False positives in universal neonatal screening for permanent childhood hearing impairment

doi:10.1016/S0140-6736(00)03267-0

The Lancet

Volume 356, Issue 9245, 2 December 2000, Pages 1903-1904

https://doi.org/10.1016/S0140-6736(00)03267-0 Get rights and content

Summary

High rates of false-positive neonatal screens for permanent childhood hearing impairment (PCHI) associated with raised hearing thresholds lead to unnecessary assessments of the baby, which may worry parents. False-positive rates need to be reduced, especially in view of the UK government's announcement that national neonatal screening will be introduced. We report screening criteria that give a six-fold reduction in false-positive rates.

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The Lancet
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There are more references available in the full text version of this article.

Cited by (44)

Limitations of hearing screening in newborns with PDS mutations
2013, International Journal of Pediatric Otorhinolaryngology
Citation Excerpt :
The importance and cost-effectiveness of UNHS has been demonstrated [10,11]. Recently, many protocols to minimize false positive rates and improve the effectiveness of UNHS testing have been developed [8,12,13]. The sensitivity of newborn screening is at or near 95% [14,15].
SLC26A4 (PDS) mutations are common cause of congenital hearing loss in East Asia. Hearing loss caused by PDS mutations tends to have delayed presentation; thus universal newborn hearing screening (UNHS) can be less effective in these patients. We examined the efficiency of newborn hearing screening test in patients with bi-allelic PDS mutations.
Forty-three patients with sensorineural hearing loss were recruited. Patients had an enlarged vestibular aqueduct and biallelic PDS mutations. Among them, newborn hearing screening test had been performed on 14. The remaining 29 patients did not undergo newborn hearing screening test. Another 15 patients without a PDS mutation but who had sensorineural hearing loss were also recruited as a comparison group. We reviewed the hearing loss history of the children using medical records and parent interviews.
Among 14 patients with PDS mutation, four (28.6%) passed newborn hearing screening test in both ears and six (42.9%) passed in one ear. In contrast, only 2 of 15 (13.3%) children without a PDS mutation passed newborn hearing screening test bilaterally. The age at confirmation of bilateral hearing loss in bilateral “pass” patients with PDS mutation was 31.5 ± 17.9 months, which was significantly delayed compared to the age for bilateral “refer” children (1.75 ± 0.96 months) (p < 0.05).
The UNHS is not an accurate tool for predicting long-term hearing loss in patients with PDS mutations. We recommend that genetic screening be combined with UNHS, particularly in communities with a high prevalence of PDS mutations, to better identify children in need of early habilitation.
Newborn hearing screening test with multiple auditory steady-state responses
2011, Acta Otorrinolaringologica Espanola
Las técnicas más utilizadas en los programas de cribado auditivo presentan limitaciones reconocidas en la detección de las pérdidas auditivas. Se presenta una nueva alternativa de cribado auditivo basada en el uso de potenciales evocados de estado estable.
Evaluar la factibilidad de una prueba semiautomática de cribado auditivo en una población de recién nacidos sanos.
Se estudiaron 50 neonatos (7-18 días de nacidos) sin factores clínicos de riesgo y con respuesta detectable bilateral a 40 db nHL con potenciales evocados auditivos de tronco cerebral. A cada uno se le realizó una prueba con potenciales evocados auditivos de estado estable a múltiples frecuencias (PEAee-MF) (500 y 2.000 Hz) y un registro sin estímulo para simulación de sordera.
Los umbrales auditivos se encontraron entre 25 y 50 dB HL para ambas frecuencias (42,5 ± 7 dB HL para 500 Hz y 35,5 ± 6 dB HL para 2.000 Hz). Los tiempos medios de registro fueron de 2,6 ± 1,6 min por cada oído y la duración total del procedimiento (incluyendo preparación y colocación de electrodos) fue de 17,8 ± 3,7 min. Con la prueba de cribado se obtuvo una adecuada eficiencia diagnóstica (sensibilidad, 100%, y especificidad, 96%).
