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Acta Oto-Laryngologica.

2014; 134: 10051010

ORIGINAL ARTICLE

Long-term prognosis of hearing loss in patients with unilateral


Mnires disease

GO SATO1, KAZUNORI SEKINE1, KAZUNORI MATSUDA1, HITOMI UEEDA1,


ARATA HORII2, SUETAKA NISHIIKE2, TADASHI KITAHARA2, ATSUSHI UNO2,
TAKAO IMAI2, HIDENORI INOHARA2 & NORIAKI TAKEDA1
1
Department of Otolaryngology, University of Tokushima Graduate School, Tokushima and 2Department of
Otolaryngology, Osaka University Graduate School of Medicine, Osaka, Japan

Abstract
Conclusion: The nding of deteriorated hearing loss at the initial visit at middle to high frequencies is a factor of poor hearing
prognosis in Mnires disease. Early intervention with instructions for lifestyle changes may lead to good outcomes in hearing.
Objective: An attempt was made to examine long-term changes in hearing loss in unilateral Mnires disease and factors
associated with prognosis of hearing loss retrospectively. Methods: Based on their last hearing level of the affected ear,
36 patients were subdivided into two groups: the poor prognosis of hearing (PPH) group and the good prognosis of hearing
(GPH) group. Results: In the PPH group, the hearing levels at the initial visit at middle and high frequencies were signicantly
worse than those in the GPH group. Moreover, the hearing loss progressed during the rst 2 years of the disease, and stayed at
to approximately 50 dB at the later stage. Conversely, the hearing loss at the onset in the GPH group showed no further
progression over the rst 2 years, and remained constant to approximately 35 dB at the later stage. In addition, the mean
intervals from the onset to the initial visit in the PPH group were signicantly longer than those in the GPH group.

Keywords: Endolymphatic hydrops, vertigo attack, early intervention

Introduction hearing loss that causes a reduction in the quality


of life in the patients [4,8,9].
Mnires disease is an intractable inner ear disease In the present study, an attempt was made to
that is characterized by episodes of recurrent vertigo retrospectively examine long-term changes in hearing
with hearing loss, tinnitus, and aural fullness. Its loss during follow-up in patients with unilateral
pathophysiology is recognized to be idiopathic Mnires disease. We divided the patients into two
endolymphatic hydrops [1,2]. groups based on their last hearing level of the affected
Previous studies reported that in most patients with ear: (1) the poor prognosis of hearing (PPH) group
Mnires disease, the frequency of vertigo attacks and (2) the good prognosis of hearing (GPH) group,
decreased gradually after the onset until it reached a and then investigated the factors associated with the
steady-state phase free of vertigo [3,4]. On the other prognosis of hearing loss.
hand, it was also reported that sensorineural hearing
loss in Mnires disease deteriorated progressively Material and methods
until it reached a moderate or severe level [57]. Since
the vertigo attacks decrease or disappear in the A total of 36 patients (14 males and 22 females; 24
long-term course, the goal of treatment for Mnires 75 years old; mean age 47.6 13.3 years) with
disease should be to prevent the progression of unilateral denitive Mnires disease according to

Correspondence: Go Sato, MD PhD, Department of Otolaryngology, University of Tokushima School of Medicine, 3-18-15 Kuramoto, Tokushima 770-8503,
Japan. Tel: +81 88 633 7169. Fax: +81 88 633 7170. E-mail: go-sato@tokushima-u.ac.jp

(Received 25 February 2014; accepted 2 May 2014)


ISSN 0001-6489 print/ISSN 1651-2251 online  2014 Informa Healthcare
DOI: 10.3109/00016489.2014.923114
1006 G. Sato et al.

