Original Article
International Journal of Audiology 2009; 48:594600
Celene McNeill+,§
Simon R.M. Freeman$,%
Catherine McMahon§
Downloaded By: [Macquarie University] At: 22:52 6 August 2009
*Healthy Hearing & Balance Care,
NSW, Australia
§
Speech Hearing and Language
Research Centre, Macquarie
University, NSW, Australia
$
The Graham Fraser Foundation,
London, UK
%
Department of Otolaryngology,
Royal Prince Alfred Hospital, Sydney,
NSW, Australia
Key Words
Meniere’s disease
Hearing fluctuation
In-situ audiometry
Self-hearing test
Portable hearing aid programmer
Audiogram configuration
Short-term hearing fluctuation in Meniere’s
disease
Abstract
Sumario
This study aimed to assess the extent and implications of
short-term hearing fluctuation in Meniere’s disease.
Thirty-six subjects diagnosed with Meniere’s were recruited to measure their own hearing using in-situ
audiometry via a hearing aid (Widex Diva) and a portable
programmer (SP3). Self-hearing tests measuring up to 14
frequency bands were conducted three times a day over
eight weeks using the expanded SensogramTM. Twentythree ears showed low frequency fluctuation while ten
fluctuated in mid frequencies with some ‘double peak’
audiogram configurations. Eight ears in the later stages of
Meniere’s, contrary to expected, also recorded fluctuation
across all frequencies. Self-hearing testing Meniere’s ears
over eight weeks revealed great hearing fluctuation with
significant changes in audiogram configuration. It suggests that as endolymphatic hydrops progresses through
the cochlea, low frequency fluctuation is followed by
fluctuation in the mid frequencies, leading to fluctuation
across all frequencies. Use of a self-hearing test may
facilitate diagnosis and hearing aid fitting for this
population, as clinical audiograms may not provide
accurate information of hearing fluctuation.
Este estudio intenta evaluar el grado y las implicaciones
de las fluctuaciones de corto plazo en la enfermedad de
Méniére. Se reclutaron treinta y seis sujetos diagnosticados con Méniére para medir su propia audición utilizando audiometrı́a in-situ a través de un auxiliar auditivos
(Widex Diva) y un programador portátil (SP3). Se
condujo una prueba de auto-evaluación auditiva midiendo hasta 14 bandas de frecuencia, realizada tres veces
al dÚa durante ocho semanas usando el Sensogram
expandido. Veintitrés oı́dos mostraron fluctuaciones en
las frecuencias graves, mientras que diez fluctuaron en las
frecuencias medias, con configuraciones audiométricas de
‘‘doble pico’’. Contrario a lo esperado, ocho oı́dos en
etapas tardı́as de Méniére también registraron fluctuaciones en todas las frecuencias. El auto-examen auditivo
en oı́dos con Méniére durante ocho semanas reveló
grandes fluctuaciones auditivas con cambios significativos en la configuración del audiograma. Se sugiere que
conforme el hidrops endolinfático progresa a lo largo de
la cóclea, la fluctuación en los graves es seguida por
fluctuación en las frecuencias medias, llevando a una
fluctuaciœn en todas las frecuencias. El uso de la prueba
de auto-evaluación auditiva puede facilitar el diagnóstico
y la adaptaciœn de auxiliares auditivos para esta población, dado que los audiogramas clı́nicos pueden no
aportar información precisa sobre la fluctuación auditiva.
