Hyperbaric oxygen treatment restores sudden hearing loss in a patient with Fabry disease.
Fabry disease is an X-linked inherited disorder of glycosphingolipid metabolism due to the deficient activity of a lysosomal enzyme, alpha-galactosidase A. The resultant systemic accumulation of sphingolipids can lead to progressive and sudden hearing loss alongside renal, cardiac and cerebrovascular complications. Although replacement therapy seems to be beneficial for cochlear function, few data are available regarding treatment of sudden hearing loss. This case report describes the course of a unilateral sudden hearing loss in a young (15-year-old) male patient and its improvement following hyperbaric oxygen treatment.
Frantz
Markus C
MC
Department of Otorhinolaryngology, Head and Neck Surgery, Klinikum rechts der Isar, Munich, Germany. m.frantz@lrz.tum.de
Pontz
Bertram F
BF
Arnold
Wolfgang
W
D000293:Adolescent; D001299:Audiometry; D001302:Audiometry, Speech; D000795:Fabry Disease / Q000150:complications* / Q000503:physiopathology; D006309:Hearing; D003639:Hearing Loss, Sudden / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy*; D046088:Hearing Loss, Unilateral / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy*; D006801:Humans; D006931:Hyperbaric Oxygenation; D008297:Male; D020127:Recovery of Function; D016896:Treatment Outcome