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INTERNATIONAL ORIGINAL ARTICLES
EUGENE N. MYERS, MD
International Editor


Role of the new imaging modalities in the investigation of
Meniere’s disease
JOHN XENELLIS, MD, LAMBROS VLAHOS, MD, ANDREAS PAPADOPOULOS, MD, PERICLES NOMICOS, MD,
KONSTANTINOS PAPAFRAGOS, MD, and GEORGE ADAMOPOULOS, MD, Athens, Greece




The incidence of nonvisualization of the vestibular               M    eniere’s disease (MD) is a well-established clini-
aqueduct and the endolymphatic duct–endolym-                      cal entity; patients with the definite type have all of the
phatic sac (ED-ES) complex as well as the type of                 following symptoms: vertigo, hearing loss, and tinni-
periaqueductal pneumatization were evaluated in                   tus or ear pressure.1 Since MD’s description by Prosper
23 patients with definite Meniere’s disease (MD) by               Meniere,2 the scientific community has tried to discover
high-resolution CT and by MRI. Fifty subjects with no             the underlying etiopathophysiologic factors responsible
previous history of any ear disease were used as a                for the characteristic vestibulocochlear dysfunction.
control group. High-resolution CT results disclosed                  Today it is widely accepted that the histopathologic
that in the control group the percentage of nonvi-                substrate of the disease is endolymphatic hydrops (EH)
sualized vestibular aqueduct (3.4%) was statistically             because of the disruption of the balance between pro-
significantly lower than in the MD group, when                    duction and absorption of the endolymph.3
either the diseased ear (27.8%) or the nondiseased                   A key role in the pathogenesis of the EH is attributed
ear of the same group (22.2%) was examined. In                    to impaired function of endolymphatic duct (ED) and
addition, no differences were observed between                    endolymphatic sac (ES).4 The ED-ES system lies partly
the diseased and nondiseased ears of the patients                 within, partly through, and partly outside of the vestibu-
with MD. The periaqueductal pneumatization was                    lar aqueduct (VA), which is a bony channel of the otic
also found to be statistically significantly lower in             capsule.
the MD group. As regards the MRI examination, the                    In the literature concerning MD, there are some
results of the analysis showed that the ED-ES com-                reports of a high incidence of radiologic nonvisualiza-
plex was visualized more frequently in the ears of                tion of the VA5,6 and poor pneumatization of the sur-
the control subjects (64.1%) than in the diseased                 rounding mastoid air cells in the diseased ears.6,7 How-
ears of the patients with Meniere’s disease (39.1%).              ever, other authors8,9 consider these observations as
This difference had a marginal statistical signifi-               nonspecific diagnostic signs seen in a variety of ear dis-
cance (P ~ 0.05). We discussed the results in relation            eases.
to possible underlying pathophysiologic mecha-                       In this study we analyzed our findings after exami-
nisms involving the flow of endolymph toward the ES               nation of the VA, ED-ES complex, and periaqueductal
during the different stages of the disease. (Oto-                 pneumatization with high-resolution (HRCT) and MRI
laryngol Head Neck Surg 2000;123:114-9.)                          of 23 temporal bones from 23 patients with definite uni-
                                                                  lateral MD. We also attempted to delineate the possible
                                                                  implications of these findings on the normal function of
                                                                  the inner ear.

                                                                  ANATOMY OF THE VA AND THE ED-ES COMPLEX
From the Departments of ENT (Drs Xenellis, Nomicos, Papafragos,
  and Adamopoulos) and Radiology (Drs Vlahos and Papadopoulos),      The VA is a bony canal that contains the ED and the
  University of Athens Medical School.                            proximal part of the ES. It extends from the medial wall
Reprint requests: J. Xenellis, MD, Sarantapichou 35, GR-114 72    of the vestibule to the posterior surface of the petrous
  Athens, Greece.                                                 pyramid, where it terminates in a bony cleft called the
Copyright © 2000 by the American Academy of Otolaryngology–
  Head and Neck Surgery Foundation, Inc.
                                                                  endolymphatic meatus, which contains the ES. In
0194-5998/2000/$12.00 + 0 23/77/102113                            adults, as the VA traverses the temporal bone, it is
doi:10.1067/mhn.2000.102113                                       shaped like an inverted J. The short limb and the bend
114
Otolaryngology–
Head and Neck Surgery
Volume 123 Number 1 Part 1                                                                             XENELLIS et al   115




                                                                   Fig 2. MRI of a normal ED-ES complex (arrow).

     Fig 1. Transaxial HRCT of a normal VA (arrow).

