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ADITYA GHOSH ROY
PGT-2 M.S. E.N.T.
N.R.S.M.C.H.
OTOSCLEROSIS
ADITYA GHOSH ROY
PGT-2 M.S. E.N.T.
N.R.S.M.C.H.
PARRT 1
 Otosclerosis is a localized hereditary disorder
affecting endochondral bone of the otic capsule that
is characterized by...
 'CLINICAL' OTOSCLEROSIS refers to a lesion that involves
the stapes bone or stapediovestibular joint and consequently is...
 The bone of the otic capsule is unique that it exhibits
very little remodeling .
 It contains small regions of immature...
BLUE MANTLES OF MANASSEH
'BLUE MANTLE'. BLUE MANTLES ARE AREAS OF
THE OTIC CAPSULE THAT STAIN MORE
BASOPHILIC THAN NORMAL
OTOSCLEROTIC FOCUS CONSISTS OF
BONE RESORPTION
NEW BONE FORMATION
VASCULAR PROLIFERATION
CONNECTIVE TISSUE STROMA
 RESORPTION OF ENCHONDRAL BONE
 ENLARGEMENT OF PERIVASCULAR SPACES
 DEPOSITION OF IMMATURE BONE
 ACTIVE RESORPTION + R...
 An otosclerotic focus may appear as
ACTIVE OR 'SPONGIOTIC', CHARACTERIZED BY
 areas of increased cellularity and vascul...
NON-CLINICAL FOCI OF
OTOSCLEROSIS
ANTERIOR FOOTPLATE INVOLVEMENT
Bipolar involvement of
the footplate
ORGAN OF CORTI
 Most common site being the area anterior to the oval
window (80-95 percent) -- Fissula ante fenestrum (anterior to
stape...
 Conductive hearing impairment ranging from 5 to
60 dB (stapes involvement).
 RECENT CONCEPT : Conductive hearing
impair...
SCHWARTZE'S SIGN
• The middle ear mucosa over
an otosclerotic focus often
shows a fibro vascular
proliferative response wi...
 Complete obstruction of the
round window membrane
means that stapes surgery in
such an ear will not be
successful.
 Rou...
The cytokines released by the remodeling bone within an
otosclerotic focus
that has reached the ligament
could diffuse int...
 When endosteum of cochlea involved there is
'hyalinization' of the spiral ligament.
PATHOLOGY OF SENSORINEURAL
HEARING I...
Liberation of toxic metabolites
into fluid of inner ear
Vascular compromise and
hypoxemia of strucure of
middle ear
Altera...
 Unsteadiness or dizziness or recurrent attacks of vertigo
 10-30%
 The incidence of vestibular symptoms in such patien...
 GENETIC
 The small histologic foci are ten-fold more common
than the larger lesions that result in clinical
manifestati...
 Type 1 osteogenesis imperfecta shares both clinical and
histologic similarities with otosclerosis.
 Approximately half ...
 Otosclerosis may be related to a persistent viral
infection of bone
 Ultrastructural and immunohistochemical evidence o...
 Otosclerosis represents a form of autoimmune
disease with humoral autoimmunity to type II
collagen.
 Elevated circulati...
 Otosclerosis occurs as a result of reactivation of
the arrested secondary remodelling process within
the cartilaginous r...
EPIDEMIOLOGY
Race incidence of microscopic otosclerosis
Caucasian 10%
Asian 5%
African American 1%
Native American 0%
(M:F=1:2.5)
Women more commonly seek medical
attention for hearing loss secondary to
otosclerosis
histologic studies pr...
The incidence of otosclerosis increases with
age.
The most common age group presenting with
hearing loss from otoscleros...
OTOMICROSCOPY
TM appears normal in the majority of
patients
Schwartze sign (flamingo flush) is observed in
10% of patie...
Rinnes –
negative
Webers –
lateralized to
more
affected ear
ABC--
normal
Stapedial
otosclerosis
Rinnes –
positive
Webers –...
 Early stage: a decrease in air conduction in the low
frequency, especially below 1000 Hz.
 As the disease progresses, t...
CARHART’S NOTCH
• Hallmark audiologic
sign of otosclerosis
• Decrease in bone
conduction thresholds
5 dB at 500 Hz
10 dB a...
 Mechanical artifact
 Reverses with stapes mobilization
PROPOSED THEORY OF CARHART’S
NOTCH
Stapes fixation disrupts the ...
 The reason why CARHART EFFECT occurs it that when the
skull is vibrated by bone- conduction sound, the sound is
detected...
 CT can characterize the extent
of the otosclerotic focus at the
oval window .
 CT scan can determine
capsular involveme...
 Ossicular discontinuity
• conductive loss of 60 db usually without sensorineural
component
• flaccid tympanic membrane o...
 Congenital stapes fixation
• Family history less likely (10%)
• usually detected in the first decade of life
• 25% incid...
 Paget’s disease
• - diffuse involvement of the bony skeleton
• - elevated alkaline phosphatase
• - CT - diffuse, bilater...
ADITYA GHOSH ROY
PGT-2 M.S. E.N.T.
N.R.S.M.C.H.
PARRT 2
 1704 – Valsalva first described stapes fixation
 1857 – Toynbee linked stapes fixation to
hearing loss
 1890 – Katz wa...
1912 – Siebenmann proposed a change of
nomenclature from otosclerosis to otospongiosis
1950– Raymond Thomas Carhart origin...
 1878– Kessel—first successful stapes surgery
 1890– Miot reported a series of 200 stapes
mobilization surgery
 1900 Po...
 1924– Sourdille
 Tympanolabyrithopexy
 Two stage procedure
 Covering fistula in HSC with skin of EAC
 1941– Lempert
...
 1953– Rosen
 first suggested mobilization of the stapes
 Immediate improved hearing
 1956– Shea
 first to perform st...
 1960– Schuknecht
 Stainless steel wire prosthesis
 Gelform to seal window
 Hearing loss
 Paracusis willisii
 Tinnitus
 Vertigo
 speech
SYMPTOMS
Cartilage persists throughout life in various
region of OTIC CAPSULE
Fissula ante fenestram
Fossula post fenestram
Int...