Los resultados descritos sugieren que los PEAee-MF (estimulación simultánea con múltiples frecuencias) pudieran ser utilizados como prueba de cribado auditivo en una población de recién nacidos sanos. Para esto, es necesario seguir perfeccionando la técnica combinando una metodología válida y sencilla con determinadas facilidades de automatización.
The techniques most frequently used within a screening context (otoacoustic emissions and click auditory brainstem response) have well-known limitations in hearing loss detection.
This study examines the feasibility of a semi-automated multiple auditory steady-state responses (MSSR) system designed for neonatal hearing screening.
A sample of 50 newborns without risk factors (well-babies) was tested within two weeks of birth. All had detectable auditory brainstem responses to clicks down to 40 dB nHL in both ears. Two amplitude modulated carrier tones of 500 and 2,000 Hz were mixed together and presented simultaneously. Each infant (and ear) was screened with the MSSR system; to simulate a hearing loss, a recording without stimulation was also obtained.
Mean auditory thresholds were 42.5 ± 7 dB HL at 500 Hz and 35.5 ± 6 dB HL at 2,000 Hz. The average duration of the MSSR recording was 2.6 ± 1.6 minutes for each tested ear and the overall duration of the screening procedure (including electrode fitting and infant preparation) was 17.8 ± 3.7 minutes. The diagnostic sensibility and the positive predictive values of the MSSR semi-automatic screening system was 100% and 96% respectively, with specificity of 96% and negative predictive values of 100%.
Although the diagnostic efficiency of the semi-automated MSSR system was found adequate, further technological improvements are still necessary to facilitate its use in the context of universal newborn hearing screening program.
Is discordance in TEOAE and AABR outcomes predictable in newborns?
2010, International Journal of Pediatric Otorhinolaryngology
Citation Excerpt :
It is therefore generally considered a more conclusive indicator of the need for diagnostic evaluation than TEOAE, though not without some limitations with regards to detecting mild sensorineural or high-frequency hearing loss as most of the instruments use 35 dBnHL threshold for the click stimulus [7]. Evidence from well-conducted TEOAE/AABR programs has shown that both tests may yield discordant but valid screening outcomes which may be interpreted as false-positives and false-negatives [2,8,9]. Sometimes this may be underpinned by specific hearing loss configurations or adverse medical conditions which cannot be detected otherwise at screening or some attributes of the screening program itself.
To determine the perinatal predictors of discordant screening outcomes based on a two-stage screening protocol with transient-evoked otoacoustic emissions (TEOAE) and automated auditory brainstem response (AABR).
A cross-sectional study of infants tested with TEOAE and AABR under a hospital-based universal newborn hearing screening program in Lagos, Nigeria. Maternal and infant factors associated with discordant TEOAE and AABR outcomes were determined with multivariable logistic regression analyses adjusting for potential confounding factors.
Of the 4718 infants enrolled under the program 1745 (36.9%) completed both TEOAE and AABR. Of this group, 1060 (60.7%) passed both TEOAE and AABR (“true-negatives”); 92 (5.3%) failed both TEOAE and AABR (“true-positive”); 571 (32.7%) failed TEOAE but passed AABR (“false-positives”) while 22 (1.3%) passed TEOAE but failed AABR (“false-negatives”). Infants with false-positives were likely to be admitted into well-baby nursery (p = 0.001), belong to mothers who attended antenatal care (p = 0.010) or who delivered vaginally (p < 0.001) compared to infants with true-negatives while infants with true-positives were also more likely to be delivered vaginally (p = 0.002) or admitted into well-baby nursery (p = 0.035) compared to infants with false-negatives. Infants with true-positives were significantly more likely to be delivered vaginally (p < 0.001) and have severe hyperbilirubinemia (p = 0.045) compared with infants with true-negatives. No association was observed between false-negatives and true-negatives. Antenatal care status, mode of delivery and nursery type were useful predictors of discordant outcomes among all infants undergoing screening (c-statistic = 0.73).
