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Objective: To assess the diagnostic yield of audiograms asso- Results: The most revealing data were the mean ASNHL at
ciated to electronystagmography (ENG) for screening vestibular 3,000 Hz ( p G 0.001), the interaural SDS asymmetry ( p G
schwannomas (VSs), to determine what definition of asymmetric 0.001), the vestibular deficit ( p G 0.049), and the absence of
sensorineural hearing loss (ASNHL) fits best for the diagnosis of vertigo ( p G 0.001). The ASNHL at 3,000 Hz was the most rep-
VS, and to determine if cochleovestibular symptoms and athero- resentative value of all the frequencies and for the SDS asym-
sclerotic potential risk factors play a role in the VS screening. metry. Interaural difference of 15 dB or more at 3,000 Hz is
Study Design: Retrospective chart review in a tertiary care sufficient to consider hearing loss as asymmetric. When the cut-
center. off for a positive test was placed at 50% probability, the receiver
Methods: One hundred twenty-two patients were included in operating characteristic curve shows a sensitivity of 73%. The
the study and divided into 2 groups: 1) patients presenting a VS grade of the tumor was also related with the degree of ASNHL at
(n = 74) and 2) patients without VS (n = 48). They had received 3,000 Hz. Caloric test does not predict the localization or the
an audiometry assessment, an ENG, and a posterior fossa mag- grade of the VS. Tinnitus and atherosclerotic potential risk fac-
netic resonance imaging (MRI). In addition, a variety of risk tors were not considered significantly linked with VS.
factors and clinical data were collected. Mean hearing threshold Conclusion: To reduce the number of negative MRI per-
by frequency, mean asymmetries by frequency, speech discri- formed in the investigation of an ASNHL, we propose the
mination score (SDS), ENG results, and presence or absence of Brule 3,000,[ ASNHL of 15 dB or more at the 3,000-Hz
vertigo are studied. Cochleovestibular symptoms and athero- frequency. In this case, an investigation with MRI is crucial.
sclerotic potential risk factors were collected. Characteristics If this ASNHL is less than 15 dB, we recommend a biannual
were studied with analysis of variance, W2 test, or a paired t audiometric follow-up. Key Words: AcousticVAsymmetricV
test. A receiver operating characteristic curve was obtained. A Hearing lossVNeurinomaVNeuromaVRule 3,000V
logistic regression with a step-wise selection based on the like- Schwannoma.
lihood ratio was used to identify the best subgroup of predictors
of the VS. Otol Neurotol 30:515Y521, 2009.
Acoustic neuroma is the most common tumor (92%) The VS is a slow-growing and well-circumscribed
of the cerebellopontine angle (CPA), accounting for 5 to benign tumor arising from the transitional zone be-
10% of all intracranial tumors in adults (1). Because it tween Schwann cells and oligodendrocytes at the point
is attached to the vestibular branch of the eight cranial where CN VIII enters the internal auditory meatus, the
nerve (CN VIII), it is more accurately called vestibular Obersteiner-Redlich zone (2). It initially grows within
schwannoma (VS). The mean age at onset is 50 years the bony auditory canal but then expands to the CPA.
except in cases of neurofibromatosis type 2, an autoso- As the tumor grows, the next cranial nerve to be affected
mal dominant disorder in which the tumors usually occur is generally the trigeminal nerve, causing facial pain and
before 21 years of age (2). sensory loss. If left untreated, the tumor will compress
the fourth ventricle, causing cerebrospinal fluid outflow
obstruction, hydrocephalus, and herniation (3). Clini-
cally, the tumor most commonly presents as an asym-
Address correspondence and reprint requests to Issam Saliba, M.D.,
Centre Hospitalier de l’Université de MontréalYHôpital Notre-Dame, Ser-
metric or unilateral sensorineural hearing loss (SNHL)
vice d’Oto-rhino-laryngologie, 1560 Sherbrooke street east, Montreal with or without unilateral tinnitus. However, only 2%
Qc - H2L 4M1, Canada; E-mail: issam.saliba@umontreal.ca of patients presenting with these classic symptoms will
515
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516 I. SALIBA ET AL.
Copyright @ 2009 Otology & Neurotology, Inc. Unauthorized reproduction of this article is prohibited.
