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Unusual otological manifestations in Camurati-Engelmann's Disease

Published online by Cambridge University Press:  17 May 2006

I Moumoulidis ,
R De ,
R Ramsden  and
D Moffat
I Moumoulidis
Affiliation:
Department of Neuro-Otology and Skull Base Surgery, University of Cambridge, Addenbrookes NHS Trust, Cambridge, UK
R De
Affiliation:
Department of Neuro-Otology and Skull Base Surgery, University of Cambridge, Addenbrookes NHS Trust, Cambridge, UK
R Ramsden
Affiliation:
Department of Otolaryngology, Manchester Royal Infirmary, Manchester, UK
D Moffat
Affiliation:
Department of Neuro-Otology and Skull Base Surgery, University of Cambridge, Addenbrookes NHS Trust, Cambridge, UK
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Abstract

Camurati-Engelmann's disease (CED) is a rare hereditary disorder affecting mainly the diaphysis of long bones but multiple cranial nerve deficits may also develop secondary to bony sclerosis of their foramina, including visual loss, facial palsy, deafness, vestibular disturbances and sensory deficits along the distribution of the trigeminal nerve. Deafness has been reported in about 18 per cent of these cases due to narrowing of the internal auditory canals caused by bony encroachment on nerves and vessels. We report an extremely rare case of a patient with CED who presented with deafness due to gross abnormalities affecting both middle ear and cochlea. The issues relating to the management of these patients with temporal bone involvement are discussed.

Keywords

Camurati-Engelmann's DiseaseDeafnessEar, InnerEar, Middle, Progressive Diaphyseal Dysplasia
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 120 , Issue 10 , October 2006 , pp. 892 - 895
Copyright
2006 JLO (1984) Limited

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