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PubMed2003

Implementation of a nation-wide automated auditory brainstem response hearing screening programme in neonatal intensive care units.

AIM: As part of a future national neonatal hearing screening programme in The Netherlands, automated auditory brainstem response (AABR) hearing screening was implemented in seven neonatal intensive care units (NICUs). The objective was to evaluate key outcomes of this programme: participation rate, first stage success rate, pass/referral rates, rescreening compliance, diagnostic referral rates, age of first diagnostic evaluation and prevalence of congenital hearing loss (CHL). METHODS: This prospective cohort study collected data on 2513 survivors. NICU graduates with one or more risk factors according to the Joint Committee on Infant Hearing were included in a two-stage AABR hearing screening programme. Conventional ABR was used to establish a diagnosis of CHL. RESULTS: A total of 2513 newborns enrolled in the programme with a median gestational age of 31.6 (range 24-43) wk and a median birthweight of 1450 (range 510-4820) g. In 25 (1%) cases parents refused screening. Four out of 2513 newborns were initially lost; 2484 newborns have been tested initially. A final 98% participation rate (2465/2513) was obtained for the whole programme. After a median postmenstrual age at the first test of 33.7 (range 27-54) wk, a pass rate of 2284/2484 (92%) resulted at the first stage. The rescreening compliance after the first test was 92% (184/200). A referral rate for diagnostic ABR of 3.1% (77/2484) resulted. Of the 77 referrals 14 (18.2%) had normal screening thresholds, 15 (19.5%) had unilateral CHL and 48 (62.3%) had bilateral CHL. The prevalence of unilateral CHL was 0.6% (15/2484) and of bilateral CHL 1.9% (48/2484). CONCLUSION: A financially supported two-stage AABR hearing screening programme can be successfully incorporated in NICU centres and detects a high prevalence of CHL in NICU graduates. Neonatal hearing screening should be part of standard clinical practice in all NICU infants.

Cohort Studies↗
PubMed1996

Survey of the definition and screening of neonatal hypoglycaemia in Australia.

OBJECTIVE: To determine the approach to identifying neonatal hypoglycaemia and the definition of neonatal hypoglycaemia used by neonatal paediatricians in Australian Level 3 neonatal intensive care units (NICU). METHODOLOGY: A questionnaire was sent to the 101 neonatal paediatricians in the 22 Level 3 NICU in Australia asking their method of screening for, and definition of, neonatal hypoglycaemia. RESULTS: Responses were received from 70 neonatal paediatricians, including all 22 directors. A bedside glucose meter is used in 19 of 22 NICU to screen for hypoglycaemia, whilst one NICU uses a glucose analyzer and another NICU uses a visual colour comparison method. One NICU does not screen, but has blood glucose measured in a satellite laboratory. If the screening method suggests hypoglycaemia, 62 of 63 neonatal paediatricians proceed to blood glucose determination in a laboratory, mostly using plasma samples. Based on the laboratory measurement, the definition of neonatal hypoglycaemia ranged from < 1.1 to 3.0 mmol/L. CONCLUSIONS: The majority of neonatal paediatricians in Australian NICU screen for neonatal hypoglycaemia using a bedside glucose meter. There is a wide range in the definition of neonatal hypoglycaemia from < 1.1 to 3.0 mmol/L.

Australia↗
PubMed1993

Congenital cytomegalovirus infection and neonatal auditory screening.

Auditory screening of newborn infants has been recommended on the basis of the presence of risk criteria, including congenital infection. We assessed the ability of risk criteria-based neonatal auditory brain stem response to identify infants with hearing loss resulting from congenital cytomegalovirus (CMV) infection. Data from 6 1/2 years of risk criteria-based neonatal auditory screening were compared with the results of screening of all newborn infants for congenital CMV infection. Infants with congenital CMV infection received follow-up hearing evaluations. Congenital CMV infection was found in 167 (1.3%) of 12,371 infants; 134 had follow-up hearing evaluations, and 14 (10.4%) had confirmed sensorineural hearing loss. The rate of sensorineural hearing loss resulting from congenital CMV infection was 14 per 12,371 infants, of 1.1 per 1000 live births; the rate of bilateral loss > or = 50 dB was 0.6 per 1000. Although 2036 infants received auditory screening because of risk criteria, only 34 (20%) of 167 infants with congenital CMV infection were included. Only 2 (14%) of 14 children with sensorineural hearing loss caused by CMV were identified by risk criteria-based screening. We conclude that congenital CMV infection is an important cause of hearing impairment. Neonatal auditory screening based on the presence of risk criteria will fail to identify the majority of cases of sensorineural hearing loss caused by congenital CMV infection.