Otic capsule
enchondral
periosteal
endosteal
ENDOSTEAL
PERIOSTEAL
ENCHONDRAL
Globuli
interossei
 These contain area of
 Cartilage cell remains + calcified cartilaginous matrix
 Calcified area – capillary bud
 Osteo...
 Early stage– low frequency conductive hearing loss
 High frequency unaffected
 AUDIOGRAM—RISING AUDIOGRAM / STIFFNESS ...
 IN COCHLEAR OTOSCLEROSIS
Air conduction worsen
Mixed or SNHL
High frequency more affected
Greatest degree hearing lo...
 Carharts notch
 Hallmark audiologic sign of otosclerosis
 Decrease in bone conduction thresholds
 5 dB at 500 Hz
 10...
 BING TEST
 Meatus occluded or pressure varied
 No shift of loudness
 Bone conduction always abnormal
 Surgery improv...
 Tympanogram—normal pressure with normal volume
 Static compliance
 low compliance
 Less than .2– footplate thick or o...
 Earliest evidence of otosclerosis
 Diphasic pattern
 Increase in compliance at onset and termination of
stimuli (probe...
 Ant footplate fixed
 Elasticity—posterior footplate
 Move independently
 Onset compliance change
 Elasticity returns...
 Stapes progressively fixed
 Ipsilateral and
contralateral affected
 CONTRACTION OF TENSOR TYMPANI TESTED SEPARATELY
 STIMULATION OF TRIGEMINAL NERVE AND DOING AUDIOMETRY
 STARTLE TYPE RE...
 If speech discrimination score (SDS) score is poor
 SNHL component to hearing loss
 Prognosis poor following surgery
...
 Transient evoked otoacoustic emission (TEOAE) have
low amplitude
 OAE less role in otosclerosis
 Lack specificity
 Ea...
ADITYA GHOSH ROY
PGT-2 M.S. E.N.T.
N.R.S.M.C.H.
COCHLEAR
OTOSCLEROSIS
 Is a term generally reserved for the occurrence
of pure sensorineural hearing impairment due to
otosclerosis in an ear w...
 Dominant family history
 Female>male
 Hearing loss started or increased during pregnancy
or following use of ocp
 Sch...
 PTA– Cookie bite
 Type 2 Tympanogram
 SDS 80 to 90 %
 SISI – high
 Stapedial reflex – present
HEARING TESTS
 In pure cochlear otosclerosis tinnitus is usually the presenting
symptom
 Endolymphatic hydrops seen as a complication ...
 1926 SHAMBAUGH ( 3 CRITERIA )
 1966 SHAMBAUGH (6 CRITERIA ) – COCHLEAR
OTOSCLEROSIS
 1981 BEALES (8 CRITERIA ) COCHLEA...
1. insidious onset + early adulthood
2. absence of other cause that may lead to hearing loss
3. conductive hearing loss in...
1. + schwartzes sign
2. Family history of otosclerosis
3. SNHL in both ear
4. Flat rising , cookie bite ; good SDS for SNH...
1. SNHL with good SDS
2. RECRUITMENt + ; high SISI ; BEKESY TYPE 2
3. progression of SNHL
4. b/l symmetrical SNHL
5. unusu...
 1. Criteria of presumption
SNHL from childhood increase during puberty +
family history of SNHL
SNHL in female increas...
 Criteria of certainity
 DIPHASIC impedance in case of SNHL
 ABG in case of SNHL and absence of stapedial reflex
 CT s...
MECHANISM OF SNHL IN COCHLEAR
OTOSCLEROSIS
 When endosteum of cochlea involved there is
'hyalinization' of the spiral ligament.
PATHOLOGY OF SENSORINEURAL
HEARING I...
MEDICAL
TREATMENT OF
OTOSCLEROSIS
 SODIUM FLOURIDE
 BIPHOSPHONATES
 CYTOKINE INHIBITORS
DRUGS USED
 MECHANISM OF ACTION
 Reduce bone resorption + increase bone formation
 Antienzymatic action – proteolytic enzymes cyto...
 ACID PHENYLPHOSPHATASE
 ENZYME OF BONE RESORPTION
INC IN OTOSCLEROSIS
 THERAPY OF FLUORINE
 ENZYME DECLINE
 OPTIMAL ...
 Stapedial otosclerosis + SNHL disproportionate to age
 Cochlear otosclerosis + f/h of otosclerosis +early age of
onset ...
 Chronic nephritis with nitrogen retention – toxic build up
 Rheumatoid arthritis – inc joint pain
 Pregnant and lactat...
 Early fluorosis in spine
 Hydroxyfluoric acid in stomach
Gastric disturbance
Prevented by enteric coating
 Chronic art...
 MECHANISM OF ACTION
 Antienzymatic action
 Reduces osteoclastic activity
 Stablise secondary bone formation
 ETIDRON...
 Reduce resorption of bone
 IL 1 ANTAGONIST AND TNF BINDING PROTEIN
 HALT BONE RESORPTION
 Effective only in active ph...
 Patient not fit for surgery
 Only hearing ear
 Inadequate hearing reserve / poor SDS
 Congenital fixation of stapes
...
 Unsuccessful stapes surgery in other ear
 Otosclerosis + menieres
 Stapedectomy done in advanced otosclerosis
ADITYA GHOSH ROY
PGT-2 M.S. E.N.T.
N.R.S.M.C.H.
PARRT 3
SURGICAL
TREATMENT OF
OTOSCLEROSIS
 GOOD HEALTH WITH A SOCIALLY ACCEPTABLE ABG,
 A NEGATIVE RINNE TEST,
 EXCELLENT DISCRIMINATION(>70%)
 THE DESIRE FOR S...
Absolute contraindication
Only hearing ear
Relative contra indication
Active middle ear infection
Hydrops and tinnitu...
 Procedure detail
 Risks
Failure
 CHL
 SNHL
Vestibular disturbances
TM perforation
FN injury
Perilymph fistula
C...
 Shape and mobility of
 Incus
 Malleus
 Presence of otosclerosis
 Fixation of stapes
 Patency round window
 Facial ...
 Less trauma to the oval window
 Less possibility of damaging to the inner ear
 Less complication
 Better results
 In...
 Results probably are the best
 Easy to perform
 More traumatic to the inner ear
 Increased post-op vestibular symptom...
