Eur Arch Otorhinolaryngol
DOI 10.1007/s00405-016-4057-7
MISCELLANEOUS
Surgical management of Eagle’s syndrome: an approach
to shooting craniofacial pain
Yoshihiko Kumai1 • Tadashi Hamasaki2 • Eiji Yumoto1
Received: 23 January 2016 / Accepted: 15 April 2016
Ó Springer-Verlag Berlin Heidelberg 2016
Abstract Eagle’s syndrome (ES) and glossopharyngeal
neuralgia (GPN) display very similar symptoms preopera-
tively. The objective of this study is to determine the sur-
gical outcome of intraoral resection of the styloid process
(IRSP) for ES, and to observe preoperative findings and
treatment outcome of our cases presenting shooting cran-
iofacial pain. In total, 14 symptomatic patients who pre-
sented with typical shooting craniofacial pain, had a styloid
process longer than 25 mm, and underwent surgical inter-
vention or medication alone from 2011 to 2015 were
involved. They were divided into two groups: Group I
included eight patients who underwent surgery following
3 months of medication failure, and Group II included six
patients who received medication alone. Preoperative
physical, radiographic findings and surgical outcomes were
examined. In Group I patients, six cases received IRSP and
five of those six cases experienced complete relief from
symptoms and were confirmed as ES. Two other cases in
Group I received microvascular decompression. One
showed complete relief from symptoms, and was con-
firmed as GPN. The other case showed recurrence 1 year
postoperatively, received IRSP with complete relief from
symptoms, and was confirmed as ES. In Group II, three
cases experienced complete relief from symptoms with
3 months of medication alone. IRSP is an effective treat-
ment for ES. There was no clear difference in the
& Yoshihiko Kumai
kumayoshi426yk@gmail.com
1 is relatively easy to perform, involves no extensive facial
Department of Otolaryngology Head and Neck Surgery,
Kumamoto University Graduate School of Medicine, 1-1-1
Honjo, Kumamoto, Kumamoto 860-8556, Japan
2
Department of Neurosurgery, Kumamoto University
Graduate School of Medicine, Kumamoto, Japan
preoperative findings for ES and GPN, suggesting the
difficulty in making a preoperative differential diagnosis
between the two conditions. Close cooperation between
ENT and neurosurgery surgeons is needed.
Level of evidence IV.
Keywords Eagle’s syndrome
Styloid process

