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Background The Newborn Hearing Screening Programme (NHSP) was introduced in England in 2001 to detect congenital hearing loss in the newborn. The screen is either hospital- or community-based. Objectives This is the first large-scale study of community-based NHSP published in the United Kingdom which aims to evaluate the performance of the community-based screen and compare it against national targets for NHSP and the outcome of national pilot projects. Method Hearing screening data recorded for 10 074 well babies between March 2004 and December 2005 were analysed. Babies who were admitted to the Special Care Baby Unit were excluded. The case notes of all children who failed the initial hearing screen, either unilateral or bilateral, were reviewed retrospectively. Specific performance measures include coverage rate, referral rate and yield. Reasons for failure to complete the screen were identified. Results The community programme met all the standards set by the NHSP and the results are comparable with the average of the pilot sites reported in 2004. Conclusion The data demonstrate that a community-based hearing screening programme conducted by Health Visitors meets all the current national standards and could be implemented across wider areas in this country. Its advantages include a low false positive rate and convenience for parents living in rural areas. The babies identified can be diagnosed and rehabilitated in a time which meets national standards. [ABSTRACT FROM AUTHOR] more...

Hearing impairment (HI) is the most frequent sensory disorder, with a genetic etiology in >50% of all cases, due to mutations in >44 identified genes. Autosomal recessive inheritance explains the majority, with GJB2 (connexin 26) mutations accounting for 15-50% of paediatric HI. Delayed presentation of HI to 11-60 months in cases of biallelic GJB2 mutations is a concern, necessitating a good audiological follow-up in addition to neonatal hearing screening. Providing a genetic diagnosis in congenital HI has implications for the prognosis, the possible risk of associated medical manifestations, and precise genetic counseling of the family, and should be integrated into the medical examinations done in order to diagnose syndromic features. Large-scale mutation detection methods, such as micro arrays, are promising for wider genetic testing, but few studies on their clinical utility have been published, so far. Limitations of interpretation of genetic test results, combined with significant ethical issues, currently do not justify to institute genetic screening for GJB2 mutations in neonates before a diagnosis of HI is established. [ABSTRACT FROM AUTHOR] more...

Summary: Objective: To determine the feasibility and effectiveness of hospital-based universal newborn hearing screening programme for the early detection of permanent congenital or early-onset hearing loss (PCEHL) in Lagos, Nigeria. Methods: A cross-sectional pilot study based on a two-stage universal newborn hearing screening by non-specialist health workers using transient evoked otoacoustic emissions (TEOAE) and automated auditory brainstem-response (AABR) in an inner-city maternity hospital over a consecutive period of 40 weeks. The main outcome measures were the practicality of screening by non-specialist staff with minimal training, functionality of screening instruments in an inner-city environment, screening coverage, referral rate, return rate for diagnosis, yield of PCEHL and average age of PCEHL confirmation. Results: Universal hearing screening of newborns by non-specialist staff without prior audiological experience is feasible in an inner-city environment in Lagos after a training period of two-weeks. Notwithstanding excessive ambient noise within and outside the wards, it was possible to identify a test site for TEOAE screening within the hospital. The screening coverage was 98.7% (1330/1347) of all eligible newborns and the mean age of screening was 2.6 days. Forty-four babies out of the 1274 who completed the two-stage screening were referred yielding a referral rate of 3.5%. Only 16% (7/44) of babies scheduled for diagnostic evaluation returned and all were confirmed with hearing loss resulting in an incidence of 5.5 (7/1274) per 1000 live births or a programme yield of 5.3 (7/1330) per 1000. Six infants had bilateral hearing loss and the degree was severe (≥70dBnHL) in three infants, moderate (40dBnHL) in one infant and mild (<40dBnHL) in two infants. The age at diagnosis ranged from 46 days to 360 days and only two infants were diagnosed within 90 days. Conclusions: Hospital-based universal hearing screening of newborns before discharge is feasible in Nigeria. Non-specialist staff are valuable in achieving a satisfactory referral rate with a two-stage screening protocol. However, a more efficient tracking and follow-up system is needed to improve the return rate for diagnosis and age of confirmation of hearing loss. [Copyright &y& Elsevier] more...