                                                             VA, which also shows a narrow external aperture. In
                                                             MD there is a high incidence of type III pneumatiza-
of the VA together are called the isthmus, and this is the   tion.6,7
most narrow part of it.
   The distal end of the VA expands to accommodate           METHODS AND MATERIAL
the terminal enlargement of the ED, the ES. Lund-                Between 1994 and 1996, 23 patients with definite unilateral
quist10 divided the ES into 3 parts: (1) a proximal part     MD were evaluated in the ENT Department of the University
located within the VA, (2) an intermediate part located      of Athens. This group of patients constitutes the source of this
partially within the VA and partially between the layers     study. The selection criteria for these patients conformed to
of dura mater outside the VA, and (3) a distal part rest-    those proposed by the Committee on Hearing and Equilibrium
ing completely within layers of dura mater.                  of the American Academy of Otolaryngology–Head and Neck
   The dimensions of the VA and the ED-ES complex            Surgery.1 None of the patients expressed the so-called
render visualization of these structures by radiologic       cochlear or vestibular types of MD.
methods technically feasible. The VA can be visualized           The patient group included 10 men and 13 women. The
on CT, and the postisthmic part of the ED and the ES,        right ear was involved in 12 patients, and the left ear was
along with their stroma, can be visualized on MRI. In        involved in 11. The mean age of the patients was 48.6 years
this context, the degree and type of periaqueductal          (range 19-72 years). In 12 patients (52%) the disease duration
pneumatization is crucial because it seems to have a         ranged between 4 and 9 years, in 4 (17.4%) it was more than
direct influence on the appearance and the length of the     9 years, and in the remaining 7 (30.6%) it was less than 4
VA.6                                                         years. The mean duration of symptoms was 5.3 years (range
   In our study we classified the type of pneumatization     0.3-24 years).
of our patients according to the proposed scheme by              The mean value of the pure-tone audiograms (500, 1000,
Stahle and Wilbrand.6 They considered 3 types of             2000, and 4000 Hz) was 48.2 dB, and the type of hearing loss
pneumatization: type I, with large-cell pneumatization;      was flat in 14 patients (60.9%), downward sloping in 6 (26%),
type II, with small air cells or bone marrow spaces; and     and upward sloping in 3 (13%). At the time of HRCT and
type III, with a complete absence of air cells. In type I    MRI, the disease in all 23 patients was in an inactive phase,
the VA is longer, and its external aperture is wider.        and none of them reported vertigo.
Contrary to type I, type III is compatible with a shorter        As a control group, we used 50 patients who had no previ-
Otolaryngology–
                                                                                                                                  Head and Neck Surgery
116      XENELLIS et al                                                                                                                        July 2000




Table 1. Frequency distribution of the pneumatiza-                                 Table 3. Descriptive characteristics of the width of
tion type among control and MD groups                                              the VA among control and MD groups
                       Control group              MD group                                                                    Average   Minimum          Maximum
                                                                                            Group                 n            (mm)      (mm)             (mm)
Pneumatization
    type               n             %           n            %          P value
                                                                                     Control patient              56           1.08          0.50          2.00
                                                                                     Diseased ear                 13           0.63          0.40          1.00
        I              12           41.4          2          11.1         0.01
                                                                                     Nondiseased ear              14           0.83          0.60          1.20
        II             10           34.5          6          33.3
        III             7           24.1         10          55.5


                                                                                   Table 4. Frequency distribution of the visualization
Table 2. Frequency distribution of the visualization                               of the MR examination among control group, dis-
of the HRCT examination among control and MD                                       eased and nondiseased ears from MD groups
groups                                                                                                                           MD group
                                             MD group
                                                                                                                Control           Diseased          Nondiseased
                               Control        Diseased            Nondiseased                                   group*               ear†              ear‡
                               group*            ear†                ear‡
                                                                                         HRCT result        n           %        n       %           n       %
      HRCT result          n        %        n        %             n       %
                                                                                        Visualized          25         64.1       9     39.1        14       63.6
     Visualized          56        96.6     13        72.2          14     77.8         Nonvisualized       14         35.9      14     60.9         8       36.4
     Nonvisualized        2         3.4      5        27.8           4     22.2    *P = 0.05 for control group versus diseased ear (Fisher exact test).
                                                                                   †P = 0.59 for control group versus nondiseased ear (Fisher exact test).
*P = 0.007 for control group versus diseased ear (Fisher exact test).
                                                                                   ‡P = 0.08 for diseased versus nondiseased ear (Fisher exact test).
†P = 0.02 for control group versus nondiseased ear (Fisher exact test).
‡P = 0.78 for diseased versus nondiseased ear (Fisher exact test).




