OTOSCLEROSIS

Sandipan naskar
CNMCH
INTRODUCTION

 Worldwide, the number of patients suffering from

otosclerosis has declined considerably.

 Cause for rejoicing / an unique predicament to modern-

day otologist.

 We (young modern-day otologists) will need to

overcome our own learning curve before being able to
perform the surgery reliably and deliver consistently
good results.

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DEFINITION:
Otosclerosis is a localized hereditary metabolic
disorder affecting endochondral bone of the otic
capsule that is characterized by disordered resorption
and deposition of bone.

This process occurrs in two phases:

(1) active phase as characterized by bone resorption
(spongiosis)
(2) phase of remission characterized by bone deposition
(sclerosis).
It is unique to human temporal bone;
Normal inhibition of bone remodelling is lost resulting in foci of bone
remodelling

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HISTORY

First described by Valsalva (early 18th century)

VonTroltsch named the final inactive stage as

‘otosclerosis’ (1869)
Siebenmann disignated the active stage as ‘otospongiosis’ (1912)
Toynbee: described different types of stapes fixation and oval window
involvement

Politzer first recognised it as primary bone disease

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EPIDEMIOLOGY

 RACE:
There appears to be a definite racial predisposition.

Caucasians (most common)

Southeast Asian (less common)
Native American (less common)
African (rare)

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 PREVALENCE:
The exact incidence remains unclear and next to
impossible to determine.
prevalence of clinically apparent otosclerosis0.3% to 0.5% of
general population (Shambaugh)

prevalence of histologic otosclerosis is 10% to 12%

of which only 1% become clinically apparent.

Many authors have noted that the prevalence otosclerosis

has declined steeply in recent times.

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 AGE OF ONSET:
It can range from 10 to 50years of age, however, it can
also occur in older people.
the incidence of presumptive clinical otosclerosis
increases with age.

 GENDER:
Not a genetically sex-linked characteristic disease; thus,
a ratio of 1:1 would have been expected.
Indeed, histologically the distribution is equal
clinically Male:Female = 1:2
progress to involve both ear in 85-90%
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 OTOSCLEROSIS AND PREGNANCY:
There are many reports that associate the onset of hearing
loss caused by otosclerosis and the onset of pregnancy.
some of them mentioned-
pregnency aggravates the hearing loss.
in bilateral stapedial otosclerosis the incidence is 33% after one
pregnency and 64% after 6 pregnencies.
Reason – unknown ;
Hormonal factors may be responsible.

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 TYPES OF OTOSCLEROSIS:

1. Histologic otosclerosis
2. Fenestral / Clinical otosclerosis
3. Cochlear otosclerosis
4. Malignant otosclerosis
5. Far advanced otosclerosis

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 HISTOLOGIC OTOSCLEROSIS:
o a finding on microscopic examination of temporal
bones.

o location of the otosclerotic changes is such that it

generally does not involve the stapes bone, the
stapediovestibular joint, or the cochlear endosteum.

o therefore asymptomatic.

o diagnosed only by post-mortem examination of the

temporal bone.
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“Histologic” Otosclerotic Focus

Stapes
Footplate

Cochlea

Vestibule

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 “Histologic” Otosclerotic Focus
Cochlea

Stapes
Footplate

Vestibule

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 FENESTRAL (Clinical) OTOSCLEROSIS:

o an otosclerotic lesion that involves the stapes bone and

/or the stapediovestibular joint.

o clinically manifested by a conductive hearing impairment

o also defined ‘presumptive clinical otosclerosis’

(British National Study of Hearing)

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Clinical
Otosclerotic
Foci

Stapes
Footplate

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 COCHLEAR OTOSCLEROSIS:

o the otosclerotic lesion invades the cochlear endosteum

o usually reserved for the occurrence of pure

sensorineural hearing loss due to otosclerosis
without any conductive component.

o hearing loss is usually bilateral and symmetrical

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 Otosclerotic Foci

saccule

utricle

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Otosclerotic Foci

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 “MALIGNANT” (OBLITERATIVE) OTOSCLEROSIS:

o active otosclerosis involving both oval and round

windows and most of the bony labyrinth
o initially by mixed hearing loss
relentlessly progress to

o profound sensorineural hearing loss

o both windows are obliterated by the otosclerotic focus

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RW

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 FAR ADVANCED OTOSCLEROSIS (FAO):

o defined as no measurable air or bone conduction

or
o air conduction no better than 95 dB and bone conduction
at 55 dB to 60 dB at one frequency only.

