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Otosclerosis
Alan L. Cowan, MD
Faculty Advisor: Tomoko Makishima, MD, PhD
Department of Otolaryngology
The University of Texas Medical Branch
Grand Rounds Presenttion
October 18, 2006
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Introduction
Otosclerosis
Primary metabolic bone disease of the otic capsule and ossicles
Results in fixation of the ossicles and conductive hearing loss
May have sensorineural component if the cochlea is involved
Genetically mediated
Autosomal dominant with incomplete penetrance (40%) and variable expressivity
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History of Otosclerosis and Stapes
Surgery
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”
1890 – Bacon describes medical therapy for the condition, and supports the common view that “surgery should not be considered for a moment.“
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History of Otosclerosis and Stapes
Surgery
Gunnar Holmgren (1923)
Father of fenestration surgery
Single stage technique
Sourdille
Holmgren’s student
3 stage procedure
64% satisfactory results
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History of Otosclerosis and Stapes
Surgery
Julius Lempert
Popularized the single
staged fenestration
procedure
John House
Further refined the
procedure
Popularized blue lining the
horizontal canal
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History of Otosclerosis and Stapes
Surgery
Fenestration procedure for otosclerosis
Fenestration in the horizontal canal with a tissue
graft covering
>2% profound SNHL
Rarely complete closure of the ABG
May exhibit vestibular disturbances
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History of Otosclerosis and Stapes
Surgery
Samuel Rosen
1953 – first suggest
mobilization of the stapes
Immediate improved
hearing
Re-fixation
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History of Otosclerosis and Stapes
Surgery
John Shea
1956 – first to perform
stapedectomy
Oval window vein graft
Nylon prosthesis from
incus to oval window
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Epidemiology
10% overall prevalence of histologic otosclerosis
1% overall prevalence of clinically significant
otosclerosis
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Epidemiology
Race Incidence of otosclerosis
Caucasian 10%
Asian 5%
African American 1%
Native American 0%
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Epidemiology
Gender
Histologic otosclerosis – 1:1 ratio
Clinical otosclerosis – 2:1 (W:M)
Possible progression during pregnancy (10%-17%)
Studies which have demonstrated changes during pregnancy are
often retrospective or lack audiometric data.
Studies comparing multigravid vs. nulligravid women with
otosclerosis have failed to show audiometric differences.
Bilaterality more common (89% vs. 65%)
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Epidemiology
Age
15-45 most common age range of presentation
Youngest presentation 7 years
Oldest presentation 50s
0.6% of individuals <5 years old have foci of
otosclerosis
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Pathophysiology
Osseous dyscrasia
Resorption and formation of new bone
Limited to the temporal bone and ossicles
Inciting event unknown
Hereditary, endocrine, metabolic, infectious, vascular,
autoimmune, hormonal
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Pathology
Two phases of disease
Active (otospongiosis phase)
Osteocytes, histiocytes, osteoblasts
Active resorption of bone
Dilation of vessels
Schwartze’s sign
Mature (sclerotic phase)
Deposition of new bone (sclerotic and less dense than normal
bone)
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Pathology
Most common sites of involvement
Fissula ante fenestrum
Round window niche (30%-50% of cases)
Anterior wall of the IAC
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Non-clinical foci of otosclerosis
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Anterior footplate involvement
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Annular ligament involvement
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Bipolar involvement of the footplate
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Round Window
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Labyrinthine Otosclerosis
1912 – Siebenmann described labyrinthine
otosclerosis
Suggested otosclerosis may cause SNHL via
Toxic metabolites
Decreased blood supply
Direct extension
Disruption of membranes
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Hyalinization of the spiral ligament
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Erosion into inner ear
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Organ of Corti
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Cochlear Otosclerosis
Audiometric studies Some studies have shown that in cases of unilateral otosclerosis ~ 60% may have decreased
sensory thresholds even after stapes surgery
Histiologic studies Cases of documented otosclerosis and a large sensory loss have shown large foci of
otosclerosis in the otic capsule.