ous history of any ear disease and had CT and MRI examina-                         RESULTS
tion of the brain for other reasons. More precisely, 29 of them                        Table 1 shows the HRCT findings regarding the
had HRCT and the other 21 had MRI.                                                 pneumatization of the temporal bones in the diseased
    HRCT of the petrous bone was performed on a 9800 GE                            and control groups. We did not consider separately the
scanner with the following technical characteristics: 1.5-mm                       2 ears of the patients with MD because we found that
slice thickness, 1-mm table increment, 200 mA, 3 seconds,                          the pneumatization was similar between the two sites.
120 kV, 10-cm field of view (FOV), and bone algorithm. The                         From the univariate analysis and the χ2 test from trend,
section plane was 30° above the anthropologic line.11 For                          we found that there was statistically significant differ-
improved demonstration of the VA, reformed images were                             ence in the type of pneumatization between the control
obtained in the sagittal plane parallel to a line connecting the                   group and the MD group. Type I pneumatization was
external aperture of the VA and the common crus of the pos-                        found in 41.4% of the control group and only 11.1% of
terior and superior semicircular canals (Fig 1).                                   the MD group. On the other hand, type III pneumatiza-
    Measurements of the VA were made by one radiologist                            tion was found in 55.5% of patients with MD and only
blinded to any clinical information. Images were magnified by                      in 24.1% of control patients. Type II pneumatization
2, and the anteroposterior diameter of the VA was manually cal-                    was similar in both groups.
culated at the midpoint of the postisthmic segment of the VA.                          Table 2 shows the percentage of visualization of VA
    MRI was performed on a Phillips 0.5-T Gyroscan unit.                           in the various groups after the tomographic examination
Two-dimensional Fourier transform images of the membra-                            of the temporal bone with HRCT. In the control group
nous labyrinth were obtained with an SE T1-weighted pulse                          both ears were considered together, whereas in the MD
sequence (recovery time, 700 ms; echo time, 15 ms; FOV, 20                         group the two ears were evaluated separately.
cm; matrix, 192 × 256) and a GRE pulse sequence (recovery                              The VA was not visualized in 3.4% of the control
time, 40 ms; echo time, 17 ms; flip angle, 90°; FOV, 16 cm;                        group. In the MD group it was not visualized in the
matrix, 205 × 256). The section plane was 30° to the anthro-                       affected ears in 27.8% and in the ears on the opposite
pologic line. Slice thickness was 3 mm, and interslice gap was                     side in 22.2%.
0.3 mm for both sequences. All examinations were performed                             Both differences, between the control group and the
with a head coil (Fig 2).                                                          diseased and nondiseased ears of the MD group, were
Otolaryngology–
Head and Neck Surgery
Volume 123 Number 1 Part 1                                                                            XENELLIS et al   117




                                                               Fig 4. MRI of a patient with MD: ED-ES system is not visual-
Fig 3. HRCT of a patient with MD: VA is not visualized. Poor   ized.
periaqueductal pneumatization (arrow).




found to be statistically significant. On the contrary,        DISCUSSION
examining the affected and nonaffected ears of the MD              The ED-ES complex has long been recognized as a
group, no statistically significant differences were           possible explanation of the pathogenesis of EH, which
observed.                                                      is the histopathologic substrate of MD.4,12 Studies sug-
   Performing the Mann-Whitney U and the Wilcoxon              gest that the ED and ES are both actively involved in
nonparametric tests, we compared also the width of the         endolymph regulation. In experimental animals it was
visualized VA between the control and the MD groups.           proved that the destruction of the ES or obstruction of
More precisely, as shown in Table 3, the average width         the ED resulted regularly in progressive EH.4
of the VAs in the control group was 1.08 mm (range                 Clemis and Valvassori5 first reported that there is a
0.50-2.0 mm), whereas it was only 0.63 mm (range               higher incidence of nonvisualization of the VA in
0.40-1.0 mm) in the diseased ears (P < 0.001) and 0.83         patients with MD on conventional tomography. Their
mm (range 0.60-1.2 mm) in the nondiseased ears (P <            observation was criticized by Yuen and Schuknecht,13
0.01) in the MD group.                                         who reported that measurements of VAs in histologic
   Table 4 depicts the percentages of visualized and           sections of temporal bones of patients with MD failed to
nonvisualized ED-ES complex with MRI in the con-               establish any statistical evidence of narrowing, in com-
trol and the MD group. In the latter group the dis-            parison with measurements in normal temporal bones.
eased and nondiseased ears were also considered sep-           However, these authors measured the isthmic and not
arately.                                                       the postisthmic part of the VA, as was recommended by
   The ED-ES complex was visualized in 64.1% of the            Clemis and Valvassori. Sando and Ikeda14 investigating
control group and only in 39.1% of the diseased ears.          this controversy confirmed the finding of Clemis and
This difference was proved to have a marginally statis-        Valvassori of hypoplasia of the postisthmic segment of
tical significance (P ~ 0.05). The visualization of the        the VAs in histologic examinations of temporal bones of
ED-ES complex in the nondiseased ears of the MD                patients with MD. Absence and morphologic abnormal-
group was almost similar to that of the control group          ities of the VA-ES complex, along with poor pneumati-
(63.6%), without any statistical significance.                 zation of the periaqueductal mastoid air cells, have been
Otolaryngology–
                                                                                                    Head and Neck Surgery
118   XENELLIS et al                                                                                             July 2000




