Newborn hearing screening project using transient evoked otoacoustic emissions: Western Sicily experience

doi:10.1016/j.ijporl.2006.09.011

International Journal of Pediatric Otorhinolaryngology

Volume 71, Issue 1, January 2007, Pages 107-112

https://doi.org/10.1016/j.ijporl.2006.09.011 Get rights and content

Summary

Objective

To study the incidence of congenital sensorineural hearing loss in all newborns introducing a screen test with a protocol no expensive, with a good “screen sensitivity” that could let an earlier identification of hearing impairment beginning early intervention by 2 months of age and increasing the probability of having language development within the normal range of development.

Methods

The study was conducted in Sciacca hospital from the beginning of 2003 to our days and was carried out with transient evoked otoacoustic emission using the criteria for PASS or RETEST and considering eventual prenatal and perinatal risk factors. All the newborns were divided into four groups each one with its personal secondary step program.

Results

In the years 2003–2004 the number of the newborns in Sciacca hospital was: 538 for 2003, 653 for 2004 with a total of 1191; all these infants were divided in three groups: resident in Sciacca, resident in the Sciacca borderlands and resident out of the district of Sciacca. The coverage (percentage of the target population who undergo the screen) was of 90% in the 2003 (483 newborns) and of 90% in the 2004 (585 newborns) with two cases of congenital sensorineural hearing loss identified.

The incidence of sensorineural hearing loss, in the District of Sciacca, was estimated to be 2.07/1000 in 2003 and 1.70/1000 in 2004.

Conclusions

The higher incidence of sensorineural hearing loss in our study is due to a high prevalence of consanguineous marriage in Sicily that was shown to be linked with hearing impairment.

The “sensitivity value” was 95% at the first step but became 99% after the second step with a few number of false positive (0.74%).

All the infants with a diagnosis of sensorineural hearing loss began a rehabilitation program before the age of 5 months and they have a good speech development and speech intelligibility.

Introduction

Hearing loss is one of the commonest disorders present at birth; the literature report a large range of childhood hearing impairment (PCHI) from 0.7 to 4.2/1000 live births [1], [2], [3], [4], [5], [6], [7], [8], [9], [10], [11], [12], [13], [14], [15], [16]; it is probably due to many elements that each author studied:

•
unilateral or bilateral hearing loss;
•
severity of hearing loss;
•
age, sex, ethnicity and socio-economic status in a particular population;
•
presence in the group observed of prenatal and perinatal risk factors.

However, it should be mentioned that if we considered all the newborns to estimate the PCHI incidence, this value should be underestimated.

It has long been believed that an earlier identification of hearing impairment must lead to better outcomes, and currently there is a reliable evidence that this is the case in the domains of communication, educational achievement and quality of life [17], [18], [19], [20].

The reason of this way of thinking is founded on the fact that although the development of the cochlea is almost complete at birth the inner ear active mechanisms need a further maturation, a process which is related to and dependent upon auditory stimulation [21]. Thus a congenital permanent hearing impairment (PHI) is likely to result in physiological deprivation unless compensated and may result in insufficient development of the central auditory structures [22], [23].

Knowledge on the prevalence of congenital permanent childhood hearing impairment, as well as later acquired HI, forms the basis for the planning and provision of paediatric hearing health services and it is a strict requirement for the introduction of hearing screening as a public health matter.

Usually screening can be regarded as a specific medical intervention with the aim of detecting disease or a certain health condition in individuals who are not primarily suspected of having them [24]. In contrast to the usual diagnostic interventions, which are carried out when a condition is suspected or known to exist, screening is applied to a population regarded primarily as healthy; in our case the neonatal hearing screening procedure must be able to detect with adequate certainty the congenital hearing loss;

The American National Institutes of Health Consensus Statement [25] and more recently the European Consensus Statement [26] recommended the implementation of universal screening before 3 months of life.

