Hyperbaric Oxygen Treatment Restores Sudden Hearing Loss in a Patient with Fabry Disease

Abstract

Fabry disease is an X-linked inherited disorder of glycosphingolipid metabolism due to the deficient activity of a lysosomal enzyme, α-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.

Hyperbaric oxygen therapy as salvage treatment for sudden sensorineural hearing loss: review of rationale and preliminary report

“…Working from a hypothesis of predominantly ischaemic origin of sudden SNHL, oxygen has a therapeutic advantage in that it easily diffuses across the blood–brain barrier to reach target tissues, and high concentrations are well tolerated 9 , 10 . Hyperbaric oxygen therapy has been favoured as a treatment for sudden SNHL by many otolaryngologists, and has resulted sometimes in dramatically improved hearing 7 , 11 , 12 …”

Cochlear Implantation: A Treatment Option for Severe to Profound Hearing Loss Associated with Fabry Disease

“…Hyperbaric oxygen treatment was used successfully to treat sudden hearing loss in a 15-year-old patient with FD. 19 ERT is the most recent medical advancement in the treatment of FD and is the recommended treatment of choice for symptomatic individuals. The therapy has improved neuropathic pain, peripheral nerve function, gastrointestinal symptoms, acroparesthesia, and cardiomyopathy and has stabilized renal function in some but not all patients.…”