Michel (complete labyrinthine) aplasia
Patient was brought for investigation due to deafness since birth.
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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.
Michel aplasia (complete labyrinthine aplasia) is a rare congenital anomaly characterized by total absent of inner ear structure developments bilaterally thus leading to anacusis.
- 1. Kathlyn Marsot-Dupuch, Alessandro Dominguez-Brito, Karim Ghasli and Claude-Henri chourand. CT and MR findings of Michel anomaly: inner ear aplasia. AJNR Am J Neuroradiol. 1999;20 (2): 281-4 http://www.ajnr.org/content/20/2/281.full