International Journal of Pediatric Otorhinolaryngology 76 (2012) 492–499

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International Journal of Pediatric Otorhinolaryngology

journal homepage: www.elsevier.com/locate/ijporl

Coronal CT scan measurements and hearing evolution in enlarged vestibular

aqueduct syndrome
Issam Saliba a,*, Marie-Eve Gingras-Charland b, Karine St-Cyr b, Jean-Claude Décarie c
a
Department of Pediatric Otolaryngology – Head and Neck Surgery, Sainte-Justine University Hospital Center (CHU SJ), University of Montreal, Montreal, Quebec, Canada
b
University of Montreal, Canada
c
Department of Medical Imaging, Sainte-Justine University Hospital Center (CHU SJ), University of Montreal, Montreal, Quebec, Canada

A R T I C L E I N F O A B S T R A C T

Article history: Objective: To assess the correlation between the enlarged vestibular aqueduct (EVA) diameter and (1) the
Received 23 December 2011 hearing loss level (mild, moderate, severe and profound and (2) the hearing evolution. The secondary
Received in revised form 3 January 2012 objective was to obtain measurement limits on the coronal plane of the temporal bone CT scan for the
Accepted 5 January 2012
diagnosis of EVA.
Available online 26 January 2012
Methods: Retrospective study in a tertiary pediatric center. Mastoid CT scans were reviewed to measure
the VA diameter at its midpoint and operculum on axial and coronal planes in a pathologic and normal
Keywords:
population. We used their serial audiograms to assess the evolution of hearing.
Enlarged vestibular aqueduct
Results: 101 EVA was identified out of 1812 temporal bones CT scan from our radiologic database in 8
Large vestibular aqueduct
LVA years. Bone conduction was stable after a mean follow-up of 40.9  32.9 months. PTA has been the most
EVA affected in time by the EVA (p = 0.006). No correlation was identified between impedancemetry and the
Vestibular aqueduct diameter of the EVA. On the diagnostic audiogram, 61% of hearing loss were in the mild and moderate hearing
Hearing loss levels; at the end of the follow-up 64% of hearing loss are still in the mild and moderate hearing levels. The
Inner ear cut-off values for the coronal midpoint and operculum planes on the CT scan to diagnose an EVA are 2.4 mm
Malformation and 4.34 mm respectively.
Operculum
Conclusions: Conductive or mixed hearing loss might be the first manifestation of EVA. Coronal CT scan
Inverted J
cuts can provide additional information to evaluate EVA especially when axial cuts are not conclusive.
ß 2012 Elsevier Ireland Ltd. All rights reserved.

1. Introduction Temporal bone fetal development study showed that the

Enlarged vestibular aqueduct syndrome (EVAS) is a relatively
common etiology of sensorineural hearing loss (SNHL) in the
pediatric population. It has been associated with other congenital
disorder [1–4].
The vestibular aqueduct is a bony canal in the otic capsule
which follows a J-shaped course from the medial wall of the
vestibule of the inner ear to open onto the posterior surface of the
petrous pyramid. The developing endolymphatic sac overlies the
sigmoid sinus in the posterior fossa. The otic labyrinth reaches
adult dimensions by mid-term, but development of the posterior
fossa structures continue, resulting in downward pull on the distal
endolymphatic system, bending the vestibular aqueduct into the
normal adult shape of an inverted ‘J’ [5].
* Corresponding author at: CHU Sainte-Justine, 3175, Côte Sainte-Catherine,
Service ORL, Montreal, Quebec H3T 1C5, Canada. Tel.: +1 514 345 4857;
fax: +1 514 737 4822.
E-mail address: issam.saliba@umontreal.ca (I. Saliba).
vestibular aqueduct continues to grow after birth. By 3 years of
age the vestibular aqueduct has reached adult size [6]. Pyle
reported that large vestibular aqueducts are the result of
continued aberrant growth rather than an arrest of development
or failure of narrowing early in embryogenesis [7]. This
morphological abnormality of the temporal bone has been
identified as a result of both the duct and the endolymphatic
sac enlargement [1,3,8].
Since Valvassori and Clemis first described clinical cases of
EVAS in 1978, the radiologic diagnosis criterion used for EVA has
been 1.5 mm at its midpoint and 2.0 mm at the operculum on axial
CT scan plane [1–9]. EVA is most likely bilateral and predisposes to
an early hearing loss and vestibular disorders.
Hearing loss usually presents at birth and initially at high
frequencies. Often it progresses during early life and could be
sudden, fluctuating or a stepwise fashion. Reports have suggested
that hearing loss can result from minor head trauma, rapid
barometric pressure change or Valsalva manoeuvre [1,3,8,9].
Madden et al. reported 39% of his patients to have a down-sloping
look to the audiogram, 35% to have a flat, 17% to have a cookie-
cutter appearance, and 10% to have an upsloping [1]. Even though

