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Table 1: Comparison of temporal bone CT findings by visual inspection between children with Cornelia de Lange Syndrome and the control group
Structure Abnormalities CdL (20 ears) Control (20 ears)
Classic Mild
Middle ear Ossicle dysmorphic 10 2 0
Cleft of incus body present 8 2 0
Soft-tissue opacification 8 0 0
Mastoid hypoplastic 10 2 0
Eustachian tube patulous 0 0 0
Tensor tympani absent 0 0 0
Inner ear 
    Cochlea Incomplete-partitioned 7 2 0
Modiolus deficient 10 2 0
    Vestibule Focal protrusion present 9 6 0
    VA Enlarged 0 0 0
Facial nerve Canal dehiscent 3 0 0
Note:—CdL indicates Cornelia de Lange syndrome; VA, vestibular aqueduct.

Structure	Abnormalities	CdL (20 ears)		Control (20 ears)
Structure	Abnormalities	Classic	Mild	Control (20 ears)
Middle ear	Ossicle dysmorphic	10	2	0
	Cleft of incus body present	8	2	0
	Soft-tissue opacification	8	0	0
	Mastoid hypoplastic	10	2	0
	Eustachian tube patulous	0	0	0
	Tensor tympani absent	0	0	0
Inner ear
Cochlea	Incomplete-partitioned	7	2	0
	Modiolus deficient	10	2	0
Vestibule	Focal protrusion present	9	6	0
VA	Enlarged	0	0	0
Facial nerve	Canal dehiscent	3	0	0