J Neurosurg 107:1228–1230, 2007

Paroxysmal otalgia due to compression of the intermediate

nerve: a distinct syndrome of neurovascular conflict
confirmed by neuroimaging

Case report

DAMIANOS E. SAKAS, M.D.,1 IOANNIS G. PANOURIAS, M.D.,1 GEORGE STRANJALIS, M.D.,1

MARIA P. STEFANATOU, M.D.,1 NIKOS MARATHEFTIS, M.D.,1
AND NIKOS BONTOZOGLOU, M.D.2
1
Department of Neurosurgery, University of Athens Medical School, Evangelismos General Hospital;
and 2Department of Magnetic Resonance Imaging, Iatrikon Medical Center, Athens, Greece

PThe authors present the case of a 52-year-old female patient with a 6-year history of intractable paroxysmal otalgia.
Preoperative magnetic resonance (MR) angiography demonstrated an anterior inferior cerebellar artery loop compress-
ing the intermediate nerve in the seventh/eighth cranial nerve complex inside the internal auditory canal. The pain
resolved completely after a microvascular decompression via a retromastoid craniotomy. To the best of the authors’
knowledge, the combined neuroimaging and intraoperative findings of this case provide a unique demonstration that
vascular compression of the intermediate nerve can be the exclusive cause of paroxysmal otalgia. Magnetic resonance
imaging and MR angiography can establish the causative mechanism and distinguish this otalgia due to vascular com-
pression of the intermediate nerve from other pain syndromes that are designated as geniculate neuralgia (GN). The pre-
sent case indicates that intermediate nerve neuralgia is a distinct syndrome of neurovascular conflict and a variant of
GN. The causative classification of GN should be reexamined with the use of advanced MR imaging.
(DOI: 10.3171/JNS-07/12/1228)

KEY WORDS • cochleovestibular nerve • facial nerve • intermediate nerve •

magnetic resonance angiography • neurovascular compression • otalgia

otalgia is a disabling, chronic, and epi- mediate nerve (sensory branch of the facial nerve) was re-

P
AROXYSMAL
sodic ear canal pain, which has remained a diagnostic
and treatment challenge. There are various types of
chronic pain in the ear canal, and the term geniculate neu-
ralgia (GN) has been used extensively to describe them.3,5,11,
12,15,21
Given the poor understanding of the causative mech-
anism of GN, the surgical treatment of GN has frequently
had an exploratory character and included MVD or division
of cranial nerves (fifth, seventh, ninth, and 10th), section-
ing of the intermediate nerve or greater superficial petrosal
nerve, and/or partial excision of the GG.8,12,15 The variety of
surgical treatments for this condition is because of the ana-
tomical variations of the GG, intermediate nerve, and their
connections,12,14,16 and their proximity with the fifth, sev-
enth, ninth, and 10th cranial nerves, which participate in the
innervation of the wider ear canal area.8,12,19,20 The liberal
and rather indiscriminate use of the terms GN or INN to de-
scribe not only ear but also facial pains and the absence of
conclusive neuroimaging data have compounded the prob-
lem. During procedures for the treatment of GN, the inter-
Abbreviations used in this paper: AICA = anterior inferior cere-
bellar artery; GG = geniculate ganglion; GN = geniculate neuralgia;
IAC = internal auditory canal; INN = intermediate nerve neuralgia;
MR = magnetic resonance; MVD = microvascular decompression.
ported to be compressed by the AICA in some patients. To
the best of our knowledge, however, clear and convincing
pictorial demonstrations of intraoperative findings of such
neurovascular conflict have been very limited. Further-
more, no reports of preoperative neuroimaging studies con-
firming the neurovascular compression of the intermediate
nerve as the primary cause of paroxysmal otalgia have been
provided in the literature.
Case Report
History and Examination. This 52-year-old woman pre-
sented with a 6-year history of paroxysmal pain of the right
auditory canal, pinna, and adjacent retromastoid area. She
also described experiencing ipsilateral tinnitus, right-sided
hearing loss, and continuous unsteadiness, which was com-
plicated by vertiginous spells that resembled bouts of mo-
tion intolerance and that were precipitated by head move-
ment. She was treated with carbamazepine administration
in high doses, but this proved ineffective. Her previous
medical history was remarkable for arterial hypertension
during medication administration and for bilateral carpal
tunnel syndrome. The neurological examination revealed
disabling positional vertigo and nystagmus with no other

