Third Window Lesions

of the Inner Ear

Quinton
Quinton S.
S. Gopen,
Gopen, MD
MD

Department of Otolaryngology and Communication Enhancement

Children’s Hospital Boston
Massachusetts Eye and Ear Infirmary
Department of Otology and Laryngology
Harvard Medical School
 Third
Third Window
Window Lesions
Lesions of
of the
the Inner
Inner Ear
Ear

• Inner Ear
– Sound energy enters via
oval window
• 1st window
– Sound energy passes
through a closed system
and ends at the round
window
• 2nd window
– Any pathologic site
within the inner ear
where sound energy can
leak out of the system is
considered a “third
window”
 Overview
• Mobile “Third Window”
– Results in diminished air conduction
• Can cause air bone gap without ossicular
pathology
– Results in enhanced bone conduction
• Lowered impedance between oval and round
windows
• Auditory dysfunction
• Vestibular dysfunction
 Third Window Lesions
Auditory symptoms
– Autophony
– Tinnitus
• Own speech
• Pulsatile • Internal sounds
• Constant – Heartbeat
– Hearing loss – Eye movements
– Chewing
• Mixed – Joints
• Conductive – Digestion
• Sensorineural – Hyperacusis
– Aural Fullness
 Third Window Lesions
Vestibular Symptoms

– Vertigo spells
• With provocation
• Without provocation
– Dysequilibrium
• Chronic
• Fluctuating
– Oscillopsia
– Fatigue
 Third Window Lesions
Sites

• Semicircular Canals (Dehiscence)

– Superior
– Lateral
– Posterior
• Bony labyrinth
– Enlarged vestibular aqueduct
– Carotid-Cochlear Fistula
• Diffuse
– Paget’s disease
 Case Presentation #1
34 year old woman presents with
autophony, aural fullness and dizziness

• HPI
– Present for several years
– Gradual onset
– Does not go away when lies down
– No history of ear infections or ear
surgery
– Very bothersome to her
– Vertigo with loud sounds
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 Case Presentation #1
What conditions cause
autophony?

– Patulous Eustachian Tube

• She hears her nasal
respirations
• She lost substantial weight
recently
• Her symptoms resolve
when she lies down
• Her symptoms worsen with
exercise
• Her eardrum moves on
deep nasal inspiration
• Loud sounds DO NOT make
her dizzy
• Her flexible fiberoptic exam
shows a patulous ET
– Third Window Lesions
normal

patulous
 Case Presentation #1
Ignoring her symptoms, what other conditions
could cause the audiogram seen in this patient
(CHL with Carhart’s notch)
– Ossicular pathology
• otosclerosis
– Third Window Lesions

• How can we tell these two conditions

apart before surgery?
•CT Scan
•Acoustic reflexes (present 90% DSSC)
•VEMP testing
 Dehiscent Superior
Semicircular Canal

• CT scan
• Coronal views
• Images acquired anterior to posterior
 Dehiscent Superior
Semicircular Canal

• Poschl’s views
• Images acquired parallel to plain of
superior semicircular canal
• Capture entire dehiscence in one or
two slices
 Dehiscent Superior
Semicircular Canal Syndrome
• Superior semicircular canal missing
normal bony covering within middle
cranial fossa floor
– Defect usually quite small, millimeters
• 1st described by Lloyd Minor in 1998
– Presented 8 patients with oscillopsia and
vestibular symptoms
• Subsequent reports of conductive hearing
loss with or without vestibular symptoms
– Failed stapedectomy
 Dehiscent Superior
Semicircular Canal Syndrome
• Epidemiology
– Typically manifests in the 5th decade of
life (ranges from pediatric to elderly)
– No male to female prevalence
– Left side affected 2X right side
– ? Cause
– No known risk factors
 Dehiscent Superior
Semicircular Canal Syndrome
• Presentation
– Hearing loss
• Gradual or sudden
• Progressive or stable