correlated with ES fibrosis, which is one of the pro-        of endolymph in cases of advanced EH. MRI when the
posed causes of MD.7                                         endolymph has no access to the ES may fail to depict
   There is no universally accepted classification of the    the ED-ES complex. However, the physician must
different morphology types of VAs, nor is there a stan-      always keep in mind the small possibility of an anatomic
dard point where measurements should be taken. In this       aberration of the ES20 as a cause of a failure in visual-
study we measured the width of the VA at the midpoint        ization of the ED-ES complex. In our study we did not
of the postisthmic segment.                                  find any statistically significant difference (P ~ 0.1) in
   Valvassori and Dobben15 classified the VA into 5          visualization of ES between the diseased and nondis-
types, based on measurements at the anteroposterior          eased ears and only a marginally statistical difference
diameter of the midpoint of the postisthmic segment, as      between the diseased ears of the MD group and those of
follow: (1) normal, 0.5 to 1 mm; (2) filiform, less than     the control group. In this particular group of patients,
0.3 mm; (3) large, 1.5 cm or more; and (4) obliterated,      the disease was in an inactive phase, and no patients
when a portion of the postisthmic segment is not visu-       showed hearing loss of more than 65 dB (mean value
alized; and (5) nonvisualized.                               48.2 dB). In this context it is important to note the
   Our data are in accordance with those of others,6,7       observation of Tanioka et al,17 who found that in
who found that MD is associated with an increased inci-      patients having acute episodes of MD, the ED-ES com-
dence of abnormally decreased periaqueductal pneuma-         plex was not adequately visible in the affected ear but
tization and small or nonvisualized VA (Fig 3). More         was seen well in the unaffected ear; Tanioka et al also
precisely, type III was found in 55.5% of ears of            found that during remission the ED-ES complex was
patients with MD and only 24.1% of the ears of control       not visible in patients with clinically advanced disease
subjects (Table 1). Likewise the VA was not visualized       but was seen well during the early-to-moderate stages
in 27.8% in the diseased ears and only 3.4% in the con-      of disease.
trol group ears (Table 2). Table 3 displays 2 more
important findings. First, in the studied group the aver-    CONCLUSIONS
age width of the VA is obviously smaller than in the           1. HRCT findings in our group of patients with defi-
healthy subjects, and second, the nondiseased ears in             nite MD are in accordance with those of others,
the MD group show the same abnormalities as the                   who found that MD is associated with an in-
affected ones. This evidence supports the opinion that            creased incidence of abnormally decreased peri-
the disease is bilateral and there is a progressively             aqueductal pneumatization and small or nonvisu-
increasing incidence of involvement of the second ear if          alized VA.
the patients with MD are followed up for many years.9          2. MRI is a sensitive examination of the membra-
   Preliminary studies using MRI suggest that patients            nous labyrinth and may contribute to better under-
with MD often have a small or nonvisualized ED-ES                 standing of the pathophysiology of the different
complex.16 Results of this study confirm the above                stages and phases of MD.
observation (Fig 4). Visualization of the ED-ES system         3. HRCT and MRI may be used as confirmatory
between diseased ears and ears from the control group             examination techniques when the diagnosis of MD
indicated a marginally statistically significant differ-          is in question.
ence (P ~ 0.05). On the contrary, the visualization of the     4. Further studies are necessary to confirm the abili-
ED-ES in nondiseased ears of patients with MD was not             ty of MRI to depict and differentiate the different
statistically different from that in ears of control sub-         stages of MD. If this proves to be true, MRI may
jects.                                                            become a useful means for planning individually
   It is probable that an adequately visible ED-ES com-           the best therapeutical approach for each patient.
plex with MRI has to do with the stage and phase
                                                             REFERENCES
(active-nonactive) of the disease.17 Linthicum and
Xenellis18 disclosed that the temporal bones of patients      1. Committee on Hearing and Equilibrium guidelines for the diag-
                                                                 nosis and evaluation of therapy in Meniere’s disease. Otolaryngol
with MD with hearing loss (0.5, 1.0, and 2.0 kHz) of 65          Head Neck Surg 1995;113:181-5.
dB or greater showed small or obliterated ES and a far        2. Meniere P. Sur une form de surdite grave dependant d’une lesion
advanced EH with a severe deformation of the normal              de l’oreille interne. Gar Med Paris 1861;16:29.
                                                              3. Schuknecht HF. Disorders of unknown or multiple causes. In:
histoanatomic architecture of the membranous inner               Schuknecht HF, editor. Pathology of the ear. 2nd ed. Philadel-
ear. Accordingly, the passage of endolymph from the              phia: Lea and Febiger; 1993. p. 499-519.
pars superior and inferior to the ES in cases such is         4. Kimura RS. Experimental blockage of the endolymphatic duct and
                                                                 sac and its effect on the inner ear of the guinea pig. A study on
probably obstructed. Schuknecht and Ruther19 described           endolymphatic hydrops. Ann Otol Rhinol Laryngol 1967;76:664-87.
the possible areas of blockage of the longitudinal flow       5. Clemis SD, Valvassori GE. Recent radiographic and clinical
Otolaryngology–
Head and Neck Surgery
Volume 123 Number 1 Part 1                                                                                               XENELLIS et al     119