Sensorineural hearing loss is one of the four symptoms
characterizing Meniere’s disease. Numerous studies have considered the shape or configuration of the pure-tone audiogram
that occurs in Meniere’s disease. Four main hearing loss
configurations have been described in the literature: rising (low
frequency loss), falling (high frequency loss), peak (low and high
frequency loss with better hearing at 10002000 Hz), and flat
(affecting all frequencies equally) (Savastano et al, 2006;
Mateijsen et al, 2001; Meyerhoff et al, 1981; Stahle et al, 1989;
Ries et al, 1999). In the early stages of Meniere’s disease there is
predominantly a low frequency or peak hearing loss with a
tendency to assume a flat configuration as the loss becomes
more severe with disease progression. In older patients, the peak
audiogram may be a combination of low frequency loss due to
Meniere’s, and high frequency loss due to presbyacusis. This
configuration, however, also occurs in young patients with
Meniere’s disease who have no evidence of a high frequency
loss in the contralateral ear, leading to the theory that the high
frequency damage is also due to Meniere’s disease (Savastano
et al, 2006; Ries et al, 1999).
Fluctuation is the main characteristic of hearing in Meniere’s
disease. Long-term studies have found this fluctuation to
be present in the early stages in about 70% of patients through
symptom assessment (Green et al, 1991) and audiometry (Savas-
tano et al, 2006), but becoming less prominent as the disease
progresses. Only 9% of patients reported continued fluctuation
after a minimum of nine years follow-up (Green et al, 1991).
Hearing fluctuation has mostly been associated with low
frequency hearing loss, although it has been shown to affect
high frequencies to a lesser extent (Savastano et al, 2006;
Eggermont & Schmidt, 1985; Mateijsen et al, 2001). The
magnitude, frequency, and pattern of short-term hearing fluctuation however, are not well documented in the literature.
Long-term studies inevitably suffer from a paucity of audiograms. Even where an audiogram could be performed every
three months over a time span of 10 years, only 40 audiograms
would be available.
Savastano et al (2006) recently described pure-tone audiometric fluctuation and the mean threshold shift in Meniere’s
patients in the early stages, but it was not clear how many
audiograms were used to determine fluctuation. Other studies
have analysed fluctuations but based only on the patients’
subjective assessment (Green et al, 1991; Mateijsen et al,
2001). Eggermont and Schmidt (1985) were able to present
data on hearing fluctuation effectively in a long-term study using
particular cases to illustrate observed trends.
The reliability of subjective assessment without documented
audiogram, however, is highly questioned by the first author, as
ISSN 1499-2027 print/ISSN 1708-8186 online
DOI: 10.1080/14992020802716778
# 2009 British Society of Audiology, International
Society of Audiology, and Nordic Audiological Society
Celene McNeill
1204/1 Newland St, Bondi Junction, NSW 2022, Australia.
E-mail: cmcneill@tpg.com.au
Received:
July 26, 2008
Accepted:
December 30, 2008
Downloaded By: [Macquarie University] At: 22:52 6 August 2009
patients are usually not able to detect fluctuations especially
when best hearing thresholds are at levels below 50 dBHL.
Clinical audiometry on the other hand cannot be relied upon to
record hearing fluctuation, as they may not be performed
frequently enough.
Demonstration of fluctuation requires a series of audiograms
performed at regular intervals for a period of time. Audiograms
performed several times a day would be the most reliable method
to demonstrate hearing fluctuation patterns in an individual
patient. Such procedures however are very time consuming and
not viable in clinical settings.
Audiograms are traditionally performed using an audiometer.
More recently audiologists have been able to perform in-situ
audiograms in the clinic without an audiometer, but through
hearing aid fitting software. This is a reliable way of assessing
hearing threshold using a custom made ear mould instead of
headphones with the test stimuli produced by the hearing aid.
The hearing instrument manufacturer Widex was the first to
release a portable device which measures in-situ audiograms with
the Senso Diva hearing aid. This procedure is trade marked as
‘Sensogram’ and allows the clinician to measure hearing thresholds in up to 14 sound frequency bands through the hearing aids,
either connected to a computer using Hi-Pro or Noah Link, as
well as via the SP3 which is Widex’s portable hearing aid
programmer.
Test-retest reliability with in-situ threshold measurements has
been shown to be equivalent to that of currently accepted
audiometric procedures. A previous study of in-situ hearing
threshold measurement using the Widex Senso Diva and the SP3
(Smith-Olinde et al, 2006) found excellent test-retest reliability,
which they considered equivalent to conventional audiometry.