Otoacoustic emissions (OAEs) have attracted a great deal of interest from audiologists, biophysicists, cell biologists, and engineers worldwide. Despite great achievements having been made in recent years, many theoretical and practical aspects of OAE assessment still remain to be explored. One important area of focus for further OAE research is the development of optimal recording techniques and methods of data processing, which are expected to offer: 1) an improvement in the amplitude and the SNR of the OAE response, and thus increase the pass rate in newborn hearing screening; and 2) a decrease in OAE acquisition time. This paper examines the current limitations of OAE recording for neonatal hearing screening, analyses the possible reasons for these constraints, and reviews possible clinical and technological solutions that have been considered to date. [ABSTRACT FROM AUTHOR] more...

We performed a retrospective analysis of TEOAEs from 1415 neonatal ears to investigate whether the application of linear averaging techniques may provide greater screening specificity than conventional nonlinear averaging techniques. Pass criteria were based on correlations between replicate averages, and pass rates were compared between linear and nonlinear averaging techniques. The linear averaging technique gave a higher pass rate as hypothesized, so that 35% of ears that passed using the linear method failed using the nonlinear method. In addition, lack of saturation was more evident for the short-latency components of the TEOAE. These results, from a large sample of recordings obtained under field conditions, indicate that there is potential to improve specificity of neonatal TEOAE screening using linear averaging techniques. A combination of linear and nonlinear techniques for the long and short latency components of the TEOAE, respectively, may offer further advantages. [ABSTRACT FROM AUTHOR] more...

Introduction: congenital hearing loss is a serious health problem affecting 1 to 3 out of 1000 neonates, and is the most common cause of neurosensorial defect; hearing screening helps to identifying early permanent childhood hearing loss (HL). Objective: to establish the percentage of hearing loss in newborns with and without risk factors through hearing screening. Material and methods: by using a cross-sectional design, a group of neonates with risk factors and a group without risk factors were included in a two-stage hearing screening program. The otoacoustic emissions (OAEs) procedure was done in the first stage and the automated auditory brain stem response (AABR) procedure in the second stage. The latter was performed only in children with abnormal results in the OAE procedure. Results: 518 newborns were included in the hearing screening procedures; 220 neonates with risk factors and 298 without risk factors. 35 had + OAE test and the ABBR procedure helped to confirm that 30 neonates had hearing loss, 26 had bilateral HL; 10 had asymmetric HL; 4 had unilateral HL and 5 were false positives. The AABR procedure ascertained 17 HL cases with a threshold > 40 dB (86 per 100 000), 11 cases (55 per 100 000) in the risk group and 6 (30 per 100 000) in the non-risk group (p < 0.05). The main risk factors were prematurity, craniofacial anomalies, mechanical ventilation, prescription of amikacin and cytomegalovirus infection. Conclusions: the two-stage hearing screening program ascertained that 5.7 % had HL. AABR (threshold > 40 dB) confirmed 17 (86 per 100 000) neonates with HL, which was more frequent in the risk group (5 %) than in the non-risk group (2 %; p < 0.05). [ABSTRACT FROM AUTHOR] more...

We aimed to investigate the incidence of false alarms that occurred with the pass/fail criteria used in a published series of neonatal hearing screening programmes, as a function of age. We analysed the database of 19137 normally hearing babies (38274 ears) tested in the Wessex Universal Neonatal Hearing Screening Project. Otoacoustic emissions were recorded prior to discharge from maternity units, using IL088 equipment. We assessed the pass/fail rate using the Wessex criteria and 10 other pass/fail criteria published in the literature. Using Pearson's correlation coefficient, a statistically significant correlation between signal-to-noise ratio at each of the frequency bands 1, 2, 3, 4 and 5 kHz and babies' age in hours at the 0.01 level was identified. The correlation was also significant (0.01 level) between age and frequency reproducibility in each of the bands at 1, 2, 3, 4 and 5 kHz as well as the whole reproducibility. The number of false alarms reduced significantly after the first 24 h of life with all the criteria examined. We conclude that in the first hours after birth due to insufficient maturation of the otoacoustic emission, there is a high rate of false alarms. This increase in the false alarm rate, whilst dependent on the criteria used, occurs with all criteria. This leads to the consideration of whether the establishment of age-dependent pass/fail criteria could reduce the false alarm rate and the subsequent strain on diagnostic centres. [ABSTRACT FROM AUTHOR] more...

