Middle ear myoclonus

1. The Laryngoscope
VC 2013 The American Laryngological,
Rhinological and Otological Society, Inc.
Clinical Characteristics and Therapeutic Response of Objective
Tinnitus Due to Middle Ear Myoclonus: A Large Case Series
Shi-Nae Park, MD, PhD; Seong-Cheon Bae, MD; Guen-Ho Lee, MD; Jee-nam Song, MD;
Kyoung-Ho Park, MD, PhD; Eun-ju Jeon, MD, PhD; Young Soo Park, MD, PhD; Sang-Won Yeo, MD, PhD
Objectives/Hypothesis: To evaluate the clinical characteristics and therapeutic response of tinnitus due to middle ear
myoclonus (MEM) and to suggest appropriate diagnostic methods.
Study Design: Retrospective chart review.
Methods: This study included 58 patients with tinnitus diagnosed with MEM, who were seen from January 2004 to July
2011. Clinical and audiological characteristics were investigated. The therapeutic responses to counseling, medical therapy,
and surgical therapy were evaluated.
Results: Patients had a mean age of 29.8 years (range, 6–70 years), 20.7% (n ¼ 12) were <10 years old, 39.7% (n
¼ 23) were <20 years old, 74.1% (n ¼ 43) were <40 years old, and 5.2% (n ¼ 3) were 60 years old. Remembered stress-
ful events or noise exposure were associated with the onset of MEM in 51.8% (n ¼ 30) and 27.6% (n ¼ 16) of patients,
respectively. The most frequent nature of the tinnitus was a crackling sound. MEM associated with forceful eyelid closure was
observed in 15% of patients. Impedance audiogram and otoendoscopic examinations of the tympanic membrane were helpful
tools for diagnosing MEM. With medical therapy, more than 75% of patients exhibited complete or partial remission of their
tinnitus. Patients with intractable MEM who underwent sectioning of the middle ear tendons had very good outcomes.
Conclusions: Tinnitus due to middle ear myoclonus seems to occur in young patients and to be related to stress or
noise. Information about the clinical characteristics and therapeutic response of this less-common type of tinnitus will help to
ensure early and appropriate diagnosis and treatment of these patients.
Key Words: Myoclonus, middle ear, tinnitus, objective, diagnosis, treatment.
Level of Evidence: 4.
Laryngoscope, 123:2516–2520, 2013
INTRODUCTION
Tinnitus is a common auditory symptom that is asso-
ciated with many otological diseases. Tinnitus can be
classified into subjective and objective tinnitus. Subjec-
tive or sensorineural tinnitus is more common and is the
phantom perception of a ringing or buzzing sound in the
ears or head in the absence of an external sound source.
Recently, researchers have proposed several mechanisms
involving brain plasticity, and most agree that subjective
tinnitus can be linked to changes at one or more points
along the peripheral and central auditory pathways.1–3
In contrast to subjective tinnitus, objective tinnitus
or somatosound originates from the para-auditory struc-
tures of the head. This symptom is much less common,
and the sound can be heard by both the patient and
examiner. Conditions that can cause objective tinnitus
include vascular abnormalities, temporomandibular joint
disease, a patulous eustachian tube, and tinnitus of
muscle origin, specifically palatal myoclonus and middle
ear myoclonus (MEM).4
Tinnitus secondary to middle
ear myoclonus is very rare, and very few English litera-
ture reports of it have been published.5–7
Most studies
about MEM are case reports. We have treated approxi-
mately 60 cases of middle ear myoclonus in our tinnitus
clinic since 2004. This study introduces the clinical and
audiological characteristics of this rare type of tinnitus
in a relatively large sample and evaluates the therapeu-
tic response to medical and surgical intervention in our
clinical setting.
MATERIALS AND METHODS
This retrospective study examined 58 patients with tinni-
tus diagnosed with MEM by the first author (S.N.P.) at the
tinnitus clinic in Seoul St. Mary’s Hospital from November 2004
to July 2011. After obtaining approval from the institutional
review board (KC11RISE0854), a database was created to docu-
ment demographic and clinical data including patient age and
sex, tinnitus questionnaires, audiological study, and the thera-
peutic response of middle ear myoclonus. The tinnitus
questionnaires included visual analog scales (VAS) of tinnitus
annoyance, awareness, loudness, and effect on life, and the Tin-
nitus Handicap Inventory (THI) developed by Newman et al.8
Additionally, audiological evaluations with a pure-tone
From the Department of Otolaryngology–Head and Neck Surgery,
The Catholic University of Korea, College of Medicine, Seoul, South Korea.
Editor’s Note: This Manuscript was accepted for publication
October 5, 2012.
Additional Supporting Information may be found in the online version of
this article.
The authors have no funding, financial relationships, or conflicts
of interest to disclose.
