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A dominant hereditary ossicular anomaly: bilateral incus anomaly and stapes fixation

Published online by Cambridge University Press:  28 January 2009

J H Lee ,
S H Jung ,
H C Kim ,
C H Park  and
S M Hong
J H Lee*
Affiliation:
Department of Otorhinolaryngology-Head and Neck Surgery, School of Medicine, Hallym University, Chuncheon, South Korea
S H Jung
Affiliation:
Department of Otorhinolaryngology-Head and Neck Surgery, Wonju College of Medicine, Yonsei University, Wonju, South Korea
H C Kim
Affiliation:
Department of Radiology, School of Medicine, Hallym University, Chuncheon, South Korea
C H Park
Affiliation:
Department of Otorhinolaryngology-Head and Neck Surgery, School of Medicine, Hallym University, Chuncheon, South Korea
S M Hong
Affiliation:
Department of Otorhinolaryngology-Head and Neck Surgery, School of Medicine, Hallym University, Chuncheon, South Korea
*
Address for correspondence: Dr J H Lee, Department of Otorhinolaryngology-Head and Neck Surgery, Chuncheon Sacred Heart Hospital, School of Medicine, Hallym University, 153 Kyo-Dong, Chuncheon, Kangwon, South Korea. Fax: 82 33 241 2909 E-mail: [email protected]
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Abstract

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.

Keywords

Ear OssiclesDeafnessCongenital
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 123 , Issue 11 , November 2009 , pp. 1280 - 1284
Copyright
Copyright © JLO (1984) Limited 2009

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