This paper aims to describe the implementation of a Neonatal Hearing Screening Program in a school hospital, focusing on the return rates among infants who failed the first screening. The population who goes to the school hospital for health services comes mainly from economically underprivileged groups. Even though our previous return rates were comparable to those reported elsewhere, we felt it was important to improve the methodology of the screening process in order to try and obtain better results. Our hypothesis was that scarce knowledge on early hearing loss detection and on the benefits of early intervention could be reasons for mothers to give less importance to the second screening. So, a strategy was developed around the idea of providing very detailed information to the mothers about the screening process since preliminary data, gathered with a different group of in-patients, had shown that mothers possessed little knowledge about neonatal hearing screening and the consequences of hearing loss for children's development. The no-return rates decreased considerably (from 39.8% to 25.8%). The findings of this research showed both the need of an adequate way of imparting information to the mothers of newborns about hearing screening in economically underprivileged populations, and the role of social work in this process. Without adequate knowledge on hearing screening and the consequences of hearing loss, a high percentage of newborns may not take advantage of free universal hearing screening programs. [ABSTRACT FROM AUTHOR] more...

Conclusion. High frequency immittance measurements demonstrate promise in clarifying middle ear status for neonates but age- and gender-specific norms should be consulted. Objective. To describe high frequency immittance measurements using a 1000 Hz probe tone for a sample of 278 neonatal ears (0–4 weeks of age) in order to compile normative tympanometric and acoustic reflex criteria. Subjects and methods. Assessment of neonatal ears included 1000 Hz probe tone immittance measurements (tympanograms and ipsilateral acoustic reflexes), and distortion product oto-acoustic emission (DPOAE) screening. Results were compared and normative values were compiled for immittance measures in ears controlled for normal middle ear functioning (n=250). Results. Comparison of immittance results to OAE screening outcome provides estimates of sensitivity and specificity for middle ear fluid with tympanometry of 57% and 95%, and 57% and 90% for acoustic reflex presence, and 58% and 87% for combined tympanogram and acoustic reflex results, respectively. Normative data indicate that static peak admittance values differ significantly across gender and age with the 5th percentile cut-off value for the entire sample at 1.4 mmho. The 90% range of tympanic peak pressure normative values increases with increasing age from 140 daPa for neonates 1 week of age to 210 daPa for neonates 2–4 weeks of age. Acoustic reflexes were elicited at 93±9 dB with a 90% normality range of 80–105 dB. [ABSTRACT FROM AUTHOR] more...

Aim: To examine the relationship between neonatal auditory function and Apgar score in term infants with depressed Apgar scores. Methods: Brainstem auditory evoked response (BAER) was recorded on day 3 after birth in term infants who had Apgar scores ≤ 7 at 1 and/or 5 min. Half of the infants also had Apgar scores at 10 min, with 17 ≤ 7. Results: No BAER variables correlated significantly with 1-min Apgar score. However, wave III and V latencies, and I–V, I–III and III–V intervals correlated significantly and negatively with 5-min Apgar score (p < 0.05–0.01). These BAER variables were significantly longer in infants with 5-min Apgar scores ≤ 7 than those > 7. Wave V latency and all intervals also correlated negatively with 10-min Apgar score (p < 0.05–0.01). Compared to normal controls, all latencies were prolonged in infants with depressed Apgar scores (all p < 0.05–0.01). All intervals were also prolonged in those with 5-min scores ≤ 7 (p<0.05–0.01). Similar results were found when defining the depression of Apgar score as ≤ 6.Conclusion: A depressed 5- and/or 10-min Apgar score is an indicator associated with neonatal auditory, mainly central, impairment. Apgar score ≤ 7 or 6 at 1 min alone is unlikely to be associated with central impairment. [ABSTRACT FROM AUTHOR] more...

Conclusion. Term infants with a low Apgar score have cochlear impairment, mainly at the frequencies 1–3 kHz. Compared with infants with both a low Apgar score and hypoxic-ischaemic encephalopathy we reported before, the impairment is less severe. Objective. To detect any peripheral impairment of cochlear origin in infants with a low Apgar score. Subjects and methods. Fifty-four term infants with a low Apgar score at 1 and/or 5 min but without clinical signs of hypoxic-ischaemic encephalopathy were recruited. Distortion product otoacoustic emissions (DPOAEs) were recorded with the f 2 primary tone at 10 frequencies (0.5–10 kHz) on days 3–5 and 1 month after birth. Results. On days 3–5 DPOAE pass rates at most frequencies tended to be decreased, and were significant lower than those in normal term controls at 1, 2, 3, 5, 6 and 10 kHz (x²=4.49–40.31, p<0.05–0.005). The greatest difference occurred at 1 kHz; 18.5% failed the DPOAE test and this was significantly higher than in the controls (4.3%, x²/7.65, p<0.01). At 1 month the DPOAE pass rate at most frequencies did not show any significant improvement. The overall failure rate (14.8%) did not differ significantly from that on days 3–5. [ABSTRACT FROM AUTHOR] more...