0165-5876/$ – see front matter ß 2012 Elsevier Ireland Ltd. All rights reserved.
doi:10.1016/j.ijporl.2012.01.004
 I. Saliba et al. / International Journal of Pediatric Otorhinolaryngology 76 (2012) 492–499 493

EVAS is the most frequent cause of SNHL in children, it can also be a
conductive or a mixed hearing loss [1,9,10].
Based on the afore-mentioned information, our study was
conducted first to assess the relationship between the diameter of
the EVA and the hearing loss severity (mild, moderate, severe and
profound) and the hearing evolution. The secondary objective was
to obtain measurement limits on the coronal plane of the temporal
bone CT scan for the diagnosis of EVA.
2. Materials and methods
1812 of high resolution temporal bone CT scans from 2002 to
2009 was studied at our tertiary pediatric center. All studies were
performed using a standard temporal bone protocol with a thin cut
of 0.5 mm. Patients with radiologic conclusion of an EVA were
selected. Aqueduct diameters at the midpoint and at the
operculum were then measured from both axial and coronal
planes by two different physicians: a neuro-radiologist and a
neurotologist (two senior authors). We compared measures of
both observers and used their mean values for all our analysis. First
analysis was done to assess the reliability of the two physicians’
measurements. To do so, aqueduct diameter measures by the two
observers at the midpoint and at the operculum from both axial
and coronal planes of normal and pathologic (EVA) temporal bones
CT scan database were analyzed. On the axial plane measures were
completed when both medial and lateral edges of the VA were
identified; on the coronal plane, measures were completed when
both inferior and superior edges of the VA were identified and a
canal is well defined (Fig. 1).
The 101 temporal bones with EVA were included. Patients had
to be less than 18-year-old and to have done at least two
audiograms. 38 normal temporal bones randomly chosen from the
normal vestibular aqueduct series were included to complete the
interobserver reliability study. Those with an aqueduct diameter
less than 1.5 mm at the midpoint or less than 2.0 mm at the
operculum on axial planes were considered normal. Patients that
showed a cochlear malformation were excluded from our study to
eliminate alternative causes of hearing loss not due to the EVA.

Fig. 1. High resolution computerized tomography scan showing the measurement of the enlarged vestibular aqueduct (EVA) on an axial (A: arrowhead showing the EVA, B:
midpoint measurement, and C: operculum measurement) and on a coronal cuts (D: arrow showing the EVA, E: midpoint measurement, and F: operculum measurement).
494 I. Saliba et al. / International Journal of Pediatric Otorhinolaryngology 76 (2012) 492–499

Within included EVA cases, charts were reviewed to get
information on hearing level at frequencies from 250 to 8000 Hz
for air conduction and from 250 to 4000 Hz for bone conduction of
the first and the last audiograms performed for every patient. A
new audiogram was completed if none had been done in the last
year. The pure-tone average (PTA) was then calculated from
frequencies of 500, 1000, 2000 and 4000 Hz. No middle ear otitis
was noticed at the time of the audiogram. This study was approved
by our institutional review board at Sainte-Justine University
Hospital Center.
enables the selection of an optimal threshold value (cutoff point)
for the marker [11].
Correlation test and Student t-test for paired data were used to
determine the degree of agreement between the two observers and
Bland–Altman plot is used to analyze this agreement. It is common
to compute the limits of agreement during Bland–Altman analysis.
This is usually specified as bias  1.96  STD (average
difference  1.96 times standard deviation of the difference).
A p-value of 0.05 or less was considered statistically significant
for all analysis.

