Superior canal dehiscence: review of a new condition
REVIEW
Banerjee, A.,* Whyte, A.,† & Atlas, M.D.‡
*Department of ENT, James Cook University Hospital, Middlesbrough, UK,  Perth Radiological Clinic, Sir
Charles Gairdner Hospital, Perth, WA, Australia, and àUniversity of Western Australia, WA, Australia
Accepted for publication 15 April 2004
Keypoints
• A new cause of sound and pressure induced vertigo,
superior canal dehisence, is described.
• Auditory manifestations include hyperacusis to bone-
conducted sounds and conductive hearing loss with
normal acoustic reflexes.
• The diagnosis is reached by a directed history, docu-
mentation of upward and torsional nystagmus evoked by
sound and pressure, and radiology.
• Acoustic reflexes and VEMP (vestibular evoked myo-
genic potentials) aid in the identification of patients with
For over 100 years otologists have recognized that in cer-
tain pathological states, sound or pressure transmitted to
the inner ear may inappropriately activate the vestibular
system. The Tullio phenomenon (sound-induced vertigo
or nystagmus or both) is associated with syphilis, and has
been reported in perilymphatic fistula, Menière’s disease,
congenital deafness, chronic otitis media and Lyme dis-
ease.1–6
The controversial condition of spontaneous perilymph
fistula has been proposed as a cause of sound or pres-
sure-evoked dizziness. However, doubt has been expressed
on the existence of perilymph fistula in the absence of
obvious trauma.7 A major and new cause for sound- and
pressure-induced vertigo has been described by Minor
et al.8 consisting of a syndrome of sound- and/or pres-
sure-induced vertigo as a result of dehiscence of bone
overlying the superior semicircular canal.
Some patients with vestibular symptoms and signs of
superior canal dehiscence have also been noted to have
auditory manifestations of the disorder. These patients
may have hyperacusis to bone-conducted sounds and
conductive hearing loss8 with normal acoustic reflexes.
As with any new condition there have been a number of
articles exploring the clinical features, investigations and
Correspondence: Anirvan Banerjee MS, FRCSEd (ORL-HNS), Consult-
ant ENT, James Cook University Hosptial, Marton Road, Middlesbrough
TS4 3BW, UK, E-mail: anirvan.banerjee@stees.nhs.uk and anirvan@
banerjee.me.uk.

2005 Blackwell Publishing Limited, Clinical Otolaryngology, 30, 9–15
an apparent conductive loss with normal acoustic
reflexes or have an asymptomatic dehiscense on
radiology.
• Treatment involves avoidance of the precipitating
stimuli. Surgical treatment, by resurfacing the dehis-
cence, is considered in patients with more severe
symptoms.
Keywords: review, superior semi-circular canal, dehiscence,
disequilibrium.
surgical treatment. This paper reviews the current literature
to help in the better understanding of the condition.
Incidence
Studies on cadaveric temporal bones have found a dehis-
cence of the bone overlying the superior canal occurred in
approximately 0.4–0.5% of temporal bone specimens.9,10 A
study by Carey et al.10 showed that in an additional 1.4%
of specimens (1.3% of individuals), the bone was markedly
thin (<0.1 mm), such that it might appear dehiscent even
on ultra-high resolution computed tomography.
Aetiology
Superior canal dehiscence probably arises from failure of
postnatal bone development.9,10 The bilateral nature of
the thinning and the mature lamellar bone on the mar-
gins of the thin areas point towards a developmental
cause. Tsunoda & Terasaki11 has described the embryolo-
gical basis for dehiscence. The precartilage bordering the
developing membranous labyrinth dedifferentiates into
loose reticular mesenchyme. During this stage if the oto-
cyst is situated close to the developing brain there is
inadequate space for the growth of the superior surface of
the superior semicircular canal as the otocyst may lie
against the dura. Brantberg et al.12 reported two brothers
suffering from the condition raising the possibility that
it may be inherited. The tegmen tympani and tegmen
9
10 A. Banerjee et al
mastoideum are typically thin or absent in patients, sup-
porting the hypothesis of a developmental abnormality.13
The affected area over the superior canal is typically
dumbbell shaped.14 This area of thinned bone is predis-
posed to disruption by trauma. This might explain the
clinical features in some cases of a precipitating event
leading to the onset of symptoms.10,14 The precipitating
events described have been direct trauma, and activities
that may have produced changes in middle ear or intra-
cranial pressure.
