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. 2021 Jun 10;41(6):BSR20203740. doi: 10.1042/BSR20203740

Table 1. NGS results from our center.

No. Age Gene Nucleotide change Amino acid change Novel ACMG criteria Feature of deafness Relationship
4238 1 year NA SNHL
4354 1 year POU3F4 c.346_350dup p.S117Rfs*26 Yes PVS1_Strong, PM2(LP) SNHL MATERNAL
5517 3 years POU3F4 c.421_422delinsTA p.V141* Yes PVS1_Strong, PM2(LP) Mixed MATERNAL
12701 1 year POU3F4 c.441del p.H147Qfs*94 Yes PVS1_Strong, PM2(LP) SNHL NA
13276 2 years POU3F4 c.346dup p.A116Gfs*77 Yes PVS1_Strong, PM2(LP) NA NA
14548 8 years NA SNHL
M32 5 months POU3F4 c.232C>T p.Q78* No (Clinvar: 426228) PVS1_Strong, PM2(LP) NA MATERNAL
M40 7 years NA NA
J0006 6 months POU3F4 c.401_404dup p.Q136Lfs*58 Yes PVS1_Strong, PM2(LP) SNHL MATERNAL
J0007 11 years NA SNHL
J0011 2 years NA SNHL
J0012 6 years NA SNHL

Abbreviation: SNHL, sensorineural hearing loss. NA in nucleotide change means no available NGS test; NA in feature of deafness means no audiogram.