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Updated: Aug 13, 2026

Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
[The cochlea in Fabry disease: a sensorineural hearing loss model of vascular origin?]
D Malinvaud1, P Avan, D P Germain
1Laboratoire de Recherche CNRS 7060 Neurobiologie des Systèmes Sensorimoteurs, Département d'ORL et de Chirurgie Cervicofaciale, Faculté de Médecine Paris-V, Université René-Descartes, Hôpital Européen Georges-Pompidou, 20, rue Leblanc, 75015 Paris, France.
Purpose:
Fabry disease is an inborn error of metabolism due to a deficient activity of the lysosomal enzyme alpha-galactosidase A. The enzyme defect leads to the systemic accumulation of neutral glycosphingolipids in tissues, mainly in the vascular endothelium.
Strong Point:
The aim of this paper is to present a review of the auditory manifestations in Fabry disease, and to discuss hypothesis on the vascular origin of deafness.
Perspectives:
Sensorineural hearing loss in Fabry disease could be the first documented vascular pathology of the inner ear.
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