Many cases of large otic capsule foci without sensory loss or of sensory loss without foci have also been described.
Biochemical studies Some authors have noted increased levels of perilymph protein during stapedotomy in
patients with radiographic evidence of otic capsule foci and sensory hearing loss.
Conclusion Many experts believe that extensive involvement of the cochlea will produce sensorineural
hearing deficits, although it is not known how this occurs or why it only occurs in a subset of patients with cochlear foci.
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Diagnosis
of Otosclerosis
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History
Most common presentation
Women age 20 - 30
Conductive or Mixed hearing loss
Slowly progressive,
Bilateral (80%)
Asymmetric
Tinnitus (75%)
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History
Age of onset of hearing loss
Progression
Laterality
Associated symptoms
Dizziness
Otalgia
Otorrhea
Tinnitus
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History
Family history
2/3 have a significant family history
Particularly helpful in patients with severe or profound mixed hearing loss
Prior otologic surgery
History of ear infections
Vestibular symptoms
25%
Most commonly dysequilibrium
Occasionally attacks of vertigo with rotatory nystagmus
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Physical Exam
Otomicroscopy
Most helpful in ruling out other disorders Middle ear effusions
Tympanosclerosis
Tympanic membrane perforations
Cholesteatoma or retraction pockets
Superior semicircular canal dehiscence
Schwartze’s sign Red hue in oval window niche area
10% of cases
Pneumatic otoscopy
Distinguish from malleus fixation
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Physical Exam
Tuning forks
Hearing loss progresses form low frequencies to
high frequencies
256, 512, and 1024 Hz TF should be used
Rinne
256 Hz – negative test indicates at least a 20 dB ABG
512 Hz – negative test indicates at least a 25 dB ABG
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Differential Diagnosis
Ossicular discontinuity
Congenital stapes fixation
Malleus head fixation
Paget’s disease
Osteogenesis imperfecta
Superior semicircular canal dehiscence
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Audiometry
Tympanometry
Impedance testing
Acoustic reflexes
Pure tones
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Tympanometry
Jerger (1970) – classification of tympanograms
Type A
Type A
Type As
Type Ad
Type B
Type C
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Acoustic Reflexes
Result from a change in the middle ear
compliance in response to a sound stimulus
Change in compliance
Stapedius muscle contraction
Stiffening of the ossicular chain
Reduces the sound transmission to the vestibule
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Acoustic Reflexes
Otosclerosis has a predictable pattern of
abnormal reflexes over time
Reduced reflex amplitude
Elevation of ipsilateral thresholds
Elevation of contralateral thresholds
Absence of reflexes
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Pure Tone Audiometry
Most useful audiometric test for otosclerosis
Characterizes the severity of disease
Frequency specific
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
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Pure Tone Audiometry
Low frequencies affected
first
Below 1000 Hz
Rising air line
“Stiffness tilt”
Secondary to stapes fixation
With disease progression
Air line flattens
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Pure Tone Audiometry
Carhart’s notch
Proposed theory
Stapes fixation disrupts the normal ossicular resonance
(2000 Hz)
Normal compressional mode of bone conduction is
disturbed because of relative perilymph immobility
Mechanical artifact
Reverses with stapes mobilization
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Pure Tone Audiometry
Committee on Hearing and Balance
Set standards for reporting results in cases of otosclerosis procedures. Operative hearing results should be reported using post-operative
data, specifically, the post-operative air-bone gap.
This prevents exaggeration of surgical results and “overclosure.”
Adopted by the AAOHNS in 1994
Important in reviewing literature regarding surgical outcomes Studies prior to this time often use pre-op bone lines and post-op air
conduction measurements which may exaggerate results.
This convention is not uniform in all parts of the world, so the methods is very important in determining the consistency of data.
