. bilateral hearing impairment occurs in approximately 1 to 5 per 1000 live births and when permanent unilateral hearing loss is included. Intervention: The neonates were subjected to a two stage sequential screening using the BERAphone. Design: Observational prospective cohort study. 30 babies had one or more risk factors. Results: 164 babies were identified as suspected for hearing loss. Conclusion: Universal neonatal hearing screening using BERAphone is a feasible service. the incidence increases to 8 per 1000 live births [3-5]. Setting: Tertiary care referral center.

exhibit a moderate to profound hearing loss [9] i. et al. 50% of the children with moderate to profound congenital hearing loss exhibit no risk factors for hearing loss [10]. The test is also seldom affected by ambient noise making it suitable for use in the postnatal ward [17].AUGUSTINE. UNIVERSAL NEONATAL HEARING SCREENING between 1 and 8 per 1000 babies screened [6-8]. where disposable electrodes have to be applied before the procedure). a very short preparation time (unlike the other aABR equipments. Early identification and intervention for hearing loss by 6 months of age provides better prognosis in language development. The other advantages of aABR include rapidity. It consists of a hand-held headphone unit which incorporates a set of three fixed reusable electrodes. The Maico MB11 BERAphone is an aABR system employing a special headphone and the time-course step-stimulus algorithm offers a quick and reliable tool for universal hearing screening in newborns [16].9% when used in a 2-stage screening protocol which is comparable to that of OAE. easy-to-use and high sensitivity (0.87) [14. using the BERAphone (2 stage sequential screening protocol) in order to provide early and effective intervention for those with hearing loss. screening all neonates for hearing loss is the only effective measure for detection of congenital deafness. This study was undertaken with the primary objective of exploring the feasibility of setting up a universal neonatal hearing screening program in a tertiary care hospital (handling an average of 10 000 deliveries/year). Tests used for screening newborns for hearing loss include Otoacoustic emissions (OAE) and automated Auditory Brainstem Response audiometry (aABR). academic success. Hence. hyperbilirbinaemia.99) and specificity (0. low birth weight. Although hearing screening programs using different screening protocols have been set up in some centres procedures for systematic identification and rehabilitation on a large scale are yet to be tested and implemented in the Indian setting. prolonged NICU stay. thereby providing an opportunity to rehabilitate the child to its fullest potential. social integration and successful participation in the society [5]. Two to 4% of neonates with one or more risk factors such as birth asphyxia. While OAE is cheap. It has been tested and found to have a sensitivity of 99. The secondary objectives include estimating the INDIAN PEDIATRICS 3                OCTOBER 5.15].02% [11-13]. 2013 [E-PUB AHEAD OF PRINT]  .9% and specificity of 97. aABR has the additional advantage of identifying neonates with auditory neuropathy unlike testing for OAE. a fast integrated impedance check and a short measuring time (median of 25 seconds with a range of 15-112 seconds in each ear). quick. BERAphone has the advantages of having a very low cost of disposables because of the permanent stainless steel electrodes. In addition. simple and reliable with a sensitivity of 100% and specificity of 99.Implementation of a high risk screening program would therefore result in 50% children with congenital hearing loss being missed out.e 96 to 98% of neonates with one or more risk factors for hearing loss have normal hearing. in-utero TORCH infections etc.

