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Osteopathia striata with cranial sclerosis and hearing loss

Osteopathia striata with cranial sclerosis and hearing loss Osteopathia striata is a manifestation of several bone dysplasias. In association with cranial sclerosis, it represents a separate entity that is not limited to the bones, but may also affect other structures, leading to an abnormal face, cleft palate, deafness, heart defects and vertebral anomalies. Neurological findings range from normal development to marked retardation with hydrocephalus, cranial nerve deficiencies and deafness. So far, only a few patients have been described in the literature, demonstrating autosomal dominant inheritance. Here, we report a spontaneous mutation of inheritance. The parents and three sisters of the affected child are healthy. In 78.6% of the reported cases with osteopathia striata and hearing loss, a conductive hearing loss was described. Surgical attempts to mobilize the middle-ear bones were unsuccessful because of swelling and augmented growth of the bones in the middle ears. Here the mobilization of the eardrum was not attempted because the middle ear cavities were epidermized. This is the second case in the literature in which a myringotomy was not done because the middle ear was completely epidermized. As a possible alternative, children with osteopathia striata and cranial sclerosis with hearing loss should be fitted with hearing aids because surgery of the middle ear probably will be difficult or perhaps not possible. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png European Archives of Oto-Rhino-Laryngology Springer Journals

Osteopathia striata with cranial sclerosis and hearing loss

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References (17)

Publisher
Springer Journals
Copyright
Copyright © 2006 by Springer-Verlag
ISSN
0937-4477
eISSN
1434-4726
DOI
10.1007/s00405-005-0972-8
pmid
16010569
Publisher site
See Article on Publisher Site

Abstract

Osteopathia striata is a manifestation of several bone dysplasias. In association with cranial sclerosis, it represents a separate entity that is not limited to the bones, but may also affect other structures, leading to an abnormal face, cleft palate, deafness, heart defects and vertebral anomalies. Neurological findings range from normal development to marked retardation with hydrocephalus, cranial nerve deficiencies and deafness. So far, only a few patients have been described in the literature, demonstrating autosomal dominant inheritance. Here, we report a spontaneous mutation of inheritance. The parents and three sisters of the affected child are healthy. In 78.6% of the reported cases with osteopathia striata and hearing loss, a conductive hearing loss was described. Surgical attempts to mobilize the middle-ear bones were unsuccessful because of swelling and augmented growth of the bones in the middle ears. Here the mobilization of the eardrum was not attempted because the middle ear cavities were epidermized. This is the second case in the literature in which a myringotomy was not done because the middle ear was completely epidermized. As a possible alternative, children with osteopathia striata and cranial sclerosis with hearing loss should be fitted with hearing aids because surgery of the middle ear probably will be difficult or perhaps not possible.

Journal

European Archives of Oto-Rhino-Laryngology – Springer Journals

Published: Feb 1, 2006

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