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.