OtosclerosisOtosclerosis

Department of OtorhinolaryngoglogyDepartment of Otorhinolaryngoglogy

the 2nd Hospital affliatted to Medical college the 2nd Hospital affliatted to Medical college

Zhejiang UniversityZhejiang University

Xu YapingXu Yaping

Introduction

bull Otosclerosis

1 Primary metabolic bone disease of the otic capsule and ossicles

2 Results in fixation of the ossicles and conductive hearing loss

3 May have sensorineural component if the cochlea is involved

1048708Genetically mediated via autosomal dominant transmission with incomplete penetrance (40) and variable expressivity

History of Otosclerosis and Stapes surgery

1048708 1704 ndash Valsalva first described stapes fixation

1048708 1857 ndash Toynbee linked stapes fixation to hearing loss

1048708 1890 ndash Katz was first to find microscopic evidence of

otosclerosis

1048708 1893 ndash Politzer described the clinical entity of

ldquootosclerosisrdquo

1048708 1890 ndash Bacon describes medical therapy for the

condition and supports the common view that

ldquosurgery should not be considered for a momentldquo

Epidemiology

bull 10 overall prevalence of histologic otosclerosis

bull 1 overall prevalence of clinically significant otosclerosis

bull Clinical otosclerosis ndashndash21 (WM)

bull Possible progression during pregnancy (10-17)

bull Bilaterality more common (89 vs 65)

bull 15-45 most common age range of presentation increases with age

Pathophysiology

bull Osseous dyscrasia

1 Resorption and formation of new bone

2 Limited to the temporal bone and ossicles

3 Inciting event unknown

many theories

Hereditary endocrine metabolic infectious vascular autoimmunehormonal

bull Most common sites of involvement

1 1048708Fissula ante fenestrum

2 1048708Round window niche (30-50 of cases)

3 1048708Anterior wall of the IAC

Histology

otosclerosis has two main forms

1 an early of spongiotic phase (otospongiosis)

multiple active cell groups including

osteocytes osteoblasts and histiocytes

2 a late or sclerotic phase dense sclerotic bone forms

Non-clinical foci of otosclerosis

Bipolar involvement of the footplate

Round Window

Diagnosis of Otosclerosis

10487081 Most common presentation

1048708Women age 20 - 30

10487082 Conductive or Mixed hearing loss

slowly progressivebilateral (80)asymmetric

1048708Tinnitus (75)

a complete history

1 Age of onset of hearing loss

2 Progression

3 Laterality

4 Associated symptomsbull Dizzinessbull Otalgiabull Otorrheabull Tinnitus

bull Family history 104870823 have a significant family history 1048708Particularly helpful in patients with severe or profound mixed hearing loss

bull Prior otologic surgerybull History of ear infectionsbull Vestibular symptoms 104870825 1048708Most commonly dysequilibrium 1048708Occasionally attacks of vertigo with rotatory nystagmu

s

Physical Exam

bull Otomicroscopy

Most helpful in ruling out other disorders

1048708Middle ear effusions

1048708Tympanosclerosis

1048708Tympanic membrane perforations

1048708Cholesteatoma or retraction pockets

1048708Superior semicircular canal dehiscence

Schwartzersquorsquos signs ( by Schwartze in 1873)

1048708Red hue behind the tympanic membrane (in oval window niche area)

104870810 of casesbull Pneumatic otoscopy

1048708Distinguish from malleus fixation

bull Tuning forks10487081 Hearing loss progresses form low frequencies to high

frequencies1048708 2 256 512 and 1024 Hz TF should be used 3 Rinne1048708256 Hz ndashndashnegative test indicates at least a 20 dB ABG 1048708512 Hz ndashndashnegative test indicates at least a 25 dB ABG (air-bone gaps)

Differential Diagnosis

1Ossicular discontinuityAconductive loss of 60 db

B type Ad tympanogram

2Congenital stapes fixationA25 incidence of other

congenital anomalies

B non-progressive CHL

3Malleus head fixation when congenital associated with other stigmata (aural atresia)

4Pagetrsquorsquos disease diffuse involvement of the bony skeleton

5Osteogenesis imperfecta presence of blue sclera

6Superior semicircular canal dehiscence vertigo or eye movements with loud noise

Audiometry

1048708Tympanometry

1048708Impedance testing

Acoustic reflexes

1048708Pure tones

As (s-stiffness curve) tympanogram is characteristic of advanced otosclerosis

Acoustic Reflexes

bull Otosclerosis has a predictable pattern of abnormal reflexes over time

1 Reduced reflex amplitude

2 Elevation of ipsilateral thresholds

3 Elevation of contralateral thresholds

4 Absence of reflexes

Pure Tone Audiometry

bull Most useful audiometric test for otosclerosis 1048708Characterizes the severity of disease 1048708Frequency specific

bull Carhartrsquorsquos notch 1048708Hallmark audiologic sign of otosclerosis 1048708Decrease in bone conduction thresholds 10487085 dB at 500 Hz 104870810 dB at 1000 Hz 104870815 dB at 2000 Hz 10487085 dB at 4000 Hz

early stage

middle stage

bull late stage

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

Introduction

bull Otosclerosis

1 Primary metabolic bone disease of the otic capsule and ossicles

2 Results in fixation of the ossicles and conductive hearing loss

3 May have sensorineural component if the cochlea is involved

1048708Genetically mediated via autosomal dominant transmission with incomplete penetrance (40) and variable expressivity

History of Otosclerosis and Stapes surgery

1048708 1704 ndash Valsalva first described stapes fixation

1048708 1857 ndash Toynbee linked stapes fixation to hearing loss

1048708 1890 ndash Katz was first to find microscopic evidence of

otosclerosis

1048708 1893 ndash Politzer described the clinical entity of

ldquootosclerosisrdquo

1048708 1890 ndash Bacon describes medical therapy for the

condition and supports the common view that

ldquosurgery should not be considered for a momentldquo

Epidemiology

bull 10 overall prevalence of histologic otosclerosis

bull 1 overall prevalence of clinically significant otosclerosis

bull Clinical otosclerosis ndashndash21 (WM)

bull Possible progression during pregnancy (10-17)

bull Bilaterality more common (89 vs 65)

bull 15-45 most common age range of presentation increases with age

Pathophysiology

bull Osseous dyscrasia

1 Resorption and formation of new bone

2 Limited to the temporal bone and ossicles

3 Inciting event unknown

many theories

Hereditary endocrine metabolic infectious vascular autoimmunehormonal

bull Most common sites of involvement

1 1048708Fissula ante fenestrum

2 1048708Round window niche (30-50 of cases)