procedure of the screening test. Their knowledge. parents were counseled and diagnostic testing using Auditory Steady State Response Audiometry (ASSR) was done. The first screening test was done in the postnatal wards or NICU after obtaining informed consent from the mother. Repeated phone calls and letters were used to contact parents of babies who failed to return for follow-up. the babies were examined. In the first month of the study they were trained in the anatomy and physiology of hearing. record keeping and reporting to the investigator. effects of hearing loss. Parents of babies who failed (‘refer’) the screening test were counseled and asked to return after 1 week for second screening. If response is detected the test produces a “PASS” result while failure to detect a response within 180 seconds produces a “REFER” result. social and cultural effects of hearing loss. et al.AUGUSTINE. METHODS This observational prospective cohort study was conducted between January and November of 2010 at our tertiary care centre after institutional research and ethical committee clearance was obtained. Newborns admitted in the neonatal intensive care unit (NICU) were screened prior to discharge from the NICU (once their general condition was stable). need for follow-up and further tests if the neonate failed the screening test and the interventions available if hearing loss was confirmed. Four women with a graduate qualification in biological sciences were recruited for the study. handling of the screening equipment and its use on neonates. All normal newborn babies delivered in the tertiary care hospital were screened by the 4 trained technicians using BERAphone between 24 hours and 72 hours after birth. The BERAphone conssits of a handheld headphone unit which is positioned on the babies head after application of electrode gel at the points of contact with the electrodes (vertex and mastoid). Mothers of all babies born in the tertiary care hospital were counseled regarding the benefits of hearing screening. UNIVERSAL NEONATAL HEARING SCREENING prevalence of neonatal hearing loss in a tertiary care setting and assessing the associated risk factors in those identified with hearing loss. An optimized chirp stimulus is used at 35dB and the system automatically detects the presence of an auditory brainstem response based on an implemented statistical test algorithm. These babies underwent a second testing in a quiet room. magnitude of the problem of hearing loss in the community. Those who passed on the second screening were discharged from the study while those who failed a second time were referred for further evaluation in the audiovestibular clinic (AVC) at the same centre. counsel parents and perform the screening test was assessed formally at the end of the training period. where a detailed history for risk factors [10] was obtained. 2013 [E-PUB AHEAD OF PRINT]  . INDIAN PEDIATRICS 4                OCTOBER 5. ability to obtain informed consent. congenital hearing loss and the benefits of early identification and rehabilitation.

ratios and proportions were calculated. The results of these 46 children are summarized in Figure 2. Thirty nine were confirmed to have hearing loss and 7 had bilateral normal hearing by ASSR. 9448 (97. 713 (82. Cytomegalovirus and Herpes simplex virus) and genetic testing for the connexin 26 gene mutation.4%) registered in the clinic. (Figure 1) Of the 9448 babies screened 2339 were NICU graduates. The discharge rate after the 2 stage sequential screening with BERAphone was 98. The babies who were referred after screening twice with BERA phone and whose parents consented for blood tests also underwent serological tests for known infective causes of hearing loss (Toxoplasma. An average of 200 babies were screened each week. Data obtained was analysed using SPSS. Rates. Three neonates had other INDIAN PEDIATRICS 5                OCTOBER 5. Table II shows the associated risk factors [10] identified in the screened babies who had been ‘referred’ after the second screening. RESULTS Among 9671 neonates born between 1st January and 30th November 2010. as well as to obtain frequency specific thresholds to enable more effective and appropriate hearing aid fitting.7%) babies were screened for hearing deficit . The most frequent risk factors were consanguineous marriage.9% with single screening. to detect those with auditory neuropathy. only 58 (35. history of in-utero infection and prematurity. These infants were referred to the AVC for further evaluation. (Table I) Among 164 babies referred to the AVC. The ASSR was done between 1 and 3 months of age. Among 58 neonates. The causal association of these risk factors cannot however be ascertained. The remaining 106 babies failed to come for follow-up despite repeated attempts (phone calls and letters) to contact the families. A total of 223 babies could not be screened since they were critically ill in the nursery and later died or were discharged at request. Eleven of these children were lost to further follow up and did not come back for confirmatory tests despite repeatedly contacting them.AUGUSTINE. Rubella. 30 had one or more risk factors. confirmation of hearing loss and appropriate rehabilitation. A total of 863 babies were referred on first screening which implies a discharge rate of 90. UNIVERSAL NEONATAL HEARING SCREENING ASSR was used as the diagnostic procedure to confirm hearing loss. Of the 39 with confirmed hearing loss 8 were NICU graduates.6%) came for second screening and 164 of them were referred again. Those confirmed with hearing loss were followed up in the AVC for further evaluation and appropriate rehabilitation. One child had died and therefore 46 children underwent confirmatory testing.2%. 2013 [E-PUB AHEAD OF PRINT]  . Of these 150 were referred on 1st screening and 14 were referred on 2nd screening. et al. Distortion product otoacoustic emission (DPOAE) testing was used in addition.

