The document details the historical development, pathology, and surgical management of otosclerosis, a condition involving abnormal bone growth in the ear that leads to hearing loss. It outlines the evolution of stapes surgery from early techniques to modern procedures such as stapedectomy, alongside diagnostic criteria, audiological evaluations, and medical therapies. Additionally, it discusses complications, contraindications, and future considerations for patients seeking treatment for this condition.

1. OTOSCLEROSIS
POONGKAMALI J
MS ENT-HNS, PG 1 YR
AIIMS, BHOPAL.

2. GOING BACK IN DATES
• ANTONIO MARIA VALSALVA-Professor of Anatomy in
Bologna: first described stapes fixation as a cause of
hearing loss in 1704.
• TOYNBEE: 1841 firmly established the link between
osseous ankylosis of the stapes as a common causes of
deafness.”
• ADAM POLITZER: in 1893 described the pathology as
OTOSCLEROSIS.
• SIEBENMANN: in 1912 proposed a change in
nomenclature from otosclerosis to otospongiosis.

3. EVOLUTION OF STAPES SURGERY
• THE PRE-ANTIBIOTIC ERA
JOHANNES KESSEL - first to describe stapes surgery in 1876.
THE FENESTRATION ERA
 HOLMGREN - three-staged “closed” fenestration operation on the
lateral semicircular canal.
SOURDILLE- three-stage “open” operation.
LEMPERT- “single-stage” endaural fenestration operation.
• THE MOBILIZATION ERA
SAMUEL ROSEN- first to describe stapes mobilization in 1953
The shortcoming of the mobilization procedure was that many
patients would refixate shortly after the operation
• THE MODERN STAPEDECTOMY ERA
JOHN SHEA- first described the stapedectomy procedure
Used vein graft followed by Teflon prosthesis in 1956
SCHUKNECHT - developed a steel wire-adipose tissue prosthesis

4. DEFINITION
• Primary disease of bony labyrinth in which one or more foci of irregularly laid spongy bone
replace part of normally dense enchondral layer of bony otic capsule.
• More aptly called as otospongiosis.

5. CLASSIFICATION
• CLINICAL OTOSCLEROSIS:
1. STAPEDIAL OTOSCLEROSIS- refers to lesions that affect the stapes, stapediovestibular
joint or round window membrane and thus cause conductive hearing loss.

6. 2. COCHLEAR OTOSCLEROSIS- refers to lesions involving the cochlear endosteum without
affecting the stapes or the stapediovestibular joint, thus causing pure sensorineural
hearing loss, with no conductive element
• HISTOLOGIC OTOSCLEROSIS: refers to histopathological lesions that do not affect the
stapes, stapediovestibular joint or cochlear endosteum, and thus remain asymptomatic
during life.

7. SITES OF INVOLVEMENT
• The most common location of involvement of otosclerosis is the bone just anterior to the
oval window at a small cleft known as the fissula ante fenestram. – 80-90% (Guild 1994)
• Round window –30 to 50% (Guild 1944; Nylen 1949).
• Other sites -apical medial wall of the cochlea, posterior to the oval window, the posterior
internal auditory canal, the cochlea aqueduct, and the semicircular canals.

8. FISSULA ANTE FENESTRUM

9. HISTOLOGIC PHASES OF THE DISEASE
TWO PHASES:
1. Active(Otospongiosis phase)
 areas of increased cellularity and vascularity
Areas of bone resorption and new bone formation
Positive Schwartze sign
Blue mantles of manasseh
2. Inactive ( Sclerotic Focus)
Dense sclerotic bone

10. ETIOLOGY
• Genetic predisposistion:OTSC1/2/3, COLA1, TGFB1,SERPINF1
• Viral infection: measles virus
• Autoimmune immunity: humoral autoimmunity to type II collagen
• Enzymatic actions: trypsin-anti trypsin imbalance, collagenase, cathepsins, etc
• Cytokines:TGF-B1,BMP
• Hormonal factors

11. PATHOGENESIS
• CONDUCTIVE HEARING LOSS-Narrowing and ankylosis of the annular ligament, specially the posterior
vestibular joint space.
• SORINEURAL HEARING LOSS-
Liberation of toxic metabolites into the fluids of the inner ear (Causse and Chevance 1978; Nager
1969)
Vascular compromise and hypoxemia of the structures of the inner ear (Ruedi and Spondlin 1966)
Alteration of the mechanism of motion within the cochlear duct because of endosteal
involvement and hyalinization of the spiral ligament of the cochlea (Linthicum et al 1975).
• VESTIBULAR SYMPTOMS-
1) The otosclerotic focus could produce end organ or neural degeneration or both (Gussen 1973;
Sando et al 1974).
(2) Vertigo is produced when the otosclerotic focus comes in contact with the perilymph, which
then results in a change in the biochemistry of the perilymph (Ghorayeb and Linthicum 1978).