– Types
• Purely conductive
• Mixed (both conductive and sensorineural)
• Purely sensorineural
– Least common
 Dehiscent Superior
Semicircular Canal Syndrome
Auditory symptoms
– Autophony 94%
• Hear internal sounds
• Own voice
– Ear fullness (62%)
– Hyperacusis (loud sounds bothersome)
– Tinnitus (19%)
• Can be pulsatile or non-pulsatile
– Suprathreshold responses in 60% (250, 500Hz)
 Dehiscent Superior
Semicircular Canal Syndrome
• Vestibular symptoms (86%)
– Vertigo
• Loud sounds (50%)
• Changes in middle ear pressure
• Changes in intracranial pressures
– Chronic dysequilibrium
– Oscillopsia
 Dehiscent Superior
Semicircular Canal Syndrome

• Symptoms
– May be difficult for the patient to describe
– Occasionally change with position
– Do not resolve spontaneously
– Do not change with exercise
– Do not have autophony with nasal
breathing
 Dehiscent Superior
Semicircular Canal Syndrome
• Additional testing
• VEMP testing
– Appears more sensitive and specific
than CT scanning for detection of a
dehiscence
– Can help rule in or out equivocal cases
based on history, symptoms, or image
findings
 Dehiscent Superior Semicircular
Canal Syndrome
• VEMP testing
– Vestibular evoked myogenic potential
– Test of vestibule-spinal reflex via saccule function
• Saccule to inferior vestibular nerve centrally
– Test involves detecting an EMG signal from the tonically
contracting SCM
– Tonic baseline EMG signal is normally inhibited by a brief
loud sound (inhibitory potential)
– Loud sound usually presented as a 0.1ms pure tone at
250Hz or 500Hz around 85dB
– In a third window lesion, the threshold for inhibition is
lowered along with an increased amplitude of the response
 VEMP Waveforms
P1 P1

90 dB nHL

90 dB nHL
80 dB nHL
N1
70 dB nHL
80 dB nHL
60 dB nHL
N1
50 dB nHL 75 dB nHL

45 dB nHL
70 dB nHL

0 5 10 15 20 25 30 35 0 5 10 15 20 25 30 35

ms ms
Dehiscent Superior Semicircular
Canal Syndrome: Treatment
• No medical management available
• Surgical correction
– Middle fossa craniotomy
– Extradural elevation of temporal lobe
– Floor of middle fossa inspected for dehiscence
– Bone wax into dehiscent canal
– Resurfaced with fascia, bone (from craniotomy
flap) or bone pâté
• Better at correcting vestibular symptoms
and autophony than for hearing loss
 Case presentation
• 27y.o. male with otorrhea, intermittent otalgia,
hearing loss and vertigo
• DDX?
 Case Presentation - Audiogram
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Case Presentation - CT scan

Lateral canal fistula

 Dehiscent Posterior
Semicircular Canals
• Much less reported buy may not be less
frequent!
• Krombach 2003 CT review of 507 patients
imaged with vertigo or inner ear symptoms
– 29 with dehiscent superior semicircular canal
– 23 with dehiscent posterior semicircular canal
• Dehiscence is often into a high jugular bulb
• Symptoms and presentation similar to DSSC
(not well studied yet)
 Posterior Semicircular Canal
Dehiscence
• 12 patients were identified with PSCD
– Axial and coronal thin section CT of the
temporal bone
– All symptomatic
• Auditory dysfunction
• Vestibular dysfunction
– Audiometric evaluation
– Confirmation with VEMP testing
• Increased amplitude response at reduced
threshold
 Epidemiology
• Ages
Ages 2-67
2-67 years
years old
old (mean
(mean 26y.o.)
26y.o.)
– Seven
Seven pediatric
pediatric patients
patients
• 55 males,
males, 77 females
females
• 11
11 right
right ears,
ears, 22 left
left ears
ears (one
(one patient
patient bil.)
bil.)
• 22 patients
patients with
with iatrogenic
iatrogenic dehiscence
dehiscence
– Mastoidectomy,
Mastoidectomy, vestibular
vestibular schwannoma
schwannoma resection
resection
• Syndromes:
Syndromes: aural
aural atresia
atresia (1
(1 patient),
patient), Apert’s
Apert’s
syndrome
syndrome (1
(1 patient)
patient)
 Symptoms