3 1048708Anterior wall of the IAC

Histology

otosclerosis has two main forms

1 an early of spongiotic phase (otospongiosis)

multiple active cell groups including

osteocytes osteoblasts and histiocytes

2 a late or sclerotic phase dense sclerotic bone forms

Non-clinical foci of otosclerosis

Bipolar involvement of the footplate

Round Window

Diagnosis of Otosclerosis

10487081 Most common presentation

1048708Women age 20 - 30

10487082 Conductive or Mixed hearing loss

slowly progressivebilateral (80)asymmetric

1048708Tinnitus (75)

a complete history

1 Age of onset of hearing loss

2 Progression

3 Laterality

4 Associated symptomsbull Dizzinessbull Otalgiabull Otorrheabull Tinnitus

bull Family history 104870823 have a significant family history 1048708Particularly helpful in patients with severe or profound mixed hearing loss

bull Prior otologic surgerybull History of ear infectionsbull Vestibular symptoms 104870825 1048708Most commonly dysequilibrium 1048708Occasionally attacks of vertigo with rotatory nystagmu

s

Physical Exam

bull Otomicroscopy

Most helpful in ruling out other disorders

1048708Middle ear effusions

1048708Tympanosclerosis

1048708Tympanic membrane perforations

1048708Cholesteatoma or retraction pockets

1048708Superior semicircular canal dehiscence

Schwartzersquorsquos signs ( by Schwartze in 1873)

1048708Red hue behind the tympanic membrane (in oval window niche area)

104870810 of casesbull Pneumatic otoscopy

1048708Distinguish from malleus fixation

bull Tuning forks10487081 Hearing loss progresses form low frequencies to high

frequencies1048708 2 256 512 and 1024 Hz TF should be used 3 Rinne1048708256 Hz ndashndashnegative test indicates at least a 20 dB ABG 1048708512 Hz ndashndashnegative test indicates at least a 25 dB ABG (air-bone gaps)

Differential Diagnosis

1Ossicular discontinuityAconductive loss of 60 db

B type Ad tympanogram

2Congenital stapes fixationA25 incidence of other

congenital anomalies

B non-progressive CHL

3Malleus head fixation when congenital associated with other stigmata (aural atresia)

4Pagetrsquorsquos disease diffuse involvement of the bony skeleton

5Osteogenesis imperfecta presence of blue sclera

6Superior semicircular canal dehiscence vertigo or eye movements with loud noise

Audiometry

1048708Tympanometry

1048708Impedance testing

Acoustic reflexes

1048708Pure tones

As (s-stiffness curve) tympanogram is characteristic of advanced otosclerosis

Acoustic Reflexes

bull Otosclerosis has a predictable pattern of abnormal reflexes over time

1 Reduced reflex amplitude

2 Elevation of ipsilateral thresholds

3 Elevation of contralateral thresholds

4 Absence of reflexes

Pure Tone Audiometry

bull Most useful audiometric test for otosclerosis 1048708Characterizes the severity of disease 1048708Frequency specific

bull Carhartrsquorsquos notch 1048708Hallmark audiologic sign of otosclerosis 1048708Decrease in bone conduction thresholds 10487085 dB at 500 Hz 104870810 dB at 1000 Hz 104870815 dB at 2000 Hz 10487085 dB at 4000 Hz

early stage

middle stage

bull late stage

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

History of Otosclerosis and Stapes surgery

1048708 1704 ndash Valsalva first described stapes fixation

1048708 1857 ndash Toynbee linked stapes fixation to hearing loss

1048708 1890 ndash Katz was first to find microscopic evidence of

otosclerosis

1048708 1893 ndash Politzer described the clinical entity of

ldquootosclerosisrdquo

1048708 1890 ndash Bacon describes medical therapy for the

condition and supports the common view that

ldquosurgery should not be considered for a momentldquo

Epidemiology

bull 10 overall prevalence of histologic otosclerosis

bull 1 overall prevalence of clinically significant otosclerosis

bull Clinical otosclerosis ndashndash21 (WM)

bull Possible progression during pregnancy (10-17)

bull Bilaterality more common (89 vs 65)

bull 15-45 most common age range of presentation increases with age

Pathophysiology

bull Osseous dyscrasia

1 Resorption and formation of new bone

2 Limited to the temporal bone and ossicles

3 Inciting event unknown

many theories

Hereditary endocrine metabolic infectious vascular autoimmunehormonal

bull Most common sites of involvement

1 1048708Fissula ante fenestrum

2 1048708Round window niche (30-50 of cases)

3 1048708Anterior wall of the IAC

Histology

otosclerosis has two main forms

1 an early of spongiotic phase (otospongiosis)

multiple active cell groups including

osteocytes osteoblasts and histiocytes

2 a late or sclerotic phase dense sclerotic bone forms

Non-clinical foci of otosclerosis

Bipolar involvement of the footplate

Round Window

Diagnosis of Otosclerosis

10487081 Most common presentation

1048708Women age 20 - 30

10487082 Conductive or Mixed hearing loss

slowly progressivebilateral (80)asymmetric

1048708Tinnitus (75)

a complete history

1 Age of onset of hearing loss

2 Progression

3 Laterality

4 Associated symptomsbull Dizzinessbull Otalgiabull Otorrheabull Tinnitus

bull Family history 104870823 have a significant family history 1048708Particularly helpful in patients with severe or profound mixed hearing loss

bull Prior otologic surgerybull History of ear infectionsbull Vestibular symptoms 104870825 1048708Most commonly dysequilibrium 1048708Occasionally attacks of vertigo with rotatory nystagmu

s

Physical Exam

bull Otomicroscopy

Most helpful in ruling out other disorders

1048708Middle ear effusions

1048708Tympanosclerosis

1048708Tympanic membrane perforations

1048708Cholesteatoma or retraction pockets

1048708Superior semicircular canal dehiscence

Schwartzersquorsquos signs ( by Schwartze in 1873)

1048708Red hue behind the tympanic membrane (in oval window niche area)

104870810 of casesbull Pneumatic otoscopy

1048708Distinguish from malleus fixation

bull Tuning forks10487081 Hearing loss progresses form low frequencies to high

frequencies1048708 2 256 512 and 1024 Hz TF should be used 3 Rinne1048708256 Hz ndashndashnegative test indicates at least a 20 dB ABG 1048708512 Hz ndashndashnegative test indicates at least a 25 dB ABG (air-bone gaps)

Differential Diagnosis

1Ossicular discontinuityAconductive loss of 60 db

B type Ad tympanogram

2Congenital stapes fixationA25 incidence of other

congenital anomalies

B non-progressive CHL

3Malleus head fixation when congenital associated with other stigmata (aural atresia)

