Fig. 1

Pedigree drawing for family 4667 and audiograms for the affected family members. Panel a Pedigree drawing displaying family members with NSHI as filled symbols and unaffected family members as clear symbols. Males are represented by squares and females by circles. For the three unaffected and two affected family members genotypes for the PCDH15 variant NM_033056:c.3101G > A and USH1G variant NM_173477:c.1093G > A are shown under each family member and demonstrate digenic inheritance. The DNA sample from Individual IV:4 was exome sequenced. Panel b Audiograms for affected family members IV:3 (top) and IV:4 (bottom). Pure-tone audiometry was performed between 250 and 8000 Hz and x represents the results for the left ear and o for the right ear. Affected individual IV:3 was 34 years old, and affected individual IV:4 was 22 years old at the time of pure-tone audiometry and physical examination