Detecting auditory neuropathy spectrum disorders (ANSD) is challenging in congenital occurrence. Common features in ANSD are functional inner ear hair cells, but insufficient transmission of the generated signals.
Here we report on a pair of siblings with ANSD that elucidate the importance of ABR for newborn hearing screening (NHS). The 10 y.o. sister was born premature (38. gestational week) and hypotrophic. After failure of speech development and the diagnosis of ANSD she was sequentially implanted with CI at the age of 4 and 6. By now, speech acquisition is average. The younger brother is 10 month old and passed the NHS without remarks. Nevertheless, the parents presented during consultation-hours. An ANSD-characteristic combination of results with missing ABRs, but detectable otoacoustic emissions (OAEs) and cochlear microphonics was found. For the NHS, OAEs had been used. After insufficient testing of hearing aids, cochlear implantation was indicated.
Conclusion:
Using OAEs for NHS by nature is insensitive for children with ANSD and should be replaced by ABR-based techniques.