4Pagetrsquorsquos disease diffuse involvement of the bony skeleton

5Osteogenesis imperfecta presence of blue sclera

6Superior semicircular canal dehiscence vertigo or eye movements with loud noise

Audiometry

1048708Tympanometry

1048708Impedance testing

Acoustic reflexes

1048708Pure tones

As (s-stiffness curve) tympanogram is characteristic of advanced otosclerosis

Acoustic Reflexes

bull Otosclerosis has a predictable pattern of abnormal reflexes over time

1 Reduced reflex amplitude

2 Elevation of ipsilateral thresholds

3 Elevation of contralateral thresholds

4 Absence of reflexes

Pure Tone Audiometry

bull Most useful audiometric test for otosclerosis 1048708Characterizes the severity of disease 1048708Frequency specific

bull Carhartrsquorsquos notch 1048708Hallmark audiologic sign of otosclerosis 1048708Decrease in bone conduction thresholds 10487085 dB at 500 Hz 104870810 dB at 1000 Hz 104870815 dB at 2000 Hz 10487085 dB at 4000 Hz

early stage

middle stage

bull late stage

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

Epidemiology

bull 10 overall prevalence of histologic otosclerosis

bull 1 overall prevalence of clinically significant otosclerosis

bull Clinical otosclerosis ndashndash21 (WM)

bull Possible progression during pregnancy (10-17)

bull Bilaterality more common (89 vs 65)

bull 15-45 most common age range of presentation increases with age

Pathophysiology

bull Osseous dyscrasia

1 Resorption and formation of new bone

2 Limited to the temporal bone and ossicles

3 Inciting event unknown

many theories

Hereditary endocrine metabolic infectious vascular autoimmunehormonal

bull Most common sites of involvement

1 1048708Fissula ante fenestrum

2 1048708Round window niche (30-50 of cases)

3 1048708Anterior wall of the IAC

Histology

otosclerosis has two main forms

1 an early of spongiotic phase (otospongiosis)

multiple active cell groups including

osteocytes osteoblasts and histiocytes

2 a late or sclerotic phase dense sclerotic bone forms

Non-clinical foci of otosclerosis

Bipolar involvement of the footplate

Round Window

Diagnosis of Otosclerosis

10487081 Most common presentation

1048708Women age 20 - 30

10487082 Conductive or Mixed hearing loss

slowly progressivebilateral (80)asymmetric

1048708Tinnitus (75)

a complete history

1 Age of onset of hearing loss

2 Progression

3 Laterality

4 Associated symptomsbull Dizzinessbull Otalgiabull Otorrheabull Tinnitus

bull Family history 104870823 have a significant family history 1048708Particularly helpful in patients with severe or profound mixed hearing loss

bull Prior otologic surgerybull History of ear infectionsbull Vestibular symptoms 104870825 1048708Most commonly dysequilibrium 1048708Occasionally attacks of vertigo with rotatory nystagmu

s

Physical Exam

bull Otomicroscopy

Most helpful in ruling out other disorders

1048708Middle ear effusions

1048708Tympanosclerosis

1048708Tympanic membrane perforations

1048708Cholesteatoma or retraction pockets

1048708Superior semicircular canal dehiscence

Schwartzersquorsquos signs ( by Schwartze in 1873)

1048708Red hue behind the tympanic membrane (in oval window niche area)

104870810 of casesbull Pneumatic otoscopy

1048708Distinguish from malleus fixation

bull Tuning forks10487081 Hearing loss progresses form low frequencies to high

frequencies1048708 2 256 512 and 1024 Hz TF should be used 3 Rinne1048708256 Hz ndashndashnegative test indicates at least a 20 dB ABG 1048708512 Hz ndashndashnegative test indicates at least a 25 dB ABG (air-bone gaps)

Differential Diagnosis

1Ossicular discontinuityAconductive loss of 60 db

B type Ad tympanogram

2Congenital stapes fixationA25 incidence of other

congenital anomalies

B non-progressive CHL

3Malleus head fixation when congenital associated with other stigmata (aural atresia)

4Pagetrsquorsquos disease diffuse involvement of the bony skeleton

5Osteogenesis imperfecta presence of blue sclera

6Superior semicircular canal dehiscence vertigo or eye movements with loud noise

Audiometry

1048708Tympanometry

1048708Impedance testing

Acoustic reflexes

1048708Pure tones

As (s-stiffness curve) tympanogram is characteristic of advanced otosclerosis

Acoustic Reflexes

bull Otosclerosis has a predictable pattern of abnormal reflexes over time

1 Reduced reflex amplitude

2 Elevation of ipsilateral thresholds

3 Elevation of contralateral thresholds

4 Absence of reflexes

Pure Tone Audiometry

bull Most useful audiometric test for otosclerosis 1048708Characterizes the severity of disease 1048708Frequency specific

bull Carhartrsquorsquos notch 1048708Hallmark audiologic sign of otosclerosis 1048708Decrease in bone conduction thresholds 10487085 dB at 500 Hz 104870810 dB at 1000 Hz 104870815 dB at 2000 Hz 10487085 dB at 4000 Hz

early stage

middle stage

bull late stage

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

Pathophysiology

bull Osseous dyscrasia

1 Resorption and formation of new bone

2 Limited to the temporal bone and ossicles

3 Inciting event unknown

many theories

Hereditary endocrine metabolic infectious vascular autoimmunehormonal

bull Most common sites of involvement

1 1048708Fissula ante fenestrum

2 1048708Round window niche (30-50 of cases)

3 1048708Anterior wall of the IAC

Histology

otosclerosis has two main forms

1 an early of spongiotic phase (otospongiosis)

multiple active cell groups including

osteocytes osteoblasts and histiocytes

2 a late or sclerotic phase dense sclerotic bone forms

Non-clinical foci of otosclerosis

Bipolar involvement of the footplate

Round Window

Diagnosis of Otosclerosis

10487081 Most common presentation

1048708Women age 20 - 30

10487082 Conductive or Mixed hearing loss

slowly progressivebilateral (80)asymmetric

1048708Tinnitus (75)

a complete history

1 Age of onset of hearing loss

2 Progression

3 Laterality

4 Associated symptomsbull Dizzinessbull Otalgiabull Otorrheabull Tinnitus

bull Family history 104870823 have a significant family history 1048708Particularly helpful in patients with severe or profound mixed hearing loss

bull Prior otologic surgerybull History of ear infectionsbull Vestibular symptoms 104870825 1048708Most commonly dysequilibrium 1048708Occasionally attacks of vertigo with rotatory nystagmu

s

Physical Exam

bull Otomicroscopy

Most helpful in ruling out other disorders

1048708Middle ear effusions

1048708Tympanosclerosis

1048708Tympanic membrane perforations

1048708Cholesteatoma or retraction pockets

1048708Superior semicircular canal dehiscence

Schwartzersquorsquos signs ( by Schwartze in 1873)

1048708Red hue behind the tympanic membrane (in oval window niche area)

104870810 of casesbull Pneumatic otoscopy

1048708Distinguish from malleus fixation

bull Tuning forks10487081 Hearing loss progresses form low frequencies to high

frequencies1048708 2 256 512 and 1024 Hz TF should be used 3 Rinne1048708256 Hz ndashndashnegative test indicates at least a 20 dB ABG 1048708512 Hz ndashndashnegative test indicates at least a 25 dB ABG (air-bone gaps)

