September 2019 HSC Section 1 Congenital and Pediatric Problems

B.J. Liming et al. / International Journal of Pediatric Otorhinolaryngology 90 (2016) 251 e 258

Section 3: Workup of child with auditory neuropathy spectrum disorder (ANSD)

bone and brain imaging (MRI) is recommended in the setting of unilateral ANSD and should be considered in the setting of bilateral ANSD. MRI may reveal abnormalities in up to 2/3 of patients [6] . Given that 40 e 50% of ANSD has a genetic basis, comprehensive genetic testing should be offered to children in whom ANSD is identi fi ed.

The diagnosis of auditory neuropathy spectrum disorder (ANSD) is given when otoacoustic emissions and/or cochlear microphonics are present in the setting of absent or abnormal ABR. Temporal

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