Under ideal conditions (sound proof room and a quiet sleeping child) the BERAphone screening test takes five minutes to complete. 1 child has undergone bilateral cochlear implant and has joined regular school. entry of data and supervision of the technicians is required. et al. 2013 [E-PUB AHEAD OF PRINT]  . a prevalence of 4. training of staff and establishing procedures for screening were found to be feasible and can be effectively done in any secondary or tertiary level hospital provided adequate knowledge about the importance of the programme. All children with confirmed bilateral hearing loss of moderate degree or more have been fitted with hearing aids and are on follow-up. thrombocytopenia. Those with severe to profound hearing loss have been advised cochlear implantation. Among 46 babies who underwent confirmatory testing. a frequency of suspected hearing loss of 17. Frequent evaluation of test procedures. However this is an underestimate considering the attrition at follow-up after second screening. Parents of 34 neonates consented for blood tests: screening for TORCH infections and Connexin 26 gene was done. 2 had mild to moderate hearing loss and one had normal hearing on ASSR. However. DISCUSSION Setting up an universal neonatal screening programme in a large tertiary centre and tailoring it such that the existing flow of patient care remains undisturbed can be a challenge.4 per thousand babies. screening took 10 to 15 minutes to complete in the postnatal ward since the ambient noise in the ward was more than 50 dB (as recorded in the wards with a sound level meter). Six neonates were positive for TORCH infections: 5 were positive for Cytomegalovirus while one was positive for Rubella. It is possible however to train a non-ENT surgeon for the supervisory role in the screening procedure.AUGUSTINE. Among 9448 babies who were initially screened. Of these 6 neonates. hepatosplenomegaly and patent ductus arteriosus. The BERAphones were quite easy to use and worked very well in high ambient noise surroundings. UNIVERSAL NEONATAL HEARING SCREENING congenital anomalies: 1 was diagnosed to have Down’s syndrome. The selection. 1 neonate had hydrocephalus. On an average. the procedure and equipment is available with the supervisory staff. The mutation found in this neonate was the common founder mutation W24X. ventricular septal defect and ectopic left kidney and 1 had microcephaly. 39 were confirmed to have hearing loss by ASSR. maintenance of equipment and interpretation of results. the equipment required frequent servicing by the company and the INDIAN PEDIATRICS 6                OCTOBER 5. 3 had severe to profound hearing loss. 164 babies were referred twice.. One neonate out of the 34 was positive for connexin 26 gene mutation and the ASSR showed severe to profound hearing loss. The screening program required intense supervisory input from the primary investigator as well as an audiologist.1 per 1000 babies screened.