Cytomegalovirus Infections↗
PubMed2002

Screening of neonatal instability and of developmental dislocation of the hip. A survey of 132,601 living newborn infants between 1956 and 1999.

Between 1956 and 1999, 132,601 living children were born in Malmö, and screened for neonatal instability of the hip. All late diagnosed patients have been followed and re-examined clinically and radiologically. During the first years of screening, less than five per 1,000 living newborn infants were treated. This figure increased to 35 per 1000 in 1980, but later diminished again to about six per 1,000 annually after 1990. The number of referred cases decreased from 45 per 1,000 in 1980 to between 10 to 15 per 1,000 from 1990. During the period of high rates of referral and treatment a larger number of paediatricians were involved in the screening procedure than during the periods with low rates of ferral and treatment. Altogether 21 patients (0.16 per 1,000) with developmental dislocation of the hip were diagnosed late, after one week. At follow-up, 18 were free from symptoms and 15 considered to be diologically normal.

Child↗
PubMed1999

Automated auditory brainstem response in neonatal hearing screening.

Severe congenital hearing impairment is an important handicap affecting 0.1% of apparently healthy liveborn 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 mo. Universal screening is preferable, since about 50% of infants with hearing loss are not discovered if neonatal hearing screening is restricted to high-risk groups. The automated auditory brainstem response (AABR) screener is a dedicated hearing screening device which provides information not only about the outer/middle ear and cochlea but also about the auditory pathway up to the brainstem. AABR has an agreement with conventional auditory brainstem response up to 98%. It uses a 35 dB near hearing level click. No operator interpretation is needed and it can be used on the ward and during oxygen therapy without disturbance from ambient noise. Reported referral rates in a hospital-based screening programme at the time of discharge vary, with an average of 4%. AABR has also been used in a home-based setting, with the same results. The time necessary for screening varies with the setting, but ranges from 4 to 15 min. Initial costs range from $15 to $25 per test, which is similar to neonatal screening for metabolic diseases. In addition to individual healthcare savings, early diagnosis may lead to savings on costs of intensive speech-language intervention and educational facilities.

Audiometry↗
PubMed1987

The effect of perinatal screening in Norway on the magnitude of noninherited risk factors for congenital dislocation of the hip.

Several investigators have noted an increase in the rate of congenital dislocation of the hip shortly after the initiation of neonatal screening procedures. This increase has been attributed to the detection of temporarily unstable hips which require no corrective treatment. To test whether neonatal screening had low specificity, the authors obtained data on 17,145 offspring of 7,896 twins from the Norwegian Twin Panel. Information from maternal reproduction history questionnaires was available on the presence or absence of congenital dislocation of the hip, type of obstetric delivery, and parity. The reported prevalence of the disorder did indeed begin to rise sharply during the late 1950s, at which time neonatal screening started in Norway. Infants were then grouped by year of birth (born before or after 1960), and odds ratios were calculated for breech delivery and early (first or second) parity. For the pre-screening group, the odds ratio of congenital dislocation of the hip was 7.7 among children delivered by breech presentation and 2.6 among those of early parity. These values are similar to those found in other studies. In the post-screening group, the odds ratios for breech delivery and early parity were 1.5 and 1.2, respectively. Breech delivery and early parity have been consistent risk factors for congenital dislocation of the hip. Their diminished influence in the post-screening group, as well as sharply increased rates of the disorder, suggests that in Norway neonatal screening programs may have had low specificity in detecting cases that required treatment.

Cross-Sectional Studies↗
PubMed2001

Parents' Experience of Universal Neonatal Hearing Screening: A Critical Review of the Literature and Its Implications for the Implementation of New UNHS Programs.

This article presents a critical review of literature reporting the effects on parents of universal neonatal hearing screening (UNHS). It explores how parents experience the process and outcome of screening and in particular focuses on debates surrounding false positive identification, the evidence base for maternal/parental anxiety, consent, and family cultural/sociodemographic influences on the effective implementation of UNHS programs. These issues are then set within the context of events in the United Kingdom, where the Department of Health has recently initiated a program to pilot the introduction of universal neonatal hearing screening at 20 sites in England.

Journal Article↗
PubMed2006

Assessing perinatal hepatitis B screening and neonatal prophylaxis in a large, multiethnic county.

