Michel aplasia

Case contributed by Dr Dalia Ibrahim

Presentation

Deafness.

Patient Data

Age: 3-year-old
Gender: Female
Modality: MRI

Absence of inner ear structures on both sides (complete labyrnithine aplasia).

Absent 8th nerves on both sides and right 7th nerve.

Both internal auditory canals are indistinct/atretic.

Small left cerebellopontine angle arachnoid cyst.

Modality: CT

Absent inner ear structures on both sides. Atretic both internal auditory canals. 

Hypoplastic both petrous apices.

Hypoplastic right middle ear cavity showing complete opacfication with slightly dysmorphic right ossicular chain. Hypoplastic right mastoid air cells.

Well aerated left middle ear cavity and mastoid air cells.

Hypoplastic stapes, absent round & oval windows on both sides.

Case Discussion

Absence of inner ear structures on both sides (complete labyrnithine aplasia).

Absent 8th nerves on both sides and right 7th nerve.

Both internal auditory canals are atretic.

Small left cerebellopontine angle arachnoid cyst.

Hypoplastic petrous apices.

Michel aplasia is a rare developmental anomaly refers to the total aplasia of the inner ear

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Case Information

rID: 51882
Case created: 11th Mar 2017
Last edited: 13th Mar 2017
System: Head & Neck
Inclusion in quiz mode: Included

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