2.1. Statistical analysis 3. Results

Correlations of continuous data were calculated using Pearson r
correlation coefficient. Student t-test was used to compare means
and variances of samples. Categoric data were investigated using
chi-square analysis. A general linear model was used to determine
the evolution of PTA and the mean of bone conduction thresholds.
Receiver operating characteristic (ROC) curves were used to
determine the cut-off values of the VA diameter at the midpoint
and at the operculum on a coronal planes of the temporal bones.
The Youden Index is a frequently used summary measure of the
ROC curve. It measures the effectiveness of a diagnostic marker and
71 patients with EVAS were investigated in our study (42 male,
29 female). 42.5% presented a bilateral EVA, 28% on the right and
29.5% resulting in 101 EVA to be analyzed. Ages range from 13
months to 15.3 years with a mean age of 5.9-year-old. There was no
difference in the mean VA size between left and right ears in the 30
patients with bilateral EVA.
In the pathologic group of patients, the VA diameter ranged
from 0.83 to 6.82 mm at the axial midpoint (N = 92), 2.00 to
12.71 mm at the axial operculum (N = 92), 2.58 to 9.34 mm at the
coronal midpoint (N = 57) and 3.17 to 11.17 mm at the coronal

Fig. 2. High resolution computerized tomography scan of the patient with a borderline measures to be considered an enlarged vestibular aqueduct (EVA) on the axial cuts.
Axial cuts showing the vestibular aqueduct (VA): (A) arrow, (B) midpoint measure, and (C) operculum measure. Coronal cuts showing the vestibular aqueduct (VA): (D) arrow,
(E) midpoint measure, and (F) operculum measure.
 I. Saliba et al. / International Journal of Pediatric Otorhinolaryngology 76 (2012) 492–499
Fig. 3. (A) Mean air conduction thresholds of the first and last audiograms, (B) mean
bone conduction thresholds of the first and last audiograms, and (C) mean air-bone
gap of the first and last audiograms.
Fig. 4. Hearing level: N1, number of patients on the first audiogram in each level and N2, number of patients on the last audiogram in each level. Hearing level: <20 dB, normal
hearing (N1 = 7, N2 = 3); 21–40 dB, mild hearing loss (N1 = 21, N2 = 16); 41–70 dB, moderate hearing loss (N1 = 16, N2 = 23); 71–95 dB, severe hearing loss (N1 = 10,
N2 = 11); >95 dB, profound hearing loss (N1 = 7, N2 = 8) (black arrow represents the shift of patient from one hearing level to a worst one; dashed black arrow represents the
shift of patient from one hearing level to a better one).
495
operculum (N = 52); their corresponding means were 2.5 
0.96 mm, 6.01  2.2 mm, 5.58  1.91 mm and 6.90  2.09 mm respec-
tively (Fig. 1).
Note that we were not able to get the coronal measurements for
all temporal bones by the lack of coronal reconstruction. In 9 cases
the VA was completely widened by a high placed jugular bulb
leaving no bone edges to take the measurements. The 0.83 mm
value for the axial midpoint was encountered only by one of the
two observers for one patient and the corresponding measure by
the second observer was 2 mm. After reexamination of this axial
cut, the two observers agreed that the 2 mm corresponds to the
operculum measure more than to the midpoint. Since it is on the
borderline to be considered a large vestibular aqueduct, it was kept
in the study. Its related measure on the coronal plane was 3.5 mm
and 4.45 mm at the midpoint and at the operculum respectively
(Fig. 2).
3.1. Hearing tests
The first audiogram was done on diagnosis. Intervals between
the first and last audiograms ranged from 3 to 139 months with a
mean of 40.9  32.9 months.
The mean air and bone conduction thresholds for 250 Hz,
500 Hz, 1000 Hz, 2000 Hz, 3000 Hz, 4000 Hz and 8000 Hz (only for
air conduction) of both the first and last audiograms as well as the
air-bone gaps at each of these frequencies are shown in Fig. 3. PTA
from first and last audiograms was 53.68  30.24 dB and
58.12  28.41 dB. Hearing level is represented in Fig. 4. Sixty one
patients had complete audiograms and were used for analysis.
3.2. Hearing and EVA diameter correlation
3.2.1. The first audiogram
Significant correlation was seen between: (1) the midpoint
diameter of the VA on the axial plane and the air conduction
threshold at 8000 Hz (r = 0.514, p = 0.012), (2) the operculum
diameter of the VA on the axial plane and the air conduction
thresholds at 250 Hz (r = 0.301, p = 0.047) and (3) the operculum
diameter of the VA on the coronal plane and the air conduction
thresholds at 500 Hz and 1000 Hz (r = 0.461, p = 0.016, r = 0.440,
p = 0.022 respectively). No significant correlation was seen
between bone conduction thresholds and the diameter of the
VA.
496 I. Saliba et al. / International Journal of Pediatric Otorhinolaryngology 76 (2012) 492–499

