Nonsyndromic Hearing Loss and Deafness DFNB1 is an inherited disorder that only affects hearing. No other medical problems are associated with the hearing loss. DFNB1 is present at birth and does not worsen over time; however, the severity of the hearing loss varies among individuals. Hearing loss for individuals with DFNB1 can be helped with the use of hearing aids or the possibility of cochlear implants for those with profound deafness. In 98% of cases, the hearing loss is caused by two pathogenic variants in the GJB2 gene, while 2% of cases are due to pathogenic variants in GJB2 and another gene named GJB6.