Hyperbaric oxygen treatment restores sudden hearing loss in a patient with Fabry disease.
نویسندگان
چکیده
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.
منابع مشابه
Fabry disease presenting with sudden hearing loss and otosclerosis: a case report
INTRODUCTION Fabry disease is an X-linked lysosomal storage disorder resulting in a multiple-system disorder with a wide spectrum of physical signs and symptoms, predominantly affecting the central and peripheral nervous systems, skin, heart, kidneys, and eyes. CASE PRESENTATION We describe the case of a 26-year-old European Caucasian man who had Fabry disease and who presented with episodic ...
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RATIONALE According to the World Health Organization reports, adult-onset hearing loss is the 15th leading cause of burden of disease, and is projected to move up to 7th by the year 2030, especially in high-income countries. Sudden sensorineural hearing loss is considered by otologists as a true otologic emergency. The current standard treatment for sudden hearing loss is a tapered course of or...
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ورودعنوان ژورنال:
- ORL; journal for oto-rhino-laryngology and its related specialties
دوره 70 3 شماره
صفحات -
تاریخ انتشار 2008