Given the available screening technologies, discordant TEOAE and AABR may be inevitable for some categories of hearing loss among apparently healthy newborns whose mothers received prenatal care. The potential limitations of perinatal morbidities as basis of targeted screening for such cases therefore merit further consideration.
Costs and performance of early hearing detection programmes in Lagos, Nigeria
2009, Transactions of the Royal Society of Tropical Medicine and Hygiene
In line with global progress towards early detection of permanent congenital and early-onset hearing loss (PCEHL), the costs and performance of hospital-based and community-based infant hearing screening models were evaluated in Lagos, Nigeria. The protocol consisted of two-stage screening with transient-evoked otoacoustic emissions and automated auditory brainstem response followed by diagnostic evaluation for all infants referred after the second-stage screening. The main outcome measures were referral rates at screening, cost per baby screened, cost per case detected and yield for PCEHL. First-stage referrals were 32.2% for universal and 31.7% for targeted screening in the hospital, compared with 14.3% and 15.2%, respectively for the community-based programme. Second-stage referrals ranged from 3.3% under hospital-based universal screening to 4.9% under community-based targeted screening. The highest yields of 27.4 and 22.5 per 1000 were recorded under community-based targeted and universal screening, respectively. Screening cost per child was lowest (US$7.62) under community-based universal screening and highest (US$73.24) under hospital-based targeted screening. Similarly, cost per child detected with PCEHL was lowest (US$602.49) for community-based universal screening and highest (US$4631.33) for hospital-based targeted screening. Community-based universal screening of infants during routine immunisation clinics appears to be the most cost-effective model for early detection of PCEHL in low-income countries.
Challenges in management of childhood sensorineural hearing loss in sub-Saharan Africa, Nigeria
2006, International Journal of Pediatric Otorhinolaryngology
The evaluation of a hearing-impaired child attempts to determine the aetiology, the degree of hearing loss and intervention to aid speech and language. This remains a challenge to practising otolaryngologists, especially in the developing countries as 85–90% of causes of hearing losses were never discovered leading to delayed intervention and irreversible effects.
In a review of children presenting at the otolaryngology outpatient in the University College Hospital, Ibadan, Nigeria, sensorineural hearing loss was found in 103, giving a hospital prevalence rate of 14%, we still perceived this figure to be unrepresentatively and low, probably due to poor access to medicare, poverty and other factors. Genetic factor accounted for 25%, followed by measles infections 13% and meningitis 8%. About 60% of them had educationally significant hearing loss at presentation. Access to hearing aid was poor as only 12.5% of the patients could afford it and the rest were managed by deaf training.
We conclude by suggesting an audiologic programme which has the comprehensive function of neonatal and infant hearing screening, subsidized hearing aid services and hearing rehabilitation surgery.
Universal newborn screening for permanent childhood hearing impairment: An 8-year follow-up of a controlled trial
2005, Lancet
An 8-year follow-up study of the birth cohort of babies enrolled in the Wessex controlled trial of universal newborn screening (UNS) for permanent childhood hearing impairment (PCHI) was undertaken to establish whether UNS would increase the proportion of all true cases of PCHI in children aged 7–9 years who are referred early. The proportion referred before 6 months of age increased from 11 of 35 (31%) children with true PCHI born during periods without UNS to 23 of 31 (74%) born during periods with UNS (difference 43%, 95% CI 19–60). UNS leads to early referral of PCHI.

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Research LettersFalse positives in universal neonatal screening for permanent childhood hearing impairment

Summary

Controlled trial of universal neonatal screening for early identification of permanent childhood hearing impairment

The Lancet

The costs of hearing screening in the first year of life in England and Wales

Arch Dis Child

Research Letters
False positives in universal neonatal screening for permanent childhood hearing impairment