12. CLINICAL EVALUATION

13. HISTORY
• Gradual onset , slowly progressive hearing loss
• Usually becomes apparent in the third or fourth decade.
• Female predominance/ aggravated during pregnancy
• Paracusis of Willis- due to Lombard effect
• Tinnitus- incidence 70% (Wiet et al 1991).
• Vestibular symptoms- 27- 30%

14. CLINICAL EXAMINATION
• OTOSCOPE/ MICROSCOPIC EXAMINATION:
Tympanic membrane- normal
Schwartze’s sign-This is a sign of an active
otosclerotic focus
• PNEUMATIC OTOSCOPE:
If the malleus is fixed, the mobility of tympanic
membrane is minimal
• TFT:
• pure stapedial otosclerosis
Rinne’s test is negative.
Weber’s test is lateralized to the more
affected
ABC test is normal.
• In pure cochlear otosclerosis,
Rinne’s test is positive
Weber’s being lateralized to the better
hearing ear.
ABC test is reduced,
• In combined otosclerosis,
 Rinne’s test is negative, ABC test is reduced.
• Gelle’s test- No change in hearing in fixed ossicles

15. AUDIOLOGICAL EVALUATION
• PTA:
A gradually progressive low-frequency
conductive hearing loss is first seen-
“stiffness tilt.”
 later stages- flat pattern
Cochlear otosclerosis- mixed or
sensorineural, with the high frequencies
becoming severely affected - COOKIE BITE
PATTERN.
Carhart’s notch- It is characterized by the
elevation of bone conduction thresholds of
approximately 5 dB at 500 Hz, 10 dB at
1000 Hz, 15 dB at 2000 Hz, and 5 dB at
4000 Hz.
Figure 4–1 Pure tone audiogram of a patient
suffering
from cochlear otosclerosis. The multiple
sloping
pattern gives it the appearance of a cookie
bite. Dotted

16. • TYMPANOMETRY: As type curve
• STATIC COMPLIANCE:
static compliance = peak compliance – compliance at
200daPa
Normal static compliance values fall in the range of 0.3
to 1.6 cc.
Greater than 0.6 cm- relatively thin footplate.
less than 0.2 cm3- fairly thicker footplate/ obliterative.
For symmetrical hearing loss- helps in selection of ear
to be operated
• ACOUSTIC REFLEX:
• NON ACOUSTIC REFLEX:
• SPEECH AUDIOMETRY:

17. RADIOLOGY
HRCT TEMPORAL BONE
• cochlear otosclerosis –
typical finding is DOUBLE RING EFFECT - is because of confluence of multiple spongiotic foci with
each other within the thickness of the capsule may be limited to a segment of the capsule or follow
the entire cochlear contour
• Fmilial (hereditary) otosclerosis- more extensive lesions on CT scanning seem to indicate the familial forms
(Shin et al 2001)
• Far advanced otosclerosis- when cochlear implantation is being actively considered
• Postoperative evaluation- position of prosthesis/status of incus
MRI
inner ear anomalies( Enlarged vestibular aqueduct/cochlear acqueduct/large IAM)
persistant stapedial artery
congenital stapes fixation
CT DENSITOMETRY
SINGLE PHOTON EMISSION COMPUTED TOMOGRAPHY (SPECT)

18. DIFFRENTIAL DIAGNOSIS
• Ossicular Discontinuity
• Malleus head fixation.
• Tympanosclerosis
• Congenital Stapes Fixation
• Osteopetrosis (Albers-Schönberg Disease)
• Osteogenesis Imperfecta
• Paget’s Disease

19. MANAGEMENT
• Amplification
• Surgery
stapedotomy/ Stapedectomy/ STAMP technique
• Medical therapy

20. • INDICATIONS FOR HEARING AID:
Patient cannot undergo surgery because of major systemic illnesses.
Only hearing ear
Patient has inadequate hearing reserve and/or poor speech discrimination score.
Congenital fixation of the stapes is present,
Surgery is not elected by the patient.
Affected ear shows early (mild) conductive hearing loss.
Unsuccessful surgery for otosclerosis on the other ear has been attempted.
Patient has both otosclerosis and Meniere’s disease.
 Far-advanced otosclerosis.
HEARING AIDS & IMPLANTATIONS

21. SURGICAL MANAGEMENT

22. CANDIDACY SELECTION
• Bone conduction- loss of 0 to 25db
Air conduction – loss of 45 to 65 dB
• AB gap- atleast 20 db
• Good speech discrimination(70%)
• Negative Rinne’s test
• Desire for surgery
• Good general health condition
CONTRAINDICATIONS
ABSOLUTE
• only hearing ear
RELATIVE
• Active middle ear or external ear infections
• symptoms of hydrops- symptom free interval of
6 months
• middle ear atelectasis/
• positive Schwartze’s sign
• Pregnancy
• Children
• Elderly
• professional activities ( scuba/boxing/pilots)

