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 probable inheritance pattern of this condition is discussed.
Daneshi
Ahmad
A
Department of Otolaryngology-Head and Neck Surgery, Hazrat Rasoul Akram Hospital, Iran University of the Medical Sciences, St. Niayesh, Ave. Satarkhan, Tehran, Iran.
Farhadi
Mohammad
M
Asghari
Alimohamad
A
Emamjomeh
Hesamedin
H
Abbasalipour
Parvaneh
P
Hasanzadeh
Saeed
S
D002648:Child; D002675:Child, Preschool; D007758:Ear, Inner / Q000002:abnormalities* / Q000000981:diagnostic imaging*; D005260:Female; D030342:Genetic Diseases, Inborn / Q000150:complications / Q000000981:diagnostic imaging* / Q000534:rehabilitation; D006319:Hearing Loss, Sensorineural / Q000209:etiology; D006801:Humans; D008297:Male; D010375:Pedigree; D014057:Tomography, X-Ray Computed