the 1995 guidelines of the American Academy of was estimated by the formula of the averaged hearing
Otorhinolaryngology and Head and Neck Surgery level of 1258000 Hz at the 3-month period when
(AAO-HNS) were included in the present study patients experienced vertigo attacks minus the aver-
(Table I) [10]. They were referred to University aged level of the pre-vertigo attack period. The pro-
Hospital from private ENT clinics, because of their gression of hearing levels after vertigo attack was
intractable Mnires attacks. estimated by subtracting the averaged hearing level
The onset of Mnires disease was determined by between 125 and 8000 Hz at the 3-month period
detailed interview at the initial visit to the hospital. when patients experienced vertigo attacks from that
After the initial visit, hearing levels were evaluated and of the post-vertigo attack period.
the frequency of vertigo attacks was requested at each After the initial visit, the patients received betahis-
follow-up visit. The mean follow-up period was 49.2 tine and/or anti-emetic during a vertigo attack, and
34.3 months after the initial visit to University isosorbide, an osmotic diuretic, for at least 3 months
Hospital. In 17 patients, their referrals reported the after a vertigo attack. They also received instructions
audiograms containing hearing level at the onset of to avoid mental and physical stress in their lifestyle.
the disease. This retrospective study was approved Patients who underwent surgery such as endolym-
by the Committee for Medical Ethics of Tokushima phatic sac surgery and intratympanic injection with
University Hospital. gentamicin were excluded from the present study.
According to a grading system to assess the severity The MannWhitney test was used for statistical
of symptoms in patients with Mnires disease analysis and p < 0.05 was considered statistically
proposed by the Intractable Vestibular Disorder signicant.
Committee of the Ministry of Health and Welfare
of Japan, the patients were subdivided into two groups Results
based on their last hearing level of the affected ear: (1)
the poor prognosis of hearing (PPH) group, whose The mean hearing levels at the initial visit to the
hearing levels were over 40 dB in all 125, 250, 500, hospital in Mnires patients with PPH were
1000, 2000, 4000, and 8000 Hz; (2) the good prog- 48.0 19.8 dB at low frequency, 41.8 18.7 dB at
nosis of hearing (GPH) group, whose hearing levels middle frequency, and 45.6 15.2 dB at high fre-
were lower than 40 dB in at least one of 125, 250, 500, quency, while in those with GPH, they were 40.3
1000, 2000, 4000, and 8000 Hz [11]. 14.1 dB, 28.9 11.3 dB, and 32.4 11.9 dB, respec-
The averaged hearing levels were calculated every tively (Table I). Thus the mean hearing levels at
3 months and changes in hearing levels at every the initial visit in patients with PPH at middle and
3-months period after the onset of the disease were high frequencies, but not at low frequencies, were
evaluated. The hearing levels at the low (125500 Hz), signicantly worse than those in patients with GPH.
middle (5002000 Hz), and high (20008000 Hz) In patients with PPH, the mean hearing levels at the
frequencies were then analyzed separately. The onset of the disease that were reported in their refer-
progression of hearing levels during vertigo attack rals at low, middle, and high frequencies were 39.3
12.7 dB, 31.2 8.5 dB, and 38.8 8.6 dB, respec-
Table I. Patients with unilateral Mnires disease at the initial tively. Two years after the onset, they deteriorated
visit. rapidly to 53.5 22.2 dB, 50.9 21.7 dB, and 51.1
PPH group GPH group
16.3 dB before stabilizing, respectively, at 44.4
Characteristic (n = 19) (n = 17) 5.9 dB, 50.6 7.9 dB, and 59.4 6.7 dB 8 years
later (Figure 1). On the other hand, in patients with
Mean age (years) 47.1 13.3 46.8 16.4 GPH, the mean hearing levels at the onset of the
Sex disease that were reported in their referrals at the same
Male (%) 6 (32) 8 (47) frequencies were 39.8 15.1 dB, 26.5 10.7 dB, and
Female (%) 13 (68) 9 (53) 30.3 11.3 dB, respectively. Two years after the
Mean hearing level at 48.0 19.8 40.3 14.1
onset, they deteriorated slightly to 44.3 13.4 dB,
low frequencies (dB) 33.8 10.2 dB, and 35.7 11.9 dB, and then became
Mean hearing level at 41.8 18.7* 28.9 11.3
stable at 35.8 27.1 dB, 34.2 24.6 dB, and 34.2
middle frequencies (dB) 5.9 dB, respectively, 8 years later (Figure 2).
Mean hearing level at 45.6 15.2* 32.4 11.9
The mean progression of hearing levels during
high frequencies (dB) vertigo attacks was signicantly larger than that after
vertigo in patients with both PPH and GPH
Values are shown as mean SD. GPH, good prognosis of hearing;
PPH, poor prognosis of hearing. (Figure 3). The mean progressions of hearing levels
*p < 0.05. during vertigo attack in patients with PGH and GPH
Long-term prognosis of hearing loss in Mnires disease 1007

(Years)
1 2 3 4 5 6 7 8
0

10 Low frequency
Middle frequency
20 High frequency

30
(dB)
40

50

60

70

Figure 1. Time course of the mean hearing levels at low, middle, and high frequencies in Mnires patients with poor prognosis of hearing
after the onset of the disease. Arrowhead indicates their mean interval from the onset to the initial visit.

