CT and MR Findings of Michel Anomaly: Inner Ear Aplasia

In 1863, Michel described a condition characterized by a total absence of differentiated inner ear structures associated with other skull base anomalies, including an abnormal course of the facial nerve and jugular veins. Michel aplasia clearly differs from Michel dysplasia, in which arrest of embry...

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Veröffentlicht in:American journal of neuroradiology : AJNR 1999-02, Vol.20 (2), p.281-284
Hauptverfasser: Marsot-Dupuch, Kathlyn, Dominguez-Brito, Alessandro, Ghasli, Karim, Chouard, Claude-Henri
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container_title American journal of neuroradiology : AJNR
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creator Marsot-Dupuch, Kathlyn
Dominguez-Brito, Alessandro
Ghasli, Karim
Chouard, Claude-Henri
description In 1863, Michel described a condition characterized by a total absence of differentiated inner ear structures associated with other skull base anomalies, including an abnormal course of the facial nerve and jugular veins. Michel aplasia clearly differs from Michel dysplasia, in which arrest of embryologic development occurs later. Recently, the role of otic capsule formation on mesenchymal differentiation was reported as well as the impact of the genetic deletion of the homeobox gene on the development of the ear, cranial nerves, and hindbrain. We report two patients with a total absence of inner ear structures bilaterally, illustrating the characteristic appearance of Michel aplasia and associated skull base anomalies.
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source MEDLINE; EZB-FREE-00999 freely available EZB journals; PubMed Central
subjects Adolescent
Biological and medical sciences
Child
Ear, auditive nerve, cochleovestibular tract, facial nerve: diseases, semeiology
Ear, Inner - abnormalities
Ear, Inner - diagnostic imaging
Female
Head and Neck
Hearing Loss, Sensorineural - congenital
Hearing Loss, Sensorineural - etiology
Hearing Loss, Sensorineural - genetics
Humans
Magnetic Resonance Imaging
Male
Medical sciences
Non tumoral diseases
Otorhinolaryngology. Stomatology
Skull Base - abnormalities
Tomography, X-Ray Computed
title CT and MR Findings of Michel Anomaly: Inner Ear Aplasia
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