Send correspondence to Shi-Nae Park, MD, Associate Professor,
Department of Otolaryngology–HNS, The Catholic University of Korea,
College of Medicine, 505 Banpo-dong, Seocho-gu, Seoul 137-701, South
Korea. E-mail:
DOI: 10.1002/lary.23854
Laryngoscope 123: October 2013 Park et al.: Tinnitus Due to Middle Ear Myoclonus
2516

2. audiogram, tympanogram, acoustic reflex decay, and tinnitus
matching test were analyzed. Endoscopic examination of the
tympanic membrane and a physical examination for palatal
myoclonus were also performed. Brain magnetic resonance
imaging (MRI) was checked in most patients. Once diagnosed,
the patients with MEM were treated using reassurance and
medication, including an anticonvulsant (carbamazepine) or
benzodiazepine (clonazepam) and a muscle relaxant (baclofen),
for up to 3 months before considering a surgical procedure.
Factors causing or triggering their middle ear myoclonus were
evaluated thoroughly, and the patients were educated to avoid
those etiologic factors as much as possible. For intractable cases
after conservative treatment, the middle ear tendons, both the
tensor tympani and stapedius, were sectioned. The therapeutic
responses to medical and surgical intervention were evaluated.
Statistical analysis was performed using SPSS version
15.0, (SPSS Inc., Chicago, IL). Descriptive analysis, frequency
analysis, and paired t test were used; P values .05 were
considered significant.
RESULTS
Clinical and Audiological Characteristics of
Tinnitus Due to MEM
The mean age of our patients was 29.8 years
(range, 6–70 years); 26 were male and 32 were female.
The age of onset of the MEM was assessed using the tin-
nitus questionnaire. We found that MEM more
commonly occurred in younger patient 50 years old.
Only 11.3% (n ¼ 6) of patients had an onset of tinnitus
after the age of 50 years (Fig. 1). Mean duration of tinni-
tus was 19.3 months (range, 1 month–10 years). Table I
lists the nature of the tinnitus due to MEM for all study
subjects. Most patients (72.4%) had crackling or buzzing
tinnitus. About 53% of the patients had unilateral tinni-
tus, and 47% of the patients had bilateral MEM (Fig. 2).
The most common factors related to the onset of
MEM were stressful events followed by noise exposure.
Other associated factors were forceful eyelid closure syn-
drome and palatal myoclonus. Interestingly, the patients
included 10 professional musicians (17.2%). We found
that approximately 45% of the patients (n ¼ 26) had
subjective hyperacusis. The triggering or causative
factors for MEM are described in Table II.
To-and-fro, or inward-and-outward motion, mostly
in the posterior part of the tympanic membrane, was
frequently observed on endoscopic examination (39.5%, n
¼ 38; see supplementary video). This motion seemed to
be subtle enough to be easily overlooked without endo-
scopic or microscopic magnification and was not related
to patient breathing. Cog-wheel perturbation of the tym-
panogram was observed in eight (14.3%) of the 58
patients. Perturbations in the stapedial reflex and acous-
tic reflex decay were observed in 28.6% and 68.8% of the
58 patients, respectively (Table III). Figure 3 presents
perturbations on representative stapedial reflex and
acoustic reflex decay tests. The average air-conduction
hearing level at 0.5, 1, 2, and 4 kHz was 12.4 6 8.2 dB
HL (mean 6 standard deviation) at speech frequency.
Abnormal brain MRI findings that seemed to be related
to MEM were observed only in one of the 35 patients
Fig. 1. Age distribution of patients with middle ear myoclonus
(n ¼ 58).
TABLE I.
Demographic and Clinical Characteristics of Patients With Middle
Ear Myoclonus (N 5 58).
Age, yr 29.8 (range, 6–70)
Male:female 26:32
Essential:symptomatic 57:1
Duration, mo 19.3 6 29.6
Site (right ear:left ear:bilateral) 14:17:27
Nature (clicking:crackling:
buzzing:tapping:bubbling:no data)
5:24:17:6:1:4
Fig. 2. Involved ear of study subjects with middle ear myoclonus
(n ¼ 58). [Color figure can be viewed in the online issue, which is
available at wileyonlinelibrary.com.]
TABLE II.
Predisposing Factors and Accompanying Symptoms in the Study
Subjects (N 5 58).
Clinical Factors
Tinnitus Due to
Middle Ear Myoclonus
Predisposing factors
Stressful event 30/58 (51.8%)
Noise exposure 16/58 (27.6%)
Professional musician 10/58 (17.2%)
Accompanying symptoms
Concomitant palatal myoclonus 4/58 (6.9%)
Forceful eye closure syndrome 7/58 (12.1%)
Subjective hyperacusis 26/58 (44.8%)
Laryngoscope 123: October 2013 Park et al.: Tinnitus Due to Middle Ear Myoclonus
2517

3. who underwent MRI (2.9%); this patient had a neuro-
genic tumor involving the trigeminal ganglion (Fig. 4).