3.2.2. The last audiogram
Statistical analysis showed no correlation between hearing and
VA diameter on any of the planes.
3.3. Hearing evolution
A linear model was used for each of the frequencies 250, 500,
1000, 2000, 3000 and 4000 Hz to compare the evolution in time
of the air conduction, the bone conduction, the ABG and the PTA.
Bone conduction and ABG showed no difference between the
first and the last audiograms but the air conduction showed a
significant difference only on the frequencies 2000 Hz
(p = 0.001) and 4000 Hz (p = 0.002) therefore the PTA which is
the mean of the frequencies 500, 1000, 2000 and 4000 Hz has
been affected in time by the pathological EVA (p = 0.006). The
mean follow-up time was 40.9  32.9 months; our results did not
show a bone conduction hearing loss even at this follow-up period
of time.
high due to the air conduction loss at the 500 Hz and 1000 Hz
frequencies; with time and after 40.9  32.9 months of follow-up
the PTA still the most affected factor but at higher frequencies:
2000 Hz and 4000 Hz. Zalzal et al. noted that the severity of hearing
loss was unrelated to the size of the vestibular aqueduct [12].
EVA has been frequently associated with a SNHL, however
hearing loss could be mixed or conductive [1,13,14]. Ears with
mixed hearing loss demonstrated significantly larger VA than ears
with only SNHL [15]. Conductive hearing loss is observed in 17–
28% of patients, especially in the lower frequencies, even though
the middle ear is normal. The conductive gap is, on average, 15–
22 dB, with a range of 10–50 dB [9]. There have been several
theories as to why this conductive component occurs [1]. Such
theories include decrease of stapes mobility from increased
endolymphatic sac pressure, stapes ossification, and other ossic-
ular dysfunction. The abnormal communication provided by the

3.4. Coronal measures utility to diagnose EVA

For the coronal midpoint, the two index of Youden were 2.36
and 2.45 mm; the mean result for observers 1 and 2 was 2.4 mm,
with a sensitivity and specificity of 100%. For the coronal
operculum, the two index of Youden were 2.63 and 5.05 mm.
The mean result was 4.34 mm with a sensitivity of 94% and
specificity 100% for the observer 1 and a sensitivity of 91% and
specificity 92% for the observer 2 (Fig. 5).