Differential Diagnosis

1Ossicular discontinuityAconductive loss of 60 db

B type Ad tympanogram

2Congenital stapes fixationA25 incidence of other

congenital anomalies

B non-progressive CHL

3Malleus head fixation when congenital associated with other stigmata (aural atresia)

4Pagetrsquorsquos disease diffuse involvement of the bony skeleton

5Osteogenesis imperfecta presence of blue sclera

6Superior semicircular canal dehiscence vertigo or eye movements with loud noise

Audiometry

1048708Tympanometry

1048708Impedance testing

Acoustic reflexes

1048708Pure tones

As (s-stiffness curve) tympanogram is characteristic of advanced otosclerosis

Acoustic Reflexes

bull Otosclerosis has a predictable pattern of abnormal reflexes over time

1 Reduced reflex amplitude

2 Elevation of ipsilateral thresholds

3 Elevation of contralateral thresholds

4 Absence of reflexes

Pure Tone Audiometry

bull Most useful audiometric test for otosclerosis 1048708Characterizes the severity of disease 1048708Frequency specific

bull Carhartrsquorsquos notch 1048708Hallmark audiologic sign of otosclerosis 1048708Decrease in bone conduction thresholds 10487085 dB at 500 Hz 104870810 dB at 1000 Hz 104870815 dB at 2000 Hz 10487085 dB at 4000 Hz

early stage

middle stage

bull late stage

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

bull Most common sites of involvement

1 1048708Fissula ante fenestrum

2 1048708Round window niche (30-50 of cases)

3 1048708Anterior wall of the IAC

Histology

otosclerosis has two main forms

1 an early of spongiotic phase (otospongiosis)

multiple active cell groups including

osteocytes osteoblasts and histiocytes

2 a late or sclerotic phase dense sclerotic bone forms

Non-clinical foci of otosclerosis

Bipolar involvement of the footplate

Round Window

Diagnosis of Otosclerosis

10487081 Most common presentation

1048708Women age 20 - 30

10487082 Conductive or Mixed hearing loss

slowly progressivebilateral (80)asymmetric

1048708Tinnitus (75)

a complete history

1 Age of onset of hearing loss

2 Progression

3 Laterality

4 Associated symptomsbull Dizzinessbull Otalgiabull Otorrheabull Tinnitus

bull Family history 104870823 have a significant family history 1048708Particularly helpful in patients with severe or profound mixed hearing loss

bull Prior otologic surgerybull History of ear infectionsbull Vestibular symptoms 104870825 1048708Most commonly dysequilibrium 1048708Occasionally attacks of vertigo with rotatory nystagmu

s

Physical Exam

bull Otomicroscopy

Most helpful in ruling out other disorders

1048708Middle ear effusions

1048708Tympanosclerosis

1048708Tympanic membrane perforations

1048708Cholesteatoma or retraction pockets

1048708Superior semicircular canal dehiscence

Schwartzersquorsquos signs ( by Schwartze in 1873)

1048708Red hue behind the tympanic membrane (in oval window niche area)

104870810 of casesbull Pneumatic otoscopy

1048708Distinguish from malleus fixation

bull Tuning forks10487081 Hearing loss progresses form low frequencies to high

frequencies1048708 2 256 512 and 1024 Hz TF should be used 3 Rinne1048708256 Hz ndashndashnegative test indicates at least a 20 dB ABG 1048708512 Hz ndashndashnegative test indicates at least a 25 dB ABG (air-bone gaps)

Differential Diagnosis

1Ossicular discontinuityAconductive loss of 60 db

B type Ad tympanogram

2Congenital stapes fixationA25 incidence of other

congenital anomalies

B non-progressive CHL

3Malleus head fixation when congenital associated with other stigmata (aural atresia)

4Pagetrsquorsquos disease diffuse involvement of the bony skeleton

5Osteogenesis imperfecta presence of blue sclera

6Superior semicircular canal dehiscence vertigo or eye movements with loud noise

Audiometry

1048708Tympanometry

1048708Impedance testing

Acoustic reflexes

1048708Pure tones

As (s-stiffness curve) tympanogram is characteristic of advanced otosclerosis

Acoustic Reflexes

bull Otosclerosis has a predictable pattern of abnormal reflexes over time

1 Reduced reflex amplitude

2 Elevation of ipsilateral thresholds

3 Elevation of contralateral thresholds

4 Absence of reflexes

Pure Tone Audiometry

bull Most useful audiometric test for otosclerosis 1048708Characterizes the severity of disease 1048708Frequency specific

bull Carhartrsquorsquos notch 1048708Hallmark audiologic sign of otosclerosis 1048708Decrease in bone conduction thresholds 10487085 dB at 500 Hz 104870810 dB at 1000 Hz 104870815 dB at 2000 Hz 10487085 dB at 4000 Hz

early stage

middle stage

bull late stage

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

Histology

otosclerosis has two main forms

1 an early of spongiotic phase (otospongiosis)

multiple active cell groups including

osteocytes osteoblasts and histiocytes

2 a late or sclerotic phase dense sclerotic bone forms

Non-clinical foci of otosclerosis

Bipolar involvement of the footplate

Round Window

Diagnosis of Otosclerosis

10487081 Most common presentation

1048708Women age 20 - 30

10487082 Conductive or Mixed hearing loss

slowly progressivebilateral (80)asymmetric

1048708Tinnitus (75)

a complete history

1 Age of onset of hearing loss

2 Progression

3 Laterality

4 Associated symptomsbull Dizzinessbull Otalgiabull Otorrheabull Tinnitus

bull Family history 104870823 have a significant family history 1048708Particularly helpful in patients with severe or profound mixed hearing loss

bull Prior otologic surgerybull History of ear infectionsbull Vestibular symptoms 104870825 1048708Most commonly dysequilibrium 1048708Occasionally attacks of vertigo with rotatory nystagmu

s

Physical Exam

bull Otomicroscopy

Most helpful in ruling out other disorders

1048708Middle ear effusions

1048708Tympanosclerosis

1048708Tympanic membrane perforations

1048708Cholesteatoma or retraction pockets

1048708Superior semicircular canal dehiscence

Schwartzersquorsquos signs ( by Schwartze in 1873)

1048708Red hue behind the tympanic membrane (in oval window niche area)

104870810 of casesbull Pneumatic otoscopy

1048708Distinguish from malleus fixation

bull Tuning forks10487081 Hearing loss progresses form low frequencies to high

frequencies1048708 2 256 512 and 1024 Hz TF should be used 3 Rinne1048708256 Hz ndashndashnegative test indicates at least a 20 dB ABG 1048708512 Hz ndashndashnegative test indicates at least a 25 dB ABG (air-bone gaps)

Differential Diagnosis

1Ossicular discontinuityAconductive loss of 60 db

B type Ad tympanogram

2Congenital stapes fixationA25 incidence of other

congenital anomalies

B non-progressive CHL

3Malleus head fixation when congenital associated with other stigmata (aural atresia)