Technological developments have led to the worldwide introduction of universal neonatal screens for hearing impairment so that the three techniques of evoked otoacoustic emissions (EOAE_S), automated auditory brainstem response (AABR) and a combination [27], [28], [29], [30], have now been accepted by Universal Newborn Hearing Screening (UNHS). All this techniques, that had many problems in the beginning, were developed by training and modifications of their own protocols.

Advantages of screening by TEOAE_S include [31]:

1.
a sophisticated examiner is not required to administer the test,
2.
actual testing time per infant ranges from 1 to 5 min,
3.
acoustic stimuli is non-invasive and costs less than use of disposable electrodes and ear cushions.

Screening tests do not identify deafness but underline those individuals who demonstrate a greater probability of having a hearing loss so they may be further evaluated to determine if a hearing impairment does exist, and if so, its extent. Furthermore, false-positive and false-negative results are inevitable in any economically feasible procedure.

The aim of this work is to develop a neonatal screening, based on transient evoked otoacoustic emissions (TEOAE_S), introducing a screen test with a low-cost protocol and need no medical control, increasing options for successful treatment.

Section snippets

Material and methods

To reduce the number of the fugitives and to have an adequate coverage (percentage of the target population who undergo the screen), we though to begin this screening program in a province (Sciacca) in which there is only a paediatric unit; the families of the babies who were born in others hospitals were informed by paediatrics about this study and decided to undergo or not undergo OAE_S screening.

The study was conducted in Sciacca hospital from the beginning of 2003 to our days and it was

Results

In the years 2003–2004 the number of the newborns in the Sciacca hospital was 1191 and of all 1068 underwent screening; all the infants were divided in three groups:

(1)
resident in Sciacca;
(2)
resident in Sciacca borderlands;
(3)
resident out of the district of Sciacca (Table 2).

In the 2003 the newborns born in the hospital of Sciacca were 538 and the number of infants whose underwent screening was 483 (90%) with a 10% (55 neonates) of escaped.

In the 2004 the number of newborns born in the hospital of

Discussion and conclusions

Universal newborn hearing screening results in earlier diagnosis of hearing loss. The diagnosis of hearing loss within the first few months of life allows the opportunity to begin early intervention services for families with infants.

Yoshinaga-Itano, Coulter and Thomson in a study of 294 children with congenital hearing loss, found that children born in a Colorado hospital which had a newborn hearing screening program were identified earlier than those born in hospitals without UNHS programs.

References (34)

B.R. Vohr et al.
Factor affecting the interpretation of transient evoked otoacoustic emission results in neonatal hearing screening, 1993–1996
J. Pediatr.
(1998)
H.S. Habib et al.
Neonatal hearing screening with transient evoked otoacoustic emissions in Western Saudi Arabia
Int. J. Pediatr. Otorhinolaryngol.
(2005)
W.K. Frankenburg et al.
Common pitfalls in the evaluation of developmental screening tests
J. Pediatr.
(1988)
H.M. Fortnum et al.
Health service implications of changes in aetiology and referral patterns of hearing-impaired children in Trent 1985–1993
Report of the MRC Institute of Hearing Research
(1997)
D. Nekahm et al.
Epidemiology of permanent childhood hearing impairment in the Tyrol, 1980–1994
Scand Audiol.
(2001)
H.M. Fortnum
Epidemiology of permanent childhood hearing impairment
Audiol. Med.
(2003)
H.M. Fortnum et al.
Prevalence of permanent childhood hearing impairment in the United Kingdom and implications for universal neonatal hearing screening: questionnaire based ascertainment study
Br. Med. J.
(2001)
C.D. Drews et al.
Hearing impairment among 10-year-old children: metropolitan Atlanta, 1985 through 1987
Am. J. Pub. Health
(1994)
Z. Naeem et al.
Prevalence of sensorineural hearing loss in Asian children
Br. J. Audiol.
(1996)
G.J. Sutton et al.
Risk factors for childhood sensorineural hearing loss in the Oxford region
Br. J. Audiol.
(1997)

T. Pitt

The Epidemiology of Childhood Hearing Impairment in South-east Ireland

(1996)

K. Van Naarden et al.

Prevalence and characteristics of children with serious hearing impairment in metropolitan Atlanta, 1991–1993

Pediatrics

(1999)

R. Morton et al.

Disability in children from different ethinc populations

Child: Care, Health and Dev

(2002)

K.P. Steel et al.