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DISTRIBUTION OF OTOSCLEROTIC LESIONS

anterior to the oval window (80-95%) Most common

fissula ante fenestrum
round window niche (about 30%)
apical medial wall of the cochlear labyrinth (about 15 %)
stapes footplate (about 12%)
posterior to the oval window (5-10%)

walls of the internal auditory canal

Less
around the vestibular and cochlear aqueducts
Commonly
around the semicircular canals
around malleus and incus involved

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HISTOPATHOLOGY OF OTOSCLEROSIS

 Otosclerosis is characterized by the following:

1. Bone resorption
2. New bone formation
3. Vascular proliferation
4. Connective tissue stroma.

 The otic capsule contains small regions of immature cartilaginous

tissue called the “globuli interossei.” This may be the loci of the
earliest lesions of otosclerosis.

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Can be divided into :
early phase / spongiotic phase
late phase / sclerotic phase

Early Phase:
The lesion consists of—
histiocytes
osteoblasts
osteocytes
osteoclasts

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First Stage:
resorption of enchondral bone around blood vessels by
histiocytes and osteoclasts enlargement of perivascular
spaces
Second stage:
widening of the vascular channels and dialatation of the
microcirculation
Third stage:
deposition of cellular fibrous connective tissue within this enlarged
perivascular space

reticular cells and fibroblasts within the connective tissue are

transformed into osteoblasts and osteocytes

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these osteoblasts lay down immature basophilic bone, rich in
ground substance and deficient in collagen;
the osteocytes themselves becomes active and elongated;
microfoci of otospongiosis forms and they fuse to form large foci.

Fourth stage:
Resorption and deposition of immature bone goes on continuously
within an otosclerotic focus.

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So, histologically an active focus is indicated by—

o Areas of nonosseous tissues that demonstrate

increased cellularity and vascularity

o Areas of bone resorption and/or bone deposition

o Increased vascularity, thickening and fibrosis of the

overlying mucosa

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 Late Phase:
the newly laid down immature bone is replaced by osseous tissue
containing more collagen and less ground substance

formation of dense sclerotic bone in areas of previous bone

resorption ; the vascular channels become narrowed

the otosclerotic focus is larger in volume than the bone it replaces

and thus it causes thickening of the structure it involves.

At the interface of normal and otosclerotic bone two types of

changes are seen:
sharply demarcated line an advancing front that appeared serrated

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Blue Mantle Of Manasse:
o basophilic staining regions that are seen in the otic capsule near
the otosclerotic foci in the temporal bone that has been stained with
hemotoxylin and eosin ( Manasse ; 1922 )

o non-specific histologic changes

characterised by plexus like projection

o probably represents bone

that has been remodelled recently

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Schwartz’s Sign:
the dialated vessels within the otospongiotic focus forms
anastomosis with the vessels of the bony labyrinth

as the foci enlarges and reaches the

surface mucosa, vascular shunts forms
between these dialated vessels and the
submucosal vessels, resulting in
hyperemia of the mucosa

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MOLECULAR BIOLOGY

osteoclast

Bone
Remodelling

osteoblast

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Bone remodeling at a local level is very tightly regulated
by a delicate balance among the 3 cytokines:
(1) OPG (osteoprotegrin) {fibroblast > perilymph}
(2) RANK {osteoclast}
(3) RANK-L{osteoblast}

RANK-L

RANK

OPG

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 In otosclerosis the balance is
tipped in favor of remodeling at
specific areas within the otic
capsule.

osteoclast

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AETIOLOGY

 GENETIC PREDISPOSITION:
Otosclerosis represents a heterogeneous group of genetic disorders
in which different genes may be involved.

It is postulated that in response to the different gene variants and

environmental factors, the physiologic inhibition of bone turnover in
the otic capsule is overruled, which then results in otosclerosis.

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o Eight genetic loci OTSC1 to OTSC8 have been published to date
of which OTSC1 was mapped to 15q25–q26 in an Indian family

o Association analysis has revealed a significant association between

defected COL1A1 gene and both familial and sporadic cases of
clinical otosclerosis. (association with osteogenesis imperfecta type 1)

o Autosomal-dominant transmission with incomplete penetrance in

familial otosclerosis.

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 MEASLES:

There is strong evidence that MeV infection is associated with the

sporadic form of otosclerosis. (McKenna et al)

Suggestion of a possible persistent measles virus infection similar to

what occurs in the central nervous system in subacute sclerosing
panencephalitis (SSPE)

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 Detection of measles-like structures and antigenicity in active otosclerotic
lesions

 Measles RNA has been found in temporal bone specimen with

otosclerosis . It was also detected in fresh otosclerotic footplate
specimens taken at the time of stapedectomy

 Raised levels of antimeasles antibodies have also been reported in the

perilymph of otosclerotic patients

 Epidemiological data reveals a clear and significant decline in the incidence

of otosclerosis following the introduction of the measles vaccine.)

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The measles virus has not been isolated so far
from otosclerotic tissue.