ASYMMETRIC HEARING LOSS AND VESTIBULAR SCHWANNOMA 517
TABLE 1. Mean hearing threshold and MASNHL by frequency for the affected and best ear
Control group VS group Control group VS group
MHT of affected MHT of best MHT of affected MHT of best
Frequencies, Hz ear, dB ear, dB ear, dB ear, dB MASNHL, dBa MASNHL, dBa p
250 11.67 T 11.31 12.08 T 13.64 18.33 T 12.75 9.93 T 7.10 9.33 T 11.90 9.55 T 10.15 0.469
500 17.60 T 16.14 20.00 T 17.47 28.04 T 17.32 11.49 T 9.53 12.22 T 16.29 14.20 T 12.93 0.048
1,000 18.54 T 17.68 20.00 T 19.41 33.51 T 21.16 11.50 T 9.53 11.44 T 16.43 19.91 T 15.83 G0.001
2,000 22.60 T 19.52 23.33 T 20.09 44.93 T 22.48 14.80 T 11.39 13.56 T 16.50 28.84 T 19.68 G0.001
3,000 23.80 T 22.48 21.96 T 18.99 45.92 T 19.65 17.11 T 15.58 12.00 T 13.96 30.27 T 18.72 G0.001
4,000 24.48 T 20.19 23.75 T 20.82 47.67 T 21.39 20.54 T 17.45 12.78 T 14.87 26.70 T 19.94 0.043
All statistics are represented by mean T standard deviation.
MHT indicates mean hearing threshold; MASNHL, mean asymmetries of sensorineural hearing loss; VS, vestibular schwannoma.
a
Difference between mean hearing thresholds of affected ear and best ear in absolute value.
was reported in 7 patients. None of the 74 patients had a Considering the results of the t test performed and the
superimposed Ménière’s disease (Table 3). p values obtained, the variables linked to the presence of
In the control group, 23 of the 48 patients had posi- VS were the following: mean asymmetries of SNHL
tional nystagmus in the ENG. Five patients had abnormal between 500 and 4,000 Hz, mean difference of speech
pursuit test, and 3 had abnormal headshaking test. The discrimination, vestibular deficit, and absence of vertigo.
mean vestibular deficit with the caloric tests was 27%. Of The ROC curves and the areas under the curves obtained
the 48 patients, 34 presented with dizziness, 25 with from these variables and the logistic regressions were
vertigo, and 32 with tinnitus. Dyslipidemia was reported performed. A subgroup of parameters with the best repre-
in 12 patients. Diabetes was reported in 6 patients. Of the sentative capacities was determined: 1, mean asymmetry
48 patients, 5 were diagnosed with Ménière’s disease of SNHL at 3,000 Hz; 2, absence of vertigo; and 3, ves-
(Table 3). The smoking status and the blood hyperten- tibular deficit. The larger area under the curve links to the
sion factor were rarely reported in the charts to be con- mean asymmetries of SNHL at the 3,000-Hz frequency
sidered in the analysis. Saccades and optokinetic were and is equal to 0.799. This is the reason why this hearing
normal in all patients of both groups. frequency was consequently used in the logistic regres-
sion model.
Categories were then established for the continue vari-
ables (asymmetries of SNHL and vestibular deficit) to
determine the best and most clinically useful model to
predict the probability of VS. A mean asymmetry of
SNHL at 3,000 Hz of 15 dB or more was found statisti-
cally significant ( p G 0.001), as well as a vestibular deficit
of 25% or more, and the absence of vertigo. No statistical
difference was found for an asymmetric SNHL of less
than 15 dB ( p = 0.077).
Following this categorization of the variables, a new
logistic regression was performed. The new model ob-
tained did not include the vestibular deficit anymore. Add-
ing the vestibular deficit information obtained by an ENG
would not improve our predicting capacity once the asym-
metry of SNHL at 3,000 Hz and the absence/presence of
vertigo were considered (Table 4). Because the 3,000-Hz
frequency data were missing in 19 patients, at this stage,
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518 I. SALIBA ET AL.
TABLE 3. Electronystagmography, symptoms, and potential TABLE 5. Predicted probabilities of VS for each
risk factors for atherosclerosis results combination of categories
Control group, VS group, Predicted
n = 48 n = 74 p Effective, probability
n = 103 average
Positional nystagmus 23 33 0.719
Abnormal pursuit test 5 3 0.165 Vertigo Absence Asymmetry of G15 dB 27 0.48135
Abnormal headshaking test 3 8 0.378 SNHL at Q15 dB 43 0.86054
Vestibular deficit, % 27 45 0.049 3,000 Hz
Dizziness 34 40 0.077 Presence Asymmetry of G15 dB 23 0.13058
Vertigo 25 9 G0.001 SNHL at Q15 dB 10 0.49966
Tinnitus 32 59 0.078 3,000 Hz
Dyslipidemia 12 13 0.448
Diabetes 6 7 0.670 SNHL indicates sensorineural hearing loss; VS, vestibular schwannoma.
Ménière’s disease 5 0 0.005
VS indicates vestibular schwannoma.
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ASYMMETRIC HEARING LOSS AND VESTIBULAR SCHWANNOMA 519
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520 I. SALIBA ET AL.
