14-otosclerosis
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Transcript of 14-otosclerosis
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Otosclerosis
Dr. Vishal Sharma
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Definition
• Hereditary disease of bony labyrinth showing
replacement of lamellar enchondral bone by
irregularly laid new bone.
• New bone is spongy + more vascular in active
Otospongiosis but thicker & more cellular in
inactive Otosclerosis.
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Antonio Valsalva
First described
ankylosis of stapes
in 1741
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Adam Politzer
Coined the term
Otosclerosis
in 1893
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Friedrich Siebenmann
Coined the term
Otospongiosis
in 1912
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Epidemiology• Exact etiology is unknown (? Viral)
• Autosomal dominant: variable penetrance
• Race: common in white races & Indians
• Female : Male = 2 : 1
• Age: Common in 20 - 40 years
• Hormonal influence: es in pregnancy,
menopause, stress (trauma, surgery)
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Van der Hoeve syndrome Otosclerosis + osteogenesis imperfecta +
blue sclera
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Types of Otosclerosis
A. Stapedial
B. Cochlear: otosclerotic focus is seen over
Round window Promontory
C. Stapedial + cochlear: mixed type
D. Malignant: rapidly progressing cochlear
lesion with severe sensori-neural deafness.
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Types of Stapedial Otosclerosis1. Anterior focus (commonest): 2 mm anterior to oval
window. 2. Posterior focus: 2 mm behind oval window.
3. Circumferential: involves footplate margin only.
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Types of Stapedial Otosclerosis
4. Biscuit type: footplate involved, margin is free.
5. Obliterative: obliterates oval window completely.
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Symptoms of Otosclerosis
1. Deafness: Bilateral, slowly progressive
Conductive: stapedial otosclerosis
Sensori-neural: cochlear otosclerosis
Mixed: stapedial + cochlear otosclerosis
2. Soft, modulated, monotonous voice
3. Tinnitus & vertigo: in cochlear lesion
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Symptoms of Otosclerosis4. Paracusis Willisii: Pt has better hearing in noisy
surroundings (people increase their voice intensity
& pt’s speech discrimination becomes better).
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Thomas Willis (1621-1675)
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Otoscopy
Normal T.M. is seen in
most cases. Pinkish
colour over promontory
seen in otospongiosis
(2 - 10 % cases)
Schwartze sign /
Flamingo pink blush.
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Tuning Fork TestsRinne Weber A.B.C.
Stapedial Negative (BC > AC)
Lateralizes toDeaf ear
Normal
Cochlear Positive (AC > BC)
Lateralizes to Better ear
Decreased
Mixed Negative (BC > AC)
Lateralizes to Better ear
Decreased
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Gelle & Bing TestsVibrating tuning fork placed over mastoid & then:
External auditory canal is blocked in Bing test or
E.A.C. pressure ed by Siegalization in Gelle test
Bing Gelle
Otosclerosis No change No change
Normal / SNHL Intensity es Intensity es
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Pure Tone Audiometry• Low frequency conductive deafness
• Carhart’s notch in bone conduction at 2 KHz
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Carhart’s notch• Proposed theories
1. Stapes fixation disrupts normal ossicular
resonance (2000 Hz)
2. Normal compression mode of bone
conduction is disturbed because of relative
perilymph immobility
3. Mechanical artefact
• Carhart’s notch reverses with stapes surgery
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Speech Audiometry
Speech Discrimination Score (maximum score
achieved) is almost 100 %.
Speech Reception Threshold (intensity at which
50 % words are heard) is increased by the
amount of conductive hearing loss.
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Speech Audiometry
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Impedance Audiometry
• As curve seen in 40 %
cases of otosclerosis.
• Normal middle ear
pressure + decreased
middle ear compliance.
• Others have A curve.
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Stapedial reflex present
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Stapedial reflex absent
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C.T. scan temporal bone
200 coronal oblique cuts are taken
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Stapedial otosclerosis (coronal)
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Cochlear otospongiosis (axial)
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Differential Diagnosis
• Otitis Media with Effusion: type B tympanogram
• Adhesive Otitis Media: absence of T.M. mobility
• Tympanosclerosis: white patch on T.M.
• Ossicular discontinuity: type Ad tympanogram
• Congenital ossicular chain fixation: tympanotomy
• Malleus head fixation: tympanotomy
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History of development of stapes surgery
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Stapes mobilization: Kessel (1880), Rosen (1952)
Lateral semicircular canal fenestration: Holmgren
(1923), Sourdille (1932), Lempert (1938)
Complete Stapedectomy: Jack (1893), Shea (1956)
Partial Stapedectomy (posterior 1/3): Plester (1960)
Stapedotomy: Shea (1962), Marquet (1965)
Laser Stapedotomy: Perkins & Di Bartolomeo (1980)
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Johannes Kessel
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Samuel Rosen
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Gunnar Holmgren
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Maurice Sourdille
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Julius Lempert
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John J. Shea Jr.
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Inclusion criteria for surgery
• Pure Tone Average between 30 - 60 dB
• Air bone gap > 15 dB
• Speech discrimination score > 60 %
• Absence of sensorineural deafness
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Contraindications for surgery
Only hearing ear Meniere’s disease
Otitis media Otitis externa
Extremes of age Pregnancy
Professions: divers, high construction workers,
frequent travelers, noisy surroundings
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Surgical steps for
Stapedotomy
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Right T.M. (upright)
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Right T.M. (supine)
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Per-meatal Incision
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Tympanomeatal flap raised
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Bony overhang curetted
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Bony overhang curetted
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Chorda tympani preserved
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Chorda tympani separated
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Confirm footplate fixation
Checking for absence of round window reflex
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Depth measurement prosthesis
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Incudo-stapedial joint broken
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Footplate perforation made
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Fenestration with burr
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Posterior crus fractured
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Teflon piston
• Length of piston =
medial surface of
incus to stapes
footplate + 0.25 mm
• Range = 3.75 – 4.25
mm
1
cm
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Piston placed in perforation
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Stapedius tendon cut
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Stapedius tendon cut
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Piston crimped around incus
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Anterior crus fractured
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Stapes superstructure removed
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Footplate perforation sealed
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Tympanomeatal flap put back
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Laser Stapedotomy
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Stapedius vaporization
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Footplate fenestration
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Rossette formation
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Stapedotomy Piston
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Stapedectomy
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Footplate Fenestration
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Stapes superstructure removed
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Footplate removal
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Prosthesis placed over vein graft
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Complications of stapes surgery
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Intra-operative Post-operative
Floating footplate Otitis media
Submerged footplate Oval window granuloma
Dislocated incus Perilymph fistula
Perforated TM Sensori-neural HL
Damage to facial nerve Persistent air-bone gap
or chorda tympani Vestibular dysfunction
Persistent stapedial artery Delayed facial palsy in
Perilymph flooding laser surgery
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Sodium Fluoride• Given in cochlear otosclerosis & active focus
of otospongiosis (Schwartze sign).
• Acts by:
a. Decreases bone resorption
b. Increases bone formation
c. Prevents enzymatic damage to cochlea
• 20 mg orally, thrice daily for 3 - 6 months
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Hearing Aid
For patients who:
• Are unfit for surgery
• Refuse surgery
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Thank You