Pathophysiology
The dehiscence of bone overlying the superior semicircu-
lar canal creates a third mobile window into the inner
ear.14 Vestibular activation occurs with sound or pressure
stimuli because the lack of bone overlying the membran-
ous superior canal causes greater compliance.8 Stimuli that
result in inward pressure at the round or oval window
(e.g. valsalva against pinched nostrils and tragal compres-
sion) results in an outward bulging of the membranous
canal in the area of dehiscence, with consequent ampullo-
fugal deflection of the cupula of the superior canal. Stim-
uli that result in outward pressure at the round or oval
window (e.g. valsalva against closed glottis, jugular venous
compression, and negative pressure in the external canal)
causes an inward bulging of the membranous canal at the
dehiscence with an ampullopetal deflection of the cupula.
Some reports suggest that the signs and symptoms arise
from the utricle11 because of the abnormally close prox-
imity between the stapes footplate and utricle,15 with or
without abnormal stapes hypermobility.16 The reason for
implicating the utricle is the presence of a sustained isola-
ted torsional eye movement for the duration of the tone.
According to the first law of Ewald,17 stimulation of the
superior semicircular canal should produce eye rotation
in the plane, i.e. upward, laterally and torsional. Cremer
et al. demonstrated that the reason for the isolated tor-
sional eye movement is that these studies reported eye
movements in light, and were therefore subject to visual
suppression.18 This suppression alters eye movement to
stimuli to either a transient vertical eye movement17 or
sustained ocular torsion.11 A vectorial analysis of eye
movements by Ostrowski et al.19 in patients without vis-
ual fixation, showed that the patient vector best matched
the vector representing the superior semicircular canal.
The response with otolith stimulation is expected to be
tonic rather than the phasic torsional response seen.
Some patients with superior canal dehiscence have
bone conduction thresholds that are <0 dB HL.14 In these
patients the third mobile window seems to act as an
amplifier for bone conducted sounds.20 The two main
proposed causes are: (i) an increased sensitivity to bone-
conducted sounds because the dehiscence acts as an
amplifier and improves thresholds in patients with an
underlying sensorineural loss, or (ii) dissipation of acous-
tic energy through the dehiscence creates an air–bone gap
and an apparent conductive hearing loss. Following surgi-
cal resurfacing of the superior canal an improvement
(lowering) of the air conduction thresholds with no
change in bone conduction thresholds was noted suggest-
ing that the cause of the air–bone gap is dissipation of
acoustic energy through the dehiscence.21
Symptoms
Sound-induced symptoms are typically described as the
appearance of motion of objects in response to loud
noises. These can be of a particular frequency, i.e. dial-
tone of a phone, a child’s scream9 or a particular note on
the church organ.19 The perceived motion is often in a
vertical-torsional plane. Several patients describe torsional
oscillopsia, ‘objects move as if they were ‘rotating on the
face of a clock’.8
Symptoms can be induced by manoeuvres that change
middle ear or intracranial pressure. The activities that
evoke vertigo and oscillopsia include blowing of the nose,
coughing, lifting heavy objects, significant straining as
when having a bowel movement or gardening, and apply-
ing pressure to the external auditory canal by pressing on
the tragus. Changes in pressure in the external canal,
while flying or during application of a cotton swab, can
also bring on the symptoms.22
Some patients experienced symptoms that can be des-
cribed as ‘conductive hyperacusis’. Hearing or feeling the
pulse in the affected ear,12 hearing eye movements, and
hearing the heel strike when running.9,14
Many patients experience a chronic sensation of dis-
equilibrium and motion intolerance.8 This can be associ-
ated with a feeling that the world is tilted when there are
periods of marked imbalance. One report detailed a
patient experiencing bradycardia and hypotension evoked
by sound and pressure.14 This symptom is explained by
the role the vestibular system plays in cardiovascular
regulation and counteracting orthostasis.14
Clinical examination
Identification of the eye movements with sound or pres-
sure stimuli requires Frenzels glasses. This eliminates vis-
ual suppression of nystagmus to evoked stimulus and
magnifies the eyes.18 Typically the eye movements have a
vertical and torsional component (the lateral component
is not obvious; Fig. 1a,b).