Grade of VS sudden hearing loss cases in our study was too small to
The grade of the tumor was also related with the evaluate statistically this parameter. In a study from
degree of asymmetric SNHL at 3,000 Hz. This asymme- Takebayashi et al. (23), 506 patients with sudden hearing
try rises with the grade of the tumor and is statistically loss were investigated by audiometry. Thirteen patients
significant for Grades I, II, and III. This difference was (2.8%) consisted with VS, and 493 patients consisted
not significant between Grades I and IV ( p = 0.209) with idiopathic sudden hearing loss. Ten of 13 patients
because of the small number of cases of Grade IV with VS (77%) and 40 of 493 patients with idiopathic
(n = 10) in the study and because 1 of the 10 patients sudden hearing loss (8%) presented with the main hear-
of Grade IV had normal hearing in both ears. The con- ing loss on the high frequencies. The group of patients
troversy between normal hearing and large tumor can be with VS had a less important hearing loss ( p G 0.05) on
related to tumor invading into the CPA without com- the 125-, 250-, and 500-Hz frequencies than the group
pressing CN VIII. By correlating these clinical features, with idiopathic acute hearing loss. In another study by
one can explain why the cisternal VSs are more likely to Sauvaget et al. (24), 28 patients with a history of sudden
be diagnosed late and present when large in size (17). hearing loss were found beyond 138 cases of VS (20%).
These studies point toward a same conclusion: a sudden
Vestibular Deficit, Dizziness, and Vertigo hearing loss occurring mostly on the higher frequencies
Although the vestibular deficit more than 25% on the leads us toward VS. On the other hand, 25 of the 28
caloric test does not predict the localization or the grade patients with VS presented as sudden SNHL are classi-
of the VS, Tringali et al. (18) found that caloric deficits fied as Grade I or II.
were higher in Grade IV VS compared with other grades The average duration of hearing loss or other unilat-
probably because of the higher number of cases (n = 734) eral symptoms in patients with VS is less than 3 years,
included in their study and the statistical power conse- with a range of 6 months to 7 years (5). Even if there is
quently generated. The vestibular symptoms that were stability of hearing loss for more than 3 years, in the
included in the analysis were dizziness and vertigo. Diz- presence of asymmetry at the 3,000-Hz frequency, we
ziness has an incidence approaching 50% in the presence recommend doing a posterior fossa MRI. Vestibular
of VS (19), but it was not found to be predictive of this schwannoma can grow just in the CPA without hearing
disease probably because dizziness has a high prevalence deterioration.
in the general population. On the opposite, the absence of
vertigo had a strong association with VS in the presence Limitation of the Study
of asymmetric SNHL of 15 dB or more. The incidence of This study is hampered by the limited number of our
vertigo is 9% in this population (20). The presence of population and the important number of VS-referred
vertigo can generally point toward other diagnosis such cases. As we have shown, by the rule 3,000 screening
as Ménière’s disease, cochlear hydrops, perilymphatic method, there is a small risk to reject an MRI and to
fistula, or cholesteatoma. In patients with asymmetric underdiagnosed VS; this will not have immediate grave
SNHL of 15 dB or more, the predicted probability of implications but is nevertheless undesirable. A prospec-
VS declines from 86% in the absence of vertigo to tive study with a high number of patients chosen from a
49% if vertigo is an associated symptom. population who seeks first-line care by the general prac-
titioner instead of referred patients to a specialized care
Tinnitus and VS facility in otolaryngology would improve the validity of
Tinnitus was not considered a symptom significantly our study.
linked with VS. This finding was also reported in a study
by Lustig et al. (21) reviewing 542 patients with VS and
CONCLUSION
symmetric hearing. He found only 4 patients with asym-
metric tinnitus as the unique symptom; these results are To reduce the number of negative MRIs performed in
supported by Obholzer and Harcourt (4) comparing 36 the investigation of an asymmetric SNHL and the costs
patients with VS and 92 without. These studies consid- generated, we propose the rule 3,000. When the cutoff
ered that this symptom might have been more revealing for a positive test was placed at 50% probability, the
if its variability was better clarified (unilateral or bilat- ROC curve shows a rule 3,000 sensitivity and specificity
eral, consistently present or not, present in quiet or noisy of 73% and 76%, respectively. The selection of patients
environment, relationship with exogenous stress and dis- who need MRI to exclude retrocochlear pathology
turbed sleep). should be based on an asymmetric SNHL of 15 dB or
more at the 3,000-Hz frequency. This should raise suspi-
Application of the Rule 3,000 to the Sudden cion of a retrocochlear disease. If the asymmetric SNHL
Hearing Loss at 3,000 Hz is less than 15 dB, we recommend a biannual
A sudden hearing loss has also been associated with audiometry testing follow-up. The ENG can be done for
VS. Sudden hearing loss is defined as an asymmetry of documenting the case and for postoperative prognosis
more than 30 dB on 3 consecutive frequencies appearing purposes, but the vestibular deficit obtained does not
in less than 3 days (22). Unfortunately, the number of help significantly in predicting the presence of a VS.
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ASYMMETRIC HEARING LOSS AND VESTIBULAR SCHWANNOMA 521
Copyright @ 2009 Otology & Neurotology, Inc. Unauthorized reproduction of this article is prohibited.