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 |
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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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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.</description><identifier>ISSN: 0195-6108</identifier><identifier>EISSN: 1936-959X</identifier><identifier>PMID: 10094354</identifier><identifier>CODEN: AAJNDL</identifier><language>eng</language><publisher>Oak Brook, IL: Am Soc Neuroradiology</publisher><subject>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</subject><ispartof>American journal of neuroradiology : AJNR, 1999-02, Vol.20 (2), p.281-284</ispartof><rights>1999 INIST-CNRS</rights><rights>Copyright © American Society of Neuroradiology 1999</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC7056116/pdf/$$EPDF$$P50$$Gpubmedcentral$$H</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC7056116/$$EHTML$$P50$$Gpubmedcentral$$H</linktohtml><link.rule.ids>230,314,723,776,780,881,53769,53771</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=1701552$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/10094354$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Marsot-Dupuch, Kathlyn</creatorcontrib><creatorcontrib>Dominguez-Brito, Alessandro</creatorcontrib><creatorcontrib>Ghasli, Karim</creatorcontrib><creatorcontrib>Chouard, Claude-Henri</creatorcontrib><title>CT and MR Findings of Michel Anomaly: Inner Ear Aplasia</title><title>American journal of neuroradiology : AJNR</title><addtitle>AJNR Am J Neuroradiol</addtitle><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.</description><subject>Adolescent</subject><subject>Biological and medical sciences</subject><subject>Child</subject><subject>Ear, auditive nerve, cochleovestibular tract, facial nerve: diseases, semeiology</subject><subject>Ear, Inner - abnormalities</subject><subject>Ear, Inner - diagnostic imaging</subject><subject>Female</subject><subject>Head and Neck</subject><subject>Hearing Loss, Sensorineural - congenital</subject><subject>Hearing Loss, Sensorineural - etiology</subject><subject>Hearing Loss, Sensorineural - genetics</subject><subject>Humans</subject><subject>Magnetic Resonance Imaging</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Non tumoral diseases</subject><subject>Otorhinolaryngology. Stomatology</subject><subject>Skull Base - abnormalities</subject><subject>Tomography, X-Ray Computed</subject><issn>0195-6108</issn><issn>1936-959X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1999</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpVkF1LwzAYhYsobk7_guRCvSu8aZq08UIYY9PBhiATvAvv0nSNZOlsNsv-vYXNr6tzcR6eA-ck6lPJRCy5fDuN-kAljwWFvBddhPAOAFxmyXnUowAyZTztR9loQdAXZP5CJtYX1q8CqUsyt7oyjgx9vUa3vydT701DxtiQ4cZhsHgZnZXogrk65iB6nYwXo6d49vw4HQ1nccUSuo0FZpIxhhRZblKAhPNc50WeC2SgBcDS8FwUuiyFLJZUasF4US4ZpiIDViAbRA8H72a3XJtCG79t0KlNY9fY7FWNVv1vvK3Uqv5UGXBBqegEd0dBU3_sTNiqtQ3aOIfe1LughBSpgCTrwOu_Sz8T3191wM0RwKDRlQ16bcMvlwHlPOmw2wNW2VXV2sao0H3oOitVbdsmoBKV5JR9AV8Hfqk</recordid><startdate>19990201</startdate><enddate>19990201</enddate><creator>Marsot-Dupuch, Kathlyn</creator><creator>Dominguez-Brito, Alessandro</creator><creator>Ghasli, Karim</creator><creator>Chouard, Claude-Henri</creator><general>Am Soc Neuroradiology</general><general>American Society of Neuroradiology</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>19990201</creationdate><title>CT and MR Findings of Michel Anomaly: Inner Ear Aplasia</title><author>Marsot-Dupuch, Kathlyn ; Dominguez-Brito, Alessandro ; Ghasli, Karim ; Chouard, Claude-Henri</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-h321t-6a79333a1a38e4002558c8d886a30c600be586dcff69db19c635dfb3a46703da3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1999</creationdate><topic>Adolescent</topic><topic>Biological and medical sciences</topic><topic>Child</topic><topic>Ear, auditive nerve, cochleovestibular tract, facial nerve: diseases, semeiology</topic><topic>Ear, Inner - abnormalities</topic><topic>Ear, Inner - diagnostic imaging</topic><topic>Female</topic><topic>Head and Neck</topic><topic>Hearing Loss, Sensorineural - congenital</topic><topic>Hearing Loss, Sensorineural - etiology</topic><topic>Hearing Loss, Sensorineural - genetics</topic><topic>Humans</topic><topic>Magnetic Resonance Imaging</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Non tumoral diseases</topic><topic>Otorhinolaryngology. Stomatology</topic><topic>Skull Base - abnormalities</topic><topic>Tomography, X-Ray Computed</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Marsot-Dupuch, Kathlyn</creatorcontrib><creatorcontrib>Dominguez-Brito, Alessandro</creatorcontrib><creatorcontrib>Ghasli, Karim</creatorcontrib><creatorcontrib>Chouard, Claude-Henri</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>American journal of neuroradiology : AJNR</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Marsot-Dupuch, Kathlyn</au><au>Dominguez-Brito, Alessandro</au><au>Ghasli, Karim</au><au>Chouard, Claude-Henri</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>CT and MR Findings of Michel Anomaly: Inner Ear Aplasia</atitle><jtitle>American journal of neuroradiology : AJNR</jtitle><addtitle>AJNR Am J Neuroradiol</addtitle><date>1999-02-01</date><risdate>1999</risdate><volume>20</volume><issue>2</issue><spage>281</spage><epage>284</epage><pages>281-284</pages><issn>0195-6108</issn><eissn>1936-959X</eissn><coden>AAJNDL</coden><abstract>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.</abstract><cop>Oak Brook, IL</cop><pub>Am Soc Neuroradiology</pub><pmid>10094354</pmid><tpages>4</tpages></addata></record> |
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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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