Copyright © 2019 Otology & Neurotology, Inc. Unauthorized reproduction of this article is prohibited.
Imaging Case of the Month
A Hurricane, Temporal Bone Paraganglioma, Cholesteatoma,
Bezold’s Abscess, and Necrotizing Fasciitis
Ã
Nathan R. Lindquist,
Ã
Eric N. Appelbaum,
Ã
Tanner Fullmer,
Ã
Vlad C. Sandulache,
and
Ã
yzAlex D. Sweeney
Ã
Department of Otolaryngology – Head and Neck Surgery; yDepartment of Neurosurgery, Baylor College of Medicine; and zDivision
of Otolaryngology, Department of Surgery, Texas Children’s Hospital, Houston, Texas
CASE PRESENTATION
A 66-year-old woman with a history of advanced
cervical cancer undergoing chemotherapy presented to
the emergency department of a tertiary care medical
center with a progressive history of right otalgia, neck
pain, and fever. At the time of presentation, it was
reported that the patient had a longstanding history of
non-descript hearing loss as well as 2 months of purulent
otorrhea, hearing loss, and tinnitus. Furthermore, in
recent weeks, her access to medical care had reportedly
been limited after being displaced into an emergency
shelter during a natural disaster. Physical examination
revealed a somnolent patient with purulent debris and
polypoid tissue obstructing the right external auditory
canal (EAC) to the level of the meatus. The right neck
was visibly erythematous and edematous, and palpation
demonstrated fluctuance of the mastoid tip with crepitus
throughout the ipsilateral neck to the level of the clavicle.
Contrasted computed tomography (CT) of the neck and
temporal bone showed erosion of the tympanic, mastoid,
and petrous portions of the temporal bone, as well as a
multiloculated abscess in the right neck with air tracking
from the skull base to the clavicle (Fig. 1A and B). There
was substantial erosion of the posterior EAC and no clear
jugular foramen destruction (Fig. 1C and D).
Once hemodynamically stable, the patient was taken to
the operating room for a sequential neck exploration and
mastoidectomy. These procedures identified a neck
abscess seemingly emanating from the mastoid tip.
Necrotic soft tissue was prevalent throughout multiple
fascial planes deep to the sternocleidomastoid muscle
extending to the sternal notch. A mastoidectomy
identified posterior EAC erosion, which was largely filled
with cholesteatoma and purulence. Debriding the previ-
ously noted polyp in the EAC revealed a distinct, vascular
tumor that was red and pulsatile. Biopsies of the tumor
mass revealed paraganglioma, which was resected where it
appeared to be associated with cholesteatoma debris
(Figs. 1E and 2). Postoperatively, the patient was treated
with antibiotics and hemodynamic support in the ICU, and
she ultimately recovered from her infection. Unfortu-
nately, she has subsequently been lost to follow-up.
DISCUSSION
Tympanic paraganglioma (TP) tumors originate from
the paraganglion cells of the tympanic plexus (1). In
general, these tumors present more commonly in middle-
aged women and are associated with conductive hearing
loss, pulsatile tinnitus, and aural fullness (2). Clinical
examination of patients with a TP frequently reveals a red
mass behind the tympanic membrane, and radiographic
evaluation with high-resolution computed tomography
(CT) of the temporal bone and/or contrast enhanced
magnetic resonance imaging (MRI) can be useful in
confirming the diagnosis and extent of disease. Gener-
ally, axial and coronal CT images of the tympanic cavity
are valuable when a temporal bone paraganglioma is
suspected. These tumors will cause bony destruction, and
destruction of the bone surrounding the jugular bulb help
to distinguish a jugular paraganglioma from a tympanic
paraganglioma. A contrast-enhanced MRI was not ini-
tially performed in this case due to the degree of the
patient’s systemic illness, though the identification of
flow voids within an otherwise enhancing tumor results
in a ‘‘salt-and-pepper’’ appearance that can be suggestive
of a paraganglioma.
The pathophysiology of this patient’s infection repre-
sents a presumably rare sequence of events and constel-
lation of imaging findings. Specifically, she had a
Glasscock-Jackson grade IV tympanic paraganglioma
that had both eroded into the mastoid as well as through
the tympanic membrane and external auditory canal (3).
Trapped keratinizing epithelium from the tympanic
membrane and ear canal injuries facilitated formation
Address correspondence and reprint requests to Alex D. Sweeney,
M.D., Department of Otolaryngology–Head and Neck Surgery, Baylor
College of Medicine, 1977 Butler Blvd., Suite E5.200, Houston,
TX 77030; E-mail: Alex.Sweeney@bcm.edu
Financial Material & Support: Internal departmental funding was
utilized without commercial sponsorship or support.
ADS is a consultant for Advanced Bionics Corp., Cochlear Corp., and
Oticon Medical.
DOI: 10.1097/MAO.0000000000002532
ß 2019, Otology & Neurotology, Inc.