Table 2

GJB2 genotypes with unknown consequences in 16 families

Number of families
Genotype**
Hearing loss****
Mode of inheritance

3
35delG/+
1P progressive; 1M; 1U
1SC,1AR, 1un
2
R127H/M34T
2P
1AR,1SC
1
R127H/W24X
P to M ***
1AR
1
M34T/V37I
1 mild
1SC
2
M34T/+
1 mild, 1M
2AD
1
(IVS1-12C>T)2*/-34T/G*
P
un
2
IVS1-12C>T*/+
1P progressive, 1P
1SC, 1un
2
V37I/+
1M, 1P
1AR, 1un
1
V153I/+
M
1SC
1
G160S/+
P
1SC

* novel sequence variant ** + designates the wild type allele *** variable phenotype within the family **** P: profound; M: moderate; U: unilateral; un: unknown

Roux et al. BMC Medical Genetics 2004 5:5   doi:10.1186/1471-2350-5-5