Table 2.

Adults with DFNB1

Parameter	Patient 1	Patient 2	Patient 3
Sex	F	F	F
Age, y	18	34	49
Estimated onset of HL	Birth	Birth	3 y prior
Mutation	35delG homozygous	35delG/GJB6 heterozygote	G4D/V371
Severity of HL	Severe to profound	Profound	Moderate to severe, progressive
Audiogram configuration	Gently sloping	Gently sloping	Unilateral, sloping
Pattern of inheritance	Autosomal recessive	Autosomal recessive	Autosomal dominant

Abbreviations: F, female; HL, hearing loss.