2005 Blackwell Publishing Limited, Clinical Otolaryngology, 30, 9–15

Fig. 1. Demonstrating the eye movements in superior canal de-
hiscence. The nomenclature for the description of torsional eye
movements is referenced with respect to the patient’s point of
regard (10). A conjugate clockwise torsional eye movement
refers to intorsion of the superior pole of the patient’s left eye
and extorsion of the superior pole of the patient’s right eye.
Such an eye movement will appear anticlockwise to the exami-
ner standing in front of the patient.
The direction of the eye movements can be predicted
based on the direction of endolymph flow within the
superior canal (ampullofugal or ampullopetal) in
response to valsalva manoeuvres or pressure in the exter-
nal canal.21 A valsalva manoeuvre against pinched nostrils
(forcing air into the middle ear through the Eustachian
tube) results in an inward displacement of the stapes, and
an ampullofugal (excitatory) motion of the superior canal
ampulla. Valsalva manoeuvre against a closed glottis (tak-
ing a deep breath and bearing down) results in increased
intra-thoracic pressure, decreased jugular venous return,
and increased intracranial pressure. The membranous
superior canal, in the area of the dehiscence, is com-
pressed by the increased intracranial pressure, and am-
pullopetal (inhibitory) motion of the superior canal
ampulla is induced. A similar ampullofugal motion is
induced by positive pressure in the external canal by
pressing on the tragus or by a pneumatic otoscope.
Increased intracranial pressure by jugular venous com-
pression will cause ampullopetal motion.
A tilt of the patients head in the plane of the affected
superior canal13 was noted in response to loud sounds

2005 Blackwell Publishing Limited, Clinical Otolaryngology, 30, 9–15
Superior canal dehiscence 11
and there is a postural sway induced by pressure applied
to the external auditory canal.14
Some patients with vestibular symptoms and signs
indicative of superior canal dehiscence have also noted
auditory manifestations of the disorder. The Weber tun-
ing fork test typically lateralizes to the affected ear, and
patients may also hear a low frequency tuning fork (128
and 256 Hz) placed on the lateral malleolus of the foot.9
Investigations
Audiometry
The signs of ‘conductive hyperacusis’ can manifest on
audiometry as bone conduction thresholds that are lower
than 0 dB hearing level. Therefore, an air–bone gap can
exist even when air conduction thresholds are nor-
mal.14,20,23 Thresholds are increased in the low to mid
frequency range. Some patients present with mild-to-
moderate sensorineural loss in the affected ear.14 These
patients have a normal speech discrimination score and
acoustic reflexes.8,9,23 Tympanometry shows normal pres-
sures, although the pressure changes may evoke vestibular
symptoms.13
Vestibular function testing
Routine electronystagmography shows no objective
abnormal findings on sound or pressure stimuli, because
of the vertical and torsional nature of the nystagmus,22
although the patient reports vertigo. Eye movements are
best documented with either video-oculography or mag-
netic field search coil recordings.8 These tests are useful
for demonstrating the presence of spontaneous, sound
and pressure-evoked and position-induced nystagmus.
Not all patients exhibit the typical findings expected on
sound and pressure stimuli; results are more predictable
using magnetic field scleral search coils.12 Caloric testing
showed normal function in most patients.12,24 Patients
with a large dehiscence (>0.5 mm) showed reduced ves-
tibular function on the affected side.10,18 Minor et al.13
postulate that a large region of bony dehiscence allows
the overlying dura of the temporal lobe to compress the
membranous superior canal, thereby impeding the flow
of endolymph during testing.
