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Spectrum of congenital inner ear malformations as proposed by Sennaroglu and Saatci13
Time of Insult (week)MalformationFeatures
ThirdLabyrinthine aplasia (Michel deformity)Complete absence of cochlea and vestibule
Third-to-fourthCochlear aplasiaComplete absence of cochlea; vestibule present
FourthCommon cavitySingle cystic cavity representing cochlea and vestibule, without any differentiation
FifthCystic cochleovestibular anomaly (IP-I)Cystic-appearing cochlea lacking entire modiolus and cribriform area; large cystic vestibule
SixthCochlear hypoplasiaCochlea and vestibule are separate but are smaller than normal; hypoplastic cochlea resembles small bud off the IAC
SeventhIncomplete partition type II (IP-II)Cochlea consists of 1.5 turns, in which middle and apical turns coalesce to form a cystic apex; vestibule and VA may be enlarged