• Auditory
Auditory
–– All
All patients
patients had
had hearing
hearing loss
loss (selection
(selection
criteria)
criteria)
–– Autophony,
Autophony, pulsatile
pulsatile tinnitus,
tinnitus, aural
aural fullness
fullness
• Vestibular
Vestibular
–– Vertigo
Vertigo with
with loud
loud sounds,
sounds, vertigo
vertigo attacks,
attacks,
chronic
chronic dysequilibrium
dysequilibrium

–– Caution:
Caution: over
over half
half pediatric
pediatric patients!
patients!
 Audiometric Data
• Hearing loss
–– Mixed
Mixed 9/12
9/12
–– Conductive
Conductive 2/12
2/12
–– Sensorineural
Sensorineural 1/12
1/12

–– Suprathreshold
Suprathreshold responses
responses (softer
(softer than
than 0dB)
0dB)
–– Sloping
Sloping configuration
configuration most
most common
common
–– Air
Air bone
bone gaps
gaps usually
usually largest
largest at
at lower
lower
frequencies
frequencies
 Imaging
Imaging

• Dehiscence
Dehiscence into
into high
high riding
riding jugular
jugular
bulb
bulb in
in 70%
70% of
of cases
cases
• Other
Other cases
cases dehiscent
dehiscent into
into posterior
posterior
cranial
cranial fossa
fossa
• Right
Right sided
sided predominance
predominance (85%)
(85%)
• Concurrent
Concurrent superior
superior canal
canal dehiscence
dehiscence
(17%)
(17%)
 CT Images

Dehiscence
Dehiscence into
into high
high riding
riding
jugular
jugular bulb
bulb

Axial
Axial and
and Coronal
Coronal sections
sections
Coronal
Coronal Sections
Sections
 CT
CT Images
Images

Dehiscence
Dehiscence into
into posterior
posterior cranial
cranial
fossa
fossa

Axial
Axial and
and Coronal
Coronal sections
sections
MRI
MRI Images
Images –– Axial
Axial
MRI
MRI Images
Images –– Sagittal
Sagittal
 Case Presentation #3
• 5 year old girl fails school screening
test
– Newborn screening is normal
– Full term, no perinatal issues
– Otherwise healthy
– Parents have not noted any hearing issues
– No ear or balance problems
– FHx: negative for hearing loss
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 Imaging
• Does her hearing test surprise you?
• Do you order a CT scan or an MRI
scan?
 Case Presentation #3
• Would you see the enlarged vestibular
aqueduct on an MRI scan?
 CT scan versus MRI scan
• Highly correlated
• Rare cases when CT scan shows
enlargement of vestibular aqueduct but MRI
scan shows normal endolymphatic duct and
sac
• Also rare cases when MRI scan shows
enlargement of endolymphatic duct and sac
but CT scan is normal
• Both excellent at demonstrating the
pathology, MRI scan may be marginally
better
 Enlarged Vestibular Aqueduct
• 1st described by Valvassori in 1978
• Most common congenital inner ear abnormality seen
on CT scan (15% pediatric SNHL)
• Female to male 3:2
• Bilateral to unilateral 2:1
• Early onset auditory dysfunction
– Usually postlingual hearing loss but ranges from birth to
adult
• Stable (20-50%)
• Fluctuating (15-30%)
• Progressive (studies 20-65%)
• Vestibular symptoms less common (peds)
– 5-30% (dysequilibrium, vertigo)
• Minor head trauma with sudden decrease in hearing
• Family history of hearing loss 15-40%
 Enlarged Vestibular Aqueduct
Audiograms
• Mixed or conductive loss in 28%
(usually lower frequencies)
• Our series 82 ears with 80% mixed or
conductive hearing loss and 50%
bilateral
– May not be testing bone conduction
correctly above 10-20dB
Enlarged Vestibular Aqueduct
Size criteria

– Diameter >1.5mm at midpoint (Valvassori)

– Upper limit normal (95% confidence) is 0.9
midpoint and 1.9 operculum in normals
(Vijayaserkaran)
– Consequently, Cincinnati criteria is >1.0mm
midpoint and/or >2.0mm at operculum
– Rough correlation between size of operculum
and risk of hearing loss progression
Enlarged Vestibular Aqueduct
Concurrent malformations

– Cochlear anomalies in 76% (or in all pt’s?)

