Table 1.

Radiological features of SNHL classification in children

DIAGNOSIS	Cochlea	Vestibule	IAC	Semi-circular canal	Facial nerve canal
CLA	Absent	Absent	Aplasia/ hypoplasia	Absent	Aberrant
Rudimentary Otocyst	Absent	Absent	Absent (usually)	Tiny parts may be present	Absent
CA with normal labyrinth	Absent	Normal	Hypoplasia	Normal	Aberrant
CADV	Absent	Variably dilated	Hypoplasia	Variably dilated	Aberrant
CC	Rudimentary	Rudimentary	Usually enters at its centre	Often horizontal SCC	Aberrant
IP-1	Normal size, cystic outline, no modiolus/ISS	Usually enlarged	Enlarged	Often horizontal SCC	Normal
IP-2	Normal size, cystic apex, normal modiolus/ISS	Minimally dilated	Normal	Normal	Normal
IP-3	Normal size, no modiolus, ISS present but dysplastic	Normal	Bulbous, direct cochlear connection	Normal	Aberrant
CH-1	Small, bud-like, no ISS/modiolus	Normal	Direct cochlear connection	Normal	Normal
CH-2	Small, normal outline, defective ISS/modiolus	Normal	Direct cochlear connection	Normal	Normal
CH-3	Small (<2 turns), the “unwound cochlea” in BOR, small modiolus, short ISS	Hypoplasia	Normal	Hypoplasia	Normal
CH-4	Small, hypoplastic apical/middle turns	Normal	Normal	Normal	Aberrant
Dysplastic SCC	Normal/but can be varyingly abnormal	Normal	Normal	Dysplastic	Normal
Cochlear nerve aplasia/hypoplasia Type 1	Normal	Normal	Stenotic/ atretic	Normal	Normal
Cochlear nerve aplasia/hypoplasia Type 2a	Normal	Normal	Stenotic/ atretic	Normal	Normal
Cochlear nerve aplasia/hypoplasia Type 2b	Normal	Normal	Normal	Normal	Normal

BOR, Branchio-Oto-Renal; CADV, CA with a dilated vestibule; IAC, internal auditory canal; ISS, interscalar septa; SNHL, sensorineural hearing loss.