OBJECTIVE: To assess the completeness of maternal screening and appropriateness of newborn prophylaxis for hepatitis B virus (HBV) in 2001 in Florida's largest county. STUDY DESIGN: A retrospective cohort study sampled 1% of deliveries at all hospitals in Miami-Dade County that had delivery suites. Abstractors reviewed mothers' and newborns' charts before discharge to determine whether newborns received appropriate HBV prophylaxis based on mothers' hepatitis B virus surface antigen (HBsAg) status at 12 hours postpartum. At each hospital, deliveries were sampled sequentially until the target number was reached. Sampling was donefor March-August 2001. RESULTS: Among 339 deliveries at 14 hospitals, the mother's HBsAg status was known within 12 hours of delivery for 286, or 84.4% (95% CI 80.1-88.1%). Of the 53 infants whose mothers' HBsAg status was unknown, 15 (28.3%) were vaccinated within 12 hours of delivery; both infants whose mothers were HBsAg positive received vaccine and immune globulin within 12 hours delivery. CONCLUSION: While HBsAg screening of pregnant women is widespread in Miami-Dade County, the information is not consistent used to protect infants who mothers' HBsAg status unknown.

Analysis of Variance↗
PubMed1996

Screening for tyrosinaemia type I.

AIMS: To assess the incidence of tyrosinaemia type I in the West Midlands Region, and the value of current neonatal screening programmes for phenylketonuria (PKU) for its detection. METHODS: Retrospective study of results from the PKU neonatal screening programmes in Birmingham (using plasma amino acid chromatography) and in the rest of the West Midlands (using the Guthrie microbiological assay for blood spot phenylalanine) was carried out between January 1985 and March 1994. Patients with tyrosinaemia I born in the region during the same period were identified from a regional database of patients with confirmed inherited metabolic disease. The study was carried out in a specialist children's hospital; the regional centre in the West Midlands for neonatal screening and investigation of inborn errors, and a supraregional centre for liver transplantation and management of paediatric liver disease. RESULTS: Amino acid chromatography showed increased tyrosine in 447 of 145,444 neonates born in Birmingham; this was still increased at 6 weeks of age in six cases. Five had tyrosinaemia I; the sixth had tyrosinaemia type III. Two others in whom amino acid chromatography was considered normal have since presented with tyrosinaemia I. Outside Birmingham, 525,151 children were screened using the Guthrie test. Five have presented clinically with tyrosinaemia I; screening did not contribute to diagnosis in any case. The incidence of tyrosinaemia I was 1 in 20,791 live births within Birmingham and 1 in 105,037 outside. Of the total 12 patients in the West Midlands with tyrosinaemia I, 10 (83%) were of non-oriental Asian ethnicity; the incidence of tyrosinaemia I was 3.7/10(6) head of population in this group and 0.04/10(6) in the rest of the population. CONCLUSIONS: Asians in the West Midlands have a high incidence of tyrosinaemia I. Neonatal PKU screening using amino acid chromatography may contribute to diagnosis and early treatment.

Amino Acid Metabolism, Inborn Errors↗
PubMed1982

Neonatal urine screening for metabolic disease with auxotrophic strains of Bacillus subtilis.

A simplified method for neonatal urine screening for metabolic diseases is described. This procedure involves the use of two bacterial inhibition assays which can detect uracil, lysine, and homocystine, in combination with two other assays utilizing spores of amino acid auxotrophic mutants of Bacillus subtilis which can detect several amino acids and purines. These four tests have the capability to detect several treatable inherited metabolic diseases, including three disorders of the urea cycle, using a urine specimen on filter paper. This method results in savings of cost and time over chromatographic screening procedures, since only four agar trays (56 specimens per tray) are used, which can be processed with one cycle through a semi-automated punch index machine.

Bacillus subtilis↗
PubMed1999

Neonatal ECG screening for congenital heart disease in Down syndrome.

We studied the value of routine neonatal electrocardiography (ECG) in the 1st 48 hours of life to diagnose congenital heart disease in 37 neonates with Down syndrome. Twenty-four infants had no clinical evidence of congenital heart disease, had normal ECGs and normal cardiac anatomy on echocardiography. Thirteen children (35.2%) had congenital heart disease. The ECG was normal in seven infants with congenital heart disease: four with atrial septal defect (ASD), two with tetralogy of Fallot (TOF) and one with ventricular septal defect (VSD). A left QRS axis deviation was found in six infants: five with complete atrioventricular septal defect (AVSD) and one with VSD and mitral valve prolapse. However, only the five infants with complete AVSD had a superior QRS axis. Although neonatal ECG detected the presence of complete AVSD in neonates with Down syndrome, it missed an equal number with other heart defects. Echocardiography remains the way reliably to diagnose or exclude congenital heart disease in these infants.

Down Syndrome↗
PubMed2001

[Hearing disorders screening in neonates at risk].