A genetic approach to understanding auditory function

Nat. Genet.

(2001)

M.B. Petersen

Non-syndromic autosomal-dominant deafness

Clin. Genet.

(2002)

M. Bitner-Glindzicz

Hereditary deafness and phenotyping in humans

Br. M. Bull.

(2002)

A. Parving

Prevalence of congenital hearing impairment and risk factors

Cited by (26)

Newborn hearing screening protocols and their outcomes: A systematic review
2018, International Journal of Pediatric Otorhinolaryngology
Citation Excerpt :
TEOAEs have however been reported to be the most common screening measure in NHS programmes worldwide as they are easier to conduct, have a shorter test time and are considered less expensive in terms of the need for consumables [16]. Repeated use of TEOAEs within a multi-stage screening protocol has also been reported to aid in reducing the number of more expensive, secondary level evaluations [2,17,18]. Berninger and Westling [2] also found that the specificity of TEOAE measurements increased with repeated TEOAE screening.
To conduct a review of the most current research in objective measures used within newborn hearing screening protocols with the aim of exploring the actual protocols in terms of the types of measures used and their frequency of use within a protocol, as well as their outcomes in terms of sensitivity, specificity, false positives, and false negatives in different countries worldwide.
A systematic literature review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis. Electronic databases such as PubMed, Google Scholar and Science Direct were used for the literature search. A total of 422 articles were identified, of which only 15 formed part of the current study. The 15 articles that met the study's criteria were reviewed. Pertinent data and findings from the review were tabulated and qualitatively analysed under the following headings: country; objective screening and/or diagnostic measures; details of screening protocol; results (including false positive and negative findings, sensitivity and/or specificity), conclusion and/or recommendations. These tabulated findings were then discussed with conclusions and recommendations offered.
Findings reported in this paper are based on a qualitative rather than a quantitative analysis of the reviewed data. Generally, findings in this review revealed firstly, that there is a lack of uniformity in protocols adopted within newborn hearing screening. Secondly, many of the screening protocols reviewed consist of two or more tiers or stages, with transient evoked otoacoustic emissions (TEOAEs) and automated auditory brainstem response (AABR) being most commonly used. Thirdly, DPOAEs appear to be less commonly used when compared to TEOAEs. Lastly, a question around routine inclusion of AABR as part of the NHS protocol remains inconclusively answered.
There is sufficient evidence to suggest that the inclusion of AABR within a NHS programme is effective in achieving better hearing screening outcomes. The use of AABR in combination with OAEs within a test-battery approach or cross-check principle to screening is appropriate, but the inclusion of AABR to facilitate appropriate referral for diagnostic assessment needs to be systematically studied.
Speech perception outcomes after cochlear implantation in prelingually deaf infants: The Western Sicily experience
2013, International Journal of Pediatric Otorhinolaryngology
Citation Excerpt :
Among CI users’ characteristics instead, only daily CI use (85.71% of the cohort) resulted an important predictor of good speech improvement (p < 0.0001). Considering the high percentage (44.68%) of profound SNHL in Western Sicily, mainly related to genetic causes, it is clear that several children, who do not benefit from hearing aids, could be candidates to cochlear implantation [17–20]. Many studies have demonstrated that CI is a suitable method to manage profound SNHL in children; however, because of a high variability in speech performances among CI users, several studies were conducted to identify the main significant predictors of cochlear implantation outcomes [1,2,5,7,8,11–13,16,26–36].
To describe audiometric characteristics and speech perception performances of prelingually deaf Sicilian children after cochlear implantation; to identify the influence of cochlear implant (CI) user and family's characteristics on speech recognition and intelligibility outcomes.
Twenty-eight infants with a congenital or acquired hearing impairment and implanted before the 3rd year of life were studied; all children suffered from bilateral sensorineural hearing loss (SNHL) with evidence of lack of hearing aids benefit and no evidence of intellectual disability. The study of the main characteristics associated with CI user and family's profile was performed with a clinical assessment including pre-implant and post-implant (1, 3, 6, 12 and 18 months) behavioural audiometry (evaluating average threshold for the frequencies 0.5, 1, 2 and 4 KHz) and speech recognition tests (IT-MAIS, MUSS, CAP and SIR).
Our cohort was characterized by an early diagnosis of SNHL (5.77 and 12.17 months for congenital and acquired HL respectively), a short length of deafness (average = 6.78 months) and an implantation before the 3rd year of life (mean = 24.25 months; range from 10 to 36). Analysis of audiometric threshold revealed a significantly improved capacity to detect sounds within the conversational speech spectrum after 12 months from implantation (r = 0.99; p < 0.001). The main speech recognition test evidenced speech perception and speech intelligibility performances (CAP median value of 3; SIR category = 3 in 46.42%) equal to those children with same characteristics reported by literature. With the exception of ‘daily CI use’ (p < 0.001), none of the variables associated with CI user and family's profile resulted significant predictor of speech perception improvement.