(Years)
1 2 3 4 5 6 7 8
0

10

20

30
(dB)

40

50
Low frequency
60
Middle frequency
High frequency
70

Figure 2. Time course of the mean hearing levels at low, middle, and high frequencies in Mnires patients with good prognosis of hearing
after the onset of the disease. Arrowhead indicates their mean interval from the onset to the initial visit.

were 3.3 11.4 dB and 2.6 11.9 dB, respectively. indicating an improvement of hearing levels after
After vertigo attacks, the mean progression of hearing vertigo attacks.
levels in patients with PPH was 0.48 11.6 dB, The percentage of patients who suffered from
whereas that of those with GPH was 3.6 10.5 dB, vertigo attacks every 3 months decreased rapidly

A B
* *
40 40
30 30
20 20
10 10
(dB)

(dB)

0 0
-10 -10
-20 -20
-30 -30
-40 -40
During vertigo After vertigo During vertigo After vertigo
attack attack attack attack

Figure 3. Changes in hearing levels during and after vertigo attack in Mnires patients with poor prognosis of hearing (A) and good prognosis
of hearing (B). Mean SD. *p < 0.05.
1008 G. Sato et al.

100%
90%

Percentages of patiens with


80%
70%

vertige attack
60%
50%
40%
30%
20%
10%
0%
1 2 3 4 5 6 7 8
Years after the onset

Figure 4. Time course of the percentages of Mnires patients who suffered from a vertigo attack during every 3-month period. Filled circles,
patients with poor prognosis of hearing; open circles, patients with good prognosis of hearing.

during the rst 3 years after the onset of the disease in early stage of the disease [5] and that at high frequen-
both PPH and GPH groups, and then 8 years later cies tends to be irreversible and progressive [6,7].
stabilized at less than 20% (Figure 4). The mean The present study also showed that in patients with
interval from the onset to the initial visit in patients PPH, the hearing loss progressed during the rst
with PPH (15.5 18.7 months) was signicantly 2 years after the onset of the disease, and stayed
longer as compared with that of those with GPH at to approximately 50 dB at the later stage of
(7.6 11.7 months) (Figure 5). Mnires disease. Conversely, the hearing loss at
the onset in patients with GPH showed no further
Discussion progression over the rst 2 years, and became
constant to approximately 35 dB at the later stage
In the present study, the mean hearing levels at the of the disease. These ndings suggest that the rapid
initial visit to the hospital in Mnires patients with progression of hearing loss during the rst 2 years
PPH were signicantly worse than those with GPH at leads to poor hearing outcomes in patients with
middle and high frequencies but not at low frequen- Mnires disease. Indeed, many studies found that
cies. It is suggested that the deteriorated hearing loss the hearing loss in Mnires disease deteriorated over
at the initial visit at middle to high frequencies is a time within the rst several years of the disease and
factor of poor prognosis of the hearing loss in patients was followed by a stabilization of moderate to
with Mnires disease, because the hearing loss at severe hearing loss [4,12,13]. Thomas and Harrison
low frequencies is uctuating and reversible at the reported that the progression of hearing loss was more

Months
40 *
Intervals from onset to initial visit

30

20

10

0
PPH group GPH group

Figure 5. The mean intervals from the onset to the initial visit in Mnires patients with poor prognosis of hearing (PPH) and good prognosis
of hearing (GPH). Mean SD. *p < 0.05.
Long-term prognosis of hearing loss in Mnires disease 1009

