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Otosclerosis
Dr. Vishal Sharma
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.
Antonio Valsalva
First described
ankylosis of stapes
in 1741
Adam Politzer
Coined the term
Otosclerosis
in 1893
Friedrich Siebenmann
Coined the term
Otospongiosis
in 1912
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)
Van der Hoeve syndrome
Otosclerosis + osteogenesis imperfecta +
blue sclera
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.
Types of Stapedial Otosclerosis
1. Anterior focus (commonest): 2 mm anterior to oval
window. 2. Posterior focus: 2 mm behind oval window.
3. Circumferential: involves footplate margin only.
Types of Stapedial Otosclerosis
4. Biscuit type: footplate involved, margin is free.
5. Obliterative: obliterates oval window completely.
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
Symptoms of Otosclerosis
4. Paracusis Willisii: Pt has better hearing in noisy
surroundings (people increase their voice intensity
& pt’s speech discrimination becomes better).
Thomas Willis (1621-1675)
Otoscopy
Normal T.M. is seen in
most cases. Pinkish
colour over promontory
seen in otospongiosis
(2 - 10 % cases) 
Schwartze sign /
Flamingo pink blush.
Tuning Fork Tests
Rinne
Weber
A.B.C.
Stapedial
Negative
(BC > AC)
Lateralizes to
Deaf ear
Normal
Cochlear
Positive
(AC > BC)
Lateralizes to
Better ear
Decreased
Mixed
Negative
(BC > AC)
Lateralizes to
Better ear
Decreased
Gelle & Bing Tests
Vibrating 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
Pure Tone Audiometry
• Low frequency conductive deafness
• Carhart’s notch in bone conduction at 2 KHz
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
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.
Speech Audiometry
Impedance Audiometry
• As curve seen in 40 %
cases of otosclerosis.
• Normal middle ear
pressure + decreased
middle ear compliance.
• Others have A curve.
Stapedial reflex present
Stapedial reflex absent
C.T. scan temporal
bone
200 coronal oblique cuts are taken
Stapedial otosclerosis (coronal)
Cochlear otospongiosis (axial)
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
History of
development of
stapes surgery
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)
Johannes Kessel
Samuel Rosen
Gunnar Holmgren
Maurice Sourdille
Julius Lempert
John J. Shea Jr.
Inclusion criteria for surgery
• Pure Tone Average between 30 - 60 dB
• Air bone gap > 15 dB
• Speech discrimination score > 60 %
• Absence of sensorineural deafness
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
Surgical steps for
Stapedotomy
Right T.M. (upright)
Right T.M. (supine)
Per-meatal Incision
Tympanomeatal flap raised
Bony overhang curetted
Bony overhang curetted
Chorda tympani preserved
Chorda tympani separated
Confirm footplate fixation
Checking for absence of round window reflex
Depth measurement prosthesis
Incudo-stapedial joint broken
Footplate perforation made
Fenestration with burr
Posterior crus fractured
1
cm
Teflon piston
• Length of piston =
medial surface of
incus to stapes
footplate + 0.25 mm
• Range = 3.75 – 4.25
mm
Piston placed in perforation
Stapedius tendon cut
Stapedius tendon cut
Piston crimped around incus
Anterior crus fractured
Stapes superstructure removed
Footplate perforation sealed
Tympanomeatal flap put back
Laser Stapedotomy
Stapedius vaporization
Footplate fenestration
Rossette formation
Stapedotomy Piston
Stapedectomy
Footplate Fenestration
Stapes superstructure removed
Footplate removal
Prosthesis placed over vein graft
Complications of
stapes surgery
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
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
Hearing Aid
For patients who:
• Are unfit for surgery
• Refuse surgery
Thank You