Otosclerosis

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Author:
jvirbalas
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209885
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Otosclerosis
Updated:
2013-03-27 16:33:17
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Otosclerosis
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Otosclerosis
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  1. This is the mode of inheritance of otosclerosis
    Autosomal dominant with incomplete penetrance (~60% have family history)
  2. Thisis the most commonly involved site of involvement in otosclerosis.
    Anterior to the oval window (fissula ante fenestrum)
  3. This is the manner in which pregnancy effects otosclerosis?
    Pregnancy can accelerate the effects of otosclerosis
  4. Microscopic evidence of otosclerosis can be found in this percentage of caucasians, though only 1% are affected with clinical otosclerosis.
    • •10% of Caucasians
    • •1% of African Americains
  5. Histopathologic evaluation of involved bone in a patient with clinical otosclerosis reveals hypercellular bone with pockets of resorption. The borders of the focus are irregular and project into normal bone along blood vessels. This is the name given to those blue-stained projections.
    Blue mantles of Manasse
  6. A 20 year old man has fixation of the stapes confirmed intraoperatively.  He has had multiple fractures of his long bones and you note blue sclera on exam. What diagnosis should be considered before otosclerosis?
    Ostgeogenesis Imperfecta is an autosomal dominant condition in which a defect in osteoblast activity results in fragile bones and blue sclera. Half of these patients develop stapes fixation. The short term surgical response to stapes surgery in these patients is similar to that seen in otosclerosis, but progressive postoperative sensorineural hearing loss is more common.
  7. A 15 year old boy has had stable CHL since birth. He has an abnormally small tympanic membrane.  What diagnosis should be considered before otosclerosis?
    Congenital fixation of the stapes may also occur in conjunction with an abnormally small tympanic membrane, partial meatal atresia, or a shortened manubrium.  The sometimes abnormal course of the facial nerve in these patients makes operative repair a greater challenge.
  8. This is the greatest risk of performing a stapedectomy on a patient with congenital fixation of the stapes.
    Perilymph gusher.  Thought to be due to a widened cochlear aqueduct or defect in the fundus of the IAC.
  9. SNHL and radiolucent areas near the cochlea on fine cut CT provide evidence for this diagnosis.
    Cochlear otosclerosis. The vast majority of cases of otosclerosis are associated with conductive or mixed hearing loss. Occasionally, a case of pure sensorineural otosclerosis will be described, but its existence is controversial.
  10. In this disease, resorption begins in the periosteal bone instead of the endochondrial layer as it does in otosclerosis.  There will also be diffuse involvement of the skull rather than being confined solely to the temporal bone.
    Paget's disease begins in the periosteal bone instead of in the endochondral capsule like otosclerosis. It causes sensorineural loss, probably due to hydrolytic enzymes entering the cochlear fluid. Conductive hearing loss is rare in Paget's disease.
  11. A red hue behind the tympanic membrane in a patient with active otosclerosis in indicative of hyperemia of this structure.
    Increased vascularity of the promontory mucosa
  12. As otosclerosis worsens, a rinne test will demonstrate bone conduction greater than air conduction first in which tuning fork? (256hz; 512 hz; or 1024 hz)
    The tuning fork exam may indicate a conductive hearing loss, with bone conduction greater than air conduction first at 256 Hz, then 512Hz, and finally 1024 Hz.
  13. In otosclerosis, Carhart’s notch is a characteristic depression of bone conduction at this frequency.
    2000 Hz
  14. It the late stages of otosclerosis, this reflex becomes attenuated.
    In pre- clinical otosclerosis, tympanometry may show an "on-off" effect, in which there is an abnormal decrease in impedance at the beginning and ending of the eliciting signal. With disease progression, this "on-off" effect usually gives way to an absent stapedial reflex.
  15. It the late stages of otosclerosis, this reflex becomes attenuated.
    In pre- clinical otosclerosis, tympanometry may show an "on-off" effect, in which there is an abnormal decrease in impedance at the beginning and ending of the eliciting signal. With disease progression, this "on-off" effect usually gives way to an absent stapedial reflex.
  16. Schwartze’s sign, though pathognomonic for otosclerosis, is present in only this percentage of patients.
    10%
  17. Which tuning fork must generate a Rinne (bone>air) prior to undertaking surgical management of otosclerosis?
    When the 512-Hz fork reveals bone conduction greater than air conduction, the patient has at least a 15-20 dB CHL. 1024-Hz corresponds with 30 dB CHL.
  18. Stapedotomy can be performed with a microdrill or one of these two types of lasers.
    • •Visible argon or KTP-532
    • •Infrared CO2
  19. What are the benefits of stapedotomy vs stapedectomy?
    Proponents of stepedotomy argue that a limited opening to the vestibule carries a lower risk of damage to the inner ear (SNHL) and vertigo.
  20. This nonsurgical treatment of cochlear otosclerosis has been shown to slow the progression of hearing loss
    Flouride therapy has been shown to reduce the progression of SNHL.
  21. 45F with poorly controlled Meniere’s and a 50dB low-frequency CHL.  What is the best way to address the patient’s hearing loss.
    Fluoride and hearing aids.  Stapedectomy is contraindicated in a patient with poorly-controlled Meniere’s and HL 45 dB or greater at 500 Hz.  Risk of dead ear due to the close relationship of saccular membrane or Reissner’s membrane with the stapes footplate.
  22. Stretching  of this nerve can occur in 30% of stapedectomies.  It may result in temporary dry mouth, tongue soreness, or metallic taste.
    Chorda tympani
  23. The distance between these two structures is what determines the size of the prosthesis after stapedectomy.
    From the incus to the footplate
  24. Though this is a risk in primary stapedectomy, the risk is 7-8 times greater in revision stapedectomy.
    Iatrogenic SNHL.  Success rates in primary surgery are ~90% closure of ABG.  65% in revision surgery.
  25. A patient s/p stapedectomy complains of vertigo when she burps or presses on her ear. 
    She has a positive “fistula test.” What is the most likely problem?
    Prosthesis is too long.
  26. 5 days s/p stapedectomy, patient begins to
    develop an incomplete facial palsy.  What
    is the next step?
    Rarely,delayed onset facial palsy occurs about 5 days post-op and lasts for a few weeks.  It is usually incomplete and responds quickly to prednisone.

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