 General anesthesia
 Local anesthesia
 2-3 cc of 1% lidocaine with
1:50,000 or 1:100,000
epinephrine
 4 quadrants
 Bo...
Permeatal
(Transcanal)
Endaural
 6 and 12 o’clock positions
 6-8 mm lateral to the annulus
 Annulus subluxated from groove
and middle ear cavity entered
 Bony annulus curetted
Stapedius tendon
Pyramidal eminence
Long process incus
 Chorda tympani nerve encountered
 Sep...
 All ossice inspected
 Oval window
 Facial nerve
 Stapedial artery
 Round window
 Mobility of ossicle
MIDDLE EAR CAV...
 Division of stapedial tendon
 Divided near pyramidal
eminence
 Incudostapedial joint
divided usually by right
angled p...
 Control hole made
 Stapes fractured towards
promontary
 Causse crurotomy scissors
 Too much force– floating
footplate
 Mucosa over footplate excoriated
 Prevent perilymph fistula formation
 Measurement
 Medial aspect of the long process...
 Fenestra in post 1/3 to prevent
damage to the saccule and
utricle
 Aspirator not to be used to avoid
aspiration of peri...
 Prosthesis types
 Robinson bucket handle
prosthesis
 Causse prosthesis
 Fisch/Mc Gee piston
prosthesis
 House wire p...
0.7mm diamond burr
Motion of the burr removes
bone dust
Avoids smoke production
Avoids surrounding heat
production
DRI...
Carbon dioxide (CO2)
10,000 nm
Not in visible light range
Adv
Near ideal absorption
Penetration low
Disadv
Surgica...
Argon and Potassium titanyl phosphate
(KTP/532)
Wave length 500 nm
Visible light
Absorbed by hemoglobin
Adv
Hand hel...
 Posteriorly
placed
fenestration
with the laser
 Causse also
recommends
following the
laser with the
diamond burr
to rem...
 Vaporization of anterior crus
and mobilization of posterior
part of footplate
 Preservation of the stapedius
tendon
 R...
COMPLICAIONS
OF
OTOSCLEROSIS
 Tear in tympanomeatal flap
 Subluxation of incus
 Overhanging FN
 Obliterative otosclerosis
 Otosclerosis involving ...
 Proceed & then repair
 Tragal perichondrium
 Fascia
 Gelform
TEAR IN TYMPANOMEATAL FLAP
Curettage around bony annulus
Separation of incudostapedial joint
Manipulation around oval window
Treatment
Subluxati...
OVERHANGING FN
• Usually dehiscent
• If prolapsed nerve abrupts the
promontary inferor to oval window
– surgery to be abor...
 Laser not sufficient to remove bone
 Small fenestra to be made
 Drill out the excess bone
 Blue lined vestibule
 Fen...
 Per op finding
 Leave it as it is
 Complete procedure and note
it as a finding
OTOSCLEROSIS INVOLVING ROUND
WINDOW NIC...
 Pulsatile tinnitus CHL SNHL
 Bleeding during operation
 Fenestration to be made in post half
PERSISTENT STAPEDIAL ARTE...
 Pre op diagnosis
 Reduced movement of manubrium
 Palpation of malleus
 Laser Doppler vibrometry
 Small AB gap
 Non ...
 fundal defect of IAM – prilymph gusher
 widened cochlear aqueduct – perilymph oozer
 Ct scan
 Treatment
 Elevation o...
 May be avoided if control holes are used
or by using laser fenestration
 Laser is used
 Assess movement of suprastruct...
 Perilymph fistula
 Facial palsy
 Chorda tympani dysfunction
 Otitis media
 Reparative granuloma
 SNHL
 CHL
POST OP...
• PRIMARY & SECONDARY
Prevention:
• Stapedectomy < stapedotomy
• Oval window seal
• No fat or gel-foam for seal
• Prohibit...
DIAGNOSIS:
 Fluctuation hearing Loss
 Vertigo & tinnitus
 Fullness of ear
 Audiometry– SNHL
 ENG– directional fixed p...
 Immediate or Delayed
 <3 hrs due to anesthesia
 >3 hrs due to operative procedure
ointegrity of nerve – steroids
ointe...
 Sacrificing the nerve better than stretching it
 Injury leads to hypogeusia and dysgeusia
 Stretching leads to metalli...
 Immediate post op period
 Worrisome
 Serous labyrinthitis meningitis
 Treatment
 Removal of pack
 Admission
 Broad...
 Granulation tissue formation around a stapes prosthesis and
the oval window which may extend into the vestibule.
 1-5%
...
 0.2-10%
 Serous labyrinthitis - high frequencies
 Surgical trauma
 Movement of stapes
 Rupture of membranes of inner...
 IMMEDIATE CHL
 Prosthesis malfunction
 Unrecognized malleus fixation
 Unrecognized round window otosclerosis
 Middle...
