With the advancement of next-generation sequencing genetic screening, mutations in other BAF/PBAF partner genes such as SMARCC2 (BAF170), SMARCG2 (DPF2) and PHF6 have been found in patients affected with CSS-like syndrome affected with hearing loss with varying degree [67,68,69]. The gene discussed is SMARCC2; the disease is Coffin-Siris syndrome.