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Imaging findings of the ears with labyrinthine aplasia
Patient CT/MRI CLA Otic Capsule Middle Ear Ossicle Anomaly IAC Cochlear Nerve Facial Nerve Course Jugular Anomaly Posterior Fossa Anomaly Skull Base−Other Bone Anomaly
1 +/+ R Apl Hypopl + Apl Apl Aberr + Large CPA Narrow clivus
L Apl Hypopl + Apl Apl Aberr + 
2 +/− L Hypopl Hypopl + Narrow ? Aberr + R CPA arachnoid cyst Low tegmen tympani, narrow clivus
3 +/+ R Apl Hypopl + Narrow Apl Aberr + – Tegmental defect (L), low tegmen tympani (R), narrow clivus
L Hypopl Hypopl – Narrow Apl Aberr – 
4 +/+ L Hypopl Hypopl + Narrow Apl Aberr + Split brain stem Narrow clivus
5 +/+ R Hypopl Hypopl ? Narrow Apl Aberr + Wide 4th v lateral recess Tegmental defect, narrow clivus
6 +/+ R Apl Hypopl ? Narrow Apl Aberr + Pontine malformation Encephalocele
L Apl Hypopl + Apl Apl Aberr + 
7 +/− R Hypopl N ? Apl ? ? + – –
L Hypopl N ? Narrow ? Aberr – 
8 +/+ R Hypopl Hypopl + Narrow Apl Aberr – – –
9 +/+ R Hypopl Hypopl – Narrow Apl Aberr – Chiari 1 –
L Hypopl Hypopl + Narrow Apl Aberr + 
Note:—MRI indicates MR imaging; R, right; L, left; Apl, aplasia; Hypopl, hypoplasia; N, normal; ?, could not be evaluated in detail; Aberr, aberrant; CPA, cerebellopontine angle; 4th v, fourth ventricle; +/+, both present; +/−, MRI absent; –, not found; CLA, complete labyrinthine aplasia; IAC, internal auditory canal; +, present.