ORL
Case Report
Vestibular Findings and Brainstem Pathology in Two Siblings with Cockayne’s SyndromeIwasaki S.a · Kaga K.b · Yagi M.a · Kuroda M.caDepartment of Otolaryngology, Musashino Red Cross Hospital, Tokyo, and Departments of bOtolaryngology and cPathology, University of Tokyo, Japan
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Article / Publication Details
Received: May 08, 1995
Accepted: February 28, 1996
Published online: January 13, 2010
Issue release date: 1996
Number of Print Pages: 4
Number of Figures: 0
Number of Tables: 0
ISSN: 0301-1569 (Print)
eISSN: 1423-0275 (Online)
For additional information: https://www.karger.com/ORL
Abstract
Two siblings with Cockayne’s syndrome (CS) are described. Both showed cachectic dwarfism, photosensitivity and progressive neurological dysfunction. The damped-rotation test revealed no vestibular nystagmus in one of them, and reduced perrotatory nystagmus in the other. Neuropathological examination in the former subject disclosed neuronal loss with fibrillary gliosis in the vestibular nuclei, whereas neuronal degeneration was not apparent in the abducens nuclei, the oculomotor nuclei and the paramedian pontine reticular formation. Our findings suggest that CS might cause vestibular dysfunction as well as hearing loss and that the lesions in the vestibular nuclei might be related to the vestibular dysfunction.
© 1996 S. Karger AG, Basel
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Article / Publication Details
Received: May 08, 1995
Accepted: February 28, 1996
Published online: January 13, 2010
Issue release date: 1996
Number of Print Pages: 4
Number of Figures: 0
Number of Tables: 0
ISSN: 0301-1569 (Print)
eISSN: 1423-0275 (Online)
For additional information: https://www.karger.com/ORL
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