This study measured four frequencies (500, 1000, 2000, and 4000
Hz) finding that 98%, 100%, 100%, and 93% respectively of all
patients fell between 95 dBHL with standard deviations of 3.14,
1.86, 1.97, and 4.02 dBHL. Furthermore, this hearing aid system
enables performance of more detailed audiograms than some
audiometers. It allows hearing threshold testing in 5-db steps
from 0 up to 100 dB at the frequencies of 250, 350, 500, 630, 800,
1000, 1250, 1600, 2000, 2500, 3200, 4000, 6000, and 8000 Hz,
depending on the hearing aid model.
We used this system to assess the magnitude and pattern of
short-term hearing fluctuation in Meniere’s disease by asking a
group of subjects to measure their own hearing thresholds
several times a day over a period of time. It was our hope that
these measurements would provide diagnostic confirmation and
better insight into the audiological aspects of Meniere’s disease.
Methods
Ethics and recruitment
Ethical approval for this study was obtained from Macquarie
University Ethics Review Committee (Human Research). The
study was advertised through a newsletter distributed by the
Meniere’s Support Group of New South Wales (Australia).
Volunteers were recruited if they had a hearing loss in at least
one ear due to Meniere’s disease diagnosed by a specialist in
otolaryngology. Diagnostic criteria was based on Gibson’s 10
points scale and confirmed by trans-tympanic electrocochleography (ECochG) (Conlon & Gibson, 1999). ECochG was
carried out using 1000-Hz, 100-dBHL stimulation with a 16Short-term hearing fluctuation in Meniere’s
disease
ms tone burst (1-ms rise/fall and 14-ms plateau) at a rate of 30/s.
The deflection was measured at approximately 10 ms. ECochG
was confirmative when a summating potential was more negative
than -6 mV. Previous studies using this technique have determined that using this diagnostic threshold provides the optimum
compromise between sensitivity and specificity (Gibson, 1996).
Stage and duration of the disease, degree of hearing loss, and ear
affected were not taken into account when recruiting for this
study. We also did not consider any current or previous medical
interventions that subjects may have undertaken along their
disease process or during the study, such as diet, medication,
and/or surgery. Subjects were expected to continue with their
normal life styles during the study but needed to be conversant
with technology and capable of testing their own hearing at
home for a period of at least eight weeks.
Subjects
Thirty-six volunteers diagnosed with Meniere’s disease, 20
females and 16 males aged from 33 to 78 years, were recruited
and fitted with Widex Senso Diva hearing aids. Twenty-seven
subjects were fitted monaurally and nine binaurally, according to
the hearing loss, giving a total of 45 ears fitted with hearing aids.
Four of the nine subjects with bilateral hearing loss had a contralateral sensorineural hearing loss unrelated to Meniere’s, and five
had bilateral disease.
Control group
A total of eight ears were used as control. One female subject
with bilateral moderate mixed hearing loss due to otosclerosis
and another with a congenital bilateral flat mild to moderate
sensorineural hearing loss, measured their hearing in both ears
twice a day for a period of two weeks. Four subjects of the study
group were fitted with bilateral hearing aids in spite of having
Meniere’s disease in one ear only (as described before) and were
also asked to measure their hearing bilaterally so that the contralateral ear could be used as a control.
Material and Procedure
Participants were fitted with Widex Senso Diva hearing aids
with a custom mould to suit the hearing loss at the time of
fitting. The fitting protocol was based on the Widex proprietor’s
procedure using the expanded Sensogram (Ludvigsen, 2001).
The audiologist performed this via the Noah Link measuring
the hearing thresholds at up to 14 frequency bands followed by
the ‘feedback test’ as prescribed by the Widex fitting protocol.
