Michel (complete labyrinthine) aplasia

Case contributed by Dr Swaleh Mohamed Mohamed


Patient was brought for investigation due to deafness since birth.

Patient Data

Age: 2 years
Gender: Male

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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