Otosclerosis
Otosclerosis
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Otosclerosis

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otosclerosis

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Otosclerosis

  1. 1. ADITYA GHOSH ROY PGT-2 M.S. E.N.T. N.R.S.M.C.H. OTOSCLEROSIS
  2. 2. ADITYA GHOSH ROY PGT-2 M.S. E.N.T. N.R.S.M.C.H. PARRT 1
  3. 3.  Otosclerosis is a localized hereditary disorder affecting endochondral bone of the otic capsule that is characterized by disordered resorption and deposition of bone.  An otosclerotic lesion consists of areas of bone resorption, new bone formation, vascular proliferation and a connective tissue stroma. DEFINITION
  4. 4.  'CLINICAL' OTOSCLEROSIS refers to a lesion that involves the stapes bone or stapediovestibular joint and consequently is clinically manifested by a conductive hearing impairment.  'HISTOLOGIC' OTOSCLEROSIS refers to a lesion that does not involve the stapes bone, stapediovestibular joint or cochlear endosteum, is consequently asymptomatic, and can be diagnosed only by post-mortem examination of the temporal bone.  'COCHLEAR' OTOSCLEROSIS is a term generally reserved for the occurrence of pure sensorineural hearing impairment due to otosclerosis in an ear without any conductive component to the hearing impairment. (involvement of the cochlear endosteum but without any stapes fixation.) TYPES
  5. 5.  The bone of the otic capsule is unique that it exhibits very little remodeling .  It contains small regions of immature cartilaginous tissue called globuli interossei.  Otosclerotic focus easily diagnosed due to bone remodelling. PATHOLOGY
  6. 6. BLUE MANTLES OF MANASSEH 'BLUE MANTLE'. BLUE MANTLES ARE AREAS OF THE OTIC CAPSULE THAT STAIN MORE BASOPHILIC THAN NORMAL
  7. 7. OTOSCLEROTIC FOCUS CONSISTS OF BONE RESORPTION NEW BONE FORMATION VASCULAR PROLIFERATION CONNECTIVE TISSUE STROMA
  8. 8.  RESORPTION OF ENCHONDRAL BONE  ENLARGEMENT OF PERIVASCULAR SPACES  DEPOSITION OF IMMATURE BONE  ACTIVE RESORPTION + REMODELLING MATURE(LAMELLAR BONE) NEW BONE FORMATION– OSTEOBLAST RESORPTION– OSTEOCLAST CONNECTIVE TISSUE STROMA OF FIBROBLAST AND HISTOCYTES NO INFLAMMATORY CELLS SEEN
  9. 9.  An otosclerotic focus may appear as ACTIVE OR 'SPONGIOTIC', CHARACTERIZED BY  areas of increased cellularity and vascularity  bone resorption  new bone formation INACTIVE 'SCLEROTIC' FOCUS consisting of dense mineralized bone. COMMON FOR AN OTOSCLEROTIC FOCUS TO CONTAIN BOTH ACTIVE AND INACTIVE REGION
  10. 10. NON-CLINICAL FOCI OF OTOSCLEROSIS
  11. 11. ANTERIOR FOOTPLATE INVOLVEMENT
  12. 12. Bipolar involvement of the footplate
  13. 13. ORGAN OF CORTI
  14. 14.  Most common site being the area anterior to the oval window (80-95 percent) -- Fissula ante fenestrum (anterior to stapes foot plate)  Round window niche (about 30 percent)  The apical medial wall of the cochlear labyrinth (about 15 percent)  The stapes footplate (about 12 percent)  Posterior to the oval window (5-10 percent)  Otosclerosis is usually bilateral, with involvement of both ears in 70-90 percent of cases.  Foci of clinical or histologic otosclerosis can be single or multiple within the temporal bone. DISTRIBUTION OF OTOSCLEROTIC FOCUS
  15. 15.  Conductive hearing impairment ranging from 5 to 60 dB (stapes involvement).  RECENT CONCEPT : Conductive hearing impairment appeared to be caused primarily by narrowing and impairment of the annular ligament especially at the posterior stapediovestibular joint space. PATHOLOGY OF CONDUCTIVE DEAFNESS
  16. 16. SCHWARTZE'S SIGN • The middle ear mucosa over an otosclerotic focus often shows a fibro vascular proliferative response with hypertrophy, deposition of connective tissue and increased vascularity. • Red vascular blush seen at otoscopy in patients with active otosclerosis (Schwartze's sign).
  17. 17.  Complete obstruction of the round window membrane means that stapes surgery in such an ear will not be successful.  Round window closure may be diagnosed by Intraoperative examination as well as by high resolution computed tomography (CT) scan imaging. COMPLETE OBSTRUCTION OF THE ROUND WINDOW
  18. 18. The cytokines released by the remodeling bone within an otosclerotic focus that has reached the ligament could diffuse into the spiral ligament upset the normal state of cytokine control within the spiral ligament. alteration the fluid and ion hemostasis within cochlea SNHL PATHOLOGY OF SENSORINEURAL HEARING IMPAIRMENT
  19. 19.  When endosteum of cochlea involved there is 'hyalinization' of the spiral ligament. PATHOLOGY OF SENSORINEURAL HEARING IMPAIRMENT
  20. 20. Liberation of toxic metabolites into fluid of inner ear Vascular compromise and hypoxemia of strucure of middle ear Alteration of the fluid and ion hemostasis within cochlea due to spiral ligament involvement
  21. 21.  Unsteadiness or dizziness or recurrent attacks of vertigo  10-30%  The incidence of vestibular symptoms in such patients seems to be correlated with the degree of sensorineural hearing impairment.  Scarpa's ganglion cell counts were significantly lower in patients who had vestibular symptoms.  Damage to the scrapa ganglion due to toxic substances liberated from otosclerotic bone  Vertigo could be produced a result of otosclerotic focus coming in contact with perilymph. PATHOLOGY OF VESTIBULAR SYMPTOMS
  22. 22.  GENETIC  The small histologic foci are ten-fold more common than the larger lesions that result in clinical manifestations  F:M = 2:1, Whites commonly affected  Age of onset. Deafness usually starts between 20 and 30 years of age and is rare before 10 and after 40 years.  AD transmission with incomplete penetration.  Sporadic > Familial AETIOLOGY