The present report concerns a three year, eight month hearing screening in 1 531 high-risk neonates by means of two successive transient evoked otoacoustic emissions (TEOAE) recordings followed, cin cases of suspected hearing loss, by brainstem auditory evoked potential (BAEP) recording and otolaryngology (ORL) consultation. After TEOAE1 and 2 and BAEP testing, 1 361 infants (88.9%) were declared normal, and 170 (11%) suspected of hearing loss. Of these 170, 58 showed bilateral and 26 unilateral impairment. Definite hearing loss on ORL consultation was diagnosed in 14 infants (0.9% of the screened population as a whole); 22 are still followed, while 86 (5.6%) failed to consult for diagnosis. The mean age on diagnosis of definite hearing loss on ORL consultation was 9.9 +/- 4.9 (range 4-20) months. Several auditory function risk factors have been proved to be more frequent in deaf than in normal children. Our results show that early hearing loss screening in at-risk neonates needs to be pursued.

Decision Trees↗
PubMed2001

Community based universal neonatal hearing screening by health visitors using otoacoustic emissions.

OBJECTIVES: To carry out a pilot study to test the feasibility of health visitors (HVs) performing neonatal otoacoustic emissions (OAE) hearing screening in the community using Echoport ILO288 and to evaluate its acceptability to parents and HVs. DESIGN: Prospective cohort study. SETTING: Local health centres and babies' homes in urban and rural settings in West Gloucestershire. PARTICIPANTS: Twelve HVs, 683 babies, and their parents. MAIN OUTCOME MEASURES: Coverage rate, age at testing, referral rate for formal audiology testing, and parental anxiety scores. RESULTS: Of the 683 babies registered with the study HVs, 99% (675) were tested, with a median age at first test of 18 days. Parental consent for the study was refused for six of the eight not tested. Taking a unilateral pass as a screening pass (for comparison with other studies), 4% (27/675) failed the first OAE test, and 1.9% (13/675) failed a second OAE test performed by the HV within a further two weeks and were referred for formal audiology testing. One baby (0.15%) was found to have a moderate sensorineural hearing loss on brain stem auditory evoked responses, giving a false positive rate of 1.7% (12/675). Some 18% (120/675) were tested at home, of which 80% (96/120) were combined with another planned reason for HV contact. In all, 82% (555/675) of tests were carried out in health centre clinics, of which 47% (260/555) were combined purpose visits. Mean parental anxiety scores (possible range 0-5) were 0.86, 2.27, and 3.45 before the first test, first retest, and audiology test respectively. The median time taken for one HV to complete testing was 12.2 minutes (range 3-65), compared with the 15 minutes currently allocated for two HVs to perform distraction testing. Based on the results of questionnaires, the test was very well received by parents and HVs alike. CONCLUSION: HVs are able to perform OAE testing in the neonatal period at home and in local health centre clinics. They achieve high population coverage rates and low false positive rates. Universal neonatal hearing screening by HVs using OAE testing is feasible, well received, and could be less demanding of HV time than the current distraction testing. This model of universal neonatal hearing screening should be considered by the National Screening Committee.

Attitude to Health↗
PubMed2005

High-risk neonatal hearing screening program using automated screening device performed by trained nursing personnel at Siriraj Hospital: yield and feasibility.

OBJECTIVES: To determine the prevalence and significant risk factors for pathologic hearing screening test results in high-risk neonates and the feasibility of implementing hearing screening program using automated otoacoustic emission (OAE)/ auditory brain stem response (ABR) device performed by trained nursing staffs. STUDY DESIGN: Single-center prospective, descriptive study. MATERIAL AND METHOD: All neonates admitted to the Division of Neonatology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, who met the high-risk criteria according to Joint Committee of Infant Hearing 1994, American Academy of Pediatrics, were screened with one-step protocol using an automated OAE/ABR device (AccuScreen, GN Otometrics, Denmark). Infants who failed 2 consecutive OAE tests were reconfirmed by ABR prior to discharge. Descriptive analysis was used for the prevalence of pathologic hearing test results, age at screening, duration of procedure, number of risk factors per infant. Univariate analysis using Chi-square test and multiple logistic regression analysis were used for identification of significant risk factors. RESULTS: Five hundred and seven infants were identified to be at-risk in an 18-month study period. The prevalence of pathologic hearing screening test was 6.7% with unilateral and bilateral pathologic results in 13 and 21 infants (2.6% and 4.1%). Only craniofacial anomalies and mechanical ventilation > 5 days were shown to be independent significant risk factors (42-fold and 4-fold increased risk). Median age at screening test performed was 19 days (range 1-149 days) and almost all infants (97.3%) were screened within 3-month postnatal age. The mean time for hearing screening procedure was 10.7 +/- 8.0 minutes (range 2-60 minutes), 98.1% of procedure was accomplished within 30 minutes. CONCLUSION: Hearing screening using automated OAE/ABR devices in high-risk neonates revealed approximately 7% of pathologic results with almost two-thirds having bilateral affected. The significant independent risk factors in this study population were craniofacial anomalies and mechnical ventilation > 5 days. The protocol of having trained nursing staffs to perform the screening yielded good results, i.e., the coverage of screened infants within 3 months of age (97%), feasible duration of procedure.