Abstract: The neonatal hearing screening comes within a step of secondary prevention of handicaps insofar as deafness has obvious consequences on the cognitive, linguistic and emotional development of the child. However, informing the parents of a potential sensory dysfunction from the first days of the child life can be the cause of a disorganization in the first parent–child interactions, and some professionals give an opinion that proves to be sensitive to many regards. This is why several studies examined this question and gave out contrasted conclusions on it. Thus, our works aims at evaluating the consequences, on the parental anxiety and quality of parent–child interactions, of a hearing screening program according to the moment when the test was carried out (4 days vs 2 months). To do so, we led semi-directing interviews with the mothers of the tested children. The results showed that a positive result entailed a significant increase in the maternal anxiety which deteriorated the general psychological state of the mother, especially in the neonatal period in which the test was carried out. This resulted in specific interactive profiles in which the mothers of the children with a positive result presented less insurance in the way they managed the interaction with their baby. [Copyright &y& Elsevier] more...

The impact of otoacoustic emissions in clinical practice spans a period of almost 30 years during which several important questions have been raised relative to the improved quality of the responses, the possible detection of hearing threshold, etc. In the last decade technological advances in the field of electrophysiology have provided new procedural methodologies which can be used as additional tools in otoacoustic emission testing. The purpose of this technical note is to provide a broad introduction to the technical trends we are now experiencing in clinical audiology. [ABSTRACT FROM AUTHOR] more...

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In studies to assess the accuracy of a screening test, often definitive disease assessment is too invasive or expensive to be ascertained on all the study subjects. Although it may be more ethical or cost effective to ascertain the true disease status with a higher rate in study subjects where the screening test or additional information is suggestive of disease, estimates of accuracy can be biased in a study with such a design. This bias is known as verification bias. Verification bias correction methods that accommodate screening tests with binary or ordinal responses have been developed; however, no verification bias correction methods exist for tests with continuous results. We propose and compare imputation and reweighting bias-corrected estimators of true and false positive rates, receiver operating characteristic curves and area under the receiver operating characteristic curve for continuous tests. Distribution theory and simulation studies are used to compare the proposed estimators with respect to bias, relative efficiency and robustness to model misspecification. The bias correction estimators proposed are applied to data from a study of screening tests for neonatal hearing loss. [ABSTRACT FROM AUTHOR] more...

Technological developments have led to the worldwide introduction of universal neonatal screens for hearing impairment. Although automated behavioural tests were the first to be employed, it has been the development of simple, sensitive, objective oto-acoustic emission testing and automated auditory brainstem response recording that has made such screens possible. Investigation has demonstrated that the emission test and the auditory brainstem (ABR) employing a 30 dB stimulus are equally valid. The specificity critically depends upon the age of maternity testing, but a false positive rate of 8% at discharge is typical when an initial oto-acoustic emission test is undertaken in the maternity unit. This is reduced to 1% or less by implementing a second out-patient test, or by undertaking an automated ABR before hospital discharge. Combination protocols using both techniques have therefore been recommended. A reduction in the numbers referred for diagnostic audiological assessment has been confirmed. Irrespective of test method, screens have identified around 1.2/1000 with a permanent childhood hearing impairment, and this yield is doubled if mild and unilateral losses are included. The median age at which habilitation has been introduced for all degrees of deafness has been reduced to the second half of infancy, with programmes reducing the median age of hearing aid fitting for severe or profound deafness to under 4 months. The provision of appropriate habilitation at this age presents new challenges. However, qualitative review of the many screens now being undertaken throughout the world confirms both their practicability and their effectiveness. [ABSTRACT FROM AUTHOR] more...