Vestibular evoked myogenic potentials are the short
latency sternomastoid activity in response to vestibular
stimulation.25 In a normal individual VEMP in response
to skull taps are always larger compared with those given
by 100 dB clicks. In superior canal dehiscence the ampli-
tude between skull taps and clicks are different, i.e. 90 dB
clicks presented to the affected ear caused vestibular
12 A. Banerjee et al
evoked myogenic potential of approximately the same
amplitude as skull taps.12 Normal galvanic evoked
responses imply that vestibulocolic reflex pathways are
normal proximal to the vestibular nerve terminals.9 The
low threshold for click-evoked responses is as a result of
increased sensitivity at the level of the vestibular hair
cells. Brantberg et al.23 suggest that simply relying on CT
scan findings is inappropriate because their study showed
that three patients whose CT revealed bilateral dehiscence
denied bilateral symptoms and had normal vestibular
evoked myogenic potentials in the asymptomatic side.
Watson et al.9 argue that measurement of click evoked
vestibular activation is easier to quantify and simpler to
perform and should be the preferred method for demon-
strating abnormal sound sensitivity.
The results of tests of acoustic reflexes and of vestibular
evoked myogenic potentials provide findings that aid in
the identification of patients with apparent conductive
hearing loss as a result of superior canal dehiscence. A
pattern of acoustic reflex abnormalities has been identi-
Fig. 2. CT scans. Reformatted coronal
oblique and sagittal oblique scans parallel
and perpendicular to the superior semi-
circular canal demonstrating dehiscence.

2005 Blackwell Publishing Limited, Clinical Otolaryngology, 30, 9–15

fied in cases of otosclerosis, and changes in acoustic reflex
responses have been noted with progression of stapes fix-
ation.26 The appearance of normal acoustic reflexes in the
setting of an air–bone gap on audiometry should prompt
investigations for causes other than otosclerosis.
Radiology
Computed tomography (CT) is the only imaging modal-
ity of proven value in evaluating superior semicircular
canal dehiscence. High resolution conventional single slice
CT of the temporal bones performed with 1 mm collima-
tion in the axial and coronal phase has high sensitivity
but lacks specificity in the diagnosis of superior semicir-
cular canal dehiscence.27 Multidetector helical CT allows
beam collimation of between 0.5 and 0.6 mm to be used.
The temporal bone is scanned as a volume of tissue
rather than multiple narrow ‘slices’ with conventional
CT. These factors allow extremely high resolution recon-
structions in any desired plane to be produced from the
volume data. In addition to the standard axial and coron-
al reconstructions, 0.5 mm interval oblique coronal
reconstructions of the labyrinth can be performed. Obli-
que coronal reformatted images are planned using an
axial reconstruction through the SCC and are parallel (ie.
along the longitudinal axis) of the SCC and perpendicular
to the long axis of the temporal bone. This technique
improves the positive predictive value of imaging in the
diagnosis of SCC dehiscence syndrome from 50% with
conventional CT (axial and coronal images) to 93% with
multidetector helical CT with 0.5 mm oblique coronal
reconstructions27 (Fig. 2a,b).
The roof of the SCC can be composed of one to three
layers.27 These layers, from inferior to superior, are the
extremely dense otic capsule (0.5–0.8 mm thick), lower
density trabecular bone and the cortical bone in continu-
ity with the remainder of the superior surface of the pe-
trous bone. In control subjects a single layer covering the
Table 1. Table showing the results of surgery
Author Number/method Recurrence of symptoms
Minor et al.13 5
3/Plugging 1 (Vestibular symptoms)
2/Resurfacing No recurrence
Kertesz et al.21 6 (Seven ears)
All resurfacing 1 (Vestibular symptoms)
Brantberg 2
et al.23
Transmastoid plugging No
Minor et al.21 1
Resurfacing No

2005 Blackwell Publishing Limited, Clinical Otolaryngology, 30, 9–15
Superior canal dehiscence 13
SCC is most frequent (65% of cases) and this represents
the otic capsule. The otic capsule and superior cortex
were seen in 23% of controls and all three layers in 12%.
