Michel (complete labyrinthine) aplasia

Case contributed by Dr Swaleh Mohamed Mohamed

Presentation

Patient was brought for investigation due to deafness since birth.

Patient Data

Age: 2 years
Gender: Male
Modality: CT

The brain window shows normal brain development.  Axial and coronal high resolution CT imaging shows complete absence of the differentiated inner ear structures.  The middle ear and external auditory meatus are normal.

Case Discussion

Michel aplasia (complete labyrinthine aplasia) is a rare congenital anomaly characterized by total absent of inner ear structure developments bilaterally thus leading to anacusis.

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Case Information

rID: 39682
Case created: 15th Sep 2015
Last edited: 16th Sep 2015
System: Head & Neck
Inclusion in quiz mode: Included

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