Pure tone air and bone conduction measurements from DFNB29 families segregating p.Val85Asp revealed intra- and inter-familial variability in thresholds. (a) Twenty-one year old affected individual of family PKDF505 has profound, bilateral, sensorineural hearing loss, while his twenty-three years old sibling has moderate to severe, bilateral sensorineural hearing impairment. Shown also is their 48 years old mother, a carrier of p.Val85Asp allele, with normal hearing thresholds across all frequencies. (b) Ten year old affected individual of family PKDF704 has moderate to profound, bilateral, sensorineural hearing loss, while her eleven year old sibling has severe to profound, bilateral sensorineural hearing impairment. (c) Twenty-five year old affected individual of family PKDF797 has severe to profound hearing loss, while a twenty-three old affected individual of family PKDF1092 has moderate to profound, sensorineural hearing loss.