Following a systematic review of the role of neonatal hearing screening in the identification of hearing impaired and deaf children in 1997, the Department of Health in England commissioned a national programme of newborn hearing screening in 2001 and has linked a systematic evaluation to the first 22 sites in the implementation of that policy. It is anticipated that all areas of England will participate in the programme by 2004–2005. It is expected that other regions of the UK will also aim for implementation by this date.In addition to successful advocacy by the two major consumer organisations, there were three key elements linked to the decision to implement newborn hearing screening which were primarily evidence based and also driven by two significant service developments.The evidence base showed that newborn hearing screen was considerably more effective and efficient than the infant distraction test used in many areas and that there was a high probability that early intervention increased the potential for better language and communication.The concern over raised anxiety for those parents whose babies need differential assessment as a result of the screen is reduced by the low number of referrals and increased efficiency of assessment when national protocols are used for screening and assessment.The introduction of family friendly hearing services (FFHS) provides a context in which the health, education, voluntary and social services can work together to provide an equitable and seamless service throughout the year for hearing impaired and deaf children and their families.Currently, 22 areas with 110 k births pa, have been selected for implementation, some of whom use a pilot community-based model for screening. A major barrier to rapid progress has been a lack of evidence concerning elements of the screening process, e.g. equipment, IT systems, training at all levels. It is anticipated that a further 20–40 areas with an additional 150 k birth pa will be brought into the programme by end of 2002–2003.The aims, protocols, training programme and evaluation of the NHS Newborn Hearing Screening Programme are presented with a view to highlighting the research needed to improve specification of large scale screening programmes. [Copyright &y& Elsevier] more...

The characteristics of high frequency (1000 Hz) acoustic admittance results obtained from normal neonates were described in this study. Participants were 170 healthy neonates (96 boys and 74 girls) aged between 1 and 6 days (mean = 3.26 days, SD = 0.92). Transient evoked otoacoustic emissions (TEOAEs), and 226 Hz and 1000 Hz probe tone tympanograms were obtained from the participants using a Madsen Capella OAE/middle ear analyser. The results showed that of the 170 neonates, 34 were not successfully tested in both ears, 14 failed the TEOAE screen in one or both ears, and 122 (70 boys, 52 girls) passed the TEOAE screen in both ears and also maintained an acceptable probe seal during tympanometry. The 1000 Hz tympanometric data for the 122 neonates (244 ears) showed a single-peaked tympanogram in 225 ears (92.2 %), a flat-sloping tympanogram in 14 ears (5.7 %), a double-peaked tympanogram in 3 ears (1.2 %) and other unusual shapes in 2 ears (0.8 %). There was a significant ear effect, with right ears showing significantly higher mean peak compensated static admittance and tympanometric width, but lower mean acoustic admittance at +200 daPa and gradient than left ears. No significant gender effects or its interaction with ear were found. The normative tympanometric data derived from this cohort may serve as a guide for detecting middle ear dysfunction in neonates. [ABSTRACT FROM AUTHOR] more...

Congenital deafness is a major pediatric problem, affecting about 1.5–3 per 1000 newborns. The early treatment through cochlear implantation and auditory rehabilitation has been a historic milestone. Early diagnosis of congenital deafness is an essential requirement to obtain the best results, which is achieved through neonatal screening, a diagnostic practice that we began systematically at the Hospital Clínico in Valencia (Spain) 30 years ago. Neonatal hearing screening is successful in most developed countries. Its implementation has been slow due to the multiple difficulties that its universal application entails since it involves several health professionals and must be carried out, in a short time interval after birth. In addition, it must have a good performance that prevents the overload of other services and that requires experience and continuous adjustments in search of proper protocols. The aim of this review is to shed some light on some key points of neonatal hearing screening, highlighting our experience in the solutions to common problems. We will discuss about techniques, protocols and neonatal or nutritional factors that can influence the screening results. To a summary of our work, an update on the subject is provided with the intention of sharing experiences and facilitating the start-up of the new units. [ABSTRACT FROM AUTHOR] more...