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Imaging
Computed tomography (CT) of the temporal bone
Proponents of CT for evaluation of otosclerosis
Pre-op Characterize the extent of otosclerosis
Severe or profound mixed hearing loss
Evaluate for enlarge cochlear aqueduct
Post-op Recurrent CHL
Re-obliteration vs. prosthesis dislocation
Vertigo
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“Halo sign”
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Paget’s disease
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Osteogenesis Imperfecta
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Management Options
Medical
Amplification
Surgery
Combinations
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Patient Selection
Factors
Result of tuning fork tests and audiometry
Skill of the surgeon
Facilities
Medical condition of the patient
Patient wishes
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Surgery
Best surgical candidate
Previously un-operated ear
Good health
Unacceptable ABG
25 to 40 dB
Negative Rinne test
Excellent discrimination
Desire for surgery
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Surgery
Other factors
Age of the patient
Elderly Poorer results in the high frequencies
Congenital stapes fixation (44% success rate)
Juvenile otosclerosis (82% success rate)
Occupation
Diver
Pilot
Airline steward/stewardess
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Surgery
Other factors
Vestibular symptoms
Meniere's disease
Concomitant otologic disease
Cholesteatoma
Tympanic membrane perforation
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Surgical Steps
Subtleties of technique and style
Local vs. general anesthesia
Stapedectomy vs. partial stapedectomy vs.
stapedotomy
Laser vs. drill vs. cold instrumentation
Oval window seals
Prosthesis
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Canal Injection
2-3 cc of 1% lidocaine
with 1:50,000 or
1:100,000 epinephrine
4 quadrants
Bony cartilaginous
junction
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Raise Tympanomeatal Flap
6 and 12 o’clock
positions
6-8 mm lateral to the
annulus
Take into account
curettage of the scutum
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Separation of chorda tympani nerve
from malleus
Separate the chorda
from the medial surface
of the malleus to gain
slack
Avoid stretching the
nerve
Cut the nerve rather
than stretch it
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Curettage of Scutum
Curettage a trough lateral
to the scutum, thinning it
Then remove the scutum
(incus to the round
window)
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Curettage of Scutum
Exposure
Vertical: Facial nerve to
round window
Horizontal: Pyramidal
process to malleus
Preservation of bone over incus
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Middle ear examination
Mobility of ossicles
Confirm stapes fixation
Evaluate for malleus or incus fixation
Abnormal anatomy
Dehiscent facial nerve
Overhanging facial nerve
Deep narrow oval window niche
Ossicular abnormalities
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Measurement for prosthesis
Measurement
Lateral aspect of
the long process of
the incus to the
footplate
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Total Stapedectomy
Uses
Extensive fixation of the footplate
Floating footplate
Disadvantages
Increased post-op vestibular symptoms
More technically difficult
Increased potential for prosthesis migration
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Stapedotomy/Small Fenestra
Originally for obliterated or solid footplates
Europe
1970-80
First laser stapedotomy performed by Perkins in 1978
Less trauma to the vestibule
Less incidence of prosthesis migration
Less fixation of prosthesis by scar tissue
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Drill Fenestration
0.7mm diamond burr
Motion of the burr
removes bone dust
Avoids smoke production
Avoids surrounding heat
production
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Laser Fenestration
Laser Avoids manipulation of the footplate
Argon and Potassium titanyl phosphate (KTP/532) Wave length 500 nm
Visible light
Absorbed by hemoglobin
Surgical and aiming beam
Carbon dioxide (CO2) 10,000 nm
Not in visible light range
Surgical beam only Requires separate laser for an aiming beam (red helium-neon)
Ill defined fuzzy beam
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Oval window seal
Tragal perichondrium
Vein (hand or wrist)
Temporalis fascia
Blood
Fat
Gelfoam (now discouraged)
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Reconstructing the annular ligament
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Placement of the Prosthesis
Prosthesis is chosen and
length picked
Some prefer bucket
handle to incorporate the
lenticular process of the
incus
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Stapedectomy vs. Stapedotomy
ABG closure < 10dB (PTA)
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Special Considerations
and Complications in
Stapes Surgery
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Overhanging Facial Nerve
Usually dehiscent
Consider aborting the procedure
Facial nerve displacement (Perkins, 2001)
Facial nerve is compressed superiorly with No. 24 suction (5 second periods)
10-15 sec delay between compressions
Perkins describes laser stapedotomy while nerve is compressed
Wire piston used
Add 0.5 to 0.75 mm to accommodate curve around the nerve
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Floating Footplate
Footplate dislodges from the surrounding OW niche Incidental finding
More commonly iatrogenic
Prevention Laser
Footplate control hole
Management Abort
H. House favors promontory fenestration and total stapedectomy
Perkins favors laser fenestration
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Diffuse Obliterative Otosclerosis
Occurs when the
footplate, annular
ligament, and oval
window niche are
involved
Closure of air-bone gap
< 10 dB less common.