4Pagetrsquorsquos disease diffuse involvement of the bony skeleton

5Osteogenesis imperfecta presence of blue sclera

6Superior semicircular canal dehiscence vertigo or eye movements with loud noise

Audiometry

1048708Tympanometry

1048708Impedance testing

Acoustic reflexes

1048708Pure tones

As (s-stiffness curve) tympanogram is characteristic of advanced otosclerosis

Acoustic Reflexes

bull Otosclerosis has a predictable pattern of abnormal reflexes over time

1 Reduced reflex amplitude

2 Elevation of ipsilateral thresholds

3 Elevation of contralateral thresholds

4 Absence of reflexes

Pure Tone Audiometry

bull Most useful audiometric test for otosclerosis 1048708Characterizes the severity of disease 1048708Frequency specific

bull Carhartrsquorsquos notch 1048708Hallmark audiologic sign of otosclerosis 1048708Decrease in bone conduction thresholds 10487085 dB at 500 Hz 104870810 dB at 1000 Hz 104870815 dB at 2000 Hz 10487085 dB at 4000 Hz

early stage

middle stage

bull late stage

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

Non-clinical foci of otosclerosis

Bipolar involvement of the footplate

Round Window

Diagnosis of Otosclerosis

10487081 Most common presentation

1048708Women age 20 - 30

10487082 Conductive or Mixed hearing loss

slowly progressivebilateral (80)asymmetric

1048708Tinnitus (75)

a complete history

1 Age of onset of hearing loss

2 Progression

3 Laterality

4 Associated symptomsbull Dizzinessbull Otalgiabull Otorrheabull Tinnitus

bull Family history 104870823 have a significant family history 1048708Particularly helpful in patients with severe or profound mixed hearing loss

bull Prior otologic surgerybull History of ear infectionsbull Vestibular symptoms 104870825 1048708Most commonly dysequilibrium 1048708Occasionally attacks of vertigo with rotatory nystagmu

s

Physical Exam

bull Otomicroscopy

Most helpful in ruling out other disorders

1048708Middle ear effusions

1048708Tympanosclerosis

1048708Tympanic membrane perforations

1048708Cholesteatoma or retraction pockets

1048708Superior semicircular canal dehiscence

Schwartzersquorsquos signs ( by Schwartze in 1873)

1048708Red hue behind the tympanic membrane (in oval window niche area)

104870810 of casesbull Pneumatic otoscopy

1048708Distinguish from malleus fixation

bull Tuning forks10487081 Hearing loss progresses form low frequencies to high

frequencies1048708 2 256 512 and 1024 Hz TF should be used 3 Rinne1048708256 Hz ndashndashnegative test indicates at least a 20 dB ABG 1048708512 Hz ndashndashnegative test indicates at least a 25 dB ABG (air-bone gaps)

Differential Diagnosis

1Ossicular discontinuityAconductive loss of 60 db

B type Ad tympanogram

2Congenital stapes fixationA25 incidence of other

congenital anomalies

B non-progressive CHL

3Malleus head fixation when congenital associated with other stigmata (aural atresia)

4Pagetrsquorsquos disease diffuse involvement of the bony skeleton

5Osteogenesis imperfecta presence of blue sclera

6Superior semicircular canal dehiscence vertigo or eye movements with loud noise

Audiometry

1048708Tympanometry

1048708Impedance testing

Acoustic reflexes

1048708Pure tones

As (s-stiffness curve) tympanogram is characteristic of advanced otosclerosis

Acoustic Reflexes

bull Otosclerosis has a predictable pattern of abnormal reflexes over time

1 Reduced reflex amplitude

2 Elevation of ipsilateral thresholds

3 Elevation of contralateral thresholds

4 Absence of reflexes

Pure Tone Audiometry

bull Most useful audiometric test for otosclerosis 1048708Characterizes the severity of disease 1048708Frequency specific

bull Carhartrsquorsquos notch 1048708Hallmark audiologic sign of otosclerosis 1048708Decrease in bone conduction thresholds 10487085 dB at 500 Hz 104870810 dB at 1000 Hz 104870815 dB at 2000 Hz 10487085 dB at 4000 Hz

early stage

middle stage

bull late stage

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

Bipolar involvement of the footplate

Round Window

Diagnosis of Otosclerosis

10487081 Most common presentation

1048708Women age 20 - 30

10487082 Conductive or Mixed hearing loss

slowly progressivebilateral (80)asymmetric

1048708Tinnitus (75)

a complete history

1 Age of onset of hearing loss

2 Progression

3 Laterality

4 Associated symptomsbull Dizzinessbull Otalgiabull Otorrheabull Tinnitus

bull Family history 104870823 have a significant family history 1048708Particularly helpful in patients with severe or profound mixed hearing loss

bull Prior otologic surgerybull History of ear infectionsbull Vestibular symptoms 104870825 1048708Most commonly dysequilibrium 1048708Occasionally attacks of vertigo with rotatory nystagmu

s

Physical Exam

bull Otomicroscopy

Most helpful in ruling out other disorders

1048708Middle ear effusions

1048708Tympanosclerosis

1048708Tympanic membrane perforations

1048708Cholesteatoma or retraction pockets

1048708Superior semicircular canal dehiscence

Schwartzersquorsquos signs ( by Schwartze in 1873)

1048708Red hue behind the tympanic membrane (in oval window niche area)

104870810 of casesbull Pneumatic otoscopy

1048708Distinguish from malleus fixation

bull Tuning forks10487081 Hearing loss progresses form low frequencies to high

frequencies1048708 2 256 512 and 1024 Hz TF should be used 3 Rinne1048708256 Hz ndashndashnegative test indicates at least a 20 dB ABG 1048708512 Hz ndashndashnegative test indicates at least a 25 dB ABG (air-bone gaps)

Differential Diagnosis

1Ossicular discontinuityAconductive loss of 60 db

B type Ad tympanogram

2Congenital stapes fixationA25 incidence of other

congenital anomalies

B non-progressive CHL

3Malleus head fixation when congenital associated with other stigmata (aural atresia)