  23. 23.  Type 1 osteogenesis imperfecta shares both clinical and histologic similarities with otosclerosis.  Approximately half of all patients with type 1 osteogenesis imperfecta develop hearing loss that is clinically indistinguishable from clinical otosclerosis.  Some patients with clinical otosclerosis have blue sclera, a feature that is found in virtually all patients with type 1 osteogenesis imperfecta DEFECTS IN EXPRESSION OF THE COL1A1 GENE
  24. 24.  Otosclerosis may be related to a persistent viral infection of bone  Ultrastructural and immunohistochemical evidence of measles like structure and antigenicity in active otosclerotic lesion  Measles RNA has been found in archival and fresh footplate specimens with otosclerosis.  Elevated levels of anti-measles antibody has also been reported in perilymph from patients undergoing stapedectomy for otosclerosis as compared to controls.  Low levels of anti measles antibody in patients with otosclerosis. MEASLES ASSOCIATION
  25. 25.  Otosclerosis represents a form of autoimmune disease with humoral autoimmunity to type II collagen.  Elevated circulating antibodies to type II collagen in the blood of some patients with otosclerosis has also been reported.  Immunohistochemical analysis has shown tissue bound igG in active areas AUTOIMMUNE DISEASE
  26. 26.  Otosclerosis occurs as a result of reactivation of the arrested secondary remodelling process within the cartilaginous rest areas of the otic capsule . BIOCHEMISTRY
  27. 27. EPIDEMIOLOGY Race incidence of microscopic otosclerosis Caucasian 10% Asian 5% African American 1% Native American 0%
  28. 28. (M:F=1:2.5) Women more commonly seek medical attention for hearing loss secondary to otosclerosis histologic studies prevalence of otosclerosis show no difference in men versus women SEX VARIATION
  29. 29. The incidence of otosclerosis increases with age. The most common age group presenting with hearing loss from otosclerosis is 15-45 years however it has been reported to manifest as early as 7 years and as late as the mid 50s. AGE
  30. 30. OTOMICROSCOPY TM appears normal in the majority of patients Schwartze sign (flamingo flush) is observed in 10% of patients). Most helpful in ruling out other disorders Middle ear effusions Tympanosclerosis Tympanic membrane perforations Cholesteatoma or retraction pockets PHYSICAL EXAMINATION
  31. 31. Rinnes – negative Webers – lateralized to more affected ear ABC-- normal Stapedial otosclerosis Rinnes – positive Webers – lateralized to better ear ABC-- reduced Cochlear otosclerosis TUNNING FORK TESTS
  32. 32.  Early stage: a decrease in air conduction in the low frequency, especially below 1000 Hz.  As the disease progresses, the air line flattens. because the otosclerotic focus has a mass affect on the entire system, carhart notch is noted. PURE TONE AUDIOMETRY
  33. 33. CARHART’S NOTCH • Hallmark audiologic sign of otosclerosis • Decrease in bone conduction thresholds 5 dB at 500 Hz 10 dB at 1000 Hz 15 dB at 2000 Hz 5 dB at 4000 Hz
  34. 34.  Mechanical artifact  Reverses with stapes mobilization PROPOSED THEORY OF CARHART’S NOTCH Stapes fixation disrupts the normal ossicular resonance (2000 Hz) Normal compressional mode of bone conduction is disturbed because of relative perilymph immobility
  35. 35.  The reason why CARHART EFFECT occurs it that when the skull is vibrated by bone- conduction sound, the sound is detected by the cochlea via three routes Route  (a) is by direct vibration within the skull, route  (b) is by vibration of the ossicular chain which is suspended. within the skull .  (c) is by vibrations emanating into the external auditory canal as sound and being heard by the normal air- conduction route.  Regained by successful reconstruction surgery . The reason that there is a Carhart notch at 2 kHz before the surgery is that the Carhart effect is greatest around that frequency
  36. 36.  CT can characterize the extent of the otosclerotic focus at the oval window .  CT scan can determine capsular involvement (radiolucent) when patients have significant mixed hearing loss  An enlarged cochlear aqueduct may be seen which potential causes perilymph gusher during footplate fenestration or removal.  It reveal normal round window and normal mastoid pneumatization.
  37. 37.  Ossicular discontinuity • conductive loss of 60 db usually without sensorineural component • flaccid tympanic membrane on pneumatic otoscopy • type Ad tympanogram  Malleus head fixation • when congenital, associated with other stigmata (aural atresia) • presence of tympanosclerosis • almost always associated with type As tympanogram (only in advanced otosclerosis) DIFFERENTIAL DIAGNOSIS
  38. 38.  Congenital stapes fixation • Family history less likely (10%) • usually detected in the first decade of life • 25% incidence of other congenital anomalies (3% for juvenile otosclerosis) • non-progressive CHL  Osteogenesis imperfecta • presence of blue sclera • h/o of multiple bone fractures • CT – more common involves the otic capsule
  39. 39.  Paget’s disease • - diffuse involvement of the bony skeleton • - elevated alkaline phosphatase • - CT - diffuse, bilateral, petrous bone involvement with extensive de-mineralization • - More commonly crowds the ossicles in the epitympanum, partially fixing
  40. 40. ADITYA GHOSH ROY PGT-2 M.S. E.N.T. N.R.S.M.C.H. PARRT 2
  41. 41.  1704 – Valsalva first described stapes fixation  1857 – Toynbee linked stapes fixation to hearing loss  1890 – Katz was first to find microscopic evidence of otosclerosis  1893 – Politzer described the clinical entity of “otosclerosis” HISTORY OF OTOSCLEROSIS
  42. 42. 1912 – Siebenmann proposed a change of nomenclature from otosclerosis to otospongiosis 1950– Raymond Thomas Carhart originated the term air bone gap . Reported notching in bone conduction in cases of stapedial otosclerosis
