Demographic, genetic and radiological characteristics of the patients

	Sex	Age at Imaging	Clinicoradiologic Phenotype	Genetic Mutation	Cochlear Phenotype	Imaging Where Cochlea Was Assessed
Patient 1	M	5 mo	WWS	POMT1	CH4 AOUT	MR imaging brain, CT brain
Patient 2	M	3 mo	WWS	POMT1	CH4 AOUT	MR imaging brain, CT brain
Patient 3	F	15 days	WWS	POMT2	CH4 AOUT	MR imaging brain, CT brain
Patient 4	F	1 day	WWS	POMT1	CH4 AOUT	MR imaging brain (axial T2)
Patient 5	M	1 day	WWS	FKRP	CH4 AOUT	MR imaging brain, CT brain
Patient 6	M	4 yr	WWS	Not available	CH 4 (less severe phenotype)	MR imaging brain (axial T2)
Patient 7	F	2 mo	WWS	Not available	CH4 AOUT	MR imaging brain (axial T2)
Patient 8	M	9 days	WWS	Not available	CH4 AOUT	MR imaging brain (axial T2)
Patient 9	F	4 mo	WWS	B3GNT1	CH4 AOUT	MR imaging brain, CT brain
Patient 10	F	18 mo	WWS	FKTN	CH4 AOUT	MR imaging brain, CT brain
Patient 11	F	7 mo	WWS	POMT1	CH4 AOUT	MR imaging brain, CT brain, 3D T2 IAMs
Patient 12	M	3 mo	WWS	Not available	CH4 AOUT	MR imaging brain (axial T2)
Patient 13	F	2 mo	WWS	Not available	CH4 AOUT	MR imaging brain (axial T2)
Patient 14	M	2.5 yr	MEB	POMGnT1	CH 4 (less severe phenotype)	MR imaging brain (axial T2)
Patient 15	F	7 yr	MEB	POMGNT1	Normal	MR imaging brain, CT brain
Patient 16	M	3 mo	MEB	POMGnT1	Normal	MR imaging brain, CT brain
Patient 17	M	2 days	MEB	POMGnT1	Normal	MR imaging brain (axial T2)
Patient 18	M	21 yr	MEB	POMGnT1	Normal	MR imaging brain (axial T2)
Patient 19	M	1 yr	MEB	POMT2	CH4 AOUT	MR imaging brain (axial T2), 3D-T2 IAMs
Patient 20	M	11 mo	MEB	POMGNT2	CH 4 (less severe phenotype)	MR imaging brain (axial T2)
Patient 21	M	9 mo	MEB	Not available	Normal	MR imaging brain (axial T2)
Patient 22	M	6 mo	Fukuyama	FKRP	Normal	MR imaging brain (axial T2)
Patient 23	F	2 days	Fukuyama	FKRP	CH 4 (less severe phenotype)	MR imaging brain, CT brain
Patient 24	F	14 mo	Fukuyama	Not available	Normal	MR imaging brain (axial T2)

Note:—IAMs indicates internal auditory meatus.