Glossopharyngeal neuralgia
Microvascular
decompression
Introduction
A symptom complex associated with an elongated styloid
process, also called Eagle’s syndrome (ES), occurs when
the styloid process or the ossified stylohyoid ligament is
longer than 25 mm; this was first demonstrated by Eagle
in 1949 [1]. The typical symptom is recurrent shooting
craniofacial pain that radiates from the base of the tongue
to the middle ear during swallowing; such pain is asso-
ciated with compression of the glossopharyngeal nerve by
the elongated styloid process [2–4]. The diagnosis is
usually confirmed by a typical history, aggravation of
symptoms on palpation of the tonsillar fossa, relief of
symptoms with injection of local anesthetics, and mea-
surement of the styloid process with a three-dimensional
CT scan [2–4]. Surgical excision of the elongated styloid
process is the treatment of choice and may be performed
via an intraoral or trans-cervical approach [5, 6]. The
intraoral approach is preferred by ENT surgeons since it
dissection, and does not lead to external scarring, which is
inevitable with a trans-cervical approach. Moreover, the
lengths of both the surgery and recovery period are
reduced [7, 8].
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GPN is a rare condition, characterized by the same
symptoms as Eagle’s syndrome, such as attacks of cran-
iofacial pain that are described as sharp or shooting, last
from a few seconds to 1 min, are experienced in the throat
and ear, and are usually precipitated by swallowing [9, 10].
Because microvascular compression of the nerve root has
been hypothesized as a cause, microvascular decompres-
sion (MVD) has been proposed as a surgical treatment [9,
10]. As the patterns of vascular compression are closely
related to the result of MVD, preoperative MRI assessment
of microvascular compression to the glossopharyngeal
nerve may be important in improving surgical results and
in avoiding unnecessary surgery [11].
Two etiologies, GPN and ES, are in fact both the same
pathological condition—glossopharyngeal neuralgia
caused by different anatomical compressions of the
nerve—intracranial vascular compression is found in GPN,
and extracranial compression due to the elongated styloid
process is found in ES. These two etiologies cause quite
similar symptoms and are thus, difficult to differentiate
[12]. Therefore, when medication for shooting cranial pain
is ineffective, and surgical treatment is required, close
collaborative discussions between ENT surgeons and
neurosurgeons are required to determine the appropriate
surgical pathway based on the correct differential diagno-
sis. However, such collaboration is currently uncommon.
The aims of this study were, first, to determine the
surgical outcome of intraoral resection of the styloid pro-
cess (IRSP) for the treatment of ES and, second, to observe
the preoperative findings and treatment outcome of our
cases presenting shooting craniofacial pain to aid in mak-
ing a correct diagnosis for shooting craniofacial pain, and
to shed light on the necessity for close collaboration
between ENT surgeons and neurosurgeons.
Materials and methods
Fourteen symptomatic patients (age 45–88, average
68.5 years, 5 males, 9 females) who presented with both
typical shooting craniofacial pain and the presence of an
elongated styloid process (more than 25 mm in length) on
the same side, as evidenced by three-dimensional CT
scans, were included in this study. These patients received
surgical intervention or medication alone at our institution
from 2011 to 2015. Written consent was obtained from all
patients. Patients agreed that we could use clinical data
obtained from this study for diagnosis and preoperative or
postoperative evaluation for the purposes of research.
Ethical approval was not necessary because this was a
retrospective study and included no additional examination
of the patients. For learning of the cases presenting
shooting craniofacial pain, patients were divided into two
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Eur Arch Otorhinolaryngol
groups: one group included patients who were treated
unsuccessfully with medication and then underwent
resection of the styloid process (n = 6) or MVD (n = 1) or
both (n = 1) (Group I, n = 8), and the other group inclu-
ded patients who received medication alone (Group II,
n = 6). Both groups had been receiving carbamazepine or
pregabalin for at least 3 months.
When unsatisfactory improvement was achieved with
medication, we considered the following surgical inter-
ventions. The first was IRSP, which was performed in our
department on patients who had been preoperatively
diagnosed with ES with the presence of typical symptoms
and an elongated styloid process (more than 25 mm), as
evidenced by three-dimensional CT scans. Another was
MVD of the glossopharyngeal nerve, which was performed
in the neurosurgery department of Kumamoto University
Hospital on patients who had been initially diagnosed with
GPN with the presence of typical symptoms and vascular
contact with the glossopharyngeal nerve (VCG), as evi-
denced by MRI. Patients were evaluated in terms of the
following: (1) section with shooting craniofacial pain, (2)
precipitant of shooting craniofacial pain, (3) length of the
styloid process on the affected and non-affected sides, (4)
presence of a trigger point and of a rigid funicular process
in the tonsillar fossa by palpation, (5) presence of VCG (as
evidenced by MRI) suggestive of GPN, (6) symptom
duration, (7) surgical procedures (i.e., IRSP, MVD, or
both), (8) surgical outcome, and (9) final diagnosis. The
surgical outcome was evaluated by the presence of surgical
complications during the postoperative period and the
relief of symptoms by the time of the one-month postop-
erative visit to the hospital.
The detailed procedures for each surgical intervention
follow.
Intraoral resection of the styloid process (IRSP)
Following a tonsillectomy on the affected side, the white
structure of the styloid process was identified by elevating
the connective tissue around the process with a mucosal
elevator, which was bluntly and gently uncovered without
damaging the branches of the pharyngeal artery and vein
and the glossopharyngeal nerve itself, which surrounded
the process. Manipulation under a microscope was helpful.
After uncovering the process, it was grasped with forceps,
fractured bluntly, and removed (case 2) (Fig. 1). Finally,
the pharyngeal muscle was sutured with 4-0 nylon.
Microvascular decompression of glossopharyngeal
nerve (MVD)
Exploration of the cerebellopontine angle was performed
through a retromastoid craniotomy, as described elsewhere
Eur Arch Otorhinolaryngol

Fig. 1 Surgical finding of the

styloid process, which is located
beneath the pharyngeal muscle
layer (a). Resected styloid
process (b) (from case 2)

