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Terms in this set (17)
hearing loss type - otosclerosis
primarily CHL, also mixed and snHL
physiological etiology of otosclerosis
abnormal deposition/resorption of bone
epidemiology of otosclerosis
1% of caucasian population, 2:1 female ratio
genetic inheritance of otosclerosis
autosomal dominant - incomplete penetrance
what is schwartz sign? how often is it seen?
reddish hue seen behind TM - increased vascularity of cochlear promontory in active lesions (otospongiosis). seen in 10% of patients
what is the most common location affected by otosclerosis?
fissula ante fenestram (anterior to oval window) affected in 80-90% of patients. Border of round window, apical medial wall of cochlear, posterior to cochlear aqueduct, adjacent to semicircular canals, stapes footplate itself.
3 phases of otosclerosis
1. otospongiosis (early phase: inc bone resorption and vascularity, formation of new spongy bone --> (blue mantles of Manasse on H&E staining)
2. transitional phase
3. otosclerosis (late phase): formation of dense sclerotic bone.
what type of bone does otosclerosis begin in
endochondral bone - as it evolves the endosteal and periosteal layers become involved
otosclerosis that fills oval window niche
otosclerosis involving only the footplate, sparing annular ligament (high risk of postop snHL because of minimal fixation - footplate can become mobilized inadvertently)
how often is the round window involved?
30% of clinical cases of otosclerosis. complete closure is uncommon
mechanism of snHL in patients with otosclerosis
controversial. possible liberation of toxic metabolities into inner ear, vascular compromise, extension of lesions into cochlea. snHL usu associated with significant stapedial otosclerosis.
what is cochlear otosclerosis
pure snHL without associated CHL.
shambaugh's 7 criteria for identifying patients suffering from snHL due to otosclerosis.
1. schwartze sign in either ear
2. family history of otosclerosis
3. unilateral CHL c/w OS and bilat symm snHL
4. flat or cookie bite audio with excellent discrims
5. progressive pure cochlear loss beginning at usual age of onset for OS
6. CT showing demineralization of cochlear typical of OS
7. stapedial reflex demonstrating biphasic "on-off effect" before stapedial fixation
percent of OS patients with dizziness
30% - OS lesions in lat semicirc canal. Positive results on ENG. called OS "inner ear syndrome"
absolute contraindication for stapedectomy/stapedotomy
Meniere's disease - enlarged endolymphatic space, saccule may be adherent to underside of stapes footplate, increased risk of profound snHL
clinical signs of OS inner ear syndrome
no well-defined episodes of rotational vertigo, nausea, vomiting, or fluctuating snHL. mild/persistent dizziness, low frequency snHL NOT present.
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