THERAPEUTIC RESPONSE TO CONSERVA-
TIVE AND SURGICAL THERAPY
We evaluated the therapeutic response to conserva-
tive management in 44 patients who were treated using
reassurance and medication (anticonvulsant and muscle
relaxant) and who were seen at least once after treat-
ment. Most of the patients who were not able to undergo
MRI missed the follow-up session. The mean follow-up of
the study subjects for evaluating the therapeutic
response was 6.8 6 11.4 months (maximum, 81 months).
Eleven patients (25%) exhibited complete resolution of
the middle ear myoclonus after medication. Twenty-two
patients (50%) exhibited a partial response to medication
TABLE III.
Diagnosis of Middle Ear Myoclonus in the Study Subjects
(N 5 58).
Inspection of to-and-fro
motion of the TM (endoscopic)
15/38 (39.5%)
Auscultation using a Toynbee tube 0/15 (0%)
Impedance audiogram (perturbation)
Tympanogram 8/56 (14.3%)
Stapedial reflex 14/49 (28.6%)
Acoustic reflex decay/static
compliance
33/48 (68.8%)
Brain MRI: tumor at
trigeminal ganglion
1/35 (2.9%)
MRI ¼ magnetic resonance imaging; TM ¼ tympanic membrane.
Fig. 3. Perturbations on representa-
tive stapedial reflex test (A) and
acoustic reflex decay test (B) were
observed in two different study sub-
jects with middle ear myoclonus.
[Color figure can be viewed in the
online issue, which is available at
wileyonlinelibrary.com.]
Fig. 4. Abnormal brain magnetic resonance imaging findings in a patient with symptomatic middle ear myoclonus. A tumor was observed
at the trigeminal ganglion in this patient (arrows). [Color figure can be viewed in the online issue, which is available at
wileyonlinelibrary.com.]
Laryngoscope 123: October 2013 Park et al.: Tinnitus Due to Middle Ear Myoclonus
2518

4. and could tolerate their symptoms. Eleven (25%) of the
44 patients exhibited no response to medication. Overall,
the therapeutic response to medical therapy was 75%.
The VAS of tinnitus and the THI score decreased signifi-
cantly after conservative management using reassurance
and medication (Fig. 5; P .05, paired t test).
Nine patients who did not show any improvement
after conservative treatment underwent surgical ther-
apy. The middle ear tendons (tensor tympani and
stapedius) were sectioned via a transmeatal approach in
11 ears with intractable tinnitus (unilateral in seven
patients and bilateral in two patients). Postoperatively,
all patients exhibited complete resolution or marked
improvement of their tinnitus (Table IV). The symptoms
recurred 1 year postoperatively in one patient who had
markedly hypertrophic tensor tympani tendons at
surgery. Repeat bilateral sectioning of the reattached
tensor tympani tendons was performed in this patient
and the tinnitus disappeared immediately.
DISCUSSION
MEM is one type of tinnitus of muscle origin and is
defined as a rhythmic movement of the tympanic mem-
brane secondary to repetitive contraction of the tensor
tympani and stapedial muscles.9
It is an extremely rare
cause of tinnitus, and only a few English-language case
reports have been published.5–7,9,10
This study evaluated the demographics and clinical
characteristics of middle ear myoclonus in a relatively
large number of patients. Most patients with MEM
indicated that their tinnitus began at a relatively young
age. Approximately 90% of the patients in this series were
younger than 50 years when their tinnitus began. Interest-
ingly, the incidence of MEM tended to decrease with age in
our study subjects, suggesting that MEM is a disease of
younger people, unlike sensorineural tinnitus, which is
more frequent in older people. There was no right/left pre-
dominance in unilateral MEM; bilaterality was observed
in approximately half of the patients in our series.
It has been reported that tensor tympani contraction
may produce a clicking sound, whereas stapedial contrac-
tion produces a buzzing noise.11
However, in our study
the nature of the MEM was mostly a crackling or buzz-
ing. Clicking tinnitus was very rare, and we found that
the patients with clicking tinnitus also had concomitant
palatal myoclonus. We presume that louder clicking tinni-
tus caused by palatal myoclonus may mask softer and
faster crackling or buzzing tinnitus of MEM in those
patients. Based on our results, we strongly suggest that
patients with tinnitus who have noncontinuous crackling
or buzzing sounds in their ear should be evaluated thor-
oughly for tinnitus originating from MEM. The nature of
tinnitus might be thoroughly evaluated to diagnose the
patients with muscle-origin tinnitus more accurately.