This work demonstrates that all children of our cohort, with an early diagnosis of SNHL and a CI surgery performed before the 3rd year of life, presented a progressive audiometric and speech improvement through the first 12–18 months after cochlear implantation. The study also highlights that, differently from the others variables studied, a continuous CI use influences significantly speech perception and recognition outcomes.
Prelingual sensorineural hearing loss and infants at risk: Western Sicily report
2013, International Journal of Pediatric Otorhinolaryngology
Citation Excerpt :
To date, in Italy, the goal of an early diagnosis of deafness in the childhood is not completely reached because of the absence of a newborn hearing screening (NHS) project extended to the entire country, with a certain number of SNHL remaining unidentified or diagnosed later than 6th month of age. In fact, in Sicily, where the initiatives are still left to individual hospitals, the NHS coverage percentage is less than 15% and very far from the North-West Italy coverage rate [15–17]. Literature reports underlined more times how understanding the main causes of prelingual hearing impairment – prenatal and/or perinatal risk factors – provides new information relevant to hearing loss management, coexisting medical problems and the prognosis for the child and family.
To evaluate independent etiologic factor associated with sensorineural hearing loss (SNHL) in newborn at risk; to study the role of their interaction especially in NICU infants who present often multiple risk factors for SNHL.
The main risk factors for SNHL reported by JCIH 2007 were evaluated on 508 infant at risk ranging from 4 to 20 weeks of life, transferred to the Audiology Department of Palermo from the main births centers of Western Sicily. After a global audiological assessment, performed with TEOAE, tympanometry and ABR, the prevalence and the effect of risk factors was statistically studied through univariate and multivariate analysis on the total population (normal and deaf subjects).
Fifty-one infants (10.03%) were diagnosed with SNHL (45 bilateral and 6 monolateral) with a mean hearing threshold of 87.39 ± 28.25 dB HL; from logistic regression analysis family history of hearing impairment (HI) and TORCH infections resulted independent significant risk factors (P < 0.00001 and P = 0.024 respectively). High SNHL percentages were evidenced also in NICU babies, due to the various pathologies and risk factors presented by these infants, and among newborns who suffered from hyperbilirubinemia requiring exchange transfusion (11.97% and 9.52% respectively). Craniofacial abnormalities (CFA) and syndromes associated to HI showed an important relationship (P < 0.00001) with conductive hearing loss (CHL). Multiple regression analysis of the variation in SNHL among NICU infants evidenced an increased risk for SNHL of 21.24% and of 19.33% respectively in preterm infants and in case of hyperbilirubinemia if respiratory distress is concomitant with these risk factors. It was also observed an higher risk of SNHL (99.66%) in case of coexistence of prematurity and hyperbilirubinemia. Finally among infants with very low birth weight (VLBW) it was evidenced a statistically difference between the mean weight of SNHL infants respect to NHL newborns (P = 0.048).
The high SNHL prevalence (10.03%) in our cohort underlines how infants at risk are more susceptible to suffer from SNHL; in particular NICU newborns have a 33% greater chance of developing SNHL, because of the presence of multiple risk factors (or = 1.33) and their interaction. As the number of coexisting risk factors increases, the prevalence of SNHL also increases (r² = 0.93).
Audiologic profile of infants at risk: Experience of a Western Sicily tertiary care centre
2012, International Journal of Pediatric Otorhinolaryngology
To identify the incidence of sensorineural hearing loss (SNHL) on infant at risk and to classify the degree and type of hearing loss describing the main causes associated in Western Sicily. To compare single TEOAE and combined TEOAE/ABR techniques studying the referral rate, the false-positive and false-negative rates through concordance test (κ coefficient), sensitivity (TPR) and specificity (TNR) for each protocol.
From January 2010 to June 2011, 412 infants at risk, ranging from 4 to 20 weeks of life, transferred to Audiology Department of Palermo from the births centers of Western Sicily, underwent to audiological assessment with TEOAE, tympanometry and ABR. The following risk factors were studied: family history of SNHL, consanguinity, low birth weight, prematurity, cranio-facial abnormality and syndromes associated to SNHL, respiratory distress, intensive care in excess of 5 days (NICU), pregnant maternal diseases, perinatal sepsis or meningitis, hyperbilirubinemia, ototoxic drugs administration.
Forty-seven infants (11.41%) were diagnosed with SNHL; median corrected age at final audiological diagnosis was 12 weeks. SNHL resulted moderate in 44.68%, severe in 10.64% and profound in 21 cases with a significant difference in family history and NICU infants (p < 0.0001). As the number of coexisting risk factors increases, the percentage value of SNHL in infants (χ² = 12.31, p = 0.01, r² = 0.98) and the degree of hearing loss (χ² = 13.40, p = 0.0095, r = 0.92) also increase. The study of single TEOAE and combined TEOAE/ABR showed a statistical difference (χ² = 14.89, p < 0.001) with a low concordance value (κ = 0.87) confirming the importance of combined techniques for NICU group (κ = 0.86) where four cases (0.97%) of auditory neuropathy were diagnosed.
