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Universal neonatal hearing screening: the Siena (Italy) experience on 19,700 newborns
Bruno De Capua1, Daniele Costantini, Carla Martufi
1U.O.S. Audiologia Clinica, Neonatal Intensive Care Unit, D.A.I. Materno-Infantile, Azienda Ospedaliera Universitaria Senese, Policlinico Santa Maria alle Scotte, Viale M. Bracci 16, I-53100 Siena, Italy.
Insights
Universal neonatal hearing screening (UNHS) using a two-stage strategy of Transient Evoked Otoacoustic Emissions (TEOAEs) and Auditory Brainstem Response (ABR) accurately detects congenital hearing loss (HL) in newborns. This method enables early diagnosis and intervention, crucial for infant development.
Area of Science:
- Neonatal screening
- Audiology
- Public health
Background:
- Congenital hearing loss (HL) is a common birth defect, affecting 1-3 per 1000 live births.
- Early detection and intervention are vital for speech, language, and cognitive development.
- Selective hearing risk assessments miss 33-50% of congenital HL cases, necessitating universal screening.
Purpose of the Study:
- To evaluate a universal neonatal hearing screening (UNHS) protocol.
- The protocol utilizes a two-stage approach: Transient Evoked Otoacoustic Emissions (TEOAEs) followed by Auditory Brainstem Response (ABR) for high-risk infants or those failing the initial screen.
Main Methods:
- A two-stage screening strategy was implemented: TEOAEs as the initial screen, with diagnostic ABR for infants not meeting TEOAE criteria or identified as high-risk.
- The study included 19,700 infants screened between April 1998 and July 2006.
- Sensitivity, specificity, and predictive values were calculated to assess the UNHS strategy's accuracy.
Main Results:
- The prevalence of congenital HL was 1.78 per 1000 live births.
- The two-stage UNHS strategy demonstrated high accuracy, with 100% sensitivity and 99.3% specificity.
- All diagnosed infants received intervention before six months of age.
Conclusions:
- Universal neonatal hearing screening (UNHS) is epidemiologically justified for congenital hearing loss (HL).
- A two-stage TEOAE and ABR screening is a feasible, accurate, and minimally invasive method for early HL detection.
- Congenital HL screening strategies using only TEOAEs must account for potential false negatives.
Background:
Hearing loss (HL) is likely to be the most common congenital abnormality in humans, with a reported prevalence of 1 to 3 per 1000 live births. Early detection and intervention is critical to prevent the adverse consequences of a delayed diagnosis on speech, language and cognitive development. As 33-50% of all congenital HLs cannot be detected in a selective hearing risk, use of universal neonatal hearing screening (UNHS) programs is expanding.
Aims:
We tested the value of a UNHS protocol, based on a two-stage strategy of Transient Evoked Otoacoustic Emissions (TEOAEs) in all infants, followed by diagnostic auditory brainstem response (ABR) testing in those infants who did not meet TEOAE pass criteria and those infants at high risk for hearing loss.
Methods:
TEOAES (292 DP Echoport OAE Analyzer) served as the initial screen, followed by diagnostic ABR (Amplaid MK12) in newborns that did not meet pass criteria for TEOAEs. Additionally, all infants at high audiologic risk according to the Joint Committee on Infant Hearing received a diagnostic ABR evaluation. Of 21,125 total live births, 19,700 were tested (April 1, 1998-July 31, 2006). Accuracy of the UNHS strategy in predicting congenital HL was evaluated by calculating sensitivity, specificity, positive predictive value and negative predictive value.
Results:
Prevalence for all HLs in the neonatal period was 1.78/1000 l.b. (35/19,700), with bilateral HL in 1.42/1000 l.b. (28/19,700) [low risk rate: 0.43/1000 l.b. (8/18,356); high risk infants rate: 14.88/1000 l.b. (20/1344)]. All the HL infants were diagnosed <3 and received intervention <6 months age. ROC curves results showed 100% sensitivity (95% C.I.: 89.0-100) and 99.3% specificity (95% C.I.: 99.2-99.4) of the two-stage strategy in detecting congenital HLs [area under the ROC curve: 0.997 (95% C.I.: 0.995-0.997)].
Conclusions:
(1) The epidemiology of congenital HLs widely justifies UNHS; (2) a two-stage TEOAE and diagnostic ABR screening for congenital HL is feasible, minimally invasive and accurate in the early detection of congenital HL; and (3) a congenital HL screening strategy based exclusively on the use of TEOAEs should always consider the possibility of false negative cases.
