GJB2 and sensorineural hearing loss disorder: Since the frequencies of GJB2 and SLC26A4 gene mutations among the patients with nonsyndromic severe to profound congenital SNHL are 27.0% based on our database, mutation frequency of OTOF among the total of severe to profound recessive nonsyndromic SNHL is considered to be about 3.2-7.3% (which is calculated by ((7-16)/160×(100/73))×100%).