4Pagetrsquorsquos disease diffuse involvement of the bony skeleton

5Osteogenesis imperfecta presence of blue sclera

6Superior semicircular canal dehiscence vertigo or eye movements with loud noise

Audiometry

1048708Tympanometry

1048708Impedance testing

Acoustic reflexes

1048708Pure tones

As (s-stiffness curve) tympanogram is characteristic of advanced otosclerosis

Acoustic Reflexes

bull Otosclerosis has a predictable pattern of abnormal reflexes over time

1 Reduced reflex amplitude

2 Elevation of ipsilateral thresholds

3 Elevation of contralateral thresholds

4 Absence of reflexes

Pure Tone Audiometry

bull Most useful audiometric test for otosclerosis 1048708Characterizes the severity of disease 1048708Frequency specific

bull Carhartrsquorsquos notch 1048708Hallmark audiologic sign of otosclerosis 1048708Decrease in bone conduction thresholds 10487085 dB at 500 Hz 104870810 dB at 1000 Hz 104870815 dB at 2000 Hz 10487085 dB at 4000 Hz

early stage

middle stage

bull late stage

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

Round Window

Diagnosis of Otosclerosis

10487081 Most common presentation

1048708Women age 20 - 30

10487082 Conductive or Mixed hearing loss

slowly progressivebilateral (80)asymmetric

1048708Tinnitus (75)

a complete history

1 Age of onset of hearing loss

2 Progression

3 Laterality

4 Associated symptomsbull Dizzinessbull Otalgiabull Otorrheabull Tinnitus

bull Family history 104870823 have a significant family history 1048708Particularly helpful in patients with severe or profound mixed hearing loss

bull Prior otologic surgerybull History of ear infectionsbull Vestibular symptoms 104870825 1048708Most commonly dysequilibrium 1048708Occasionally attacks of vertigo with rotatory nystagmu

s

Physical Exam

bull Otomicroscopy

Most helpful in ruling out other disorders

1048708Middle ear effusions

1048708Tympanosclerosis

1048708Tympanic membrane perforations

1048708Cholesteatoma or retraction pockets

1048708Superior semicircular canal dehiscence

Schwartzersquorsquos signs ( by Schwartze in 1873)

1048708Red hue behind the tympanic membrane (in oval window niche area)

104870810 of casesbull Pneumatic otoscopy

1048708Distinguish from malleus fixation

bull Tuning forks10487081 Hearing loss progresses form low frequencies to high

frequencies1048708 2 256 512 and 1024 Hz TF should be used 3 Rinne1048708256 Hz ndashndashnegative test indicates at least a 20 dB ABG 1048708512 Hz ndashndashnegative test indicates at least a 25 dB ABG (air-bone gaps)

Differential Diagnosis

1Ossicular discontinuityAconductive loss of 60 db

B type Ad tympanogram

2Congenital stapes fixationA25 incidence of other

congenital anomalies

B non-progressive CHL

3Malleus head fixation when congenital associated with other stigmata (aural atresia)

4Pagetrsquorsquos disease diffuse involvement of the bony skeleton

5Osteogenesis imperfecta presence of blue sclera

6Superior semicircular canal dehiscence vertigo or eye movements with loud noise

Audiometry

1048708Tympanometry

1048708Impedance testing

Acoustic reflexes

1048708Pure tones

As (s-stiffness curve) tympanogram is characteristic of advanced otosclerosis

Acoustic Reflexes

bull Otosclerosis has a predictable pattern of abnormal reflexes over time

1 Reduced reflex amplitude

2 Elevation of ipsilateral thresholds

3 Elevation of contralateral thresholds

4 Absence of reflexes

Pure Tone Audiometry

bull Most useful audiometric test for otosclerosis 1048708Characterizes the severity of disease 1048708Frequency specific

bull Carhartrsquorsquos notch 1048708Hallmark audiologic sign of otosclerosis 1048708Decrease in bone conduction thresholds 10487085 dB at 500 Hz 104870810 dB at 1000 Hz 104870815 dB at 2000 Hz 10487085 dB at 4000 Hz

early stage

middle stage

bull late stage

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

Diagnosis of Otosclerosis

10487081 Most common presentation

1048708Women age 20 - 30

10487082 Conductive or Mixed hearing loss

slowly progressivebilateral (80)asymmetric

1048708Tinnitus (75)

a complete history

1 Age of onset of hearing loss

2 Progression

3 Laterality

4 Associated symptomsbull Dizzinessbull Otalgiabull Otorrheabull Tinnitus

bull Family history 104870823 have a significant family history 1048708Particularly helpful in patients with severe or profound mixed hearing loss

bull Prior otologic surgerybull History of ear infectionsbull Vestibular symptoms 104870825 1048708Most commonly dysequilibrium 1048708Occasionally attacks of vertigo with rotatory nystagmu

s

Physical Exam

bull Otomicroscopy

Most helpful in ruling out other disorders

1048708Middle ear effusions

1048708Tympanosclerosis

1048708Tympanic membrane perforations

1048708Cholesteatoma or retraction pockets

1048708Superior semicircular canal dehiscence

Schwartzersquorsquos signs ( by Schwartze in 1873)

1048708Red hue behind the tympanic membrane (in oval window niche area)

104870810 of casesbull Pneumatic otoscopy

1048708Distinguish from malleus fixation

bull Tuning forks10487081 Hearing loss progresses form low frequencies to high

frequencies1048708 2 256 512 and 1024 Hz TF should be used 3 Rinne1048708256 Hz ndashndashnegative test indicates at least a 20 dB ABG 1048708512 Hz ndashndashnegative test indicates at least a 25 dB ABG (air-bone gaps)

Differential Diagnosis

1Ossicular discontinuityAconductive loss of 60 db

B type Ad tympanogram

2Congenital stapes fixationA25 incidence of other

congenital anomalies

B non-progressive CHL

3Malleus head fixation when congenital associated with other stigmata (aural atresia)

4Pagetrsquorsquos disease diffuse involvement of the bony skeleton

5Osteogenesis imperfecta presence of blue sclera

6Superior semicircular canal dehiscence vertigo or eye movements with loud noise

Audiometry

1048708Tympanometry

1048708Impedance testing

Acoustic reflexes

1048708Pure tones

As (s-stiffness curve) tympanogram is characteristic of advanced otosclerosis

Acoustic Reflexes

bull Otosclerosis has a predictable pattern of abnormal reflexes over time

1 Reduced reflex amplitude

2 Elevation of ipsilateral thresholds

3 Elevation of contralateral thresholds

4 Absence of reflexes

Pure Tone Audiometry

bull Most useful audiometric test for otosclerosis 1048708Characterizes the severity of disease 1048708Frequency specific

bull Carhartrsquorsquos notch 1048708Hallmark audiologic sign of otosclerosis 1048708Decrease in bone conduction thresholds 10487085 dB at 500 Hz 104870810 dB at 1000 Hz 104870815 dB at 2000 Hz 10487085 dB at 4000 Hz

early stage

middle stage

bull late stage

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

a complete history

1 Age of onset of hearing loss

2 Progression

3 Laterality

4 Associated symptomsbull Dizzinessbull Otalgiabull Otorrheabull Tinnitus

bull Family history 104870823 have a significant family history 1048708Particularly helpful in patients with severe or profound mixed hearing loss

bull Prior otologic surgerybull History of ear infectionsbull Vestibular symptoms 104870825 1048708Most commonly dysequilibrium 1048708Occasionally attacks of vertigo with rotatory nystagmu

s

Physical Exam

bull Otomicroscopy

Most helpful in ruling out other disorders

1048708Middle ear effusions

1048708Tympanosclerosis

1048708Tympanic membrane perforations

1048708Cholesteatoma or retraction pockets

1048708Superior semicircular canal dehiscence

Schwartzersquorsquos signs ( by Schwartze in 1873)

1048708Red hue behind the tympanic membrane (in oval window niche area)

