. Author manuscript; available in PMC: 2009 Sep 1.

Published in final edited form as: Int J Pediatr Otorhinolaryngol. 2008 Jun 27;72(9):1301–1309. doi: 10.1016/j.ijporl.2008.05.007

Table 1.

Demographic features, audiometric features, radiological abnormalities, familial history and genotypes of the patients

Case	Sex	Age	Degree of hearing loss ^*		Temporal bone CT	Familial Hx	Mutation
Case	Sex	Age	Rt	Lt	Temporal bone CT	Familial Hx	GJB2	SLC26A4
1	M	10	70	71.25	normal	+	-
2	F	19	95	100	-	+	-
3	F	5	NR^**	NR^**	normal	−	-
4	M	6	96.25	96.25	EVA	−		IVS9+3A>G/ H723R
5	M	2	87.5	93.75	mondini	+		-
6	M	5	97.5	95	mondini	+		-
7	M	5	NR	NR	normal	−	-
8	F	4	NR	NR	EVA, mondini	−		-
9	F	14	48.75	48.75	normal	+	-
10	M	7	65	53.75	normal	+	-
11	F	38	42.5	48.75	-	+	-
12	F	27	87.5	85	normal	+	-
13	M	23	95	96.25	normal	+	-
14	M	9	NR	NR	normal	+	235delC/235delC
15	F	3	NR^**	NR^**	normal	−	-
16	M	10	77.5	82.5	EVA	−		Q421P/H723R
17	F	2	80^**	80^**	normal	+	T86R/T86R
18	F	11	90	96.25	normal	−	235delC/235delC
19	F	15	96.25	91.25	-	+	-
20	F	9	71.25	77.5	EVA	−		H723R/H723R
21	M	12	100	82.5	normal	−	-
22	F	51	87.5	91.25	-	+	-
23	F	49	100	100	-	+	-
24	M	44	85	85	-	+	-
25	M	47	98	98.75	-	+	-
26	M	42	93.75	97	-	+	-
27	F	2	90^**	80^**	normal	+	-
28	F	6	80^**	50^**	EVA, mondini	−		H723R/H723R
29	F	44	46.25	43.75	-	+	-

Notes: pure tone average (dB HL) at 500, 1000, 2000, and 3000 Hz except the patients with ** marking

^**

the result of auditory brainstem responses Rt; right ear, Lt; left ear, Hx; history, NR; no response, EVA; bilateral enlarged vestibular aqueduct, mondini; mondini’s malformation.