It is estimated that one in every 1,000-9,000 people suffer from Fabry Disease. A milder form of the disorder that comes on later in life is more common than the more severe form that can render in young children. Fabry Disease is a pan-ethnic disorder, meaning that it can be found in all racial and ethnic populations affecting males and females. While Fabry Disease can affect many different aspects of one’s health, this guide will outline how it can impact the hearing of sufferers.
Everything You Need To Know About Fabry Disease & Hearing
What is Fabry Disease?
Fabry Disease is a rare genetic disorder caused by a defect in the GLA gene. Fabry Disease is also referred to as alpha-galactosidase-A deficiency and impacts the neurological system of the human body. This inherited defect is most commonly caused by a deficiency of the enzyme alpha-galactosidase A which is necessary for the daily breakdown of lipids. This lack of breakdown can lead to cell damage which may result in mild to severe symptoms including kidney failure, heart failure and strokes.
What are the symptoms of Fabry Disease?
Fabry Disease causes episodes of pain to sufferers, particularly in the hands and feet. It can also cause clusters of small, dark red spots on the skin, a decreased ability to sweat, cloudiness in the eyes as well as problems with the gastrointestinal system. Fabry Disease can also cause ringing in the ears also known as Tinnitus as well as as temporary and permanent hearing loss.
Is Fabry Disease life-threatening?
All cases of Fabry Disease are unique but it can come with some potentially life-threatening complications such as progressive kidney failure, heart failure and stroke. Many affected Fabry Disease sufferers only experience problems with the heart, kidneys or blood vessels in the brain in a more mild fashion.
How does Fabry Disease impact hearing?
The hearing-related symptoms of Fabry Disease include:
- Ringing in the ears or Tinnitus
- Vertigo with dizziness and nausea
- Progressive hearing loss especially in the high-tone range
- Sudden deafness in one or both ears
Audiology symptoms are becoming more commonly reported as medical professionals research and learn more about Fabry Disease education and awareness.
The audiology symptoms that result from Fabry Disease are thought to be associated with lipid accumulation causing narrowing and blockage of the blood vessels in the inner ear which can lead to auditory nerve fiber damage.
The current treatment for hearing loss and hearing-related symptoms typically includes anti-nausea medication for vertigo, hearing aids, infusion of vasodilators and steroids for sudden hearing loss as well as cochlear implants for instances of profound deafness.
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