3.5. Interobserver reliability

38 normal and 101 pathologic (EVA) temporal bones were used

to study the interobserver reliability. A total of 106 CT scans were
used for axial planes, 72 for midpoint and 70 for operculum on
coronal planes. The number of axial and coronal measurements
used is lower than the total number of cuts because reconstruc-
tions of images were not available for all patients.
The VA measurement on the CT scan did not demonstrate
significant differences between the two physicians. For the axial
planes, correlations were 0.817 and 0.748 at midpoint and
operculum respectively. For the coronal planes, they were 0.935
and 0.894. All these with a p-value lower than 0.001, indicating a
high level of reliability.
The Bland–Altman analysis showed that the measurement
agreement is stronger at the midpoint measurement than at the
operculum on the axial as well as on the coronal planes (Fig. 6). The
agreements between physicians tend to diminish as the diameter
got larger for all planes but both observers considered the EVA
pathologic when this is the case.

4. Discussion

EVAS is considered to be rare. Estimates fall between as high as

5–15% in pediatric patients [3]. In our 8 years presented data base,
5.57% of the 1812 temporal bones had an EVA. In opposition to the
literature, our series showed less female than male cases. Madden
et al. reported a female to male ratio EVAS of 3:2 [3].
The increased use of high resolution CT scans has revealed EVA
to be the most commonly identified inner ear malformation in
children with unknown causes of SNHL. Our study attempted to
correlate radiologic measurements and audiologic findings among Fig. 5. Receiver operating characteristic (ROC) curve. Sensitivity and specificity
patients with EVAS in an effort to offer some prognostic insight for values of the vestibular aqueduct diameter measured on a coronal model for the
our young patients. Therefore we found a correlation between the observer 1 (A) and the observer 2 (B). At the coronal midpoint, results were 2.36 mm
and 2.45 mm for the observers 1 and 2 respectively, with a sensitivity and
diameter of vestibular aqueduct and the severity of the air specificity of 100%. For the coronal operculum, the mean of the two index of Youden
conduction hearing loss on the diagnostic audiogram (first was 4.34 mm giving for the observer 1 a sensitivity of 94% and specificity of 100%
audiogram). The more the diameter is large, the more the PTA is and for the observer 2 a sensitivity of 91% and a specificity of 92%.
 I. Saliba et al. / International Journal of Pediatric Otorhinolaryngology 76 (2012) 492–499
Fig. 6. Bland–Altman plot shows the correlation between observers for the vestibular aqueduct width size expressed in millimeter. We notice that the average difference
between the two observers for the midpoint on the axial (A) and coronal (B) cuts is 0.16  0.78 mm and 0.43  1.46 mm respectively.
enlarged endolymphatic duct and sac in EVA may act as the ‘‘third
window’’ for sound conductance explaining this finding [10,16].
Hearing loss severity was evaluated by comparing the first
diagnostic audiogram to the last one. On the diagnostic audiogram,
61% of hearing loss was in the mild and moderate hearing levels; at
the end of the follow-up 64% of hearing loss remained in the mild
and moderate hearing levels. We noted 57.4% of stable hearing
over the period of 40.9  32.9-month follow-up. Some patients shift
back to a better hearing level but this improvement was not clinically
significant (less than 10 dB) (Fig. 4). The majority of patients had a
moderate–severe (46%) and severe–profound SNHL (38%) [14].
Madden was following the exact same patients suffering from EVAS
for three years to see how their audiograms changed. He noted 50% to
have stable hearing over those three years. 28% had fluctuating or
fluctuating with progressive picture and 10% had a progressive type of
audiogram [1].
The theory of hearing loss after minor trauma that has really
stood the test of time thus far is in Levenson’s 1989 reflex theory
[17]: the endolymphatic sac sits in the posterior cranial fossa and
with head trauma we can get increased pressure or fluctuations of
cerebrospinal fluid (CSF). This might compress the endolymphatic
sac and compress its hyperosmotic contents through the EVA to
the endolymphatic duct and push it into the cochlea. The
hyperosmotic contents may cause neuroepithelial damage and
