The proband with a reciprocal X/21 translocation that affects ARHGEF6 expression shows SNHL in addition to intellectual disability, while hearing deficit was not reported in the Dutch family with the Arhgef6 IVS1-11T→C mutation (Yntema et al., 1998; Kutsche et al., 2000). Here, ARHGEF6 is linked to sensorineural hearing loss disorder.