      observations on the vestibular aqueduct (a preliminary report).      13. Yuen SS, Schuknecht H. Vestibular aqueduct and endolymphatic
      Otolaryngol Clin North Am 1968;1:339-46.                                 duct in Meniere’s disease. Arch Otolaryngol 1972;96:553-5.
 6.   Stahle J, Wilbrand H. The vestibular aqueduct in patients with       14. Sando J, Ikeda M. The vestibular aqueduct in patients with
      Meniere’s disease. A tomographic and clinical investigation. Acta        Meniere’s disease: a temporal bone histopathological investiga-
      Otolaryngol (Stockh) 1974;78:36-48.                                      tion. Acta Otolaryngol (Stockh) 1984;97:558-70.
 7.   Stephen FH, Fitzgerald O’Connor, et al. Significance of tomog-       15. Valvassori G, Dobben G. Multidirectional and CT of the vestibu-
      raphy in Meniere’s disease: periaqueductal pneumatization.               lar aqueduct in Meniere’s disease. Ann Otol Rhinol Laryngol
      Laryngoscope 1983;93:1551-3.                                             1983;93:547-50.
 8.   Kraus EM, Dubois PJ. Tomography of the vestibular aqueduct in        16. Tanioka H, Zusho H, Machida T et al. High resolution MR imag-
      ear disease. Arch Otolaryngol 1979;105:91-8.                             ing of the inner ear: findings in Meniere’s disease. Eur J Radiol
 9.   Sackett JF, Korareck JA, Arenberg IK. The clinical significance          1992;15:83-8.
      of tomographic visualization or nonvisualization of the vestibular   17. Tanioka H, Kimitaka K, Zusho H, et al. MR of the endolymphat-
      aqueduct. Otolaryngol Clin North Am 1980;13:657-64.                      ic duct and sac: findings in Meniere disease. AJNR Am J
10.   Lundquist PG. The endolymphatic duct and sac in the guinea pig.          Neuroradiol 1997;18:45-51.
      An electron microscopic and experimental investigation. Acta         18. Linthicum FH Jr, Xenellis JE. Pathophysiological mechanisms to
      Otolaryngol (Stockh) 1965;Suppl 201:1.                                   Meniere’s syndrome based on infrared oculography and
11.   Brogan M, Chakeres DN, Schmalbrock P. High resolution 3DF                histopathology. Proceedings of the 4th International Symposium
      MR imaging of the endolymphatic duct and soft tissues of otic            on Meniere’s disease.
      capsule. AJNR Am J Neuroradiol 1991;12:1-11.                         19. Schuknecht HF, Ruther A. Blockage of longitudinal flow in
12.   Egami T, Sando I. Pathology of the vestibular aqueduct and               endolymphatic hydrops. Eur Arch Otorhinolaryngol 1991;248:
      endolymphatic sac in temporal bones with endolymphatic hy-               209-17.
      drops. In: Hood JD, editor. Vestibular mechanisms in health and      20. Feghali JG, Linthicum FH Jr, Xenellis SE. Is the endolymphatic sac
      disease. London: Academic Press; 1978. p. 122-8.                         always accessible? Otolaryngol Head Neck Surg 1985;93:21-4.




                         Brachytherapy Workshop

                            The 3rd Workshop on Brachytherapy in Head and Neck Cancer will be held August
                         27-29, 2000, in Kiel, Germany. Chairmen are Prof Dr H. Rudert and Prof Dr G.
                         Kovács.
                             For further information, contact S. Gottschlich, MD, Department of Otorhino-
                         laryngology–Head and Neck Surgery, University of Kiel, Arnold-Heller-Strasse 14, D-
                         24105 Kiel, Germany; phone, 49-431-5972321; fax, 49-431-5972272; e-mail, rud-
                         ert@hno-uni-kiel.de.

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Role of the new imaging modalities in the investigation of meniere disease

