Title:
Histopathological and neuroradiological features of Usher syndrome type II
Authors:
A. Ciorba*, A. Schrott-Fisher**, A. Berto*, R. Glueckert**, A. Janecke*** and A. Martini*
Institutions:
*Audiology Department, University Hospital of Ferrara, Ferrara, Italy; **Medizinische Universitàt Innsbruck, Universitàts-HNO Klinik, Labor fur Innenohrbiologie, Innsbruck, Austria; ***Medical University Innsbruck, Department of Medical Genetics, Molecular and Clinical Pharmacology, Innsbruck, Austria
Keywords:
Usher syndrome; cochlear findings; histopathology; neuroimaging
Pages:
201 - 206
Abstract:
Histopathological and neuroradiological features of Usher syndrome type II. Objective: To study the histopathological and neuroradiological features of Usher syndrome (USH), with particular focus on USH type II, an inherited disorder characterized by moderate to severe congenital hearing impairment and retinitis pigmentosa with onset in the late teens. Methodology: A report of four cases and literature review. Results and conclusion: Rare examples of histopathological and neuroradiological findings from four USH type II cases are presented. More studies like these are encouraged so that correlation studies between the morphological and clinical findings can be performed on the path to elucidate the pathogenesis of this heterogeneous disorder.
Issue:
Vol. 4, 2008, 4th trimester


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Histopathological and neuroradiological features of Usher syndrome type II