Otosclerosis

Otosclerosis (otosclerosa, otospongiosis, otospongiosa) is an AD or multifactorial hereditary disease of the capsule of the labyrinth. Women are more often affected. The median incidence is 35 years. In women, the disease often manifests itself during pregnancy. The incidence (diagnosed, histologically verified) is about 1%.

Pathogenesis
There is gradual spongiotization (sclerotization) of the capsule of the labyrinth, especially in the area of ​​the oval window (fenestra ovalis), which leads to fixation of the stirrup and subsequent narrowing of the labyrinth lumen. Finally, the lumen disappears completely and is replaced by fibrous tissue (labyrintitis ossificans).

Clinical symptoms
The main clinical signs include:
 * hypacusia (the hearing defect in otosclerosis can be conductive, mixed or even perceptual; in the case of a perceptual defect, the oval window is not affected by otosclerosis and we speak of the so-called retrocochlear otosclerosis) );
 * vertigo;
 * tinnitus.

Diagnostics

 * Otoscopy - the eardrum is normal.
 * Audiology - we use audiology to diagnose conductive hearing loss.
 * Tympanometry - we get the A curve.
 * Stapedial reflex is disabled.

Treatment
The treatment is symptomatic, trying to correct the transmission defect. It can be divided into conservative and surgical treatment. Surgical treatment: we distinguish 2 methods:
 * Conservative therapy: is indicated in patients with cochlear otosclerosis and when surgical treatment is impossible. Includes p.o. application of fluorides.
 * Stapedectomy - is the complete removal of the stapes + its replacement with a so-called Schuknecht prosthesis.
 * Stapedotomy - removal of only the suprastructures of the stirrup (arms and head) + piston implantation.
 * Stapedoplasties are demanding operations with the possible occurrence of serious postoperative complications (meningitis, labyrinthitis, deafness).

Related Articles

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 * Examination methods in ENT/overview