Chi-Square Distribution↗
PubMed2004

A molecular epidemiologic study of thalassemia using newborns' cord blood in a multiracial Asian population in Singapore: results and recommendations for a population screening program.

DNA technology provides a new avenue to perform neonatal screening tests for single-gene diseases in populations of high frequency. Thalassemia is one of the high-frequency single-gene disorders affecting Singapore and many countries in the malaria belt. The authors explored the feasibility of using PCR-based diagnostic screening on 1,116 unselected sequential cord blood samples for neonatal screening. The cord blood samples were screened for the most common reported alpha- and beta-thalassemia mutations in each ethnic group (Chinese, Malays, and Indians) in a multiracial population. The carrier frequency for alpha-thalassemia mutations was about 6.4% in the Chinese (alpha deletions = 3.9%, alpha deletions = 2.5%), 4.8% in Malays, and 5.2% in Indians. Only alpha deletions were observed in the Chinese. The carrier frequency for beta-thalassemia mutations was 2.7% in the Chinese, 6.3% in Malays, and 0.7% in Indians. Extrapolating to the population distribution of Singapore, the authors found a higher overall expected carrier frequency for alpha- and beta-thalassemia mutations of 9% compared with a previous population study of 6% by phenotype. The highly accurate results make this molecular epidemiologic screening an ideal method to screen for and prevent severe thalassemia in high-risk populations.

China↗
PubMed1980

Measuring thyroxine and thyrotropin simultaneously in a dried blood sample on filter paper, to screen for neonatal hypothyroidism.

We have developed a highly sensitive radioimmunoassay of thyroxine and thyrotropin for mass screening for neonatal hypothyroidism. This assay involves a single disc (3 mm diameter) of dried blood on filter paper. The minimum detectable concentrations are 15 pg/tube (10 microgram/L) for thyroxine and 15 nano-int. units/tube (10 milli-int. units/L) for thyrotropin; intra- and interassay CV's are less than 15% in both assays. The high sensitivity of this method is due to use of labeled thyroxine with high specific activity (3 kCi/g) and of an anti-thyrotropin serum with high affinity (Keq = 7.8 x 10(11) L/mol). With this method, 11337 newborns were screened; a follow-up study revealed that only newborns with both high thyrotropin and low thyroxine concentrations had permanent hypothyroidism. We conclude that this method is sensitive, simple, and reliable and that the recall rate with this method is much lower than that of tests for measuring thyroxine or thyrotropin alone.

Blood Specimen Collection↗
PubMed1998

Asymptomatic vesicoureteral reflux detected by neonatal ultrasonographic screening.

Renal ultrasonography was performed in 2,384 healthy and asymptomatic neonates. The definition and grading of hydronephrosis was according to the system of the Society for Fetal Urology. Voiding cystourethrography was performed in the cases with moderate to severe hydronephrosis or persistent mild hydronephrosis. In cases with vesicoureteral reflux (VUR), a dimercaptosuccinic acid (DMSA) renal scan was arranged immediately to evaluate the renal parenchyma. VUR was diagnosed in 30 infants with a prevalence of 1.26%; 7 had bilateral VUR. The male/female ratio was 4:1 and the right/left ratio was 1.85:1. Comparing with the abnormal ultrasonographic findings, VUR appeared ipsilaterally in 23 ureters and contralaterally in 14 ureters. Using ultrasonography for diagnosing VUR, the sensitivity, specificity, positive predictive value, and negative predictive value were 62.2%, 36.1%, 11.0%, and 88.2%, respectively. DMSA renal scan was performed in 31 refluxing kidneys, and congenital renal scarring was found in 9 (29.0%) kidneys. Six neonates underwent reimplantation surgery. We conclude that although ultrasonography is not a reliable tool for diagnosing VUR, it can detect many cases of VUR during the newborn stage using our screening program, which thus makes early treatment from the neonatal period possible. Whether our screening program can improve the outcome of VUR will require further follow-up.

Female↗