Often babies required multiple attempts to obtain a satisfactory result because of artifacts produced by upper respiratory tract infections and failure of the baby to achieve deep sleep. However babies with risk factors are more likely to be brought back for follow-up as these children require frequent hospital visits for various other reasons. Frequently. This also had the advantage that those children who failed the test the second time could be referred for the diagnostic test earlier. In practice. The causal association of the identified risk factors is also difficult to ascertain. The fact that nearly 98% of the babies born in the hospital were recruited for the first screening and more than 80% of those identified on the first screening completed the 2nd stage of screening establishes the feasibility of a 2 stage sequential hearing screening protocol using automated ABR (BERAphone) in a tertiary care set-up. UNIVERSAL NEONATAL HEARING SCREENING software required frequent reinstallation. et al. our study showed a large attrition of patients.1 per 1000 babies screened. it is still an underestimation considering the large number of babies who were lost to follow-up. Follow up (after failing the test the first time) was intended at 6 weeks after birth. The laptop required constant recharging of batteries which added to delays and disruption in work and consequently limited the number of children who could be screened on a given day. it was found that the follow up was poor at 6 weeks and IgM testing for infective causes required an early sample. 2013 [E-PUB AHEAD OF PRINT]  . In spite of these measures. Only 46 of the 164 neonates identified on screening underwent confirmatory tests. However ensuring follow up of children who were referred twice proved to be the biggest hurdle. Implications of the results obtained The estimated prevalence of hearing loss among neonates in this study was 4. Nearly 50% of neonates who attended the AVC after failing the screening test twice had one or more risk factors for hearing loss. Although this value is similar to that obtained in other studies done in India [6-8]. This was because of the availability of only one testing facility for both the routine diagnostic testing of patients attending tertiary care and the neonates identified during the study. Hence the follow up appointment had to be advanced to one week after discharge. The waiting time for confirmatory testing was between 1 to 3 months. patients did not keep appointments and so had to be rescheduled for another date. Babies with confirmed hearing loss could be fitted with appropriate hearing aids by 6 months of age and started on auditory verbal therapy thereby initiating the process of early rehabilitation. Parents were more likely to come a week after discharge from the hospital for a checkup hence decreasing the dropouts.AUGUSTINE. High usage was the reason attributed. Most parents were INDIAN PEDIATRICS 7                OCTOBER 5. Most parents required repeated counseling and multiple telephone calls to return for confirmatory tests.

A large loss to follow-up is the biggest hurdle in the implementation of such a program. UNIVERSAL NEONATAL HEARING SCREENING unable to accept the fact that their child may have a hearing loss. its effect on the individual and society. its effects and the benefits of early identification in order to ensure that babies who are identified on screening are brought for further confirmatory testing and rehabilitation offered when indicated. We conclude that the BERA phone is an easy to use and reliable tool for screening newborns. The sensitivity and specificity of the equipment in the test setting however. We are also grateful to the technicians Ms Ramya. What this study adds?  The feasibility of a universal neonatal hearing screening program at a tertiary care set up in a developing country using automated ABR has been emphasized and the potential hurdles including a large number of loss to follow-up have been highlighted. The stigma associated with the use of a hearing aid was also a factor which deterred parents from bringing their children for further testing. 2013 [E-PUB AHEAD OF PRINT]  . The key to successful universal neonatal hearing screening lies in improving awareness regarding congenital hearing loss. Acknowledgments: The investigators wish to thank ICMR for providing the funds to carry out this screening programme. In the United States where nearly 95% of neonates are screened only half of those who do not pass the initial screening undergo confirmatory testing and rehabilitation [18. Others failed to understand the need for early rehabilitation due to the popular belief that hearing would improve as the child grows older. The problem of a huge loss to follow-up is a reality even in developed countries which have established universal neonatal hearing screening programs. which can be effectively used by trained technicians for the implementation of a screening program. Mrs. INDIAN PEDIATRICS 8                OCTOBER 5. et al. Selvi.19]. Some mothers who were from far off places and had come to the tertiary care hospital only for their delivery had returned to their hometown and could not therefore return for further follow-up. Angel. are to be ascertained. Revathy and Thenmozhi for carrying out the ASSR and the nurses in the wards for providing assistance. Measures to increase awareness regarding neonatal hearing loss.AUGUSTINE. What is already known?  Universal neonatal hearing screening has been widely instituted in most developed countries in view of the high prevalence of congenital hearing loss and the positive outcomes associated with early diagnosis and rehabilitation. available rehabilitation modalities and the effectiveness of early identification and rehabilitation are essential for the successful implementation of such a program. Indu and Bamini for performing the screening.