  • 1. INTERNATIONAL ORIGINAL ARTICLES EUGENE N. MYERS, MD International Editor Role of the new imaging modalities in the investigation of Meniere’s disease JOHN XENELLIS, MD, LAMBROS VLAHOS, MD, ANDREAS PAPADOPOULOS, MD, PERICLES NOMICOS, MD, KONSTANTINOS PAPAFRAGOS, MD, and GEORGE ADAMOPOULOS, MD, Athens, Greece The incidence of nonvisualization of the vestibular M eniere’s disease (MD) is a well-established clini- aqueduct and the endolymphatic duct–endolym- cal entity; patients with the definite type have all of the phatic sac (ED-ES) complex as well as the type of following symptoms: vertigo, hearing loss, and tinni- periaqueductal pneumatization were evaluated in tus or ear pressure.1 Since MD’s description by Prosper 23 patients with definite Meniere’s disease (MD) by Meniere,2 the scientific community has tried to discover high-resolution CT and by MRI. Fifty subjects with no the underlying etiopathophysiologic factors responsible previous history of any ear disease were used as a for the characteristic vestibulocochlear dysfunction. control group. High-resolution CT results disclosed Today it is widely accepted that the histopathologic that in the control group the percentage of nonvi- substrate of the disease is endolymphatic hydrops (EH) sualized vestibular aqueduct (3.4%) was statistically because of the disruption of the balance between pro- significantly lower than in the MD group, when duction and absorption of the endolymph.3 either the diseased ear (27.8%) or the nondiseased A key role in the pathogenesis of the EH is attributed ear of the same group (22.2%) was examined. In to impaired function of endolymphatic duct (ED) and addition, no differences were observed between endolymphatic sac (ES).4 The ED-ES system lies partly the diseased and nondiseased ears of the patients within, partly through, and partly outside of the vestibu- with MD. The periaqueductal pneumatization was lar aqueduct (VA), which is a bony channel of the otic also found to be statistically significantly lower in capsule. the MD group. As regards the MRI examination, the In the literature concerning MD, there are some results of the analysis showed that the ED-ES com- reports of a high incidence of radiologic nonvisualiza- plex was visualized more frequently in the ears of tion of the VA5,6 and poor pneumatization of the sur- the control subjects (64.1%) than in the diseased rounding mastoid air cells in the diseased ears.6,7 How- ears of the patients with Meniere’s disease (39.1%). ever, other authors8,9 consider these observations as This difference had a marginal statistical signifi- nonspecific diagnostic signs seen in a variety of ear dis- cance (P ~ 0.05). We discussed the results in relation eases. to possible underlying pathophysiologic mecha- In this study we analyzed our findings after exami- nisms involving the flow of endolymph toward the ES nation of the VA, ED-ES complex, and periaqueductal during the different stages of the disease. (Oto- pneumatization with high-resolution (HRCT) and MRI laryngol Head Neck Surg 2000;123:114-9.) of 23 temporal bones from 23 patients with definite uni- lateral MD. We also attempted to delineate the possible implications of these findings on the normal function of the inner ear. ANATOMY OF THE VA AND THE ED-ES COMPLEX From the Departments of ENT (Drs Xenellis, Nomicos, Papafragos, and Adamopoulos) and Radiology (Drs Vlahos and Papadopoulos), The VA is a bony canal that contains the ED and the University of Athens Medical School. proximal part of the ES. It extends from the medial wall Reprint requests: J. Xenellis, MD, Sarantapichou 35, GR-114 72 of the vestibule to the posterior surface of the petrous Athens, Greece. pyramid, where it terminates in a bony cleft called the Copyright © 2000 by the American Academy of Otolaryngology– Head and Neck Surgery Foundation, Inc. endolymphatic meatus, which contains the ES. In 0194-5998/2000/$12.00 + 0 23/77/102113 adults, as the VA traverses the temporal bone, it is doi:10.1067/mhn.2000.102113 shaped like an inverted J. The short limb and the bend 114
  • 2. Otolaryngology– Head and Neck Surgery Volume 123 Number 1 Part 1 XENELLIS et al 115 Fig 2. MRI of a normal ED-ES complex (arrow). Fig 1. Transaxial HRCT of a normal VA (arrow). VA, which also shows a narrow external aperture. In MD there is a high incidence of type III pneumatiza- of the VA together are called the isthmus, and this is the tion.6,7 most narrow part of it. The distal end of the VA expands to accommodate METHODS AND MATERIAL the terminal enlargement of the ED, the ES. Lund- Between 1994 and 1996, 23 patients with definite unilateral quist10 divided the ES into 3 parts: (1) a proximal part MD were evaluated in the ENT Department of the University located within the VA, (2) an intermediate part located of Athens. This group of patients constitutes the source of this partially within the VA and partially between the layers study. The selection criteria for these patients conformed to of dura mater outside the VA, and (3) a distal part rest- those proposed by the Committee on Hearing and Equilibrium ing completely within layers of dura mater. of the American Academy of Otolaryngology–Head and Neck The dimensions of the VA and the ED-ES complex Surgery.1 None of the patients expressed the so-called render visualization of these structures by radiologic cochlear or vestibular types of MD. methods technically feasible. The VA can be visualized The patient group included 10 men and 13 women. The on CT, and the postisthmic part of the ED and the ES, right ear was involved in 12 patients, and the left ear was along with their stroma, can be visualized on MRI. In involved in 11. The mean age of the patients was 48.6 years this context, the degree and type of periaqueductal (range 19-72 years). In 12 patients (52%) the disease duration pneumatization is crucial because it seems to have a ranged between 4 and 9 years, in 4 (17.4%) it was more than direct influence on the appearance and the length of the 9 years, and in the remaining 7 (30.6%) it was less than 4 VA.6 years. The mean duration of symptoms was 5.3 years (range In our study we classified the type of pneumatization 0.3-24 years). of our patients according to the proposed scheme by The mean value of the pure-tone audiograms (500, 1000, Stahle and Wilbrand.6 They considered 3 types of 2000, and 4000 Hz) was 48.2 dB, and the type of hearing loss pneumatization: type I, with large-cell pneumatization; was flat in 14 patients (60.9%), downward sloping in 6 (26%), type II, with small air cells or bone marrow spaces; and and upward sloping in 3 (13%). At the time of HRCT and type III, with a complete absence of air cells. In type I MRI, the disease in all 23 patients was in an inactive phase, the VA is longer, and its external aperture is wider. and none of them reported vertigo. Contrary to type I, type III is compatible with a shorter As a control group, we used 50 patients who had no previ-
