Three familial cases of Michel's aplasia

Three familial cases of Michel's aplasia

Complete agenesis of the bony labyrinth, first described by Michel, represents the most severe form of inner ear defect. A search of the literature yielded only one report of this rare anomaly, affecting two siblings. Three familial cases of bilateral inner ear aplasia are reported here, and the pro...

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Veröffentlicht in:Otology & neurotology 2002-05, Vol.23 (3), p.346-348
Hauptverfasser: DANESHI, Ahmad, FARHADI, Mohammad, ASGHARI, Alimohamad, EMAMJOMEH, Hesamedin, ABBASALIPOUR, Parvaneh, HASANZADEH, Saeed
Format: Artikel
Sprache:eng
Schlagworte:
Biological and medical sciences
Child
Child, Preschool
Ear, auditive nerve, cochleovestibular tract, facial nerve: diseases, semeiology
Ear, Inner - abnormalities
Ear, Inner - diagnostic imaging
Female
Genetic Diseases, Inborn - complications
Genetic Diseases, Inborn - diagnostic imaging
Genetic Diseases, Inborn - rehabilitation
Hearing Loss, Sensorineural - etiology
Humans
Male
Medical sciences
Non tumoral diseases
Otorhinolaryngology. Stomatology
Pedigree
Tomography, X-Ray Computed
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Zusammenfassung:Complete agenesis of the bony labyrinth, first described by Michel, represents the most severe form of inner ear defect. A search of the literature yielded only one report of this rare anomaly, affecting two siblings. Three familial cases of bilateral inner ear aplasia are reported here, and the probable inheritance pattern of this condition is discussed.
ISSN:1531-7129
1537-4505
DOI:10.1097/00129492-200205000-00020