The Widex Compass software automatically programmed the
hearing aids based on these two measurements. The ‘expanded
Sensogram’ procedure was selected from the Compass menu
(Ludvigsen, 2001). This procedure allows the measurement of
hearing thresholds at 13 frequency bands from 250 to 6000 Hz
in the behind-the-ear SD-9 and SD-19 models, and 14
frequency bands from 250 to 8000 Hz, in the custom devices
SD-X ITC and SD-CIC models. A maximum stimulation level
of 85 dB HL at 250 and 350 kHz, 90 dB at 500, 630, and 800
Hz, 95 dB at 1000 and 1250 Hz, and 100 dB at 2000, 3200,
4000, and 6000 Hz can be achieved using the SD19 model,
while the custom aids produce frequency stimulation up to 8000
Hz at 80 dB with the ITC SD-X, and 75 dB with the SD-CIC
models.
McNeill/Freeman/McMahon
595
Downloaded By: [Macquarie University] At: 22:52 6 August 2009
The participants were then taught how to connect the SP3
device to their hearing aid and to perform their own expanded
Sensogram. Their ability to carry out this procedure was checked
in the clinic, by asking them to repeat the measurements made
by the audiologist earlier in the session. They were provided with
an SP3 to take home and were requested to perform their
Sensograms three times per day over a period of at least eight
weeks and record these on a spreadsheet. They were instructed
to perform these tests in as quiet an environment as possible
while plugging the contralateral ear with their index finger.
Whenever possible, tests were to be performed in the same room
every time. They were also requested to inspect the earmould
prior to testing to exclude the presence of any obstruction, which
would interfere with sound delivery. A follow-up visit was
carried out two weeks after the initial fitting to ensure
Sensograms could be performed with ease. Telephone followups were also conducted as needed.
Prior to measuring their hearing, subjects were also asked to
rate their symptoms of ear fullness, tinnitus, and vertigo on a
scale 15, to be recorded on the spreadsheet along with their
hearing thresholds at each tested frequency.
Figure 2. Example from the same patient as Figure 1 where the
hearing fluctuation is displayed as the standard deviation of the
mean change in hearing at each frequency. The dashed line
represents the expected fluctuation from conventional test-retest
measurements.
Results
Figure 1. Example of a low frequency fluctuating hearing loss
showing data from 247 audiograms where the greatest changes in
hearing thresholds were measured at 250500 Hz.
All thirty-six participants with Meniere’s disease plus two others,
with hearing losses from causes other than endolymphatic
hydrops, complied with the task of measuring their own hearing
on a regular basis. The minimum number of Sensograms
provided by a participant was 25 and the maximum was 381,
giving a total of 5316 audiograms. These records provided an
equivalent mean of 118 audiograms per ear over a three month
period.
Visual classification of audiograms resulted in 23 ears defined
as having a low frequency hearing fluctuation, 10 ears with a mid
frequency fluctuation, eight ears with an all frequency fluctuation, and eight ears presented no fluctuation. Inter-observer
agreement was achieved by all three audiologists for 47 ears, with
the remaining two classified by majority decision.
Figure 1 is an example of one subject with a low frequency
fluctuating hearing loss showing 247 audiograms where the
greatest changes in hearing thresholds were measured at 250500
Hz. Figure 3 is an example of a mid-frequency fluctuating
hearing loss showing 110 audiograms of another subject where
the greatest fluctuation peaked at any one frequency around
8003000Hz. Figure 4 exemplifies a case of an all frequency
fluctuating hearing loss showing 188 audiograms where the
fluctuation appeared similar for the majority of frequencies.
The standard deviation of the mean change in hearing for
these three different patients is respectively demonstrated in
Figure 2, 5, and Figure 6. The area below the dashed line
represents the expected fluctuation in hearing thresholds for
subjects with normal hearing or a stable sensorineural hearing
loss. Figure 7 shows the group data of the 23 ears with a low
frequency fluctuating loss, Figure 8 shows 10 ears with mid
frequency fluctuation, and Figure 9 displays the eight ears with
all frequency fluctuation.
