Hearing loss in fabry disease: A 16 year follow-up study of the Danish nationwide cohort.

ABSTRACT:

Background

Fabry disease (FD) is a lysosomal storage disorder resulting in systemic accumulation of globotriaosylceramide (Gb₃) causing multi-organ dysfunction. The audiologic involvement in FD has been neglected in previous studies; while not a lethal aspect of the disease, hearing loss can have a significantly negative impact on quality of life.

Objective

To investigate hearing loss from baseline through 16 years follow-up of the Danish FD cohort and to compare audiometric data to other clinical variables.

Methods

Data was collected prospectively and assessed retrospectively during a period of 16 years from 83 patients (age: 9-72 years; sex: 29 males and 54 females). 55 patients underwent treatment. Air conduction thresholds was assessed at six frequencie

SUBMITTER: Yazdanfard PDW 

PROVIDER: S-EPMC8857513 | biostudies-literature | 2022 Jun

REPOSITORIES: biostudies-literature