Refixation commonly
occurs
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Perilymphatic Gusher
Associated with patent cochlear aqueduct
More common on the left
Increased incidence with congenital stapes fixation
Increases risk of SNHL
Management
Rough up the footplate
Rapid placement of the OW seal then the prosthesis
HOB elevated, stool softeners, bed rest, avoid Valsalva, +/-
lumbar drain
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Round Window Closure
20%-50% of cases
1% completely closed
No effect on hearing unless 100% closed
Opening has a high rate of SNHL
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SNHL
1%-3% incidence of profound permanent SNHL Surgeon experience
Extent of disease Cochlear
Prior stapes surgery
Temporary Serous labyrinthitis
Reparative granuloma
Permanent Suppurative labyrinthitis
Extensive drilling
Basilar membrane breaks
Vascular compromise
Sudden drop in perilymph pressure
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Reparative Granuloma
Granuloma formation around the prosthesis and incus
2 -3 weeks postop
Initial good hearing results followed by an increase in the high frequency bone line thresholds
Associated tinnitus and vertigo
Exam – reddish discoloration of the posterior TM
Treatment ME exploration
Removal of granuloma
Prognosis – return of hearing with early excision
Associated with use of Gelfoam
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Vertigo
Most commonly short lived (2-3 days)
More prolonged after stapedectomy compared
to stapedotomy
Due to serous labyrinthitis
Medialization of the prosthesis into the vestibule
With or without perilymphatic fistula
Reparative granuloma
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Recurrent Conductive Hearing Loss
Slippage or displacement of the prosthesis
Most common cause of failure
Immediate
Technique
Trauma
Delayed
Slippage from incus narrowing or erosion
Adherence to edge of OW niche
Stapes re-fixation
Progression of disease with re-obliteration of OW
Malleus or incus ankylosis
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Amplification
Excellent alternative
Non-surgical candidates
Patients who do not desire surgery
Patient satisfaction rate lower than that of
successful surgery
Canal occlusion effect
Amplification not used at night
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Medical
Sodium Fluoride
1923 - Escot suggested using calcium fluoride
1965 – Shambaugh popularized its use
Mechanism
Fluoride ion replaces hydroxyl group in bone forming
fluorapatite
Resistant to resorption
Increases calcification of new bone
Causes maturation of active foci of otosclerosis
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Medical
Sodium Fluoride
Reduces tinnitus, reverses Schwartze’s sign, resolution of
otospongiosis seen on CT
OTC – Florical
Dose – 20-120mg
Indications
Non-surgical candidates
Patients who do not want surgery
Surgical candidates with + Schwartze’s sign
Treat for 6 mo pre-op
Postop if otospongiosis detected intra-op
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Medical
Sodium fluoride
Hearing results
50% stabilize
30% improve
Re-evaluate q 2 yrs with CT and for Schwartze’s sign
to resolve
If fluoride are stopped – expect re-activation within
2-3 years
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Medical
Bisphosphonates
Class of medications that inhibits bone resorption by
inhibiting osteoclastic activity
Dosing not standard
Often supplement with Vitamin D and Calcium
Studies conducted on otosclerosis patients with neurotologic
symptoms report the majority of patients with subjective
improvement or resolution.
Future application of this treatment unclear, especially with
new reports of bisphosphonate related osteonecrosis.