104870810 of casesbull Pneumatic otoscopy

1048708Distinguish from malleus fixation

bull Tuning forks10487081 Hearing loss progresses form low frequencies to high

frequencies1048708 2 256 512 and 1024 Hz TF should be used 3 Rinne1048708256 Hz ndashndashnegative test indicates at least a 20 dB ABG 1048708512 Hz ndashndashnegative test indicates at least a 25 dB ABG (air-bone gaps)

Differential Diagnosis

1Ossicular discontinuityAconductive loss of 60 db

B type Ad tympanogram

2Congenital stapes fixationA25 incidence of other

congenital anomalies

B non-progressive CHL

3Malleus head fixation when congenital associated with other stigmata (aural atresia)

4Pagetrsquorsquos disease diffuse involvement of the bony skeleton

5Osteogenesis imperfecta presence of blue sclera

6Superior semicircular canal dehiscence vertigo or eye movements with loud noise

Audiometry

1048708Tympanometry

1048708Impedance testing

Acoustic reflexes

1048708Pure tones

As (s-stiffness curve) tympanogram is characteristic of advanced otosclerosis

Acoustic Reflexes

bull Otosclerosis has a predictable pattern of abnormal reflexes over time

1 Reduced reflex amplitude

2 Elevation of ipsilateral thresholds

3 Elevation of contralateral thresholds

4 Absence of reflexes

Pure Tone Audiometry

bull Most useful audiometric test for otosclerosis 1048708Characterizes the severity of disease 1048708Frequency specific

bull Carhartrsquorsquos notch 1048708Hallmark audiologic sign of otosclerosis 1048708Decrease in bone conduction thresholds 10487085 dB at 500 Hz 104870810 dB at 1000 Hz 104870815 dB at 2000 Hz 10487085 dB at 4000 Hz

early stage

middle stage

bull late stage

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

bull Family history 104870823 have a significant family history 1048708Particularly helpful in patients with severe or profound mixed hearing loss

bull Prior otologic surgerybull History of ear infectionsbull Vestibular symptoms 104870825 1048708Most commonly dysequilibrium 1048708Occasionally attacks of vertigo with rotatory nystagmu

s

Physical Exam

bull Otomicroscopy

Most helpful in ruling out other disorders

1048708Middle ear effusions

1048708Tympanosclerosis

1048708Tympanic membrane perforations

1048708Cholesteatoma or retraction pockets

1048708Superior semicircular canal dehiscence

Schwartzersquorsquos signs ( by Schwartze in 1873)

1048708Red hue behind the tympanic membrane (in oval window niche area)

104870810 of casesbull Pneumatic otoscopy

1048708Distinguish from malleus fixation

bull Tuning forks10487081 Hearing loss progresses form low frequencies to high

frequencies1048708 2 256 512 and 1024 Hz TF should be used 3 Rinne1048708256 Hz ndashndashnegative test indicates at least a 20 dB ABG 1048708512 Hz ndashndashnegative test indicates at least a 25 dB ABG (air-bone gaps)

Differential Diagnosis

1Ossicular discontinuityAconductive loss of 60 db

B type Ad tympanogram

2Congenital stapes fixationA25 incidence of other

congenital anomalies

B non-progressive CHL

3Malleus head fixation when congenital associated with other stigmata (aural atresia)

4Pagetrsquorsquos disease diffuse involvement of the bony skeleton

5Osteogenesis imperfecta presence of blue sclera

6Superior semicircular canal dehiscence vertigo or eye movements with loud noise

Audiometry

1048708Tympanometry

1048708Impedance testing

Acoustic reflexes

1048708Pure tones

As (s-stiffness curve) tympanogram is characteristic of advanced otosclerosis

Acoustic Reflexes

bull Otosclerosis has a predictable pattern of abnormal reflexes over time

1 Reduced reflex amplitude

2 Elevation of ipsilateral thresholds

3 Elevation of contralateral thresholds

4 Absence of reflexes

Pure Tone Audiometry

bull Most useful audiometric test for otosclerosis 1048708Characterizes the severity of disease 1048708Frequency specific

bull Carhartrsquorsquos notch 1048708Hallmark audiologic sign of otosclerosis 1048708Decrease in bone conduction thresholds 10487085 dB at 500 Hz 104870810 dB at 1000 Hz 104870815 dB at 2000 Hz 10487085 dB at 4000 Hz

early stage

middle stage

bull late stage

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

Physical Exam

bull Otomicroscopy

Most helpful in ruling out other disorders

1048708Middle ear effusions

1048708Tympanosclerosis

1048708Tympanic membrane perforations

1048708Cholesteatoma or retraction pockets

1048708Superior semicircular canal dehiscence

Schwartzersquorsquos signs ( by Schwartze in 1873)

1048708Red hue behind the tympanic membrane (in oval window niche area)

104870810 of casesbull Pneumatic otoscopy

1048708Distinguish from malleus fixation

bull Tuning forks10487081 Hearing loss progresses form low frequencies to high

frequencies1048708 2 256 512 and 1024 Hz TF should be used 3 Rinne1048708256 Hz ndashndashnegative test indicates at least a 20 dB ABG 1048708512 Hz ndashndashnegative test indicates at least a 25 dB ABG (air-bone gaps)

Differential Diagnosis

1Ossicular discontinuityAconductive loss of 60 db

B type Ad tympanogram

2Congenital stapes fixationA25 incidence of other

congenital anomalies

B non-progressive CHL

3Malleus head fixation when congenital associated with other stigmata (aural atresia)

4Pagetrsquorsquos disease diffuse involvement of the bony skeleton

5Osteogenesis imperfecta presence of blue sclera

6Superior semicircular canal dehiscence vertigo or eye movements with loud noise

Audiometry

1048708Tympanometry

1048708Impedance testing

Acoustic reflexes

1048708Pure tones

As (s-stiffness curve) tympanogram is characteristic of advanced otosclerosis

Acoustic Reflexes

bull Otosclerosis has a predictable pattern of abnormal reflexes over time

1 Reduced reflex amplitude

2 Elevation of ipsilateral thresholds

3 Elevation of contralateral thresholds

4 Absence of reflexes

Pure Tone Audiometry

bull Most useful audiometric test for otosclerosis 1048708Characterizes the severity of disease 1048708Frequency specific

bull Carhartrsquorsquos notch 1048708Hallmark audiologic sign of otosclerosis 1048708Decrease in bone conduction thresholds 10487085 dB at 500 Hz 104870810 dB at 1000 Hz 104870815 dB at 2000 Hz 10487085 dB at 4000 Hz

early stage

middle stage

bull late stage

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

bull Tuning forks10487081 Hearing loss progresses form low frequencies to high

frequencies1048708 2 256 512 and 1024 Hz TF should be used 3 Rinne1048708256 Hz ndashndashnegative test indicates at least a 20 dB ABG 1048708512 Hz ndashndashnegative test indicates at least a 25 dB ABG (air-bone gaps)

