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DEFINATION: Otosclerosis is a common hereditary disease of localized bone derived from the otic capsule, where the normal laminar bone is removed by osteoclasts & replaced by unorganized bone of greater thickness, vascularity & cellularity.

SYMPTOMS:

  1. HEARING LOSS: Mainly conductive, sensorial or mixed in cochlear type. Bilateral, painless, progressive, insidious. PARACUSIS WILLISII- Patient hears better in noisy surroundings (as people tend to speak in a louder voice against a background noise).
  2. TINNITUS: Common with cochlear otosclerosis & active disease.
  3. VERTIGO: Persistent & frequent in beginning. Paroxysmal & less frequent, lasts & maximum for 3 weeks & do not reccur.
  4. SPEECH: Monotonous. Well modulated soft. [BC is more & patient hears these own voice louder].
  5. BEZOLD’S TRIAD: Absolute negative Rinne. Increase lower tone limit. Prolonged bone conduction.

SIGNS:

  1. OTOSCOPY: EAR- Wider. TM- Quite normal & mobile. A reddish hue may be seen on the promontory through the TM (Schwartz Sign or Flamingo pink blush).
  2. ET FUNCTION: Normal.
  3. TUNNING FORK TEST: 1. Rinne’s Test- Negative. 2. Weber’s Test- Lateralised to affected site. 3. ABC- Normal. 4. Gelle’s Test- Positive in case of fixed stapes foot plate.
  4. AUDIOGRAM: Air bone gap present. A notch or dip at 200 Hz in BC curve- Carhart’s Notch. Mixed hearing loss (cochlear otosclerosis). Cookie bite pattern.
  5. CT SCAN OF TEMPORAL BONE: Loss of definition of margins of oval window to narrowing & finally complete obliteration of oval window opening & niche. Cochlear otospongiosis- double ring effect due to spongiosis within the thickened capsule.

DIAGNOSIS:

  1. Ossicular discontinuity behind an intact drum.
  2. Congenital fixation of ossicles, mainly stapes-foot plate.
  3. Secretory otitis media.
  4. Fixed malleus syndrome.
  5. Tympanosclerosis.

MANAGEMENT:

  1. MEDICAL: No medical treatment that cures it. Sodium fluoride has been tried to hasten the maturity of active focus & arrest further cochlear loss.
  2. SURGICAL: Various procedures-

          1) Stapedectomy,

          2) Small fenestra stapedectomy,

          3) Stapedotomy,

          4) Stapedotomy with Stapedius tendon preservation,

          5) Vein graft Teflon interposition operation,

          6) Laser stapedotomy.

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