23. CONSENT
• Nature of the disease/procedure
• Alternatives available
• 85% improvement, 14%- no benefit & risk of SNHL/Dead ear(-1-2%)
• Vertigo and dizziness in the post operative period- transient/rarely permanent
• Post op complications
• Need for amplification in the post operative period if any.
• Occupational/ leisure activities

24. STAPEDOTOMY
• Instruments-The instruments used
in stapedectomies are standard,
with little variation

25. ANAESTHESIS
2% Lignocaine(1:30,000-1:1,00,000)
4 quadrants at bony cartilaginous junction

26. INCISION- Rosen’s incison/ extended Rosen’s incison

27. • ELEVATION OF TYMPANOMEATAL FLAP:

28. • CURETTING OF POSTERIOR SUPERIOR MEATAL WALL

29. • MIDDLE EAR EXAMINATION/ OSSICULAR STATUS/ CONFIRM STAPES MOBILITY

30. • IS JOINT IS DISARTICULATED

31. • STAPES TENDON IS CUT

32. • POST CRUROTOMY FOLLOWED BY ANTERIOR CRUROTOMY
• SUPRASTRUCTURE IS REMOVED BY DOWNFRACTURING

33. • Fenestration of footplate
Fenestration done in the posterior 1/3 rd of footplate
Fisch perforator(0.3/0.5/07mm)
Microdrill(0.6mm)
Laser

34. • MEASUREMENT OF PISTON LENGTH
Lateral surface of long process of incus upto the footplate
Usually 4.25 to 4.5 +0.25 for the fenestra

35. • PLACEMENT OF THE PROSTHESIS
• CHECKING FOR THE MOBILITY OF THE PROSTHESIS- HANGING/BENDING TEST
• CLOSURE OF FENESTRA WITH LOBULE FAT

36. TYPES OF STAPEDOTOMY
• CLASSIC STAPEDOTOMY
Stapes suprastructure removed
Fenestration of footplate
Placement of prosthesis
• MODIFIED STAPEDOTOMY
Fenestration of footplate
Prosthesis placement
Stapes suprastructure is removed
• NEO TECHNIQUES
 reattachment of the stapes tendon
Linear stapedotomy without process (STAMP)

37. OVAL WINDOW SEAL
Vein (hand or wrist)
Temporalis fascia
Blood
Fat
Tragal perichondrium

38. PROSTHESIS
• TYPES: Robinson prosthesis/Caussee Teflon
prosthesis/Fisch/McGee prosthesis/ House wire
prosthesis
• MATERIALS: steel, platinum, gold, Teflon,
titanium and alloys
• DIAMETER : 0.4 mm
• LENGTH: length measured + 0.25mm ( approx.
4mm ranges from 4.25 mm to 4.75 mm)
• Ideal prosthesis: roughness/band shaped loop /
malleable/stiff/transition zone- band
shaped/appropriated length and diameter/
biocompatible.

39. LASERS IN STAPES SURGERY
• Ideal qualities of a laser:
1. Precise optics
 2. better vaporization
3. thermal foot print should not be
deep
4. The laser should not heat the
perilymph.
• Types: Visible( argon/KTP) & infrared (
CO2)
• Advantages of LASER surgery
• Vestibular and facial nerve saftey

40. STAPEDECTOMY VS STAPEDOTOMY
STAPEDECTOMY
• Advantage
 Extensive fixation of the footplate
 Floating footplate
 Comminuted fractures of footplate
 Better gain in lower frequencies
• Disadvantages
 Increased post-op vestibular symptoms and SNHL
 Technically more difficult
 Increased potential for prosthesis migration
STAPEDOTOMY
• Originally for obliterated or
solid footplates
• Advantages
 Less trauma to the vestibule
 Less incidence of prosthesis
migration
 Less fixation of prosthesis by scar
tissue
 Better high frequency gain
 Better postoperative speech
discrimination

41. INTRA OPERATIVE COMPLICATION
• Facial nerve dehiscence.
• Floating and submerged footplate
• Perilymph flooding
• Malleus fixation
• Tympanic membrane tear
• Persistent stapedial artery
• Obliterative otosclerosis
• Round window otosclerosis
• Intra operative vertigo

42. POST OPERATIVE COMPLICATIONS
• Sensorineural hearing loss and
vestibular symptoms
• Perilymph fistula
• Dysgeusia and ageusia
• Facial paralysis
• Post operative reparative granuloma
• Cholesteatoma formation
poststapedectomy