Differential Diagnosis

1Ossicular discontinuityAconductive loss of 60 db

B type Ad tympanogram

2Congenital stapes fixationA25 incidence of other

congenital anomalies

B non-progressive CHL

3Malleus head fixation when congenital associated with other stigmata (aural atresia)

4Pagetrsquorsquos disease diffuse involvement of the bony skeleton

5Osteogenesis imperfecta presence of blue sclera

6Superior semicircular canal dehiscence vertigo or eye movements with loud noise

Audiometry

1048708Tympanometry

1048708Impedance testing

Acoustic reflexes

1048708Pure tones

As (s-stiffness curve) tympanogram is characteristic of advanced otosclerosis

Acoustic Reflexes

bull Otosclerosis has a predictable pattern of abnormal reflexes over time

1 Reduced reflex amplitude

2 Elevation of ipsilateral thresholds

3 Elevation of contralateral thresholds

4 Absence of reflexes

Pure Tone Audiometry

bull Most useful audiometric test for otosclerosis 1048708Characterizes the severity of disease 1048708Frequency specific

bull Carhartrsquorsquos notch 1048708Hallmark audiologic sign of otosclerosis 1048708Decrease in bone conduction thresholds 10487085 dB at 500 Hz 104870810 dB at 1000 Hz 104870815 dB at 2000 Hz 10487085 dB at 4000 Hz

early stage

middle stage

bull late stage

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

Differential Diagnosis

1Ossicular discontinuityAconductive loss of 60 db

B type Ad tympanogram

2Congenital stapes fixationA25 incidence of other

congenital anomalies

B non-progressive CHL

3Malleus head fixation when congenital associated with other stigmata (aural atresia)

4Pagetrsquorsquos disease diffuse involvement of the bony skeleton

5Osteogenesis imperfecta presence of blue sclera

6Superior semicircular canal dehiscence vertigo or eye movements with loud noise

Audiometry

1048708Tympanometry

1048708Impedance testing

Acoustic reflexes

1048708Pure tones

As (s-stiffness curve) tympanogram is characteristic of advanced otosclerosis

Acoustic Reflexes

bull Otosclerosis has a predictable pattern of abnormal reflexes over time

1 Reduced reflex amplitude

2 Elevation of ipsilateral thresholds

3 Elevation of contralateral thresholds

4 Absence of reflexes

Pure Tone Audiometry

bull Most useful audiometric test for otosclerosis 1048708Characterizes the severity of disease 1048708Frequency specific

bull Carhartrsquorsquos notch 1048708Hallmark audiologic sign of otosclerosis 1048708Decrease in bone conduction thresholds 10487085 dB at 500 Hz 104870810 dB at 1000 Hz 104870815 dB at 2000 Hz 10487085 dB at 4000 Hz

early stage

middle stage

bull late stage

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

Audiometry

1048708Tympanometry

1048708Impedance testing

Acoustic reflexes

1048708Pure tones

As (s-stiffness curve) tympanogram is characteristic of advanced otosclerosis

Acoustic Reflexes

bull Otosclerosis has a predictable pattern of abnormal reflexes over time

1 Reduced reflex amplitude

2 Elevation of ipsilateral thresholds

3 Elevation of contralateral thresholds

4 Absence of reflexes

Pure Tone Audiometry

bull Most useful audiometric test for otosclerosis 1048708Characterizes the severity of disease 1048708Frequency specific

bull Carhartrsquorsquos notch 1048708Hallmark audiologic sign of otosclerosis 1048708Decrease in bone conduction thresholds 10487085 dB at 500 Hz 104870810 dB at 1000 Hz 104870815 dB at 2000 Hz 10487085 dB at 4000 Hz

early stage

middle stage

bull late stage

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

As (s-stiffness curve) tympanogram is characteristic of advanced otosclerosis

Acoustic Reflexes

bull Otosclerosis has a predictable pattern of abnormal reflexes over time

1 Reduced reflex amplitude

2 Elevation of ipsilateral thresholds

3 Elevation of contralateral thresholds

4 Absence of reflexes

Pure Tone Audiometry

bull Most useful audiometric test for otosclerosis 1048708Characterizes the severity of disease 1048708Frequency specific

bull Carhartrsquorsquos notch 1048708Hallmark audiologic sign of otosclerosis 1048708Decrease in bone conduction thresholds 10487085 dB at 500 Hz 104870810 dB at 1000 Hz 104870815 dB at 2000 Hz 10487085 dB at 4000 Hz

early stage

middle stage

bull late stage

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

Acoustic Reflexes

bull Otosclerosis has a predictable pattern of abnormal reflexes over time

1 Reduced reflex amplitude

2 Elevation of ipsilateral thresholds

3 Elevation of contralateral thresholds

4 Absence of reflexes

Pure Tone Audiometry

bull Most useful audiometric test for otosclerosis 1048708Characterizes the severity of disease 1048708Frequency specific

bull Carhartrsquorsquos notch 1048708Hallmark audiologic sign of otosclerosis 1048708Decrease in bone conduction thresholds 10487085 dB at 500 Hz 104870810 dB at 1000 Hz 104870815 dB at 2000 Hz 10487085 dB at 4000 Hz

early stage

middle stage

bull late stage

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

Pure Tone Audiometry

bull Most useful audiometric test for otosclerosis 1048708Characterizes the severity of disease 1048708Frequency specific

bull Carhartrsquorsquos notch 1048708Hallmark audiologic sign of otosclerosis 1048708Decrease in bone conduction thresholds 10487085 dB at 500 Hz 104870810 dB at 1000 Hz 104870815 dB at 2000 Hz 10487085 dB at 4000 Hz

early stage

middle stage

bull late stage

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

early stage

middle stage

bull late stage

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

bull late stage

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

Imaging

bull Computed tomography (CT) of the temporal bone

1048708Proponents of CT for evaluation of otosclerosis

Pre-op

1 Characterize the extent of otosclerosis

2 Severe or profound mixed hearing loss

3 Evaluate for enlarge cochlear aqueduct

Post-op

1 Recurrent CHL

2 Re-obliteration vs prosthesis dislocation

3 Vertigo

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

Management options

bull Medical Sodium FluorideBisphosphonatesVitamin D and Calcium

bull AmplificationNon-surgical candidates-wearing hearing aids

bull Surgery Stapedectomy vs Stapedotomy

bull Combinations

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

Surgery

bull Best surgical candidate

1Previously un-operated ear

2Good health

3Unacceptable ABG

425 to 40 dB

5Negative Rinne test

6Excellent discrimination

7Desire for surgery

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

Tympanosclerosis

bull Definition a whitish plaque of the TMbull Pathology submucosal hyaline degeneration in t

he TM and middle ear mucosabull extensive involvement of the TM and ossicle am

y result in conductive hearing loss(air-bone gap gt40dB)

bull medical therapy and pressure equalization tubes (PETs) do not prevent progression of disease

The end thank youThe end thank you

The end thank youThe end thank you