Joint Committee on Infant Hearing (JICH) 1994 position statement. New York: Thieme Medical Publishers Inc. Universal infant hearing screening by automated auditory brainstem response measurement.77:1036-41. LaFranchi S. Pediatrics. Neonatal mass screening for metabolic disorders: summary of recent sessions of the committee of experts to study inborn metabolic diseases. Judith A. Nagapoornima P. Eur J Pediatr. et al. critically revising article for important intellectual content and final approval of manuscript. REFERENCES 1. 2. Thakur N. 6. J Otolaryngol Suppl. RamachandranVS. Eavey RD. Murnane O. Clayton BE. analysis and interpretation of data and final approval of manuscript. J Pediatr. 2007. Beckers R. Rai N.14:28-33. Competing interests: None stated. 2013. 10. Corrado G. INDIAN PEDIATRICS 9                OCTOBER 5. 1985. Early identification of hearing loss and centralized newborn hearing screening facilityThe Cochin experience. et al. Indian Pediatr. Herrmann MD. RRP and AT: acquisition of data. 9. Srilakshmi. critically revising article for important intellectual content and final approval of manuscript and AL. Joint Committee on infant hearing. 2009. Screening for congenital hypothyroidism: results of screening one million North American infants. children. Larsen PR. In: Madell JR. Jacobson J. 2008. Stach BA. Dussault JH. JE. Fisher DA. Universal screening of newborns to detect hearing impairment – Is it necessary?. Ramesh A. Bachmann C. Technology.140:461-72. Indian J Pediatr.74:545-9. Durieux-Smith A. Kileny P. Rao S. and Management. Kenneth R. Pediatric Audiology: Diagnosis.95:152-6.94:700-5. Klein AH. Mehra S. and adolescents. 1994.101:221-8. The epidemiology of hearing impairment in the United States: newborns. Flexer C eds. Gore M. 1979. et al. 5. Funding: Indian Council for Medical Research (ICMR). Int J Pediatr Otorhinolaryngol.AUGUSTINE. KAK. 3. Universal Hearing Screening. acquisition. 4. 2013 [E-PUB AHEAD OF PRINT]  .1981. 8. et al. Bickel H.48:355-9. Otolaryngol Head Neck Surg. Mason MS. AKJ. Brandt NJ.137:133–9. UNIVERSAL NEONATAL HEARING SCREENING Contributors: AMA: drafted the manuscript. Pediatrics. Hyde M. Foley TP. SD and AB: concept and design of the study. 1998. Hearing disorders in children. 2011. 7. Patricia PL. 3-12. Keamy DG Jr. Incidence of hearing loss in high risk and intensive care nursery infants. Sanders R. P. Paul AK.

Year 2007 Position Statement: Principles and Guidelines for Early Hearing Detection and Intervention Programs. Behrens TR. Wenzel G. 12. Passali D. Cebulla M. Forsman I. Maxon AB. INDIAN PEDIATRICS 10                OCTOBER 5. et al. Shehata-Dieler W. Bagnoli F. Shulman S. Iwasaki S. Newborn hearing screening by transient evoked otoacoustic emissions: analysis of response as a function of risk factors. Kok JH. Acta Paediatr. 15. Universal newborn hearing screening program in Wurzburg. White KR. 2003. Singer D. Evaluation of the Universal Newborn Hearing Screening and Intervention Program. Seki A. Behrens TR.Br J Audiol. Dutch NICU Neonatal Hearing Screening Working Group. White KR. von Deuster Ch. Saltzman A. Maxon AB. 1993. Nagura M. 2003. 16. UNIVERSAL NEONATAL HEARING SCREENING 11.67:1099-104. White KR. Ireys H. 19. Costantini D. 14. Hayashi Y.126:S19-27. Vohr BR. Pediatrics. 2010. 2002. J Am Acad Audiol. A model of two-stage newborn hearing screening with automated auditory brainstem response. Hashimoto Y. 2013 [E-PUB AHEAD OF PRINT]  . Dieler R. 2012. Acta Otorhinolaryngol Ital. Oshima G. Int J Pediatr Otorhinolaryngol. 13. Vohr BR.81:204-10. van Straaten HL. 2007.76:536-43. De Felice C. Pediatrics. Referral rates and cost efficiency in a universal newborn hearing screening program using transient evoked otoacoustic emissions. Using transient evoked otoacoustic emissions for neonatal hearing screening.6:271-7. Verkerk PH. German.120:898-921. et al.92:332-8. 2003. Implementation of a nation-wide automated auditory brainstem response hearing screening Program in neonatal intensive care units. Shehata-Dieler WE. Int J Pediatr Otorhinolaryngol. Experience with more than 4000 newborns and the influence of non-pathological factors on the test results Laryngorhinootologie. ABR-based newborn hearing screening with MB11 BERAphone®using an optimized chirp for acoustical stimulation. Hille ET. 1995. De Capua B. Besculides M. Joint Committee on Infant Hearing. 17.27:149-53. Keim R.23:16-20. 18.AUGUSTINE.

