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Table 2: Inner ear/IAC abnormalities in children with ANSD^a
Type of Temporal Bone Abnormality Cochlear Nerve Status
Normal (n = 69) Unilateral CND (n = 19) Bilateral CND (n = 15)
None 68 (98.6%)^1,2 2 (10.5%)¹ 1 (6.7%)²
Any labyrinthine abnormality 1 (1.4%)³ 3 (15.8%)⁴ 11 (73.3%)^3,4
    Cochlear malformations 1 (1.4%)⁵ 2 (10.5%)⁶ 8 (53.3%)^5,6
    Vestibular malformations 0 (0.0%)⁷ 2 (10.5%) 5 (33.3%)⁷
    SCC malformations 0 (0.0%)⁸ 2 (10.5%) 4 (26.7%)⁸
    Large endolymphatic duct/sac 1 (1.4%) 0 (0.0%) 0 (0.0%)
IAC stenosis/atresia 0 (0.0%)^9,10 5 (26.3%)⁹ 7 (46.7%)¹⁰
BCNC stenosis/atresia 0 (0.0%)^11,12 15 (78.9%)¹¹ 13 (86.7%)¹²

a Superscripts denote significant group pair differences: ^{1–5, 7–12}, P ≤ .001; ⁶, P =.010.

Type of Temporal Bone Abnormality	Cochlear Nerve Status
Type of Temporal Bone Abnormality	Normal (n = 69)	Unilateral CND (n = 19)	Bilateral CND (n = 15)
None	68 (98.6%)^1,2	2 (10.5%)¹	1 (6.7%)²
Any labyrinthine abnormality	1 (1.4%)³	3 (15.8%)⁴	11 (73.3%)^3,4
Cochlear malformations	1 (1.4%)⁵	2 (10.5%)⁶	8 (53.3%)^5,6
Vestibular malformations	0 (0.0%)⁷	2 (10.5%)	5 (33.3%)⁷
SCC malformations	0 (0.0%)⁸	2 (10.5%)	4 (26.7%)⁸
Large endolymphatic duct/sac	1 (1.4%)	0 (0.0%)	0 (0.0%)
IAC stenosis/atresia	0 (0.0%)^9,10	5 (26.3%)⁹	7 (46.7%)¹⁰
BCNC stenosis/atresia	0 (0.0%)^11,12	15 (78.9%)¹¹	13 (86.7%)¹²