Table 1 General clinical features of children with neurocristopathy-related hearing loss.

ID	Sex	CI_age_mon	Hearing Loss/Type	Outer/Middle Ear Abnormalities	Inner Ear Abnormalities	Auditory nerve malformation	Craniofacial dysmorphism	growth retardation	Intellectual disability/Gesell score^a
WS01	male	9 mon	Severe SNHL	-	-	-	-	-	88/96/79/29/85
WS02	female	10mon	Severe SNHL	-	-	-	-	-	82/85/80/38/79
WS03	female	8 mon	Severe SNHL	-	+	-	-	-	79/90/87/50/81
WS04	male	12 mon	Severe SNHL	-	-	-	-	-	87/82/81/46/78
WS05	female	16 mon	Severe SNHL	-	-	-	-	-	88/90/83/37/84
WS06	male	13 mon	Severe SNHL	-	-	-	-	-	76/81/80/40/75
NS01	male	16 mon	Severe SNHL	-	-	-	+	-	82/86/75/29/85
NS02	male	19 mon	Severe SNHL	-	-	-	+	+	89/83/85/61/72
NS03	female	17 mon	Severe SNHL	-	-	-	+	+	85/82/83/36/80
KS01	female	18 mon	Severe SNHL	+	+	-	+	+	71/78/73/58/74
CS01	female	26 mon	Severe SNHL	+	+	+	+	+	56/60/55/40/66
CS02	male	48 mon	Severe SNHL	+	+	+	+	+	60/71/56/39/63
CS03	male	36 mon	Severe SNHL	+	+	+	+	+	65/57/59/40/63
CS04	female	18 mon	Severe SNHL	+	+	+	+	+	58/48/55/37/50
CS05	female	17 mon	Severe SNHL	+	+	+	+	+	56/70/63/33/49
CS06	female	19 mon	Severe SNHL	+	+	+	+	+	55/45/52/30/51

^aGesell score are reported as scaled scores across five domains: Motor Skills/Fine Motor/Adaptive Behavior/Language Development/Personal-Social Skill. Scoring Interpretation: 85–115: Normal development, 70–85: Mild delay, < 70: Significant delay (may indicate need for intervention) clinical assessment including hearing dysfunction was performed in all participants (n = 16).
CI: cochlear implant, CS: CHARGE syndrome, ID: identification number, KS: Kabuki syndrome, mon: months, NS: Noonan syndrome, SNHL: Sensorineural Hearing Loss.WS: Waardenburg syndrome.

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