Three ears were found to return to normal thresholds when
their hearing was at its best (two with low frequency, and one with
all frequency hearing loss). When the shape of the audiogram was
considered, it was clear that the same one patient could change
between different configurations (e.g. peak to flat or falling to
flat), depending on the site of fluctuation, at potentially daily
intervals. Figure 10 is an example of the fluctuation that may
596
International Journal of Audiology, Volume 48 Number 8
Analysis
Including the control group, a total of 49 ears were included in
the study. Every Sensogram obtained from each individual
participant was entered in an Excel spreadsheet. These data
were plotted in one single audiogram format graph, making it
easier to visualize the hearing fluctuation for each subject
(example in Figure 1). These graphs were visually inspected by
three audiologists independently and classified according to the
fluctuation into predominantly low frequency, mid frequency, all
frequencies, or no fluctuation.
The magnitude and configuration of hearing fluctuation of
each participant was also plotted using the standard deviation
from the mean at each frequency measured (example in Figure 2).
This allowed easier comparison of the pattern in hearing
fluctuation between participants. Considering that test-retest
reliability of psychophysical hearing assessment in subjects with
sensorineural hearing loss is 10 dBHL (95 dBHL), a standard
deviation of up to 2.5 dBHL of the mean audiometric threshold
was assumed as normal. Therefore only threshold fluctuations of
greater than 2.5 dB were considered as significant.
Downloaded By: [Macquarie University] At: 22:52 6 August 2009
Figure 3. Example of a mid-frequency fluctuating hearing loss
showing data from 110 audiograms where the greatest fluctuation peaked at any one frequency around 8003000 Hz.
occur for a single patient within a period of 24 hours. When the
best thresholds obtainable at each frequency (not necessarily
from the same point in time) were examined together, 16 ears
showed a ‘double peak’ audiogram, with better hearing peaks at
8001000 Hz and 20002500 Hz, and a dip at 1600 Hz (examples
in Figures 1 and 3).
No significant fluctuation, (greater than 95 dB) were recorded
by the control group at any of the tested frequencies, suggesting
good test/retest reliability. This included the four participants who
had unilateral Meniere’s disease and recorded significant fluctuation in the contralateral ear. These participants recorded bilateral
tests performed at the same time and so would have had similar test
conditions. This suggests that the change in measured thresholds
in the active ear reflects reliable hearing threshold fluctuations, at
least for these patients.
Methodological bias
There are several potential sources of bias with regard to our
methodology as subjects tested their own hearing in a noncontrolled environment. The previous study assessing test/retest
reliability of the Sensogram was performed in a soundproof booth
(Smith-Olinde et al, 2006) whereas our participants were
instructed to conduct their hearing measurements in a ‘quiet’
room at home or at work.
Figure 4. Example of an all-frequency fluctuating hearing loss
showing data from 188 audiograms where the fluctuation
appeared similar for the majority of frequencies.
Short-term hearing fluctuation in Meniere’s
disease
Figure 5. Example from the same patient as Figure 3 where the
hearing fluctuation is displayed as the standard deviation of the
mean change in hearing at each frequency. The dashed line
represents the expected fluctuation from conventional test-retest
measurements.
A large study has compared hearing thresholds in a soundproofed environment with those from the same participants in
the workplace with a background noise of up to 68 dB (Wong
et al, 2003). This found that standard deviations of the test
differences were 811 dB, suggesting background noise has a
significant effect on test/retest reliability. It seems however
unlikely that the background noise at the test environment of
our participants was as great as the one in that study but this
effect cannot be discounted.
The participants were also not blinded to their test results, as
they would be during conventional testing. However, this was
pointed out as a positive factor by many participants suggesting
that self-testing may elicit far less false positive responses. This
factor nevertheless needs researching.
Given the large number of audiograms performed by each
participant in this study, it seems unlikely that bias had an effect
in the overall results, however further investigation is needed to
confirm reliability of self-hearing tests performed in a non
controlled environment.
