Table 3.

Neurophysiologic data of patients with novel mutations in GJB1.

Patients	Sex	Compound muscle action potential								Sensory nerve action potential						EMG
		Median		Ulnar		Tibial		Peroneal		Median		Ulnar		Sural		ADM/TA
		Amp., mv	CV, m/s	Amp., mv	CV, m/s	Amp., mv	CV, m/s	Amp., mv	CV, m/s	SNAP, uv	CV, m/s	SNAP, uv	CV, m/s	SNAP, uv	CV, m/s
1.1318
III-7	M	0.81	38.5	1.69	33.2	Abs	Abs	Abs	Abs	Abs	Abs	Abs	Abs	Abs	Abs	+/+
IV-4	F	5.2	30.6	4.7	38.6	2.7	31.8	0.3	41.9	3	37.8	1.6	40	Abs	Abs	N/+
IV-5	F	4.1	30.8	4.2	38.6	3.6	31.5	1.3	38.7	3.2	37.5	4.2	34.8	2.9	36.1	+/+
IV-7	M	3.6	38.1	3.9	35.9	0.7	30.5	Abs	Abs	2.7	33.8	1.8	36.6	0.3	31	+/+
2.1312
III-2	M	0.42	35.6	1.29	35.8	0.3	27.4	Abs	Abs	5.8	38.9	1.5	42.8	Abs	Abs	+/+
II-2	F	5.5	31.4	3.5	38.5	2.7	31.5	1.4	35.1	7.3	30.8	4.6	34.5	Abs	Abs	–/+
3.1806
III-7	M	0.37	34.3	2.5	35.2	0.13	37.5	Abs	Abs	1.5	32.2	Abs	Abs	1.0	33.2	+/+
II-4	F	9.6	51.5	5.5	59.6	0.9	37.3	0.3	38.5	3	37.2	5.7	42.8	Abs	Abs	N/+

Amp, amplitude; CV, conduction velocity; SNAP, sensory nerve action potential; CMAP, compound muscle action potential; –, not recorded; Abs, absent; ADM, abductor digiti minimi; TA, tibialis anterior; +, neurogenic; N, normal value: CMAP (mv): median >5, ulnar >5, peroneal >3, tibial >3; MCV (m/s): median >50, ulnar >50, peroneal >40, tibial >40; SNAP (uv): median >7, ulnar >5, sural >6; SCV (m/s): median >45, ulnar >45, sural >40.

Bold values signify patients with abnormal value.