In our series, a considerable number of the patients
with MEM had associated forceful eyelid closure syndrome
(FECS), which was first reported in 1983.12
The main
symptom of this syndrome is a muscular tinnitus related
only to forced eye closure, specifically the voluntary con-
traction of the periorbital muscles. Recently, we reported
six cases of FECS in children and suggested a possible
mechanism of this rare condition.13
Another interesting finding was that more than
half of our patients with MEM had identifiable factors
causing or triggering their tinnitus, such as noise expo-
sure or stressful events. Moreover, our series included a
disproportionate number of professional musicians, who
are routinely exposed to loud sounds, which led us to
postulate that a possible mechanism of MEM might be
closely related to sound exposure in addition to stress.
The underlying etiology of tinnitus is still obscure. Myo-
clonic movements are a form of segmental myoclonus
and involve muscles innervated from a limited segment
Fig. 5. Changes in tinnitus due to middle ear myoclonus after
conservative treatment. Visual analog scale of tinnitus and the tin-
nitus handicap inventory (THI) score decreased significantly after
conservative management using reassurance and medication (P
.05, paired t test). AN ¼ annoyance; AW ¼ awareness; EOL ¼
effect on life; LD ¼ loudness; Tx ¼ treatment. *P .0001, Wil-
coxon signed rank test. †P ¼ .017, Wilcoxon signed rank test.
[Color figure can be viewed in the online issue, which is available
at wileyonlinelibrary.com.]
TABLE IV.
Therapeutic Response of Middle Ear Myoclonus.
Treatment N Response N
Reassurance
with medication
(3 mo)
44 Completely
resolved
11 (25%)
Partial
improvement
22 (50%)
No improvement 11 (25%)
Tendon sectioning 9 (11 ears) Completely
resolved
7 (9 ears)
Marked
improvement
2 (2 ears)
Laryngoscope 123: October 2013 Park et al.: Tinnitus Due to Middle Ear Myoclonus
2519

5. of the brainstem. Segmental myoclonus due to vascular,
infectious, demyelinating, tumor, traumatic, or idio-
pathic causes can result in ocular, palatal, jaw, facial,
tongue, and middle ear myoclonus.5,6,9
In this study, we
discovered one brain tumor that seemed to be a cause of
MEM. Based on the clinical characteristics discovered in
this study, we propose a possible pathomechanism of
tinnitus due to MEM (Fig. 6).
An endoscopic examination and impedance audio-
gram (i.e., tympanogram, stapedial reflex and acoustic
reflex decay, or static compliance) may aid in an objective
diagnosis of MEM. In this study, to-and-fro rhythmic
motion of the posterior part of the tympanic membrane
observed in the endoscopic examination and perturba-
tions in the various impedance audiograms were
frequently found in patients with MEM. We believe that
ours is the first report to show to-and-fro motion of the
posterior part of the tympanic membrane in patients with
essential MEM (see supplementary video). We also sug-
gest that observation of the tympanic membrane with
noise stimuli may be another valuable diagnostic tool for
patients complaining of crackling or buzzing tinnitus
under noisy conditions. MRI also seems to be necessary to
rule out pathological lesion of the brain, although our
series included only one case with brain pathology.
Previous case reports have described several treat-
ments, including the use of muscle relaxants, sedatives,
anticonvulsants, and surgical therapy.5–7,9,10,11
Our
study evaluated the therapeutic response to conservative
medical treatment and surgical management of MEM.
Seventy-five percent of patients in this study exhibited
partial or complete resolution of their tinnitus after
conservative therapy using reassurance and medication.
For intractable cases of MEM, sectioning the tendons of
the middle ear muscles seems to be a very promising
treatment. Considering the possibility of reattachment of
the tensor tympani tendon, as observed in one of our
patients treated surgically, it is important to ensure a
sufficient gap between the resected margins of the
tendon during the surgery.
CONCLUSION
This study constitutes the largest single-institution
case series of tinnitus due to MEM. The clinical and
audiological characteristics and therapeutic responses to
treatment were evaluated thoroughly. We found that
patients with MEM were relatively young compared to
patients with sensorineural tinnitus. A possible patho-
logical mechanism involving brain reorganization due to
stress, noise, or brain lesions is suggested, although fur-
ther studies will be necessary to show a more direct
causal relationship or brain reorganization in these
patients. Endoscopic examination and impedance audi-
ometry are helpful tools for diagnosing and documenting
this rare condition, although negative findings should
not preclude the diagnosis. Given its high response rate,
conservative therapy with reassurance and medication
should be considered the first-line therapy in these
patients. Sectioning the middle ear tendons seems to be
a very reliable therapeutic option for MEM. Future stud-
ies should expand on our findings by investigating the
prognostic factors, long-term outcome of therapy, and
the pathomechanism of MEM.
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Fig. 6. A possible mechanism of
middle ear myoclonus. †Gun shot,
musician. ‡Tumor, demyelination,
etc. [Color figure can be viewed in
the online issue, which is available at
wileyonlinelibrary.com.]
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