  43. 43.  1878– Kessel—first successful stapes surgery  1890– Miot reported a series of 200 stapes mobilization surgery  1900 Politzer and Siebenmann condemned stapes surgery because of potential risk to cause meningitis  1916-- Gunnar Holmgren  Father of fenestration surgery  Single stage technique  Not successful in maintaining an open fenestra HISTORY OF STAPES SURGERY
  44. 44.  1924– Sourdille  Tympanolabyrithopexy  Two stage procedure  Covering fistula in HSC with skin of EAC  1941– Lempert  Popularized the single staged fenestration procedure  Extraction of incus – no reduction in hearing  Extraction of incus – more space to create a wider fenestra
  45. 45.  1953– Rosen  first suggested mobilization of the stapes  Immediate improved hearing  1956– Shea  first to perform stapedectomy  Used operating microscope  Sealed the oval window  Homograft bone graft between oval window and incus  Immediate hearing gain  Over time– hearing loss due to adhesion  1960 Shea used teflon piston– STAPES SURGERY WAS BORN
  46. 46.  1960– Schuknecht  Stainless steel wire prosthesis  Gelform to seal window
  47. 47.  Hearing loss  Paracusis willisii  Tinnitus  Vertigo  speech SYMPTOMS
  48. 48. Cartilage persists throughout life in various region of OTIC CAPSULE Fissula ante fenestram Fossula post fenestram Intracochlear area(enchondral layer) Cochlear area (round window) Semicircular canal Petrosquamous suture Base of styloid process ANATOMY OF OTIC CAPSULE
  49. 49. Otic capsule enchondral periosteal endosteal ENDOSTEAL PERIOSTEAL ENCHONDRAL Globuli interossei
  50. 50.  These contain area of  Cartilage cell remains + calcified cartilaginous matrix  Calcified area – capillary bud  Osteoblast  Deposit bone in lacunae  Small bony globules or globuli ossei  Globuli interossei (region of immature cartilage)  Loci of earliest otosclerosis
  51. 51.  Early stage– low frequency conductive hearing loss  High frequency unaffected  AUDIOGRAM—RISING AUDIOGRAM / STIFFNESS TILT  Otosclerotic focus proliferates  Mass effect added to audiogram  low frequency conductive hearing loss- stabilizes  High frequency loss occurs  Flat pattern on AUDIOGRAM AUDIOLOGICAL EVALUATION OF THE PATIENT WITH OTOSCLEROSIS
  52. 52.  IN COCHLEAR OTOSCLEROSIS Air conduction worsen Mixed or SNHL High frequency more affected Greatest degree hearing loss in mid frequency Cookie bite pattern
  53. 53.  Carharts notch  Hallmark audiologic sign of otosclerosis  Decrease in bone conduction thresholds  5 dB at 500 Hz  10 dB at 1000 Hz  15 dB at 2000 Hz  5 dB at 4000 Hz BONE CONDUCTION
  54. 54.  BING TEST  Meatus occluded or pressure varied  No shift of loudness  Bone conduction always abnormal  Surgery improves bone conduction and carharts notch disappears following surgery  Animal experiments– bone conduction poorer
  55. 55.  Tympanogram—normal pressure with normal volume  Static compliance  low compliance  Less than .2– footplate thick or obliterative otosclerosis  More than .6– footplate is thin  Acoustic reflex IMPEDANCE AUDIOMETRY
  56. 56.  Earliest evidence of otosclerosis  Diphasic pattern  Increase in compliance at onset and termination of stimuli (probe in affected ear) ACOUSTIC REFLEX
  57. 57.  Ant footplate fixed  Elasticity—posterior footplate  Move independently  Onset compliance change  Elasticity returns to normal  Remains till pull of stapedius is relaxed  Offset compliance change DIPHASIC PATTERN
  58. 58.  Stapes progressively fixed  Ipsilateral and contralateral affected
  59. 59.  CONTRACTION OF TENSOR TYMPANI TESTED SEPARATELY  STIMULATION OF TRIGEMINAL NERVE AND DOING AUDIOMETRY  STARTLE TYPE REFLEX  FATIGUEBLE IN NATURE  UNSTABLE  LONG LATENCY PERIOD NON ACOUSTIC REFLEX
  60. 60.  If speech discrimination score (SDS) score is poor  SNHL component to hearing loss  Prognosis poor following surgery  Pt. benefit more by hearing aid SPEECH AUDIOMETRY
  61. 61.  Transient evoked otoacoustic emission (TEOAE) have low amplitude  OAE less role in otosclerosis  Lack specificity  Early identification of cochlear otosclerosis OTOACOUSTIC EMISSION
  62. 62. ADITYA GHOSH ROY PGT-2 M.S. E.N.T. N.R.S.M.C.H. COCHLEAR OTOSCLEROSIS
  63. 63.  Is a term generally reserved for the occurrence of pure sensorineural hearing impairment due to otosclerosis in an ear without any conductive component to the hearing impairment. (involvement of the cochlear endosteum but without any stapes fixation.)
  64. 64.  Dominant family history  Female>male  Hearing loss started or increased during pregnancy or following use of ocp  Schwartzes sign positive  SNHL  Tinnitus vertigo SIGNS SYMPTOMS
  65. 65.  PTA– Cookie bite  Type 2 Tympanogram  SDS 80 to 90 %  SISI – high  Stapedial reflex – present HEARING TESTS
  66. 66.  In pure cochlear otosclerosis tinnitus is usually the presenting symptom  Endolymphatic hydrops seen as a complication of cochlear otosclerosis  BPPV is also commonly seen
  67. 67.  1926 SHAMBAUGH ( 3 CRITERIA )  1966 SHAMBAUGH (6 CRITERIA ) – COCHLEAR OTOSCLEROSIS  1981 BEALES (8 CRITERIA ) COCHLEAR OTOSCLEROSIS  1975 CAUSSE ET AL 3 TYPES OF CRITERIA DIAGNOSIS OF COCHLEAR OTOSCLEROSIS
  68. 68. 1. insidious onset + early adulthood 2. absence of other cause that may lead to hearing loss 3. conductive hearing loss in family member 1926 SHAMBAUGH ( 3 CRITERIA )
  69. 69. 1. + schwartzes sign 2. Family history of otosclerosis 3. SNHL in both ear 4. Flat rising , cookie bite ; good SDS for SNHL 5. SNHL early in life and other cause cannot be found out 6. fixation of stapes with SNHL 1966 SHAMBAUGH (6 CRITERIA ) – COCHLEAR OTOSCLEROSIS
  70. 70. 1. SNHL with good SDS 2. RECRUITMENt + ; high SISI ; BEKESY TYPE 2 3. progression of SNHL 4. b/l symmetrical SNHL 5. unusual configuration in audiogram 6. successful use of hearing aid 7. paracusis willis in early adulthood 8. negative RINNE test 1981 BEALES (8 CRITERIA ) COCHLEAR OTOSCLEROSIS