1228 J. Neurosurg. / Volume 107 / December, 2007

Magnetic resonance angiography in paroxysmal otalgia
neurological symptoms or signs. Pure tone audiometry of
the patient showed complete sensorineural right-sided hear-
ing loss. Hearing in the left ear was normal. The imag-
ing studies included computed tomography; MR imaging;
and MR angiography with typical T1- and T2-weighted
sequences, time-of-flight in coronal reprojection, and high-
resolution T2-weighted MR imaging at the level of the
IAC. This thorough neuroimaging sequence showed that
the AICA was extensively curved, had entered into the
IAC, and was clearly compressing the seventh and eighth
cranial nerves (Fig. 1).
Operation and Postoperative Course. The patient under-
went a right retromastoid craniotomy. After minimal re-
traction of the cerebellum, the seventh and eighth cranial
nerves were identified. The sensory branch of the seventh
cranial nerve was identified as a separate branch from the
rest of the nerve that was being compressed by the arterial
loop (Fig. 2 left). The artery was mobilized, and a Teflon
narrow band (Fig. 2 right) was placed between the artery
and the seventh and eighth cranial nerves to separate the
artery from the nerves. The postoperative course was un-
eventful, apart from mild peripheral facial nerve palsy. The
paresis resolved completely during the next 3 months. The
intensity and frequency of the otalgia (the paroxysmal pain
of the right auditory canal and the adjacent area) decreased
markedly. The pain resolved completely during the next 6
months. In addition, the patient experienced a gradual relief
from the vertigo and tinnitus and she no longer experienced
nystagmus. A postoperative audiogram demonstrated an
improvement of hearing in the right ear. Importantly, nei-
ther sectioning of the intermediate nerve nor excision of the
GG was necessary to achieve this excellent outcome.
Discussion
Neurovascular Conflict in the Seventh/Eighth Cranial
Nerve Complex
Vascular branches in close anatomical relationship to a
cranial nerve (neurovascular proximity) may cause com-
pression and dysfunction of the nerve.7 The proposed mech-
anisms for this condition include either ephaptic transmis-
sion and irritation of the nerve by the pulsating artery or
compression-induced demyelination, chronic inflammatory
response, and increased excitability.18 Neurovascular prox-
imity may be present at birth, although it may not become
clinically evident until the development of atherosclerosis
and ectatic distortion of vascular loops that compress the
adjacent nerves.9 Cochleovestibular nerve syndrome oc-
curs when a vessel compresses the eighth cranial nerve; its
FIG. 1. Axial (left) and coronal (right) views of time-of-flight
MR angiography showing the right AICA crossing the cerebello-
pontine cistern and entering into the IAC.
J. Neurosurg. / Volume 107 / December, 2007
FIG. 2. Intraoperative views of the patient through the micro-
scope under magnification. Left: The loop of the AICA is shown
running between the facial (upper) and cochleovestibular (lower)
nerves. The concave part of the loop runs around the cochleovestib-
ular nerve and the convex part of the loop compresses the facial
nerve. The sensory branch runs normally along the inner surface of
the main bundle of the facial nerve. The compression of the facial
nerve by the arterial loop is demonstrated at this area, causing the
clinical symptoms of the patient. Right: View after the placement
of the Teflon band between the AICA and the facial nerve.
symptoms include unilateral sensorineural hearing loss, tin-
nitus, and disabling positional vertigo. These symptoms
may be aggravated after changes in head position or be at-
tenuated by bed rest.10,17 The vascular compression of the
seventh cranial nerve causes hemifacial spasm, a paroxys-
mal, involuntary, synchronous contraction of the muscles
innervated by the facial nerve. In cases of compression of
both the seventh and eighth cranial nerves, there may be a
combined symptomatic picture.18
Ear Canal Pain, GN, and INN
The GG contains the cell bodies of unipolar sensory
fibers whose proximal processes run along the facial nerve
and, via the intermediate nerve, inside the IAC, cross over
to the superior vestibular nerve where they lie above and
slightly anterior to it, and reach the brainstem; the distal
processes traverse the seventh cranial nerve and its branch-
es.8,11,13,16 The intermediate nerve consists of proprioceptive
afferents (facial muscles), visceral afferents (anterior two-
thirds of the tongue), and visceral efferents (lacrimal and
salivatory glands), and carries sensation from a small part
of the medial external auditory canal and, via the posterior
auricular nerve and greater superficial petrosal nerve, from
the external auditory canal posterior wall, posterior tym-
panic membrane, and postauricular area.19,20
Geniculate neuralgia is a rare, insufficiently understood
ear canal pain. The term geniculate neuralgia has been ap-
plied rather loosely to describe various chronic ear or facial
aches of various causes. Geniculate neuralgia remains a
diagnostic challenge because it can be either idiopathic or
secondary to diseases of the ear or structures remote from
the ear. Intermediate nerve neuralgia was originally report-
ed in 19091 and also remains insufficiently understood. The
few reported patients with this condition include cases
related to herpetic infection or neuroborreliosis.2,6 Given
that GG contains the afferent fibers of the intermediate
nerve, the terms GN and INN have often been used indis-
criminately.3,6,8,11,16 In the past, without the use of preopera-
tive imaging, surgeons used exploratory craniotomies and
MVDs, sectioning of cranial nerves innervating the ear area
(fifth, seventh, ninth, and 10th), or excision of the GG to
treat ear canal pain.8,11,12,15,16 The selection of surgical treat-
1229