This study demonstrates the necessity to implement a neonatal hearing screening program in Western Sicily because of the high percentage of SNHL in infants at risk. Family history of HL is an independent significant risk factor for SNHL easily diagnosed through single TEOAE technique. Combined TEOAE/ABR is the gold standard for NICU babies which are at risk for auditory neuropathy. Coexisting risk factors are an additional risk factor for HL.
On the threshold of effective well infant nursery hearing screening in Western Sicily
2012, International Journal of Pediatric Otorhinolaryngology
Citation Excerpt :
Aim project was also to develop an easy and suitable screening programme comfortable with the current objective hearing screening technologies, with high sensitivity (the proportion of the children tested with a specified condition, detected by the screening protocol) and specificity (the proportion of the children tested who do not have a specified condition who are correctly identified by the screen protocol) reducing both the number of more expensive secondary level exams and the workload of the only third level speech and hearing centre present in Western Sicily that represent the effectiveness of UNHS recommended by JCIH [2]. This study was carried out by the Department of Audiology, University of Palermo, together with the District of Sciacca, screening all the newborns of Sciacca hospital from January 2003 to date [12]. The District of Sciacca was chosen for the screening because in this province there is only a paediatric unit and the percentage of eligible newborns that were screened before hospital discharge or within the first month of life could have been, according to quality benchmarks of JCIH, >90%.
To determine the feasibility and effectiveness of well-infant nursery hearing screening programme for the early identification of hearing impairment, based on transient evoked otoacoustic emission (TEOAE) with a high “screen sensitivity” reducing the number of more expensive secondary level exams.
The newborns were screened by non-specialist health workers in well babies nursery at the twentieth day of life for 6 years consecutive. Based on PASS/FAIL criteria and presence/absence of audiological risk factors the newborns were divided into four groups each one with its personal step programme: G1 – PASS without risk factor, free to go home; G2 – PASS with risk factor, retest at the age of 7 months; G3 – FAIL without risk factor, re-screening after 2 weeks for a maximum of four times before audiology assessment; G4 – FAIL with risk factor, retest after 2 weeks.
The coverage rate increased progressively from 89.8% to 92%. The referral rate was 1.51% after second stage with a specificity value of 98.78%. The four-stage screening performed for G3 reduced the numbers of global audiology assessment to 0.91% with a final global specificity of 99.4 ± 0.4%.
Less than 1% of infants underwent audiological assessment; the false positives resulted 0.62% with hearing loss global incidence of 2.95/1000 and a mean age of confirmation of 3.5 months of age. It is reasonable to think that this screening programme could be implement to overall 42 Western Sicily birth centres within few years.
Newborn hearing screening: An outpatient model
2009, International Journal of Pediatric Otorhinolaryngology
Citation Excerpt :
The outpatient model incorporates infant hearing screening into a return visit to a health care facility. Implementation of outpatient models along these lines has been conducted in South Africa [7] and Italy [13], where the initial screening was performed after discharge from the hospital in a cost-effective effort to increase the number of babies receiving hearing screening before the age of 3 months. The purpose of this study was to assess the feasibility of implementing an outpatient model of an NHS program in the city of Recife.
The purpose of this study was to assess the feasibility of implementing an outpatient model of a newborn hearing screening program with a particular focus on determining how compliance with the follow-up appointment related to specific socio-economic and demographic factors associated with the mother.
Mothers who delivered their babies in public hospitals in Recife, northeast Brazil, were invited to participate in a two-step program. In Step 1 they were interviewed with regard to specific socio-economic and demographic factors, and then scheduled for a hearing screening test for their baby 1 month after discharge. In Step 2, the baby's hearing was screened using transient otoacoustic emissions.
A total of 1035 mothers consented to participate in Step 1, but only 149 returned to participate in Step 2 (14.3%). Analysis of the socio-economic and demographic factors indicated that mothers who did not comply with the scheduled newborn hearing screening (NHS) test generally had less than a high school education and came from primarily lower income families who lived in rural areas outside of Recife.
The results of this study highlight some socio-economic and demographic factors of the population of northeast Brazil that contribute to a low compliance rate for an outpatient model of a newborn hearing screening program. Possible solutions to the low compliance rate are considered.