Figure 6. Example from the same patient as Figure 4 where the
hearing fluctuation is displayed as the standard deviation of
the mean change in hearing at each frequency. The dashed line
represents the expected fluctuation from conventional test-retest
measurements.
McNeill/Freeman/McMahon
597
Figure 7. The group data of the 23 ears with low frequency
fluctuating hearing loss showing standard deviation of the mean
change in hearing at each frequency.
Figure 8. The group data of the 10 ears with mid frequency
fluctuating hearing loss standard deviation of the mean change
in hearing at each frequency.
would not have been possible using routine audiological assessments in the clinic. The extent of hearing fluctuation found in
our results was more than we would have predicted based on
current literature. Furthermore hearing fluctuation seems to
occur independently of the other symptoms of Meniere’s disease
and was mostly not associated with vestibular episodes according to our preliminary findings.
The relationship between hearing fluctuation and vertigo
attacks, although of great interest, is beyond the scope of this
paper. Further analysis looking into correlations of fluctuation
amongst the symptoms of Meniere’s disease is currently underway as part of a larger study.
Some of our findings are in agreement with those of
Eggermont and Schmidt (1985) as they also demonstrated
fluctuation to be most prominent at the low frequencies and
least at the high frequencies. Our results also show that as
hearing loss progresses to more severe it does become flatter in
configuration as in other reports, but in our group fluctuation,
although reduced, still continued to occur.
A proportion of our subjects had greatest fluctuation at the mid
frequencies with relatively little at the lows, which as far as we are
aware, has not been previously described in the literature. The
peak audiogram is a prominent feature of Meniere’s disease,
particularly in the early stage, albeit with significant discrepancy
between studies as to its prevalence (Savastano et al, 2006;
Mateijsen et al, 2001; Meyerhoff et al, 1981; Stahle et al, 1989;
Ries et al, 1999). The variability in audiogram configuration for
each individual found in this study confirmed previous findings
(McNeill, 2005). It shows that a single patient can have significant
changes in audiogram configurations within a relatively short
space of time, particularly in those presenting with mid frequency
fluctuation. This may be why different studies have found
discrepancy in the prevalence of the peak audiogram. Previous
studies have also variously found that this peak occurs at either
1000 or 2000 Hz (Meyerhoff et al, 1981; Ries et al, 1999). With the
addition of intermediate frequency evaluation in this study, we
have consistently found two separate peaks with an intervening dip
at 1600 Hz, showing that the same patient may have peaks at
different frequencies at different times.
It is however possible that this double peak finding is an
artefact, secondary to an insertion loss effect due to the presence
of a hearing aid mould in the ear canal during in-situ threshold
598
International Journal of Audiology, Volume 48 Number 8
Discussion
Downloaded By: [Macquarie University] At: 22:52 6 August 2009
Figure 9. The group data of the eight ears with all frequency
fluctuating hearing loss standard deviation of the mean change
in hearing at each frequency.
To the best of our knowledge this is the first study to examine
hearing fluctuation in Meniere’s disease on a daily basis and with
the addition of intermediate frequencies. It was found that there
was a wide variety of audiogram shapes and hearing fluctuation,
with some fluctuating as much as 60 dBHL. Although the low
frequencies were the commonest site of fluctuation, a significant
proportion had greatest variation at the middle frequencies. The
same subjects could change between different audiogram shapes
(e.g. peak to flat or falling to flat), depending on the frequencies
where fluctuation occurred, at potentially daily intervals. These
shapes also included a ‘double peak’ audiogram with better
hearing thresholds at 8001000 Hz and 20002500 Hz. In
general the high frequencies (40008000 Hz) fluctuated less
than the low and mid frequencies, but there were still fluctuations in a minority of subjects.
High frequency losses were also noted in some younger patients
with normal hearing in the contralateral ear. As the mean hearing
loss became more severe, hearing tended to fluctuate less and to
assume a flat audiogram. However even with this group there
could still be a significant fluctuation of up to 30 dBHL.
