A dominant hereditary ossicular anomaly: bilateral incus anomaly and stapes fixation.
OBJECTIVE
We report three generations of one family suffering from bilateral conductive hearing loss due to a congenital anomaly of the incus and stapes fixation.


METHODS
All three female patients presented with similar symptoms and findings of hearing impairment since birth. Their computed tomography findings were the same. An abnormally shaped incus, fixed stapes and hanging tympanic portion of the facial nerve were seen at surgery. Stapedotomy could not be performed because the hanging facial nerve blocked the operating field.


CONCLUSIONS
Although several cases of familial ossicular anomaly have been reported, this is the first report of an incus anomaly and stapes fixation combined with a facial nerve anomaly.
Lee
J H
JH
Department of Otorhinolaryngology-Head and Neck Surgery, Chuncheon Sacred Heart Hospital, School of Medicine, Hallym University, Chuncheon, Kangwon, South Korea. zoonox@nate.com
Jung
S H
SH
Kim
H C
HC
Park
C H
CH
Hong
S M
SM
D000328:Adult; D001301:Audiometry, Pure-Tone; D002675:Child, Preschool; D004432:Ear, Middle / Q000002:abnormalities* / Q000601:surgery; D005260:Female; D006314:Hearing Loss, Conductive / Q000235:genetics* / Q000601:surgery; D006801:Humans; D007188:Incus / Q000002:abnormalities* / Q000601:surgery; D008307:Malleus / Q000002:abnormalities / Q000601:surgery; D008875:Middle Aged; D010375:Pedigree; D013199:Stapes / Q000002:abnormalities; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome