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Otological manifestations of a new familial polyostotic bone disorder

Published online by Cambridge University Press:  29 June 2007

D. A. Adams ,
P. K. Gormley ,
A. G. Kerr ,
G. D. L. Smyth ,
P. Osterberg  and
J. Sloan
D. A. Adams*
Affiliation:
Otolaryngology Department, Royal Victoria Hospital, Belfast.
P. K. Gormley
Affiliation:
Otolaryngology Department, Galvia Hospital, Galway.
A. G. Kerr
Affiliation:
Otolaryngology Department, Royal Victoria Hospital, Belfast.
G. D. L. Smyth
Affiliation:
Otolaryngology Department, Royal Victoria Hospital, Belfast.
P. Osterberg
Affiliation:
Orthopaedic Department, Musgrave Park Hospital, Belfast
J. Sloan
Affiliation:
Pathology Department, Royal Victoria Hospital, Belfast.
*
Mr D. A. Adams F.R.C.S., Consultant E.N.T. Surgeon, Royal Victoria Hospital, Belfast BT12 6BA.
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Abstract

Fifty members of a family with a unique autosomal dominant bone disease were investigated. Nineteen of the family members were either known to have, or were strongly suspected of having the disease. All but one of these had a hearing loss which was conductive in the younger age group and mixed in the older members. The common finding in those who had middle ear surgery was replacement of the long process of incus by a fibrous band. The histological features were similar to those found in Paget's disease. The age of onset, distribution of lesions and radiographic findings, however, were not typical of this disorder.

Keywords

DeafnessHearing lossconductiveTemporal boneBone
Type
Main Articles
Information
The Journal of Laryngology & Otology , Volume 105 , Issue 2 , February 1991 , pp. 80 - 84
Copyright
Copyright © JLO (1984) Limited 1991

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References

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