  71. 71.  1. Criteria of presumption SNHL from childhood increase during puberty + family history of SNHL SNHL in female increased by PREGNANCY ; MENSTRUATION ; MENOPAUSE ; INTAKE OF OCP SNHL + GOOD SDS  Criteria of probability + SCHWARTZES SIGN SNHL + COOKIE BITE ON AUDIOGRAM + Radiological findings 1975 CAUSSE ET AL 3 TYPES OF CRITERIA
  72. 72.  Criteria of certainity  DIPHASIC impedance in case of SNHL  ABG in case of SNHL and absence of stapedial reflex  CT scan demonstartes cochlear otosclerosis
  73. 73. MECHANISM OF SNHL IN COCHLEAR OTOSCLEROSIS
  74. 74.  When endosteum of cochlea involved there is 'hyalinization' of the spiral ligament. PATHOLOGY OF SENSORINEURAL HEARING IMPAIRMENT
  75. 75. MEDICAL TREATMENT OF OTOSCLEROSIS
  76. 76.  SODIUM FLOURIDE  BIPHOSPHONATES  CYTOKINE INHIBITORS DRUGS USED
  77. 77.  MECHANISM OF ACTION  Reduce bone resorption + increase bone formation  Antienzymatic action – proteolytic enzymes cytotoxic to cochlea  NaF acts only on active focus  Reduces osteoclastic when focus is active  Inc osteoblastic activity  HYDROXYAPATITE ---------------- FLUORAPATITE SODIUM FLOURIDE • HARDER • BETTER QUALITY • RESISTANT TO BONE RESORPTION F ION
  78. 78.  ACID PHENYLPHOSPHATASE  ENZYME OF BONE RESORPTION INC IN OTOSCLEROSIS  THERAPY OF FLUORINE  ENZYME DECLINE  OPTIMAL DOSE OF NAF– 60mg daily
  79. 79.  Stapedial otosclerosis + SNHL disproportionate to age  Cochlear otosclerosis + f/h of otosclerosis +early age of onset + audiometric pattern + good SDS  Radiological signs  + SCHWARTZES SIGN  Otosclerosis with secondary hydrops  Surgery refused by pt. And seeks an alternative form of treatment INDICATION OF NAF
  80. 80.  Chronic nephritis with nitrogen retention – toxic build up  Rheumatoid arthritis – inc joint pain  Pregnant and lactating  Children in whom skeletal growth not achieved  Skeletal fluorosis  Allergy to fluoride CONTRA INDICATION
  81. 81.  Early fluorosis in spine  Hydroxyfluoric acid in stomach Gastric disturbance Prevented by enteric coating  Chronic arthritis SIDE EFFECTS
  82. 82.  MECHANISM OF ACTION  Antienzymatic action  Reduces osteoclastic activity  Stablise secondary bone formation  ETIDRONATE– Halt progression of otosclerotic activity  Newer –  Alendronate  Residronate  zolendronate BIPHOSPHONATES
  83. 83.  Reduce resorption of bone  IL 1 ANTAGONIST AND TNF BINDING PROTEIN  HALT BONE RESORPTION  Effective only in active phase CYTOKINE INHIBITORS
  84. 84.  Patient not fit for surgery  Only hearing ear  Inadequate hearing reserve / poor SDS  Congenital fixation of stapes  Surgery not elected by patient  Mild conductive deafness HEARING AIDS
  85. 85.  Unsuccessful stapes surgery in other ear  Otosclerosis + menieres  Stapedectomy done in advanced otosclerosis
  86. 86. ADITYA GHOSH ROY PGT-2 M.S. E.N.T. N.R.S.M.C.H. PARRT 3
  87. 87. SURGICAL TREATMENT OF OTOSCLEROSIS
  88. 88.  GOOD HEALTH WITH A SOCIALLY ACCEPTABLE ABG,  A NEGATIVE RINNE TEST,  EXCELLENT DISCRIMINATION(>70%)  THE DESIRE FOR SURGERY AFTER AN APPROPRIATE PERIOD OF TIME FOR DELIBERATION.  YOUNGER PATIENTS ARE MORE LIKELY TO DEVELOP RE- OSSIFICATION OF THE STAPES FOOTPLATE OVER THEIR LIFETIME. INDICATIONS
  89. 89. Absolute contraindication Only hearing ear Relative contra indication Active middle ear infection Hydrops and tinnitus Severe atelectasis Unfit for surgery Schwartzes sign – controversy Pregnancy Boxers, wrestlers CONTRA INDICATIONS
  90. 90.  Procedure detail  Risks Failure  CHL  SNHL Vestibular disturbances TM perforation FN injury Perilymph fistula Chorda tympani injury Delayed failure CONSENT
  91. 91.  Shape and mobility of  Incus  Malleus  Presence of otosclerosis  Fixation of stapes  Patency round window  Facial neve  Chorda tympani status OPERATIVE NOTE
  92. 92.  Less trauma to the oval window  Less possibility of damaging to the inner ear  Less complication  Better results  In addition, revision surgery, if required, is easier due to preserved anatomy  Done with laser also STAPEDOTOMY
  93. 93.  Results probably are the best  Easy to perform  More traumatic to the inner ear  Increased post-op vestibular symptoms  Higher incidence of postoperative SNHL  The operation is unavoidable in:  Comminuted fracture of the footplate  Revision surgery  Floating footplate  Footplate removed accidently while removing the suprastructure STAPEDECTOMY
  94. 94.  General anesthesia  Local anesthesia  2-3 cc of 1% lidocaine with 1:50,000 or 1:100,000 epinephrine  4 quadrants  Bony cartilaginous junction PROCEDURE
  95. 95. Permeatal (Transcanal) Endaural
  96. 96.  6 and 12 o’clock positions  6-8 mm lateral to the annulus  Annulus subluxated from groove and middle ear cavity entered
  97. 97.  Bony annulus curetted Stapedius tendon Pyramidal eminence Long process incus  Chorda tympani nerve encountered  Separate the chorda from the medial surface of the malleus  Avoid stretching the nerve  Cut the nerve rather than stretch it
  98. 98.  All ossice inspected  Oval window  Facial nerve  Stapedial artery  Round window  Mobility of ossicle MIDDLE EAR CAVITY EXAMINATION
  99. 99.  Division of stapedial tendon  Divided near pyramidal eminence  Incudostapedial joint divided usually by right angled pick
  100. 100.  Control hole made  Stapes fractured towards promontary  Causse crurotomy scissors  Too much force– floating footplate
  101. 101.  Mucosa over footplate excoriated  Prevent perilymph fistula formation  Measurement  Medial aspect of the long process of the incus to the footplate  Average 4.5 mm  add .5mm  .25mm of prosthesis projects into vestibule
  102. 102.  Fenestra in post 1/3 to prevent damage to the saccule and utricle  Aspirator not to be used to avoid aspiration of perilymph  Oval window seal Tragal perichondrium Vein (hand or wrist) Temporalis fascia Blood Fat