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 AUTOIMMUNITY:

autoimmune disease with humoral autoimmunity to type II collagen

present in the sites of prediliction
o concept is supported by —
Production of lesions in the otic capsule in some
animals immunized with type II collagen

Elevated circulating antibodies to type II collagen in

the blood of some patients with otosclerosis
o criticised due to —
concurrent joint lesion
relapsing polychondritis

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CLINICAL FEATURES

Symptoms:
o Hearing Loss
Progressive over month to years
Usually bilateral ( unilateral in 15% cases)
Paracusis Willisii (20-78% of patients)
Patient talks in low voice
o Tinnitus
Occurs in 37-78% of patients
Present in the early phase
Probably due to abnormal vascularity
Pulsetile / roaring / hissing

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o Vestibular Symptoms (10-30%)
Transient attack vertigo are not uncommon
Due to action of toxic enzymes released by the lesion into the
vestibular labyrinth leads to degeneration of SCARPA’S
GANGLION
Co-existing Meniere's disease

Dizziness

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Signs:
o Otoscopy
Normal TM in 90% of cases
Schwartz’s sign ( Flemingo's flush ) in 10% cases
o Pneumatic otoscopy
o To rule out malleus fixation

o Tuning fork test

Initially conductive hearing loss
Gradually becomes mixed or sensorineural
Initially at low frequency; later, at high frequency

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 Pathology Of Conductive Hearing Impairment:

Appeared to be caused primarily by, narrowing and impairment

of the annular ligament, especially at the posterior
stapediovestibular joint space.

The size of the air-bone gap appeared to be determined by the

extent and degree of narrowing of the posterior stapediovestibular
joint space.

bony ankylosis of stepes footplate > AB gap 30 db or more but

not proportional quantitatively

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 Pathology Of Sensorineural Hearing Impairment:

Appears to occur when an otosclerotic focus reaches the

endosteum of the cochlea.
'Hyalinization' of the spiral ligament by releasing cytokines

 Pathology of vestibular symptoms:

Damage of the vestibular end organs by soluble toxic substances
liberated by otosclerotic bone
or
Changes in biochemistry of the inner ear fluids
or
Both

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DIAGNOSIS

Otosclerosis is the presumptive diagnosis of a conductive hearing

impairment with normal mobile tympanic membrane.

The 'gold' reference standard of diagnosis is surgery.

Histological confirmation of otosclerosis is more readily obtained if

a total stapedectomy is performed; now that stapedotomy is the
favoured surgical procedure, so it is usually valueless.

However the diagnosis can be suggested by some investigations

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INVESTIGATIONS
 Pure Tone Audiometry:
A low frequency conductive hearing loss in the early stages

Once the hearing loss reaches the 25 dB stage the patient

demonstrates a conductive hearing loss at all frequencies with a dip
at 2 kHz known as Carhart’s notch

Carhart’s notch-
depression of bone conduction thresholds of approximately 5 dB
at 500 Hz, 10 dB at 1 kHz, 15 dB at 2 kHz, and 5 dB at 4 kHz
mechanical artifact and not truly represent cochlear reserve
Carhart’s effect-

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In late stage mixed type and SN hearing loss occurs

Cookie bite pattern-

mixed type of hearing loss, greatest at mid-frequency and
better hering in low and high frequency

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 Impedance Audiometry:
Static Compliance –
Normal static compliance values fall in the range of 0.3 to
1.6 cc. Values < 0.3 cc are indicative of stiffness in the conductive
mechanism. Typical ‘As’ type of curve is less common.
Acoustic Reflexes –
In fixed stapes – absent reflex
Still mobile stapes- diphasic pattern

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 Speech Audiometry:
Usually speech discrimination score (SDS) is excellent.

 Otoacoustic Emissions:
OAEs are not present in otosclerosis.

 Radiology:
CT scan / MRI

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HRCT scan
Active otosclerotic foci were more likely to be identified than inactive
foci
Active foci is perceived as “hypodense” area (sensitivity 94%)

Coronal cuts are better for oval window

Axial cuts are better for round winow

Typical of cochlear otosclerosis is the formation of a double ring

effect due to multiple foci coalescing with each other.

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 Cochlear Otosclerosis.
The cochlea and semicircular canals are demineralized

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 CT Densitometry:
Whether the foci is active or inactive
Response to fluride therapy

 Single Photon Emission Computed Tomography(SPECT)

SPECT allows the detection of pathologic increases of bone
metabolism even in the absence of clinical manifestations of the
underlying process.

