Auditory neuropathy in newborns and toddlers in Croatia – is the presentmethodology of universal neonatal hearing screening appropriate?

Purpose of study: To examine how frequent auditory neuropathy (AN) is in Croatia and to find out if the testing of otoacoustic emission (OAE) is an appropriate method of universal neonatal hearing screening or the automatic auditory brainstem response (AABR) is needed.
Examinees and methods: From 1.10.2002 until 1.10.2007 about 200,000 children were born. Hearing screening was performed by OAE on about 90% of newborns before discharge. All babies with absent OAE as well as babies at some risk of neurodevelopment delay or uncertain reactions to sound underwent diagnostics of ABR. Criterions for diagnosis of AN were normal OAE and absent or aberrant ABR.
Results: Permanent hearing impairment was found in 240 children in this period of time. Bilateral AN was found in 4 children, all of them with some risk factors. It means that AN was present in 1.7% of all hearing impaired children or in 0.02‰ of all newborns. One child has Arnold-Chiari malformation and other one agenesis of corpus calosum and craniosinostosis. Conclusion: Congenital AN according to the study seems to be extremely rare in Croatia, so A-ABR as a method of universal neonatal hearing screening in Croatian delivery hospitals is not needed. In any case, hearing and speech development follow up is important in all children even with normal OAE at birth, especially if they are at risk of neurodevelopment delay. In the case of any doubt, A-ABR or diagnostic ABR is needed.

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