What is vestibular aqueduct syndrome?
Enlarged Vestibular Aqueduct (EVA) is a condition caused by a malformation in the inner ear that leads to loss of hearing and vestibular function. EVA is the most common inner ear malformation associated with sensory hearing loss, and is detected with a CT scan.
What causes Eva?
The most well-known cause of EVA and hearing loss is mutations in a gene called SLC26A4 (previously known as the PDS gene). Two mutations in the SLC26A4 gene can result in Pendred syndrome. Scientists believe that other, currently unknown, genetic or environmental factors also may lead to EVA.
Is enlarged vestibular aqueduct hereditary?
Enlarged vestibular aqueduct (EVA; MIM 600791) is an autosomal recessive genetic disease causing congenital inner ear malformation that accounts for 1–12% of sensorineural deafness in children and adolescents [1].
What is EVA treatment?
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What is EVA in kids?
Children with EVA will have a sensorineural deafness that usually affects both ears but can be unilateral (affecting one side). Sensorineural deafness is permanent and most types are stable so that the hearing doesn’t usually change over time.
What does endolymphatic sac do?
The main functions ascribed to the endolymphatic sac are the regulation of the volume and pressure of endolymph, the immune response of the inner ear, and the elimination of endolymphatic waste products by phagocytosis.
What is endolymphatic sac surgery?
Endolymphatic sac decompression is a treatment that is performed to maintain the hydrostatic pressure and endolymph homeostasis in the inner ear for patients with Meniere’s disease. This procedure can reverse the damage to the ear while maintaining the balance of your hearing levels.
Is EVAS genetic?
Causes of EVA are Likely Multifactorial The most well-known cause of EVA and hearing loss is a gene mutation, known as SLC26A4 (previously known as the PDS gene). Two mutations in the SLC26A4 gene can result in Pendred syndrome and other factors, genetic or environmental, may also lead to EVA.
Can a deaf person hear with a cochlear implant?
Cochlear implants allow deaf people to receive and process sounds and speech. However, these devices do not restore normal hearing. They are tools that allow sound and speech to be processed and sent to the brain.
Is cochlear implant a major surgery?
The cochlear implant procedure is usually considered a minimally invasive surgery. A cochlear implant is a medical device that can partially restore hearing. The implant directly stimulates the auditory nerve to appreciate the sense of sound.
What is endolymphatic fluid?
Endolymph, also known as Scarpa fluid, is a clear fluid that can found in the membranous labyrinth of the inner ear. It is unique in composition compared to other extracellular fluids in the body due to its high potassium ion concentration (140 mEq/L) and low sodium ion concentration (15 mEq/L).
How long is an endolymphatic sac surgery?
Your doctor will remove a small amount of bone and cut a hole in the outer layer of the sac. A shunt will be placed in the sac to allow the ear to drain when fluid reforms. The incision will then be stitched closed. The procedure takes approximately 60 to 90 minutes.