  • 3. Otolaryngology– Head and Neck Surgery 116 XENELLIS et al July 2000 Table 1. Frequency distribution of the pneumatiza- Table 3. Descriptive characteristics of the width of tion type among control and MD groups the VA among control and MD groups Control group MD group Average Minimum Maximum Group n (mm) (mm) (mm) Pneumatization type n % n % P value Control patient 56 1.08 0.50 2.00 Diseased ear 13 0.63 0.40 1.00 I 12 41.4 2 11.1 0.01 Nondiseased ear 14 0.83 0.60 1.20 II 10 34.5 6 33.3 III 7 24.1 10 55.5 Table 4. Frequency distribution of the visualization Table 2. Frequency distribution of the visualization of the MR examination among control group, dis- of the HRCT examination among control and MD eased and nondiseased ears from MD groups groups MD group MD group Control Diseased Nondiseased Control Diseased Nondiseased group* ear† ear‡ group* ear† ear‡ HRCT result n % n % n % HRCT result n % n % n % Visualized 25 64.1 9 39.1 14 63.6 Visualized 56 96.6 13 72.2 14 77.8 Nonvisualized 14 35.9 14 60.9 8 36.4 Nonvisualized 2 3.4 5 27.8 4 22.2 *P = 0.05 for control group versus diseased ear (Fisher exact test). †P = 0.59 for control group versus nondiseased ear (Fisher exact test). *P = 0.007 for control group versus diseased ear (Fisher exact test). ‡P = 0.08 for diseased versus nondiseased ear (Fisher exact test). †P = 0.02 for control group versus nondiseased ear (Fisher exact test). ‡P = 0.78 for diseased versus nondiseased ear (Fisher exact test). ous history of any ear disease and had CT and MRI examina- RESULTS tion of the brain for other reasons. More precisely, 29 of them Table 1 shows the HRCT findings regarding the had HRCT and the other 21 had MRI. pneumatization of the temporal bones in the diseased HRCT of the petrous bone was performed on a 9800 GE and control groups. We did not consider separately the scanner with the following technical characteristics: 1.5-mm 2 ears of the patients with MD because we found that slice thickness, 1-mm table increment, 200 mA, 3 seconds, the pneumatization was similar between the two sites. 120 kV, 10-cm field of view (FOV), and bone algorithm. The From the univariate analysis and the χ2 test from trend, section plane was 30° above the anthropologic line.11 For we found that there was statistically significant differ- improved demonstration of the VA, reformed images were ence in the type of pneumatization between the control obtained in the sagittal plane parallel to a line connecting the group and the MD group. Type I pneumatization was external aperture of the VA and the common crus of the pos- found in 41.4% of the control group and only 11.1% of terior and superior semicircular canals (Fig 1). the MD group. On the other hand, type III pneumatiza- Measurements of the VA were made by one radiologist tion was found in 55.5% of patients with MD and only blinded to any clinical information. Images were magnified by in 24.1% of control patients. Type II pneumatization 2, and the anteroposterior diameter of the VA was manually cal- was similar in both groups. culated at the midpoint of the postisthmic segment of the VA. Table 2 shows the percentage of visualization of VA MRI was performed on a Phillips 0.5-T Gyroscan unit. in the various groups after the tomographic examination Two-dimensional Fourier transform images of the membra- of the temporal bone with HRCT. In the control group nous labyrinth were obtained with an SE T1-weighted pulse both ears were considered together, whereas in the MD sequence (recovery time, 700 ms; echo time, 15 ms; FOV, 20 group the two ears were evaluated separately. cm; matrix, 192 × 256) and a GRE pulse sequence (recovery The VA was not visualized in 3.4% of the control time, 40 ms; echo time, 17 ms; flip angle, 90°; FOV, 16 cm; group. In the MD group it was not visualized in the matrix, 205 × 256). The section plane was 30° to the anthro- affected ears in 27.8% and in the ears on the opposite pologic line. Slice thickness was 3 mm, and interslice gap was side in 22.2%. 0.3 mm for both sequences. All examinations were performed Both differences, between the control group and the with a head coil (Fig 2). diseased and nondiseased ears of the MD group, were
  • 4. Otolaryngology– Head and Neck Surgery Volume 123 Number 1 Part 1 XENELLIS et al 117 Fig 4. MRI of a patient with MD: ED-ES system is not visual- Fig 3. HRCT of a patient with MD: VA is not visualized. Poor ized. periaqueductal pneumatization (arrow). found to be statistically significant. On the contrary, DISCUSSION examining the affected and nonaffected ears of the MD The ED-ES complex has long been recognized as a group, no statistically significant differences were possible explanation of the pathogenesis of EH, which observed. is the histopathologic substrate of MD.4,12 Studies sug- Performing the Mann-Whitney U and the Wilcoxon gest that the ED and ES are both actively involved in nonparametric tests, we compared also the width of the endolymph regulation. In experimental animals it was visualized VA between the control and the MD groups. proved that the destruction of the ES or obstruction of More precisely, as shown in Table 3, the average width the ED resulted regularly in progressive EH.4 of the VAs in the control group was 1.08 mm (range Clemis and Valvassori5 first reported that there is a 0.50-2.0 mm), whereas it was only 0.63 mm (range higher incidence of nonvisualization of the VA in 0.40-1.0 mm) in the diseased ears (P < 0.001) and 0.83 patients with MD on conventional tomography. Their mm (range 0.60-1.2 mm) in the nondiseased ears (P < observation was criticized by Yuen and