Table 1: Patients’ distribution according to gender, age of onset, audiological evaluation and genetic background.

ANSD Sex Age OAE ABR CM GJB2 Genotype Past History
1 F Childhood + No response - wt/wt None
2 M Congenital + No response + wt/wt Preterm baby (35wks) / neonatal unity care for 10 days
3 M Congenital + No response - wt/wt None
4 M Adolescent + No response - wt/wt None
5 M Congenital + No response - wt/wt None
6 F Congenital + No response + wt/wt Preterm baby (36 wks)
7 M Adult + No response - IVS1+G>A/wt None
8 F Congenital + No response - wt/wt None
9 F Childhood + No response - wt/wt None
10 M Congenital + No response - wt/wt None
11 M Congenital + No response + wt/wt Brother with SNHL
12 M Congenital + No response - wt/wt Kernicterus
13 F Congenital + No response - wt/wt Preterm baby (29 wks), neonatal unity care for 35 days, neonatal CRA
14 M Adult + No response - wt/wt None
15 F Congenital + No response - wt/wt Neonatal jaundice, preterm baby (36 wks), neonatal unity care for 26 days
16 F Congenital + No response - wt/wt Axial ascending muscular atrophy
17 M Adolescent + No response - wt/wt None
18 M Congenital + No response - c.35delG/c.35delG None
19 M Congenital + No response - c.35delG/c.35delG None
20 F Congenital - No response + wt/wt None
21 M Congenital - No response + wt/wt None
22 F Congenital - No response + wt/wt None
23 M Congenital - No response + wt/wt None
24 M Congenital - No response + wt/wt Charge syndrome
25 M Congenital - No response + wt/wt None
26 M Congenital - No response + wt/wt Preterm baby (27 wks), neonatal unity care for 30 days
27 M Congenital - No response + wt/wt Preterm baby (28 wks), neonatal unity care for 42 days, meningitis
28 M Congenital - No response + wt/wt Preterm baby (32 wks), neonatal unity care for 14 days
29 M Congenital - No response + wt/wt Neonatal unity care for 10 days
30 M Congenital - No response + wt/wt None
31 F Congenital - No response + wt/wt Gestational toxoplasmosis
32 F Congenital - No response + wt/wt aspiration pneumonia 3 months old
33 M Congenital - No response + c.35delG/c.35delG Preterm baby (35 wks), neonatal unity care for 05 days
34 M Congenital - No response + wt/wt Preterm baby (35 wks), neonatal unity care for 19 days
35 F Adolescent - No response + wt/wt Systemic arterial hypertension, diabetes mellitus
36 M Congenital - No response + wt/wt Brother with SNHL
37 F Adolescent - No response + wt/wt None
38 M Congenital - No response + wt/wt Preterm baby (35 wks), neonatal unity care for 16 days
39 F Congenital - No response + wt/wt None
40 M Childhood - No response + wt/wt None

Ps.: M: male; F: female; +: positive; -: negative; N/A: not available; wt: wild type.