Otosclerosis

Alan L. Cowan, MD Tomoko Makishima, MD, PhD

Department of Otolaryngology University of Texas Medical Branch Galveston, TX

October 18, 2006

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

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.

History of Otosclerosis and Stapes Surgery

Gunnar Holmgren (1923)

Father of fenestration surgery Single stage technique

Sourdille

Holmgrens student 3 stage procedure 64% satisfactory results

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

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

History of Otosclerosis and Stapes Surgery

Samuel Rosen

1953 first suggest mobilization of the stapes

Immediate improved hearing Re-fixation

History of Otosclerosis and Stapes Surgery

John Shea

1956 first to perform stapedectomy

Oval window vein graft Nylon prosthesis from incus to oval window

Epidemiology

10% overall prevalence of histologic otosclerosis 1% overall prevalence of clinically significant otosclerosis

Epidemiology
Race Caucasian Asian African American Native American Incidence of otosclerosis 10% 5% 1% 0%

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%)

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

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

Pathology

Two phases of disease

Active (otospongiosis phase)

Osteocytes, histiocytes, osteoblasts Active resorption of bone Dilation of vessels Schwartzes sign Deposition of new bone (sclerotic and less dense than normal bone)

Mature (sclerotic phase)

Pathology

Most common sites of involvement

Fissula ante fenestrum Round window niche (30%-50% of cases) Anterior wall of the IAC

Non-clinical foci of otosclerosis

Anterior footplate involvement

Annular ligament involvement

Bipolar involvement of the footplate

Round Window

Labyrinthine Otosclerosis

1912 Siebenmann described labyrinthine otosclerosis

Suggested otosclerosis may cause SNHL via

Toxic metabolites Decreased blood supply Direct extension Disruption of membranes

Hyalinization of the spiral ligament

Erosion into inner ear

Organ of Corti

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.

Diagnosis of Otosclerosis

History

Most common presentation

Women age 20 - 30 Conductive or Mixed hearing loss

Slowly progressive, Bilateral (80%) Asymmetric

Tinnitus (75%)

History

Age of onset of hearing loss Progression Laterality Associated symptoms

Dizziness Otalgia Otorrhea Tinnitus

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

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 Red hue in oval window niche area 10% of cases

Schwartzes sign

Pneumatic otoscopy

Distinguish from malleus fixation

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

Differential Diagnosis

Ossicular discontinuity Congenital stapes fixation Malleus head fixation Pagets disease Osteogenesis imperfecta Superior semicircular canal dehiscence

Audiometry

Tympanometry Impedance testing

Acoustic reflexes

Pure tones

Tympanometry

Jerger (1970) classification of tympanograms

Type A
Type A Type As Type Ad

Type B Type C

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

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

Pure Tone Audiometry

Most useful audiometric test for otosclerosis

Characterizes the severity of disease Frequency specific

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

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

Pure Tone Audiometry

Carharts 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

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.

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 Recurrent CHL Re-obliteration vs. prosthesis dislocation Vertigo

Post-op

Halo sign

Pagets disease

Osteogenesis Imperfecta

Management Options

Medical Amplification Surgery Combinations

Patient Selection

Factors
Result of tuning fork tests and audiometry Skill of the surgeon Facilities Medical condition of the patient Patient wishes

Surgery

Best surgical candidate

Previously un-operated ear Good health Unacceptable ABG

25 to 40 dB Negative Rinne test

Excellent discrimination Desire for surgery

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

Surgery

Other factors

Vestibular symptoms

Meniere's disease

Concomitant otologic disease

Cholesteatoma Tympanic membrane perforation

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

Canal Injection

2-3 cc of 1% lidocaine with 1:50,000 or 1:100,000 epinephrine

4 quadrants
Bony cartilaginous junction

Raise Tympanomeatal Flap

6 and 12 oclock positions 6-8 mm lateral to the annulus Take into account curettage of the scutum

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

Curettage of Scutum

Curettage a trough lateral to the scutum, thinning it Then remove the scutum (incus to the round window)

Curettage of Scutum

Exposure

Vertical:

Facial nerve to round window

Horizontal:

Pyramidal process to malleus

Preservation of bone over incus

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

Measurement for prosthesis

Measurement

Lateral aspect of the long process of the incus to the footplate

Total Stapedectomy

Uses
Extensive fixation of the footplate Floating footplate

Disadvantages
Increased post-op vestibular symptoms More technically difficult Increased potential for prosthesis migration

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

Drill Fenestration

0.7mm diamond burr

Motion of the burr removes bone dust Avoids smoke production Avoids surrounding heat production

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 10,000 nm Not in visible light range Surgical beam only

Carbon dioxide (CO2)

Requires separate laser for an aiming beam (red helium-neon)

Ill defined fuzzy beam

Oval window seal

Tragal perichondrium Vein (hand or wrist) Temporalis fascia Blood Fat Gelfoam (now discouraged)

Reconstructing the annular ligament

Placement of the Prosthesis

Prosthesis is chosen and length picked Some prefer bucket handle to incorporate the lenticular process of the incus

Stapedectomy vs. Stapedotomy

ABG closure < 10dB (PTA)

Special Considerations and Complications in Stapes Surgery

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 Add 0.5 to 0.75 mm to accommodate curve around the nerve

Wire piston used

Floating Footplate

Footplate dislodges from the surrounding OW niche

Incidental finding More commonly iatrogenic Laser Footplate control hole Abort H. House favors promontory fenestration and total stapedectomy Perkins favors laser fenestration

Prevention

Management

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

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

Round Window Closure

20%-50% of cases 1% completely closed

No effect on hearing unless 100% closed

Opening has a high rate of SNHL

SNHL

1%-3% incidence of profound permanent SNHL

Surgeon experience Extent of disease

Cochlear

Prior stapes surgery

Temporary

Serous labyrinthitis Reparative granuloma

Suppurative labyrinthitis Extensive drilling Basilar membrane breaks Vascular compromise Sudden drop in perilymph pressure

Permanent

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

Vertigo

Most commonly short lived (2-3 days) More prolonged after stapedectomy compared to stapedotomy

Due to serous labyrinthitis With or without perilymphatic fistula

Medialization of the prosthesis into the vestibule

Reparative granuloma

Recurrent Conductive Hearing Loss

Slippage or displacement of the prosthesis

Most common cause of failure Immediate

Technique Trauma 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

Delayed

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

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

Medical

Sodium Fluoride

Reduces tinnitus, reverses Schwartzes 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 + Schwartzes sign

Treat for 6 mo pre-op Postop if otospongiosis detected intra-op

Medical

Sodium fluoride

Hearing results
50% stabilize 30% improve

Re-evaluate q 2 yrs with CT and for Schwartzes sign to resolve If fluoride are stopped expect re-activation within 2-3 years

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.

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. Quinns 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.