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. Case report: 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. Conclusion: 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.