ment seems to have been based on the surgeon’s personal
experience and interpretation of the intraoperative findings
rather than on a firm preoperative diagnosis. The treatment
approaches for the presumed GN have even been targeted
to nerves with no known connections to the GG.8 Frequent-
ly, the MVD or sectioning of any of the cranial nerves list-
ed earlier was proven postoperatively to be insufficient for
offering pain relief, and surgeons were subsequently oblig-
ed to reoperate and perform intermediate nerve sectioning.
Pulec11,12 proposed that part of the facial nerve along with
the intermediate nerve and the GG should be excised to
offer an effective treatment for intractable INN. Lovely and
Jannetta8 noted that, in patients who underwent multiple
procedures for alleviating incapacitating symptoms of GN,
it was not until sectioning of the intermediate nerve was
performed that lasting relief was obtained.
Modern Neuroimaging Use in the Diagnosis of GN and
INN
The term GN implies that the GG is the primary struc-
ture involved in the pathogenetic mechanism of this condi-
tion, but the earlier data indicate that the intermediate nerve
plays the key role in the manifestation of ear canal pain. In
confirming the anatomical relationship between a vessel
and a nerve, special MR imaging sequences are crucial.
During the last decade, advances in high-resolution MR
imaging and MR angiography provided substantial evi-
dence in support of the neurovascular compression con-
cept. Our patient presented with symptoms of compression
of the eighth cranial nerve (such as disabling positional
vertigo, tinnitus, and unilateral [right-sided] sensorineural
hearing loss), but the most incapacitating symptom was in-
tractable paroxysmal pain in the auditory canal, pinna, and
adjacent posterior temporal area. Preoperative MR angiog-
raphy revealed the arterial loop of the AICA entering into
the IAC and positioned in close relationship with the sev-
enth and eighth cranial nerves. The excellent response to
MVD of the intermediate nerve (with neither sectioning of
the intermediate nerve nor excision of the GG required)
demonstrates that intermediate nerve compression by the
AICA can be a single underlying cause of paroxysmal otal-
gia. In our case, therefore, the diagnosis of INN due to vas-
cular compression of the intermediate nerve is well estab-
lished.
The present case does show that the problem of parox-
ysmal otalgia can be restricted only to the intermediate
nerve; in this sense, our case represents a well-document-
ed, distinct clinical entity. When paroxysmal otalgia is pro-
ven to be caused by intermediate nerve compression using
combined MR angiography and intraoperative findings, the
chronic pain should be considered a variant of GN, a dis-
tinct syndrome of neurovascular conflict. This pain syn-
drome should be distinguished from the numerous other ear
and facial pain syndromes that are encompassed in the
broader clinical entity of GN. This distinction can guide
surgical decision-making toward selective MVD of the in-
termediate nerve, and therefore prevent the untoward ef-
fects of ablative and resective procedures on other cranial
nerves. Our data suggest that the wider clinical entity of
GN and atypical facial pain syndromes should be revisited
using modern sophisticated neuroimaging. This reassess-
ment may prove necessary because, currently, neuromod-
1230
D. E. Sakas et al.
ulatory treatments of severe cephalalgias4 may gradually
become a serious alternative to the conventional microneu-
rosurgical approaches; therefore, it is expected that, in the
future, MVD procedures may need to be reserved for those
particular cases in which the relevant vessel is sufficiently
visualized by advanced digitized neuroimaging techniques.
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Manuscript submitted December 12, 2005.
Accepted March 20, 2007.
Address correspondence to: Damianos E. Sakas, M.D., Department
of Neurosurgery, Evangelismos General Hospital, 4 Marasli Street,
Athens 10676, Greece. email: sakasde@med.uoa.gr.
J. Neurosurg. / Volume 107 / December, 2007