Unlabelled: Severe congenital hearing impairment is an important handicap affecting 0.1% of live-born infants and 1%-2% of graduates of Neonatal Intensive Care Units. The prognosis for intellectual, emotional, language and speech development in the hearing-impaired child is improved when the diagnosis is made early and intervention is begun before the age of 6 months. The usual age at diagnosis of hearing impairment is at least 18-30 months (or even later in cases of less severe hearing impairment) where there are no screening programmes. When screening is carried out using distraction methods at the age of approximately 9 months some hearing-impaired infants are missed and those discovered are at least 15-18 months before intervention begins. Neonatal screening could give hearing-impaired children the best chances for optimal care and development. Universal neonatal hearing screening is necessary, because, when neonatal hearing screening is restricted to high risk groups 30%-50% of infants with hearing loss are not discovered. The methods available for neonatal hearing screening are discussed in this paper.Conclusion: In our view automated measurement of auditory brainstem responses is the most valuable method for universal neonatal hearing screening. [ABSTRACT FROM AUTHOR] more...

Very preterm infants are at a greater risk of developing neurodevelopmental impairments such as neuro-motor delays, vision and hearing deficits (Roze and Breart, 2004; Saigal and Doyle, 2008) [1,2]. The hearing difficulties in preterm infants vary depending on the co-morbid conditions. However, prematurity itself is considered as a risk factor that influence the functioning of auditory system. The current study aims to compare the DPOAEs in very preterm infants and term infants at 1 month, 3 months and 6 months of age (corrected age in preterm infants). DPOAEs were recorded in 72 very preterm infants and 30 term infants at 1 month, 3 months and 6 months of age. All these infants had obtained 'pass' results in newborn hearing screening using ABR. DPOAE f2 test frequency was measured at six frequencies (1500 Hz, 2000 Hz, 3000 Hz, 4500 Hz, 6000 Hz and 8000 Hz) with primary tone stimulus intensity L1 equal to 65 dBSPL and L2 equal to 55 dBSPL with primary tone f2/f1 frequency ratio of 1.2. Otoscopic examination and tympanometry was performed prior to DPOAE testing, to ascertain normal middle ear status. DPOAE amplitude did not change significantly between two groups from 1 month till 6 months of age (p > 0.05). DPOAE amplitude and noise floor in very preterm infants were not different from term infants and DPOAE amplitude did not vary significantly across f2 frequencies at various time periods. The current study findings provided evidence that prematurity did not constitute as a factor to influence the results of DPOAE in very preterm infants who passed newborn hearing screening test. Any significant reduction in DPOAE amplitude or absence of DPOAE in very preterm infants has to be considered and monitored effectively, as it may not reflect a developmental process of cochlear function; instead it could indicate the presence of inner ear or middle ear pathology. [ABSTRACT FROM AUTHOR] more...

In order to improve early detection of congenital permanent childhood hearing impairment the Austrian ENT society recommended in 1995 that universal neonatal hearing screening be introduced (''Millstätter Concept''). Coverage is presently about 67% for full-term healthy neonates and 86% for neonates from intensive care units. For maternity units, referral rates between 1% and 3.7% have been reported (2.7- 15% for intensive care units). The results of the screening test and follow-up in cases of failure have been documented in 37 543 neonates. Of this population, 91 infants (2.4 per 1000) showed bilateral permanent hearing loss. In these children intervention and management of the family started within the first months of life. These results justify the effort involved in introducing universal neonatal hearing screening. [ABSTRACT FROM AUTHOR] more...

The present paper reports on the implementation of a maternity based neonatal hearing-screening program in a private hospital. A retrospective analysis is performed on the test pass rate, the coverage and the number of children that become lost to follow-up. The data show a steady learning curve with a time course of several years. In the current screening practice, the test pass rate is at 99.0%, the coverage is at 96% (birth rate of 2000 per annum) and almost no babies get lost to follow-up. [ABSTRACT FROM AUTHOR] more...

The objective of this study was to establish the local incidence of hearing screening failure rate in newborns with all three stages of hypoxic ischemic encephalopathy (HIE). This retrospective cohort study was undertaken in a tertiary neonatal intensive care unit. Medical records and hearing secreening test results were collected for two years. One hundred and ninety seven infants diagnosed with HIE, 20 of them died, 177 screened. Thirty five of 177 (19%) infants failed in screening test for hearing. Screening failure rate was 10/51 (19%), 20/105 (19%) and 5/21 (23%) in stage 1, 2 and 3, respectively and did not differ between HIE stages (p = 0.88). Furthermore failure rates were similar between infants who received therapeutic hypothermia or not (20% vs 19%, p = 0.84). Hearing screening failure rate in HIE is quite high even in Stage 1 infants. Management and treatment of these infants should be made carefully concerning additional risks for hearing loss and long term follow-up even in Stage 1 HIE infants should be planned strictly. [ABSTRACT FROM AUTHOR] more...

