Cardiac and renal dysfunction is associated with progressive hearing loss in patients with Fabry disease.

ABSTRACT:

Background

Fabry disease (FD) is an X-linked recessive hereditary lysosomal storage disorder which results in the accumulation of globotriaosylceramid (Gb3) in tissues of kidney and heart as well as central and peripheral nervous system. Besides prominent renal and cardiac organ involvement, cochlear symptoms like high-frequency hearing loss and tinnitus are frequently found with yet no comprehensive data available in the literature.

Objective

To examine hearing loss in patients with FD depending on cardiac and renal function.

Material and methods

Single-center study with 68 FD patients enrolled between 2012 and 2016 at the Department of Oto-Rhino-Laryngology, Plastic, Aesthetic and Reconstructive Head and Neck Surgery of the University of Würzburg. Every subject unde

SUBMITTER: Koping M 

PROVIDER: S-EPMC5697846 | biostudies-literature | 2017

REPOSITORIES: biostudies-literature