common during the rst 5 years [12], whereas Stahle Contradicting this observation, the Cochrane Library
reported it to occur within the rst 2 years [13]. indicates that no good evidence regarding the effect of
During follow-up, the mean hearing levels wors- diuretics on Mnires disease has been previously
ened by about 3 dB during the vertigo attacks in reported [18]. Therefore, the good prognosis of
Mnires patients with both PPH and GPH. How- hearing associated with the early intervention may
ever, the deteriorated hearing loss of 3 dB improved not be due to the treatment with osmotic diuretic.
after the vertigo attack in patients with GPH but not in On the other hand, in the present study, patients with
those with PPH. It is possible that the progression of Mnires disease also received the instruction to
hearing loss during vertigo attacks became irreversible operate changes in their lifestyle, especially to avoid
in patients with PPH who suffered from repeated mental and physical stress, because Mnires disease
vertigo attacks. This is because an intractable vertigo is recognized as a stress-induced disease [7]. Indeed,
attack was suggested to be a factor for poor hearing it has been reported that counseling is more effective
prognosis, based on the ndings that hearing loss took in improving the hearing level in patients with
place in the early course of Mnires disease before Mnires disease than drug treatment [19], suggest-
the relief in vertigo attack [3,4]. However, the possi- ing that it decreases levels of stress hormones such as
bility is unlikely, because the present study showed vasopressin, which increases the extent of endolym-
that the percentages of patients who suffered from a phatic hydrops [20]. Therefore, it is suggested that
vertigo attack every 3 months decreased rapidly dur- early intervention with instructions for changes in
ing the rst 3 years after onset of the disease and lifestyle leads to good outcomes of hearing in patients
that there were no differences in these percentages with Mnires disease.
between patients with PPH and those with GPH.
Previous studies also reported that the frequency of
vertigo attacks decreased over time in most patients Conclusion
with Mnires disease [14]. Perez-Garrigues et al.
also reported that the frequency of vertigo attacks In the present study, we showed that the deteriorated
showed a rapid decline during the rst 8 years of hearing loss at the initial visit at middle and high
the disease [3]. Thus, the frequency of vertigo attack frequencies is a factor for poor hearing prognosis in
seems to have little effect on hearing prognosis of patients with Mnires disease. The hearing loss
Mnires disease. It is suggested that hydrops itself, further progressed over the rst 2 years, and stayed
but not the rupture of hydrops, leads to hair cell at at approximately 50 dB later, indicating poor
degeneration, because a vertigo attack in Mnires outcomes of hearing. In Mnires patients with
disease is induced by its rupture [15]. PPH, the hearing level worsened by about 3 dB
Another possibility of the irreversible progression of during vertigo attacks, after which the loss was irre-
hearing levels during the vertigo attack in patients versible. Because the frequency of vertigo attacks in
with PPH is the higher susceptibility of their inner ear patients with PPH was not different from that of
to endolymphatic hydrops. Recently, Moon, et al. those with GPH, it is suggested that the inner ear
reported that a high SP/AP ratio on electrocochleo- in patients with PPH is more susceptible to endolym-
gram was a predictor of poor hearing outcomes in phatic hydrops. Conversely, the hearing loss in
patients with Mnires disease and suggested that patients with GPH did not progress after the onset,
excessive endolymphatic hydrops associated with a probably because the mean interval from the onset to
high SP/AP ratio led to hair cell degeneration and the initial visit to the hospital in patients with GPH
malfunction [16]. Therefore, the inner ear of patients was shorter than that of those with GPH, indicating
with PPH may be more susceptible to endolymphatic the need for early intervention with instructions for
hydrops than that of those with GPH. changes in lifestyle. This may lead to reducing the
In the present study, patients with GPH visited the extent of endolymphatic hydrops and to good out-
hospital and were treated signicantly earlier than comes of hearing in patients with Mnires disease.
those with PPH, suggesting that early intervention
is effective in preventing the progression of hearing
loss in patients with Mnires disease. Patients Acknowledgments
received isosorbide, an osmotic diuretic, for at least
3 months after vertigo attack, because a Japanese This work was partially supported by a Grant-in-Aid
randomized controlled trial showed that isosorbide from the Ministry of Health and Welfare of Japan and
was more effective in suppressing vertiginous a Grant-in Aid from the Japan Society for Promotion
symptoms than betahistine, but had no effect on of Science. We thank Dr Kalubi Bukasa for his critical
hearing loss in patients with Mnires disease [17]. reading of the manuscript.
1010 G. Sato et al.

Declaration of interest: The authors report no [10] AAO-HNS Committee on Hearing and Equilibrium.
conicts of interest. The authors alone are responsible Committee on Hearing and Equilibrium guidelines for the
diagnosis and evaluation of therapy in Mnires disease.
for the content and writing of the paper. Otolaryngol Head Neck Surg 1995;113:1817.
[11] Yagi T, Ito J, Kubo T, Takahashi M, Takahashi M,
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