View all citing articles on Scopus

View full text

Newborn hearing screening project using transient evoked otoacoustic emissions: Western Sicily experience

Summary

Objective

Methods

Results

Conclusions

Introduction

Section snippets

Material and methods

Results

Discussion and conclusions

J. Pediatr.

Int. J. Pediatr. Otorhinolaryngol.

J. Pediatr.

Health service implications of changes in aetiology and referral patterns of hearing-impaired children in Trent 1985–1993

Report of the MRC Institute of Hearing Research

Epidemiology of permanent childhood hearing impairment in the Tyrol, 1980–1994

Scand Audiol.

Epidemiology of permanent childhood hearing impairment

Audiol. Med.

Prevalence of permanent childhood hearing impairment in the United Kingdom and implications for universal neonatal hearing screening: questionnaire based ascertainment study

Br. Med. J.

Hearing impairment among 10-year-old children: metropolitan Atlanta, 1985 through 1987

Am. J. Pub. Health

Prevalence of sensorineural hearing loss in Asian children

Br. J. Audiol.

Risk factors for childhood sensorineural hearing loss in the Oxford region

Br. J. Audiol.

The Epidemiology of Childhood Hearing Impairment in South-east Ireland

Prevalence and characteristics of children with serious hearing impairment in metropolitan Atlanta, 1991–1993

Pediatrics

Disability in children from different ethinc populations

Child: Care, Health and Dev

A genetic approach to understanding auditory function

Nat. Genet.

Non-syndromic autosomal-dominant deafness

Clin. Genet.

Hereditary deafness and phenotyping in humans

Br. M. Bull.

Prevalence of congenital hearing impairment and risk factors