In dehiscence, all three layers are absent.
Treatment
Superior canal dehiscence has been recently discovered as
a clinical entity and many patients with sound evoked
symptoms have been previously misdiagnosed as having
such conditions as Menière’s disease,22,24 benign paroxys-
mal positional vertigo24 or perilymph fistula.8,14,22 Conse-
quently, some newly diagnosed patients might have
undergone previous treatment including surgery for these
conditions.
Most patients achieve control of their symptoms by
avoidance of the sound and pressure stimuli that cause
difficulties.12,14,22,24
The surgical treatment for superior canal dehiscence is
either by plugging8,12,14 or resurfacing the superior semi-
circular canal.8,14,24 Plugging of the canal has been per-
formed by the middle fossa8,12,14 or transmastoid
approach.23 Materials used in the middle fossa approach
to plug the canal are fibrin glue, bone dust and fascia
either with or without a covering of conchal cartilage.14
Mendel used fascia to plug the canal via the trans-mas-
toid route.23 The more commonly performed operation
has been the resurfacing via the middle fossa approach.
The resurfacing has been performed using bone sand-
wiched between two layers of fascia14 or with either a
covering of hydroxyapatite24 or bone cement28 (Table 1).
Surgery resulted in the complete resolution of symp-
toms in most patients.8,24 However, in some patients the
symptoms persisted although they were markedly
decreased.12,24 The commonest symptom to persist was
dizziness on blowing the nose. The symptoms improved
in the immediate post-operative period in the patients
resurfaced with hydroyxapatite or bone cement but there
Revision Hearing
1 Moderate SNHL in the revision case
0 No change
1 2 cases of SNHL (16 and 19 dB), 1 after revision
0 1 SNHL (18 dB)
0 Improvement in conductive loss (20 dB)
14 A. Banerjee et al
was a delay in patients resurfaced with fascia and bone.
Persistence of symptoms in the immediate postoperative
period with subsequent resolution may reflect the time
required for the fascia and bone to form a rigid surface.14
Two cases have required revision for recurrence of symp-
toms. In one case oscillopsia returned and on re-explora-
tion a further region of dehiscence was identified.14 The
other case reported by Kertesz et al.28 required revision
surgery because of partial covering of the dehiscent canal
as a result of inadequate bonding.
Hearing and speech discrimination remained the same
in most operated patients, although Minor et al.21 reports
significant improvement in the air conduction hearing of
one of his patients. There have been four cases of sensori-
neural hearing loss after surgery (of 15 ears operated). In
two cases there was no explainable cause, the other two
had revision surgery. Both the revised cases resulted in
hearing loss and revision surgery appears to increase the
risk of sensorineural hearing loss. Interestingly, Brantberg
et al.23 report that the vestibular evoked myogenic poten-
tials returned to normal after plugging. Larger studies
with longer follow-up periods will be required to evaluate
results.
Conclusion
The condition of superior canal dehiscence has been relat-
ively recently discovered. The patients with this condition
present with both vestibular and auditory manifestations,
and their clinical features vary. The diagnosis is reached
by a directed history, documentation of upward and tor-
sional nystagmus evoked by sound and pressure, and
radiology. There appears to be a role for vestibular evoked
myogenic potentials in the diagnosis of cases that have an
apparent conductive loss with normal acoustic reflexes or
have an asymptomatic dehiscence on radiology. The treat-
ment for this condition, in most cases, involves avoidance
of the precipitating stimuli. Surgical treatment is consid-
ered in patients with more severe symptoms that are not
controlled by these measures. Surgery involves resurfacing
of the dehiscence utilizing the mid-fossa approach. Sur-
gery is generally successful with only a small probability
of recurrence or failure of the relief of vestibular symp-
toms but larger studies are required.
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Superior canal dehiscence 15
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