[2, 9–15]. Compressive arteries were dissected free from
the nerve and the brainstem, and maintained far from the
root entry zone by interposing small pieces of Teflon
between the offending artery and the brain stem.
Results
Details of the preoperative physical and radiographic
findings and surgical outcomes for Group I patients (n = 8)
are provided in Table 1. Six cases received IRSP; five of
this group experienced complete relief from symptoms
without recurrence, and were confirmed as suffering from
ES (cases 1, 4, 5, 6, and 7). One of the six cases showed
persistent symptoms postoperatively, which might have
been due to symptoms of a herpes zoster infection in the
trigeminal area that developed 6 months prior to the IRSP
(case 3). The two remaining cases in Group I received
microvascular decompression (MVD) of the glossopha-
ryngeal nerve. One showed complete relief from symptoms
with no recurrence and was confirmed as suffering from
GPN (case 8). This patient underwent MVD because, in
addition to the presence of VCG, the length of the styloid
process was only 25 mm (shortest), and the rigid process
was not palpable in the tonsillar fossa preoperatively.
Another case, who showed recurrence 1 year postopera-
tively, subsequently underwent IRSP with complete relief
from symptoms and no recurrence; this case was ultimately
confirmed as suffering from ES (case 2). This case received
MVD first, because, the length of the styloid process on the
affected side was not measured preoperatively; therefore,
this information did not contribute to the surgical decision
made in the neurosurgery department. The styloid process
was later found to be 53 mm long. Thus, in total, we per-
formed IRSP in seven patients, and six patients achieved
complete relief from symptoms. None of these eight
patients who underwent surgical procedures suffered major
complications, such as post-operative bleeding, swallowing
dysfunction, or deep neck infection. The location of the
pain was from the base of the tongue to the middle ear in

Table 1 Patient demographic (Group I)

Case Age Portion Inducing Length Length of Presence Presence Presence of Duration Surgical Symptom
number of factor of styloid of trigger of rigid arteries in contact of approach
shooting styloid process point process with the disease
pain process non- in the glossopharyngeal (years)
affected affected tonsillar nerve (VCG)
side side fossa
(mm) (mm)

1 72M DIE Swallow 46 42 ? ? ? 10 IRSP Disappear

2 70M DIE Swallow 53 54 ? ? ? 7 MVD Disappear
IRSP
3 58F DIE Phonation 35 35 ? - - 1 IRSP Continued
4 65F DIE Swallow 39 38 ? ? ? 2 IRSP Disappear
5 78M DIE. Swallow 32 31 ? - ? 6 IRSP Disappear
6 78M DIE Swallow 42 38 ? ? - 13 IRSP Disappear
7 63F DIE Swallow 36 36 ? ? ? 2 IRSP Disappear
8 45F DIE Swallow 25 26 ? - ? 3 MVD Disappear
DIE deep inside ear, IRSP intraoral resection of styloid process, MVD microvascular decompression

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all eight cases. With the exception of one case, whose pain
was triggered by phonation (case 3), symptoms were trig-
gered by swallowing. The non-affected side also showed
elongation of the styloid process in all eight cases. The
presence of a trigger point in the tonsillar fossa was
detected in all eight cases. The presence of a rigid process
in the tonsillar fossa was detected in five cases but not in
three (cases 3, 5, and 8). The presence of VCG, diagnosed
with MRI, was detected in six cases but not in two cases
(cases 3 and 6). The duration of symptoms in Group I
ranged from 1 to 10 years (average, 5.5 years).
Details of the physical and radiographic findings and
medication outcomes for the Group II patients (n = 6) are
provided in Table 2. Three cases achieved complete relief
from symptoms following 3 months of medication and no
surgical intervention or recurrence for a minimum follow-
up period of 1 year (cases 9, 10, and 14). Of the remaining
three cases, two achieved symptom relief with 2 months of
medication; however, symptoms recurred at 3 months and
1 year, respectively (cases 12 and 13). As with the Group I
patients, the pain was located from the base of the tongue
to the middle ear, and the precipitant was swallowing in all
six cases. As in Group I, all patients showed bilateral
elongations; the non-affected side also showed elongation
of the styloid process in all six cases. The presence of a
trigger point and of a rigid process in the tonsillar fossa
were detected in all six cases. Unlike the Group I patients,
the presence of VCG was not detected in any Group II
patients. The duration of symptoms in Group II ranged
from 3 months to 13 years (average 3.8 years).
Discussion
This is the first report to focus on the necessity of close
cooperation between ENT surgeons and neurosurgeons in
the management of shooting craniofacial pain. All patients,
except for three (3/14), enrolled in the present study were
initially consulted by the neurosurgery department for the
purpose of differentially diagnosing between ES and GPN.
This helped us to ‘‘excavate’’ the ES patients. ES was
previously demonstrated twice in the neurosurgical litera-
ture, with those studies identifying the etiology as entrap-
ment of the glossopharyngeal nerve caused by compression
of the nerve by bony elements as it travels medially to the
styloid process in the neck, and patients with its symptoms
are often referred to neurosurgeons for operative consid-
eration with a tentative diagnosis of GPN [2, 6]. These
patients underwent resection of the styloid process on the
symptomatic side using a lateral transcutaneous (external)
approach. Previous ENT studies [7, 8] noted that the
external approach provides good visualization and a
reduced possibility of deep neck space infection. The dis-
advantages are the presence of an external scar, longer
duration of surgery, and risk of injury to the facial nerve.
On the other hand, the advantages of the intraoral approach
that we performed are that the method is safe, simple, less
time consuming, and an external scar is avoided. However,
the disadvantages are possible infection of the deep neck
spaces, risk of injury to major vessels, and poor visual-
ization [5]. In the present study, no patient who received
the intraoral approach demonstrated complications, and all
were discharged within five postoperative days. Based on
this perspective, we wish to emphasize that the disadvan-
tages of the intraoral approach described in previous
reports can be avoided by performing the surgical proce-
dure carefully and precisely under a microscope.
Craniofacial pain syndromes of neuromusculoskeletal
origin are not well known by most clinicians working in the
head and neck area [16]. As a result, most patients with
these syndromes are either overlooked and do not receive
any efficacious treatment or are inappropriately treated
with antibiotics or surgical interventions, such as dental
extractions and tonsillectomies. These patients are typi-
cally seen by an ENT doctor, a dentist, a neurologist, or
even a psychiatrist and receive a variety of treatments that