This study allowed for a more detailed assessment of hearing
fluctuation that may occur over a short period of time, which
Frequency Hz
250
0
350
500
630
800
1000
1250
1600
2000
2500
3200
4000
6000
10
Hearing level dBHL
20
30
40
50
60
70
9am
80
2pm
7pm
90
100
Downloaded By: [Macquarie University] At: 22:52 6 August 2009
Figure 10. Example from the same patient as Figures 3 and 5 showing the daily hearing fluctuation from three audiograms during
the same 24 hour period.
measurement. This hypothesis however needs to be further
verified in future studies.
These results have different implications in the diagnosis and
treatment of Meniere’s disease. A self-testing protocol to assess
hearing fluctuation may be a useful tool in the diagnosis of
endolymphatic hydrops. The difficulty in establishing a firm
diagnosis is in many instances imposed by the fact that when the
patient reaches the clinic the symptoms may have subsided. A selfhearing assessment will allow the patients to provide the clinician
with a much more accurate picture of the cochlea status.
Our findings also help to explain the difficulties in satisfactorily fitting hearing aids to patients with Meniere’s disease. As
previously reported (McNeill, 2005), these patients usually leave
the audiology clinic satisfied with a new fitting only to return a
few days or even hours later, complaining that the sound through
the hearing aid is either too booming, too muffled, or distorted.
It has been suggested that such poor outcomes are associated
with low tolerance to amplification due to recruitment and
diplacusis (Valente et al, 2006). Our conclusion however, is that
the difficulties associated with fitting hearing aids to this
population is most likely due to the erratic fluctuation in the
hearing levels. Hearing fluctuation leading to changes in
audiogram configuration, as found in this study, explains why
neither a volume control nor even a sophisticated multi-program
hearing aid has been able to effectively improve hearing in so
many patients with Meniere’s disease.
In keeping with current theories (Braun, 1996; Xenellis et al,
2004), our findings suggest that the hearing loss of Meniere’s
disease is due to a combination of endolymphatic fluid changes
with basilar membrane displacement and outer hair cell dysfunction, leading to temporary threshold shifts, and hair cell damage,
which will ultimately turn into permanent threshold shift.
All the participants in our group had reduced low frequency
hearing at some stage, with mean thresholds usually at 4060
dBHL. The pattern found in the ‘all frequency’ fluctuating group
seems to be predominantly of those who are in the so-called ‘burnt
out’ stage of Meniere’s disease (Gibson, 1999). In this group the
Short-term hearing fluctuation in Meniere’s
disease
mean hearing thresholds and magnitude of fluctuation is reduced
across all frequencies most likely due to progressive hair cell
damage. One exception in this group was one patient with better
hearing. We speculate that hydrops in this individual may have
spread beyond the apex without concurrent permanent damage,
allowing hearing to return to normal at times. Damage to the high
frequencies of the basal turn seems to be less related to fluctuation.
Either this may be a different pathological process or these hair
cells are more readily damaged by fluid changes. Patterns of
hearing fluctuation therefore may be a clear indication as to which
part of the cochlea may be affected by fluid changes and which part
is due to hair cell damage.
Based on our findings we hypothesize that the pattern of low
frequency fluctuation may be due to hydrops in the cochlear
apical region. The mid-frequency fluctuation may occur as there
is already permanent damage in the apex so that the middle
cochlear regions become more affected by endolymphatic
hydrops. In the late stages of Meniere’s disease the whole cochlea
has been affected so that the hearing becomes more impaired
with the audiogram assuming a flatter configuration but small
fluctuations in hearing thresholds may still continue to occur
with eventual changes in endolymphatic pressure. The precise
correlation of stage and duration of disease with the degree of
fluctuation of hearing loss requires further study.
Acknowledgements
Paper presented at North of England Otolaryngology Society,
Carlisle, UK, September 2007.This is part of the ongoing PhD
studies of the first author conducted at Macquarie University
(Australia). Celene McNeill is very grateful to the financial
support of the Meniere’s Support Group NSW Inc., whose grant
made the writing of this paper possible. A special thank you to
all the subjects who participated in this study. The authors would
like to thank Prof. Bill Gibson, Prof. Eugen Molodysky, Prof.
