Regardless of the necessity of exome sequencing, the first line screening for prevalent variants of GJB2, OTOF and SLC26A4 using previously reported diagnostic kits45,46 in tandem with IAC-MRI could possibly lead to better etiologic diagnosis, as high as 58.0% in pediatric SP-SNHL (Fig. 2). The gene discussed is OTOF; the disease is sensorineural hearing loss disorder.