7 12 Ototoxic drugs 2 (gentamicin) 3.4 10 Low Apgar score (≤4 at 1 min or ≤6 at 5 min) 2 3.4 11 Mechanical ventilation ( > 5 days) 1 1.3 4 Family H/O craniofacial anomalies 2 3.2 6 Hyperbilirubinemia (> 20mg/dL) 3 5. et al.7 2 Family history of hearing loss 5 8. UNIVERSAL NEONATAL HEARING SCREENING TABLE I SCREENING RESULTS OF NORMAL BABIES AND NICU GRADUATES Babies screened Babies refered on 1st screening Babies who underwent 2nd screening Babies refered on 2nd screening Babies who underwent confirmator y tests Babies with confirmed hearing loss Normal 7109 713 563 150 32 31 NICU graduates 2339 150 150 14 14 8 Total 9448 863 713 164 46 39 TABLE II FREQUENCY OF ASSOCIATED RISK FACTORS IN 58 BABIES WHO WERE REFERRED TWICE ON SCREENING WITH BERAPHONE S.2 11                OCTOBER 5.4 5 Family H/O syndromes 3 5.2 8 Prematurity (gestation <37weeks) 6 10.No Risk Factor Frequency % 1 Consanguineous marriage 12 20.2 7 Very low birth weight <1500g 3 5.AUGUSTINE.6 3 H/O in utero infection 6 10. 2013 [E-PUB AHEAD OF PRINT]  .3 9 H/O Meningitis 2 3.4 13 Other congenital diseases 3 INDIAN PEDIATRICS 5.

AUGUSTINE. 2013 [E-PUB AHEAD OF PRINT]  . et al. UNIVERSAL NEONATAL HEARING SCREENING Total number of births 9671 Number of babies who underwent 1st screening 9448 Number refered at 1st screening 863 Number of babies who underwent 2nd screening 713 Number referred at 2nd screening 164 Number of babies registered at the AVC 58 Number of babies who underwent confirmatory testing 46 Fig 1 Flowchart showing the number of babies at each stage of the screening program INDIAN PEDIATRICS 12                OCTOBER 5.

AUGUSTINE. 2013 [E-PUB AHEAD OF PRINT]  .46 Normal Confirmed hearing loss 7 39 Unilateral 9 Mild 5 Bilateral 30 Moderate 2 Severe to profound 2 Symmetrical 20 Mild profound 14 Moderate 2 Asymmetrical 10 Severe to profound 4 Moderately severe Severe to (in better ear) 3 (in better ear) 7 Fig 2 Results of ASSR on 46 infants who underwent confirmatory testing INDIAN PEDIATRICS 13                OCTOBER 5. et al. UNIVERSAL NEONATAL HEARING SCREENING Total number of infants who underwent ASSR .