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Vestibulocochlear dysfunction progressive familial

Field	Value
synonym	Familial progressive vestibulocochlear dysfunction
image	Autosomal dominant - en.svg
caption	Autosomal dominant is the inheritance pattern of this disorder
specialty

Vestibulocochlear dysfunction progressive familial, known also as familial progressive vestibulocochlear dysfunction is an autosomal dominant disease that results in sensorineural hearing loss and vestibular areflexia. Patients report feelings of vague dizziness, blurred vision, dysequilibrium in the dark, and progressive hearing impairment.

TOC

Symptoms and signs

Reported symptoms include:

Sensorineural hearing loss
Vestibular areflexia
Hearing impairment
Vertigo
Nausea and vomiting
Head movement-dependent oscillopsia

Cause

The disease is an inherited autosomal dominant disease, but the physiological cause of the dysfunction is still unclear. An acidophyllic mucopolysaccharide-containing substance was discovered, especially in cochleas, maculas, and crista ampullaris of patients with DFNA9 (a chromosome locus), as well as severe degeneration of vestibular and cochlear sensory axons and dendrites. It is suggested that the mucopolysaccharide deposit could cause strangulation of nerve endings.

The maculas and crista ampullaris are what allow for non-visual sensation of head movements. The crista ampullaris resides in the semicircular canals of the inner ear and detects angular acceleration, while the maculas are housed within the vestibule of the inner ear and detect linear acceleration. When affected, these organs can lead to vertigo and nausea because the body would always feel off-balance.

Diagnosis

References

(2000). "Familial Progressive Vestibulocochlear Dysfunction Caused by a COCH Mutation (DFNA9)". Archives of Neurology.
Saladin, Kenneth. (2012). "Anatomy and Physiology: The Unity of Form and Function". McGraw-Hill.

Info: Wikipedia Source

This article was imported from Wikipedia and is available under the Creative Commons Attribution-ShareAlike 4.0 License. Content has been adapted to SurfDoc format. Original contributors can be found on the article history page.

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