43. REVISION SURGERY
• CAUSES OF THE REAPPEARANCE OF A CONDUCTIVE HEARING LOSS
• Immediate conductive hearing loss or poor response to primary surgery include
Failure of initial prosthesis placement / Inappropriate prosthesis length
Excessive tissue graft
Reparative granuloma
 Incorrect diagnosis such as third mobile window syndrome or ossicular fixation
from the malleus or incus
• Progressive conductive loss
Displacement of the prosthesis
Incus erosion/necrosis
Allergy to the prosthetic substance, typically nickel-titanium (Nitinol)
Footplate refixation
Ongoing otosclerosis
• THE INDICATIONS FOR REVISION SURGERY:
Hearing loss with an air-bone gap greater than 25 dB
Persistent intractable vertigo
 Facial nerve complication requiring intervention
perilymphatic fistula
Progressive, fluctuating sensorineural hearing loss
• SURGICAL PLANNING:
• RECOMMENDED INTERVAL OF TIME BETWEEN SURGERIES:
 1 year ( at least a minimum of 6 weeks)
• CONTRAINDICATIONS FOR SECOND EAR SURGERY:
 complications following the first surgery
 Tinnitus and/or persistent vertigo following surgery
 SNHL

44. OUTCOMES
THE BELFAST RULE OF THUMB : patient likely to report a significant benefit if the
postoperative hearing level was 30 dB or better and the interaural difference reduced to less
than 15 dB
GLASGOW BENEFIT PLOT: the ear that has a hearing level better than 30 dB is considered
normal or "socially acceptable", and an interaural difference of less then 10 dB is classified as
symmetric

45. MEDICAL MANAGEMENT
SODIUM FLUORIDE THERAPY:
• Dosage:
50-75 mg per day
For 3 months to 2 years
• MOA:
 Reducing bone resorption and increasing
osteoblastic bone formation,
Anti enzymatic action on proteolytic enzymes that
are cytotoxic to the cochlea.
• INDICATIONS
Progressive SNHL
Positive schwartze sign
Otosclerosis with secondary hydrops
Surgery has been refused and patient seeks
alternative therapy
CONTRAINDICATIONS
Chronic nephritis with nitrogen retention
Acute rheumatoid arthritis
Pregnancy
Skeletal fluorosis
Allergic to fluoride
When skeletal growth is not complete
SIDE EFFECTS OF THERAPY
 temporary spine fluorosis
Gastritis
Acute bone pain
OTHER MEDICATIONS:
Bisphosphonates
Cytokine inhibitors

46. COCHLEAR IMPLANTATION IN OTOSCLEROSIS
• INDICATIONS
Far advanced otosclerosis
Severe to profound SNHL
Poor speech discrimination score
• DIFFICULTIES ENCOUNTERED DURING CI IN OTOSCLEROSIS
Facial nerve stimulation
Incomplete electrode placement
Potential ossification of cochlear/ RW/ basal turn
Unstable results
REFERENCE:
1. Quaranta N, Bartoli R, Lopriore A, Fernandez-Vega S, Giagnotti F, Quaranta A. Cochlear implantation in otosclerosis [published correction appears in
Otol Neurotol. 2005 Nov;26(6):1264. Priore, Anna Lo [corrected to Lopriore, Anna]]. Otol Neurotol.
2. Burmeister J, et al, Cochlear implantation in patients with otosclerosis of the otic capsule, American Journal of Otolaryngology–Head and Neck
Medicine and Surgery (2017)
• CHOICE OF PROCEDURE
Primary management- stapedotomy with
amplification
Ossification of Basal turn of cochlear- primary CI

47. REFERENCES & PROPOSED READING:
• REFERENCES:
Scott-Brown’s Otorhinolaryngology Head & Neck surgery . Vol II- The Paediatrics, The Ear, The Skull Base.
Cummings Otolaryngology-Head & Neck Surgery
Otosclerosis and Stapedectomy-Diagnosis, Management, and Complications. Christopher de Souza, Michael E.
Glasscock.
History of Otosclerosis and Stapes Surgery -Ronen Nazarian, John T. McElveen Jr, Adrien A.
Eshraghi, MD, MSc.
The fissula ante fenestram of the human otic capsule-Developmental and normal adult structure- Barry J.
Anson, Earl w. Cauldwell, M.D. Chicago, and Theodore H. Bast, Madison.
The Stapes Prosthesis Past, Present, and Future -Alexander Sevy, Moises Arriaga, MD.
Revision Surgery for Otosclerosis- Apoorva T. Ramaswamy, MD, Lawrence R. Lustig.
• PROPOSED READING:
Cochlear otosclerosis
Obliterative otosclerosis
Bio mechanics of stapes replacement

48. THANK YOU