Table 2 Patient demographic (Group II)

Case Age Portion Inducing Length of Length of Presence Presence of Presence of arteries Duration Symptom
number of factors styloid styloid of trigger rigid in contact with the of
shooting process process non- point process in glossopharyngeal disease
pain affected affected side the tonsillar nerve (VCG) (months)
side (mm) (mm) fossa

9 64F DIE Swallow 43 30 ? ? - 3 Disappear

10 81F DIE Swallow 35 35 ? ? - 4 Disappear
11 37F DIE Swallow 28 27 ? ? - 96 Continued
12 63F DIE Swallow 30 30 ? ? - 6 Continued
13 72F DIE Swallow 30 26 ? ? - 156 Continued
14 59M DIE Swallow 35 35 ? ? - 12 Disappear
DIE deep inside ear

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Fig. 2 Case 2: Preoperative
(microvascular decompression)
MRI; green arrow shows the
vascular (PICA) contact with
the glossopharyngeal nerve
(VCG) (a). Preoperative
(intraoral resection of the
styloid process) three-
dimensional CT: the length of
the styloid process was 53 mm
on the affected side (b)
do not relieve the symptoms. This suggests that better
recognition of the pain syndromes of the neck and face
region or of entities related to the craniofacial neuromus-
culoskeletal system may result in more appropriate and
effective management of such conditions while avoiding
unnecessary medical and surgical treatments.
The differential diagnosis of these symptoms includes
ES, GPN, superior laryngeal neuralgia, carotidynia, and
hyoid bone syndrome [16]. Among these entities, ES and
GPN, in particular, present the quite similar symptom of
shooting craniofacial pain. Kent et al. demonstrated that the
styloid process was significantly closer to the tonsillar fossa
in patients with ES, compared with asymptomatic control
patients. No significant difference was found in other
measures in a comparison between ES and GPN. The dis-
tance to the tonsillar fossa may be a more appropriate
diagnostic criterion for ES than is the length of the styloid
process, and may contribute to the pathophysiology of ES
[17]; however, further analysis of a clear preoperative dif-
ferential diagnosis between ES and GPN in a prospective
cohort study with an increased number of patients is needed.
In the present study, all 14 patients demonstrated bilat-
eral elongated styloid processes that were longer than
25 mm; however, unilateral symptoms were present in all
cases. This suggests that the location of both the styloid
process and the glossopharyngeal nerve of the pharyngeal
branch, rather than the length of the styloid process per se,
might cause the typical symptoms of ES when these are
precipitated by swallowing accompanied by contraction of
the pharyngeal muscle. Thus, we assumed that the unilat-
eral symptoms were due to the unilateral compression of
the glossopharyngeal nerve between the two structures, the
styloid process and the pharyngeal muscle, precipitated by
swallowing.
We basically agree that palpation of an elongated styloid
process in the tonsillar fossa that indicates a hard, bony
spicule is typical in ES patients. However, in one case (case
5), we were unable to preoperatively detect the process in
the tonsillar fossa, but resection of the styloid process
worked effectively. This suggests that, even if palpation
did not indicate a hard, bony spicule in the tonsillar fossa,
the presence of typical observations on three-dimensional
CT and shooting craniofacial pain precipitated by swal-
lowing would make it worthwhile to perform an intraoral
resection of the styloid process if medication fails.
MVD is an effective surgical option for GPN, as
described elsewhere [9–14]. The diagnosis of GPN, like
that of ES, is based on clinical findings, and neuroimaging
plays a key role in detecting possible causes, such as vas-
cular compression and tumors. MRI can be an extremely
accurate tool for detecting VCG, although the absence of
an MRI-evidenced VCG should not exclude patients with
intractable pain from MVD [10]. Case 2 received MVD
first because of vascular contact with the glossopharyngeal
nerve evidenced by the preoperative MRI, and the length of
the styloid process on the affected side was not measured
preoperatively which did not contribute to the surgical
decision made by the neurosurgery department. The styloid
process was later found to be 53 mm in length (Fig. 2) at
ENT department. Additionally, among the six cases who
underwent IRSP and achieved complete symptom relief,
four presented with VCG preoperatively. These findings
suggest that VCG in a preoperative MRI may not guarantee
that vascular contact with the glossopharyngeal nerve is a
true cause of shooting pain. Based on this consideration,
IRSP should be prioritized over MVD due to the physical
aggressiveness of the former and given that MVD requires
a craniotomy approach.
If the preoperative diagnosis is correct, surgical inter-
ventions are the fundamental and unique treatments for
both ES and GPN [2–6, 11–15]. However, it is important to
recognize that some of these patients can be ‘cured’ with
medication alone. The present study demonstrated that
three cases (9, 10, and 14) in Group II achieved complete
123

