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Results for Query ‹ DEAFNESS, CONGENITAL, WITH INNER EAR AGENESIS, MICROTIA, AND MICRODONTIA symptoms ›
Michel aplasia – Abstract
Michel aplasia, also known as complete labyrinthine aplasia (CLA), is a congenital abnormality of the inner ear. It is characterized by the bilateral absence of differentiated inner ear structures and results in complete deafness (anacusis).
Michel aplasia should not be confused with michel dysplasia. It may affect one or both ears.
"Aplasia" is the medical term for body parts that are absent or do not develop properly. In Michel aplasia, the undeveloped (anaplastic) body part is the bony labyrinth of the inner ear. Other nearby structures may be underdeveloped as well.
Michel aplasia – Pathology | Associations
Abnormal development of the skeletal portions of the second arch
1. Nondifferentiation of the stapes, with resultant absence of round and oval window.
2. Abnormal course of the facial nerve.
Skull base abnormalities
1. Hypoplasia of the petrous temporal bone.
2. Hypoplastic and sclerotic petrous apex may mimic labyrinthitis ossificans.
3. Platybasia.
4. Aberrant course of jugular veins.