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References
Bacon, Gorham. A Manual of Otology. Lea Brothers & Co. New York, NY. 1898.
Banerjee A, Whyte A, Atlas. Superior canal dehiscence : review of a new condition. Clinical Otolaryngology. 30, 9-15.
Brooker KH, Tanyeri H. Etidronate for the Neurotologic Symptoms of Otosclerosis : Preliminary Study. Ear, Nose & Throat Journal. June 1997 ; 76 (6) : p371-377.
Causse JR et al. Sodium fluoride therapy. Am J Otol 1993;14(5):482-490
Committee on Hearing and Equilibrium. Committee on Hearing and Equilibrium guidelines for the evaluation of results of treatment of conductive hearing loss. Otolaryngology – Head and Neck Surgery. 113 (3) pp. 186-7.
Glasscock II ME, et al. Twenty-five years of experience with stapedectomy. Laryngoscope 1995;105:899-904
House HP, Kwartler JA. Total stapedectomy. Otologic Surgery, 2nd ed. edited by Brackmann, Shelton, and Arriaga, W.B. Saunders 2001;226-234
Hough J. Partial stapedectomy. Ann Otol Rhinol Laryngol 1960;69:571
House J. Otosclerosis. Otolaryngol Clinics 1993;26(3):323-502
Jerger J. Clinical experience with impedance audiometry. Arch Otolaryngol 1970;92:311
Lempert J. Improvement in hearing in cases of otosclerosis: A new, one stage surgical technique. Arch Otolaryngol 1938;28:42-97
Lippy WH, Schuring AG. Treatment of the inadvertently mobilized footplate. Otolaryngol Head Neck Surg 1973;98:80-81
Meyer S. The effect of stapes surgery on high frequency hearing in patients with otosclerosis Am J Otol 1999;20:36-40
Millman B. Giddings, NA and Cole, JM. Long-term follow-up stapedectomy in children and adolescents. Otol Head Neck Surg 1996;115(1):78-81
Minor L. Clinical Manifestiations of Superior Semicircular Canal Dehiscence. The Laryngoscope. 2005. 115: 1717-1727.
Muller, C. Gadre, A. Otosclerosis. Quinn’s online textbook of Otolaryngology. http://www.utmb.edu/otoref/Grnds/GrndsIndex.html.
Nelson EG, Hinojosa R. Questioning the Relationship between Cochlear Otosclerosis and Sensorineural Hearing Loss: A Quantitative Evaluation of Cochlear Structures in Cases of Otosclerosis and Review of the Literature. The Laryngoscope. 2004; 114: 1214-1230
Perkins RC. Laser stapedotomy. Otologic Surgery, 2nd ed. edited by Brackmann, Shelton, and Arriaga, W.B Saunders 2001;245-260
Perkins RC. Laser stapedotomy for otosclerosis. Laryngoscope 1980;91:228-241
Politzer. Primary Diseases of the Bony Labyrinthine Capsule. Archives of Otology, 1894, vol. xxiii. P. 255.
Roland PS. Otosclerosis. www.emedicine.com/ped/topic1692.htm. 2002;1-11
Roland PS, Meyerhoff WL. Otosclerosis. Otolaryngology-Head and Neck Surgery. 3rd ed., edited by Byron J. Bailey, Lippincott Williams & Wilkins, Philadelphia 2001;1829-1841
Rosen S. Restoration of hearing in otosclerosis by mobilization of the fixed stapedial footplate. An analysis of results. Laryngoscope 1955;65:224-269
Shea J Jr. Fenestration of the oval window. Ann Otol Rhinol Laryngol 1958;67:932-951
Shambaugh G. Clinical diagnosis of cochlear (labyrinthine) otosclerosis. Laryngoscope 1965;75:1558-1562
Shambaugh GE, Jr. and Glasscock ME, III. Surgery of the ear, 3rd ed. Philadelphia, W. B. Saunders, 1980;455-516
Toynbee, Joseph. The Diseases of the Ear: Their Nature, Diagnosis, and Treatment. With a Supplement by James Hinton. London, 1868.