• Modiolar deficiency
• Gross dysmorphism
• Scalar asymmetry

– Vestibular anomalies in 40%-88%

• Simple enlargement of lateral SCC
• Gross dysmorphism of lateral SCC
• Distortion of lateral SCC
• Enlarged vestibule

• Are these issues more important than the EVA?

 Enlarged Vestibular Aqueduct
Genetics
• SLC26A4 gene mutations chromosome 7q31
found in 27% with only 8% biallelic
• Correlated with wider aqueducts and
midpoint and operculum, more severe
hearing loss
• Pendred’s syndrome is AR with SNHL, goiter
and EVA
– Genetic mutation found on SLC26A4 or Pendred’s
gene (PDS) although mutations in SLC26A4 do
not necessarily result in the full Pendred
syndrome
 Enlarged Vestibular Aqueduct
Treatment
• Medical options
– Corticosteroids
• Controversial
– Studies have shown benefit and no benefit
• Surgical options
– Endolymphatic sac obliteration
• Mixed results
• High risk dead ear
– Cochlear implantation
• Good candidates
• No perilymph gushers/oozers to date!
 Paget’s disease
• Mixed hearing loss

• Theorized to cause a
distributive third window
lesion

• Fibrous dysplasia does

NOT involve the bony
labyrinth
 Conclusions
• Third window lesions present with variable auditory
and vestibular dysfunction
• Both MR and CT scans demonstrate 3 rd window lesions
well
• VEMP testing can confirm the diagnosis and be helpful
in borderline cases
• Exact mechanism and differences in presentation
between patients remains largely unknown
 References
• Zhou G, Gopen, Kenna, Delineating the Hearing Loss in Enlarged Vestibular
Aqueduct Syndrome Laryngoscope 2008, 118 11 2062-6
• Zhou GW, Gopen Q, Poe D, Clinical and Diagnostic Characterization of Canal
Dehiscence Syndrome: a Great Otologic Mimicker. Otol Neurotol. 2007
Oct;28(7):920-6.
• Som, Curtain, Textbook of Head and Neck Radiology
• Madden C, The Influence of Mutations in the SLC26A4 Gene on the Temporal
Bone in a Population with Enlarged Vestibular Aqueduct, Arch Otol Head
Neck Surg, 133: 162-8 2007.
• Dahleen R, Overlapping Thin Section Fast Spin-Echo MR of the Large
Vestibular Aqueduct Syndrome AJNR 18: 67-75 1997.
• Davidson et al, MR Evaluation of Vestibulocochlear anomalies associated
with large Endolymphatic Duct and Sac, AJNR 1999 20: 1435-1441
• Madden et al, Enlarged Vestibular Aqueduct Syndrome in the Pediatric
Population, Otol Neurotol 2003; 24: 625-32
• Vijayasekaran S et al, When is the Vestibular Aqueduct Enlarged? A Statistical
Analysis of the Normative Distribution of Vestibular Aqueduct Size, AJNR
2007, 28: 1133-38.
 References
• Krombach et al, Posterior semicircular canal dehiscence: a
morphologic cause of vertigo similar to superior semicircular canal
dehiscence, Eur Radiol 13: 1444-1450, 2003
• Zhou G, Gopen Q, Poe D, Clinical and diagnostic characterization of
canal dehiscence syndrome: a great otologic mimicker, Otol Neurotol
28 (7) 920-926, 2007.
• Poe D and Gopen Q, Eustachian Tube Dysfunctionin Ballenger’s
Otorhinolaryngology, ed P. Ashley Wackym, 2008.
• Madden C, Halsted M, et al., The influence of mutations in the SLC26a4
gene on the temporal bone in a population with enlarged vestibular
aqueduct, Arch Oto Head Neck Surg 133: 162-8, 2007.
• Merchant SN, Rosowski JJ, Conductive Hearing Loss Caused by Third
Window Lesions of the Inner Ear, Otol Neurotol 29: 282-9, 2008.
• Tong K, Harnsberger et al., Large Vestibular Aqueduct Syndrome: A
Genetic Disease? AJR 97:1097-1101, 1997.