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DIFFERENTIAL DIAGNOSIS
o Ossicular discontinuity (CHL 60 dB, Ad tympanogram)

o Congenital stapes fixation (other congenital anomalies, CHL non

progressive)

o Malleus head fixation (other congenital anomalies, aural atresia)

o Paget’’s disease (diffuse involvement of bony skeleton)

o Osteogenesis imperfecta (blue sclera, easy fracture of other bone)

o Superior semicircular canal dehiscence (vertigo, nystagmus with

loud noise)

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MANAGEMENT OPTIONS

MEDICAL MANAGEMENT
Directed at the active phase of fenestral otosclerosis, presumed
cochlear otosclerosis

 FLUORIDE THERAPY:
Daniel first noted that there is an increase of stapedial otosclerosis
in areas where the levels of fluoride were low
Shambaugh and Scott first suggested that sodium fluoride in
moderate dosages is very effective only when the otosclerotic focus
is active.

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Fluorides act by:
1. Reducing bone resorption.
2. Increasing osteoblastic bone formation.
3. Fluorides prevent the release of proteolytic enzymes that are
cytotoxic to the cochlea.
4. Reducing the vascularity of an active foci.

Indications:
Patients with progressive sensorineural hearing loss, positive family
history, radiological sign suggestive of active cochlear otosclerosis.

Stapedial otosclerosis patients who refused surgery.

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 Administration:
has been used as –
Fluoridation of drinking water
Oral fluorides

Results:
at present there is no evidence that support use of fluoride in either
forms in management of otosclerosis.

 BISPHOSPHONATES:

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 HEARING AIDS

may be considered in following situations—

1. Cannot undergo surgery because of major systemic illnesses.

2. The only hearing ear.
3. Inadequate hearing reserve
4. Surgery is not elected for by the patient.

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5. Early (mild) conductive hearing loss.
6. During post-stapedectomy rehabilitation
7. As a rescue treatment many years after surgery
8. In combination with surgery in FAO

BAHA
can be a low risk alternative to stapedectomy.

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SURGERY:

HISTORICAL PERSPECTIVE

 Three distinct phases can be identified in the development of

surgery for otosclerosis:
stapedectomy and mobilization of the stapes
(in late ninteenth century)
the fenestration procedures
(early twentieth century)
mobilization of the stapes revisited, along with stapedectomy and
stapedotomy with prostheses
(in the later half of the twentieth century.)

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 Stapedectomy And Mobilization Of The Stapes
(in late ninteenth century)
 Kessel (1878)
first described his work on the columella of pigeons and stapes of
dogs and humans.
he removed the stapes footplate 'allowing a new membrane to
form'.

 Bucheron (1888) and Miot (1890)

 Blake (1892) and Jack (1893)
performed similar procedures and achieved improved hearing in
more than half of their patients.
.

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But
the results were temporary
effective in early ankylosis
complications were more (meningitis & death)

In 6th International Otologic Congress, London (1899) these

procedures were condemned.

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 Fenestration Procedures
(early twentieth century)
 Holmgren (1930)
father of fenestration surgery

 Sourdille (Holmgren’s student)

multi-stage fenestration surgery
64% stisfactory results

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 Julius Lempert

popularised
the single staged
fenestration procedure

Fenestration surgery provided significant and lasting hearing

improvement in large series of patients

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 Mobilization Of The Stapes Revisited
(in the later half of the twentieth century)
 Samuel Rosen (1952)

Reintroduced stapes mobilisation,

unware of earlier work of Miot, Bucheron

Became accepted as the

treatment of choice for those
with only partial ankylosis, though re-ankylosis tended to
develop a few years later.

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 Stapedectomy
(in the later half of the twentieth century.)

 John Shea (1956)

Perfected stapedectomy
and introduced the concept of
ossicular continuty by a teflon prsthesis

Within a decade this procedure became the standard operation for

treatment of otosclerosis

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In the next three decades this procedure underwent furthur
refinement and has been replaced (except in some selected cases)
by
Limited Stapedectomy (only the posterior half of footplate)

and more recently

Stapedotomy

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 Best surgical candidate:

 Previously un-operated ear

 Good health
 ABG of 25 dB or more
 Negative Rinne test in 512 Hz
 Excellent speech discrimination score

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 Contraindication:

1. Perforation of ear drum

2. Otitis externa and otitis media
3. Meniere’s Disease
4. Age more than 70 yr (relative)
5. Only hearing ear (except in profound mixed hearing loss who is
a candidate for cochlear implant)
6. Contralateral ear has a disease which may threaten hearing in
future
7. Those requiring intact vestibule for professional or occupational
purpose (air travel, scuba diving, parachuting)

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 Anaesthesia:

Local vs General

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SURGICAL STEPS:

12’O clock to 6’O clock (conventional)

Incision:
1’O clock to 7’O clock (better exposure)

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Elevation Of The Flap:

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Chorda Tympani Is Dissected Free
Of Fibrous Annulus & manubrium

Flap Along With The Nerve Is

Folded Anteriorly On The Umbo

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Removal Of Scutum:

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 Thin plate of scutum is
removed by a currete

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