Schuknecht,13 0.01) in the MD group. who reported that measurements of VAs in histologic Table 4 depicts the percentages of visualized and sections of temporal bones of patients with MD failed to nonvisualized ED-ES complex with MRI in the con- establish any statistical evidence of narrowing, in com- trol and the MD group. In the latter group the dis- parison with measurements in normal temporal bones. eased and nondiseased ears were also considered sep- However, these authors measured the isthmic and not arately. the postisthmic part of the VA, as was recommended by The ED-ES complex was visualized in 64.1% of the Clemis and Valvassori. Sando and Ikeda14 investigating control group and only in 39.1% of the diseased ears. this controversy confirmed the finding of Clemis and This difference was proved to have a marginally statis- Valvassori of hypoplasia of the postisthmic segment of tical significance (P ~ 0.05). The visualization of the the VAs in histologic examinations of temporal bones of ED-ES complex in the nondiseased ears of the MD patients with MD. Absence and morphologic abnormal- group was almost similar to that of the control group ities of the VA-ES complex, along with poor pneumati- (63.6%), without any statistical significance. zation of the periaqueductal mastoid air cells, have been
  • 5. Otolaryngology– Head and Neck Surgery 118 XENELLIS et al July 2000 correlated with ES fibrosis, which is one of the pro- of endolymph in cases of advanced EH. MRI when the posed causes of MD.7 endolymph has no access to the ES may fail to depict There is no universally accepted classification of the the ED-ES complex. However, the physician must different morphology types of VAs, nor is there a stan- always keep in mind the small possibility of an anatomic dard point where measurements should be taken. In this aberration of the ES20 as a cause of a failure in visual- study we measured the width of the VA at the midpoint ization of the ED-ES complex. In our study we did not of the postisthmic segment. find any statistically significant difference (P ~ 0.1) in Valvassori and Dobben15 classified the VA into 5 visualization of ES between the diseased and nondis- types, based on measurements at the anteroposterior eased ears and only a marginally statistical difference diameter of the midpoint of the postisthmic segment, as between the diseased ears of the MD group and those of follow: (1) normal, 0.5 to 1 mm; (2) filiform, less than the control group. In this particular group of patients, 0.3 mm; (3) large, 1.5 cm or more; and (4) obliterated, the disease was in an inactive phase, and no patients when a portion of the postisthmic segment is not visu- showed hearing loss of more than 65 dB (mean value alized; and (5) nonvisualized. 48.2 dB). In this context it is important to note the Our data are in accordance with those of others,6,7 observation of Tanioka et al,17 who found that in who found that MD is associated with an increased inci- patients having acute episodes of MD, the ED-ES com- dence of abnormally decreased periaqueductal pneuma- plex was not adequately visible in the affected ear but tization and small or nonvisualized VA (Fig 3). More was seen well in the unaffected ear; Tanioka et al also precisely, type III was found in 55.5% of ears of found that during remission the ED-ES complex was patients with MD and only 24.1% of the ears of control not visible in patients with clinically advanced disease subjects (Table 1). Likewise the VA was not visualized but was seen well during the early-to-moderate stages in 27.8% in the diseased ears and only 3.4% in the con- of disease. trol group ears (Table 2). Table 3 displays 2 more important findings. First, in the studied group the aver- CONCLUSIONS age width of the VA is obviously smaller than in the 1. HRCT findings in our group of patients with defi- healthy subjects, and second, the nondiseased ears in nite MD are in accordance with those of others, the MD group show the same abnormalities as the who found that MD is associated with an in- affected ones. This evidence supports the opinion that creased incidence of abnormally decreased peri- the disease is bilateral and there is a progressively aqueductal pneumatization and small or nonvisu- increasing incidence of involvement of the second ear if alized VA. the patients with MD are followed up for many years.9 2. MRI is a sensitive examination of the membra- Preliminary studies using MRI suggest that patients nous labyrinth and may contribute to better under- with MD often have a small or nonvisualized ED-ES standing of the pathophysiology of the different complex.16 Results of this study confirm the above stages and phases of MD. observation (Fig 4). Visualization of the ED-ES system 3. HRCT and MRI may be used as confirmatory between diseased ears and ears from the control group examination techniques when the diagnosis of MD indicated a marginally statistically significant differ- is in question. ence (P ~ 0.05). On the contrary, the visualization of the 4. Further studies are necessary to confirm the abili- ED-ES in nondiseased ears of patients with MD was not ty of MRI to depict and differentiate the different statistically different from that in ears of control sub- stages of MD. If this proves to be true, MRI may jects. become a useful means for planning individually It is probable that an adequately visible ED-ES com- the best therapeutical approach for each patient. plex with MRI has to do with the stage and phase REFERENCES (active-nonactive) of the disease.17 Linthicum and Xenellis18 disclosed that the temporal bones of patients 1. Committee on Hearing and Equilibrium guidelines for the diag- nosis and evaluation of therapy in Meniere’s disease. Otolaryngol with MD with hearing loss (0.5, 1.0, and 2.0 kHz) of 65 Head Neck Surg 1995;113:181-5. dB or greater showed small or obliterated ES and a far 2. Meniere P. Sur une form de surdite grave dependant d’une lesion advanced EH with a severe deformation of the normal de l’oreille interne. Gar Med Paris 1861;16:29. 3. Schuknecht HF. Disorders of unknown or multiple causes. In: histoanatomic architecture of the membranous inner Schuknecht HF, editor. Pathology of the ear. 2nd ed. Philadel- ear. 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