Philip Newall, Ms. Amy Knott and Mr. Peter McNeill, for their
McNeill/Freeman/McMahon
599
Braun, M. 1996. Impediment of basilar membrane motion reduces
overload protection but not threshold sensitivity: Evidence from
clinical and experimental hydrops. Hear Res, 97, 110.
Conlon, B.J. & Gibson, W.P.R. 1999. Meniere’s Disease: The incidence of
hydrops in the contralateral asymptomatic ear. Laryngoscope, 109,
18001802.
Eggermont, J.J. & Schmidt, P.H. 1985. Meniere’s disease: A long-term
follow-up study of hearing loss. Ann Otol Rhinol Laryngol, 94, 19.
Gibson W.P.R. 1996. The role of electrocochleography in the understanding of pathophysiology of Meniere’s disease. Auris Nasus
Larynx, 23(Suppl. 11): s12s17.
Gibson, W.P.R. 1999. Removal of the extraosseous portion of the
endolymphatic sac. In J.P. Harris (ed.), Meniere’s Disease. The
Hague: Kugler Publications, pp. 361368.
Green, J.D, Blum, D.J. & Harner, S.G. 1991. Longitudinal follow-up of
patients with Meniere’s disease. Otolaryngol Head Neck Surg, 104,
7838.
Ludvigsen, C. 2001. Audiological background and design rationale of
Senso Diva. Widexpress, 15, 110.
Mateijsen, D.J.M., Van Hengel, P.W.J., Van Huffelen, W.M., Wit, H.P. &
Albers, F.W.J. 2001. Pure-tone and speech audiometry in patients
with Meniere’s disease. Clin Otolaryngol, 26, 37987.
McNeill, C. 2005. A hearing aid system for fluctuating hearing loss due
to Meniere’s disease: A case study. ANZJA, 27, 7884.
Meyerhoff, W.L., Paparella, M.M. & Gudbrandsson, F.K. 1981. Clinical
evaluation of Meniere’s disease. Laryngoscope, 91, 16638.
Ries, D.T., Rickert, M. & Schlauch, R.S. 1999. The peaked audiometric
configuration of Meniere’s disease: Disease related? J Speech Lang
Hear Res, 42, 82943.
Savastano, M., Guerrieri, V. & Marioni, G. 2006. Evolution of
audiometric pattern in Meniere’s disease: Long-term survey of 380
cases evaluated according to the 1995 guidelines of the American
Academy of Otolaryngology-Head Neck Surgery. J Otolaryngol, 35,
269.
Smith-Olinde, L., Nicholson, N., Chivers, C., Highley, P. & Williams,
D.K. 2006. Test-retest reliability of in situ unaided thresholds in
adults. Am J Audiol, 15, 7580.
Stahle, J., Friberg, U. & Svedberg, A. 1989. Long-term progression of
Meniere’s disease. Am J Otol, 10, 1703.
Valente, M., Mispagel, K., Valente, L.M. & Hullar, T. 2006. Problems
and solutions for fitting amplification to patients with Meniere’s
disease. J Am Acad Audiol, 17, 615.
Wong, T.W., Yu, T.S., Chen, W.Q., Chiu, Y.L., Wong, C.N., et al. 2003.
Agreement between hearing thresholds measured in non-soundproof
work environments and a soundproof booth. Occup Environ Med,
60, 66771.
Xenellis, J.E., Linthicum, F.H., Webster, P. & Lopez, R. 2004. Basilar
membrane displacement related to endolymphatic sac volume.
Laryngoscope, 114, 19539.
600
International Journal of Audiology, Volume 48 Number 8
help and support. Simon Freeman also thanks the Graham
Fraser Foundation (UK) for funding his fellowship in Australia.
Downloaded By: [Macquarie University] At: 22:52 6 August 2009
References