Screening programs examining neonatal hearing serve to detect hearing defects, as a prerequisite for hearing rehabilitation, communication skills, and the enhancement of speech development. There are two methods through which neonatal hearing screening is carried out - the transiently evoked otoacoustic emissions (TEOAE) or the automatic BERA (AABR, automated auditory brainstem response). Positive screening means the discovery of a hearing defect (permanent hearing loss), and negative screening (normal TEOAE or the AABR results) means the absence of a hearing defect. The procedural aim is to update and adjust the neonatal hearing screening, which is determined by the Bulletin of the Ministry of Health of the Czech Republic No. 7/2012. Neonatal screening is performed at three levels: at neonatological site, at the ENT (phoniatric) rescreening site and at the ENT regional centre. The activities at each level are accurately and concretely identified including the issue of billing the performance to health insurance companies and informed consent to personal data protection (GDPR). The correct functioning of screening for hearing loss is based on the simple organization of the screening, patient examination comfort, medical recovery from it, and its economic viability. The schedule for neonatal hearing screening and rehabilitation recommends the following steps: 1. screening of a newborns hearing on the second or third day after delivery by a neonatological nurse using otoacoustic emissions, alternatively AABR for newborns at risk; 2. hearing rescreening in the third to sixth week of child`s age at the ENT rescreening site; 3. completion of hearing impairment diagnostics within three to sixth months of age at the ENT regional centre. The failure to follow the procedure above is a threat to the hearing and speech development of the child with severe permanent hearing impairment. The collaboration of ENT doctors with neonatologists and paediatricians allows for creating conditions under which the functional nationwide hearing screening of newborns can be established throughout the Czech Republic. more...

Objective: To analyze the hearing assessment characteristics and follow-up of some deafness gene screening homozygous infants in Zhuhai. Method: The clinical data of 28 newborns with homozygous mutations transferred to Zhuhai Maternal and Child Health Hospital from Feb. 1, 2015 to Oct. 25, 2018 in hospitals of Zhuhai City were retrospectively analyzed. All the children were screened for hearing. The hearing characteristics and long-term follow-up results of homozygous mutations at different gene sites were analyzed by auditory diagnosis and behavioral follow-up from 1 to 3 years. Result: Fourteen cases of GJB2 c.109G>A with a homozygous mutation, 11 cases passed the hearing screening, the audiological diagnosis was normal, and the behavior test and follow-up were normal from 1 to 3 years. Hearing screening was not passed in 3 newborns, mild to moderate abnormalities of single or bilateral ears were diagnosed by audiology, 1 000 Hz without positive, and middle ear lesions were diagnosed. Eight cases of GJB2 c.235del C homozygous mutation were followed up by behavioral audiometry and follow-up from 1 to 3 years after cure. Among them, 5 cases were diagnosed as severe hearing impairment of bilateral ears and 3 cases as mild and moderate hearing impairment. One case of GJB3 547G>A homozygous mutation was followed up for 1-3 years, and all of them failed to pass the follow-up of behavioral audiometry and follow-up. Four cases of SLC26A4 IVS7-2A>G, 1 case of SLC26A4 1229C>T homozygous mutation, all of them failed to pass the neonatal hearing screening. All the patients were diagnosed as severe hearing impairment of binaural hearing, and the follow-up of 1-3 years' follow-up did not pass the follow-up tests. Conclusion: GJB2 C.235del C, SLC26A4 IVS7-2A>G locus homozygous mutation infant hearing impairment was mainly severe hearing impairment in bilateral ears, and there was no change in 1-3 years follow-up. GJB2 C.109G A homozygous mutant infants had normal hearing, and it was suggested that they should be followed up closely. It is very important to give correct and reasonable genetic counseling to parents with GJB2 C.109G A homozygous mutation without unnecessary panic., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.) more...

Summary The prevalence of hearing loss in newborns and infants is estimated between 1 to 3.47 cases per 1000 live births. Neonatal screening for hearing loss and audiological evaluation are becoming more extensively carried out. However, there is no consensus regarding the use of audiometry and other electrophysiological tests in current practices. This article is intended to provide professionals with recommendations about the "best practice" based on consensus opinion of the session's speakers, and a review of the literature on the efficacy of various assessment options for children with hearing loss., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.) more...