Fig. 3 Differential treatment
algorithm for Eagle’s syndrome
relief from symptoms with 3 months of medication and no
surgical intervention or recurrence for up to at least 1 year
of follow-up. This suggests that, even with typical symp-
toms and positive observations on a three-dimensional CT,
a trial of at least 3 months of medication is necessary
before considering surgical intervention. Moreover, we
need to inform patients and emphasize that surgical inter-
vention does not always work. Cases in which symptoms
resolved with medication alone require at least 1 year of
follow-up after symptom relief given that our study
demonstrated that, among the remaining three cases (11,
12, and 13) in Group II, two (12 and 13) showed relief from
symptoms with 2 months of medication, but the symptoms
recurred at 3 months and 1 year, respectively. In Group II,
the presence of VCG could not be detected with MRI in
any case; thus, medication should be tried first in cases that
do not show VCG as evidenced with MRI.
Overall, based on the data obtained in the present study
and our literature review, we have established a tentative
differential treatment algorithm for ES and GPN (Fig. 3).
This algorithm basically fits the complete patient profile,
treatment process, and outcome. If we find the typical
symptoms and the physical examination results presented
here, key radiographic examination points are whether the
styloid process is less than or more than 25 mm according
to a three-dimensional CT scan and VCG-positive
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Eur Arch Otorhinolaryngol
or -negative according to MRI. However, MRI findings
suggesting the presence of VCG may not guarantee true
vascular contact with the glossopharyngeal nerve. If
3 months of medication does not help, IRSP should be
prioritized for MVD even in the presence of VCG.
The limitations of this study were that it was performed
retrospectively without definite criteria for the surgical
indications and the involvement of the patients in each
group was not randomized. In the future, following the
proposed algorithm in the present study, a prospective
study with an increased number of patients, matched
medical management, and follow-up is required.
Conclusions
Intraoral resection of the styloid process is an effective
treatment for ES. There was no clear difference in the
preoperative findings for ES and GPN, suggesting the
difficulty in making a preoperative differential diagnosis
between the two conditions. Close cooperation between
ENT and neurosurgery surgeons is needed.
Compliance with ethical standards
Conflict of interest None.
Eur Arch Otorhinolaryngol
Research involving human participants and/or animals This
research involves human participants.
Informed consent Written consent was obtained from all patients.
Patients agreed that we could use clinical data obtained from this
study for diagnosis and preoperative or postoperative evaluation for
the purposes of research. Ethical approval was not necessary because
this is a retrospective study and included no additional examination of
the patients.
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