  103. 103.  Prosthesis types  Robinson bucket handle prosthesis  Causse prosthesis  Fisch/Mc Gee piston prosthesis  House wire prosthesis  Prosthesis is chosen and length picked  Some prefer bucket handle to incorporate the lenticular process of the incus
  104. 104. 0.7mm diamond burr Motion of the burr removes bone dust Avoids smoke production Avoids surrounding heat production DRILL FENESTRATION
  105. 105. Carbon dioxide (CO2) 10,000 nm Not in visible light range Adv Near ideal absorption Penetration low Disadv Surgical beam only  Requires separate laser for an aiming beam (red helium-neon) Ill defined fuzzy beam Working distance more LASER FENESTRATION
  106. 106. Argon and Potassium titanyl phosphate (KTP/532) Wave length 500 nm Visible light Absorbed by hemoglobin Adv Hand held probe Surgical and aiming beam Disadv Char formation LASER FENESTRATION
  107. 107.  Posteriorly placed fenestration with the laser  Causse also recommends following the laser with the diamond burr to remove char
  108. 108.  Vaporization of anterior crus and mobilization of posterior part of footplate  Preservation of the stapedius tendon  Reduction in hyperacusis  Reduction in risk for long-term postoperative inner ear injuries  No prosthesis complications  Very difficult technique STAMP(STAPEDOTOMY MINUS PROSTHESIS)
  109. 109. COMPLICAIONS OF OTOSCLEROSIS
  110. 110.  Tear in tympanomeatal flap  Subluxation of incus  Overhanging FN  Obliterative otosclerosis  Otosclerosis involving round window niche  Persistent stapedial artery  Malleus ankylosis  Perilymph gusher oozer  Floating or depressed footplate INTRA OPERATIVE COMPLICATIONS
  111. 111.  Proceed & then repair  Tragal perichondrium  Fascia  Gelform TEAR IN TYMPANOMEATAL FLAP
  112. 112. Curettage around bony annulus Separation of incudostapedial joint Manipulation around oval window Treatment Subluxation– incus attachment prosthesis Disarticulation – remove incus and put malleus attachment prosthesis SUBLUXATION OF INCUS
  113. 113. OVERHANGING FN • Usually dehiscent • If prolapsed nerve abrupts the promontary inferor to oval window – surgery to be aborted • Surgery usually completed by making a small fenestra in the inferior aspect • Prosthesis is usually longer to accommodate the bend of nerve
  114. 114.  Laser not sufficient to remove bone  Small fenestra to be made  Drill out the excess bone  Blue lined vestibule  Fenestra to be made  Long prosthesis OBLITERATIVE OTOSCLEROSIS INVOLVING OVAL WINDOW
  115. 115.  Per op finding  Leave it as it is  Complete procedure and note it as a finding OTOSCLEROSIS INVOLVING ROUND WINDOW NICHE
  116. 116.  Pulsatile tinnitus CHL SNHL  Bleeding during operation  Fenestration to be made in post half PERSISTENT STAPEDIAL ARTERY
  117. 117.  Pre op diagnosis  Reduced movement of manubrium  Palpation of malleus  Laser Doppler vibrometry  Small AB gap  Non acoustic reflex -- faint  Myringosclerosis  Removal of malleus head and reconstruction with malleus attachment prosthesis MALLEUS ANKYLOSIS
  118. 118.  fundal defect of IAM – prilymph gusher  widened cochlear aqueduct – perilymph oozer  Ct scan  Treatment  Elevation oh head  Introduce spinal catheter and proceed  Small fenestra stapedotomy  Tissue seal over fenestra  Complete control required as may cause post op complications  Avoid cork bottle effect PERILYMPH GUSHER OOZER
  119. 119.  May be avoided if control holes are used or by using laser fenestration  Laser is used  Assess movement of suprastructure before disarticulation  Treatment  Small hole inferior to annular ligament  Elevation by small hook  Opening sealed with tissue graft  Appropriate sized prosthesis put FLOATING OR DEPRESSED FOOTPLATE
  120. 120.  Perilymph fistula  Facial palsy  Chorda tympani dysfunction  Otitis media  Reparative granuloma  SNHL  CHL POST OPERATIVE COMPLICATIONS
  121. 121. • PRIMARY & SECONDARY Prevention: • Stapedectomy < stapedotomy • Oval window seal • No fat or gel-foam for seal • Prohibit nose blowing, flying, diving, & lifting heavy objects postoperatively PERILYMPH FISTULA
  122. 122. DIAGNOSIS:  Fluctuation hearing Loss  Vertigo & tinnitus  Fullness of ear  Audiometry– SNHL  ENG– directional fixed positional nystagmus  Fistula test  radiology—presence of air bubble in vestibule at prosthesis end TREATMENT:  Surgical closure
  123. 123.  Immediate or Delayed  <3 hrs due to anesthesia  >3 hrs due to operative procedure ointegrity of nerve – steroids ointegrity of nerve not sure– exploration odelayed facial nerve palsy -- rare FACIAL PALSY
  124. 124.  Sacrificing the nerve better than stretching it  Injury leads to hypogeusia and dysgeusia  Stretching leads to metallic taste, altered taste to various food, altered taste CHORDA TYMPANI INJURY
  125. 125.  Immediate post op period  Worrisome  Serous labyrinthitis meningitis  Treatment  Removal of pack  Admission  Broad spectrum anti biotic ACUTE OTITIS MEDIA
  126. 126.  Granulation tissue formation around a stapes prosthesis and the oval window which may extend into the vestibule.  1-5%  Gradual deterioration 5-15 days postoperativly  Vertigo, tinnitus, nystagmus towards non op side and deafness  Otoscopy: reddish discoloration of the postero-superior TM  Mixed hearing loss reduced SDS  Many surgeons would now advocate a more conservative policy of steroids and antibiotics initially and some would consider delayed surgery if no improvement occurred. REPARATIVE GRANULOMA
  127. 127.  0.2-10%  Serous labyrinthitis - high frequencies  Surgical trauma  Movement of stapes  Rupture of membranes of inner ear  Rapid loss of perilymph  hydrops SNHL
  128. 128.  IMMEDIATE CHL  Prosthesis malfunction  Unrecognized malleus fixation  Unrecognized round window otosclerosis  Middle ear effusion  Unrecognized SSCD  RECURRENCE OF CHL  Prosthesis malfunction  Incus erosion  Otosclerosis regrowth  round window otosclerosis PERSISTENCE OR RECURRENCE OF CHL

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