thus lead to the SNHL. The etiology is postulated to be related to the
transmission of intracranial pressure fluctuations stimulating the
vestibular sensory receptors. Our results did not show a bone
conduction hearing loss even after 40.9  33.9 months. However
our patients were always informed to strictly avoid head trauma and
contact sports (e.g. wrestling, football, rugby, soccer, hockey, skate
497
board jumping) and to always wear head protection if there is
possibility of head trauma. Boston et al. find no statistically significant
difference of progressive SNHL between a group of patients with EVA
and a group without EVA (24% vs 14% respectively) [15].
Several authors have speculated on possible causes for the
progressive hearing loss in EVAS such as: intracochlear membrane
rupture and associated hydrops [13], round window fistula or
abnormalities [18], stapes fixation and a cerebrospinal fluid gusher
in case of exploratory tympanostomy [19].
In adults vestibular symptoms consist of occasional vertigo and
unsteadiness, whereas in children incoordination and imbalance
were present in 30% of cases [13,20–22].
Analysis suggests that midpoint measurements on axial plane
should be preferred to operculum ones for better interobserver
agreement. In addition, coronal planes showed better agreement
than axial planes, for both midpoint and operculum values. This
brought us to the interrogation, as if it would be possible to use
coronal planes to assist in the diagnosis of the EVAS.
Contrary to Valvassori and Clemis, Boston et al. considered the
VA enlarged if one is greater than 1.9 mm at the operculum and/or
greater than 0.9 mm at the midpoint in an axial CT scan [15]. Some
authors have suggested that the vestibular aqueduct could be more
easily evaluated on coronal plane for CT scans, as seemed to be the
case in our study [23]. The present study gave us an idea of what
those criteria could be. 2.4 mm for the coronal midpoint plane
could be accepted as the cut-off value with a 100% sensitivity and
specificity. By using the mean result of the two index of Youden,
4.34 mm at the operculum in the coronal planes offer at least a
sensitivity of 91% and specificity 92% to diagnose an EVA thus it
could be helpful in cases where the axial measure is not available
498 I. Saliba et al. / International Journal of Pediatric Otorhinolaryngology 76 (2012) 492–499
Fig. 7. Right coronal view of a high resolution CT scan on the same patient showing an enlarged vestibular aqueduct (arrow). (A and B) The vestibular aqueduct operculum
width where an aqueduct shape is well defined (4.90 mm) as we described in this study; (C and D) the first point of vestibular aqueduct definition posteriorly (7.49 mm)
where measurements were defined by Murray et al. [15].
or it is on the borderline diameter like in our presented case (Fig. 2).
The limitation of these values is the small number of coronal cuts
(52 coronal planes) available in this study. Further large-scale
studies would be needed to clarify the cut-off values obtained in
our current research and their reproducibility. Since a perpendic-
ular cut of the VA at the operculum or at the midpoint shows an
oval shape than a round shape, the axial value measures differ from
the coronal value measures. Murray et al. defined the upper limit of
normal VA operculum on a coronal CT by 6.8  3.3 mm [23]. At this
point, their cut-off is higher than our upper limit. This is explained by
the fact that measurement were done posteriorly at the first point of
VA definition, whereas we measure the operculum width at the
aperture of the VA where the inferior and the superior edges were
indentified and an aqueduct shape (canal) is well defined (Fig. 7).
A multicentre study is needed to collect a higher number of
patients with EVAS therefore increasing the power of the study.
5. Conclusion
EVA is the most commonly identified inner ear bony
malformation in children with SNHL. However conductive or
mixed hearing loss might be the first manifestation. The
mechanisms of hearing loss and vertigo in EVAS may be the result
of the third window for conductive problem and cerebrospinal
fluid reflux for SNHL. When axial CT scan cuts are not conclusive,
VA diameter of 2.4 mm at the midpoint and/or 4.34 mm at the
operculum on a coronal cuts are an alternative measures to
diagnose EVA.
Funding
None.
Conflict of interest
None.
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