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Case Report

Congenital Mastoid Cholesteatoma of Adult Patient

Masquerading as Complicated Otitis Media – A Case Report
and Literature Review
Balaji Ramamourthy, Anurag Snehi Ramavat, Neemu Hage, Arun Govind
Department of Otolaryngology and Head and Neck Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Abstract
The study highlights the management protocol of a large mastoid cholesteatoma presenting with sudden‑onset facial palsy mimicking a
complicated otitis media and literature review. Congenital cholesteatoma may affect various regions of the temporal bone, the mastoid process
being the least frequently involved. The diagnosis is often missed, specifically when the patient presents with features of mastoiditis. Radiology
plays an important role in clenching the diagnosis. Congenital (mastoid) cholesteatoma manifesting with features of complicated otitis media
is a rare occurrence and needs familiarity to facilitate early diagnosis and appropriate management.

Keywords: Congenital cholesteatoma, facial palsy, mastoid abscess, temporal bone

Introduction moderate in severity, intermittent, and nonradiating. Facial

In 1965, Derlacki and Clemis [1] defined congenital
cholesteatoma (CC) for the first time by outlining criteria for
its diagnosis, which was later modified in 1986 by Levenson
et al.[2] The incidence of CC reported in the literature is 0.12
per 100,000.[3] Approximately 1%–3% of the childhood
cholesteatoma cases, and 1%–5% of the cholesteatoma in
general, are congenital.[4] CC may originate within the temporal
bone in various locations, including the petrous apex, the
middle ear, the area of the geniculate ganglion, the mastoid
process, the jugular foramen, and the squamous portion of the
temporal bone.[1] However, occurrence in the mastoid process
is the least frequent.[5] Here, we report a rare case of the CC
of the mastoid in an adult patient who presented with otalgia
and facial nerve palsy, confounding our diagnosis with that
of acute mastoiditis.
weakness was acute onset and nonprogressive. The patient
denied any history of ear discharge, vertigo, or tinnitus;
however, he reported fullness of the ear and mild diminution
of hearing on the affected side. There was no history of
fever, headache, altered sensorium, seizures, or difficulty in
ambulation.
The patient had no other ear complaints in the past, and there
was no history of trauma. The patient received a course of oral
antibiotics before presenting to our institution.
On examination, the patient was fully conscious, oriented,
and afebrile. He had House Brackmann Grade V facial paresis
on the left side. No mastoid or cymba concha tenderness
was elicited; however, the patient had tragal tenderness. On
otoscopic examination, the cartilaginous part of the external
auditory canal was unremarkable; however, the deep part

Case Report Address for correspondence: Dr. Balaji Ramamourthy,

A 50‑year male patient presented to the emergency department Department of Otorhinolaryngology and Head & Neck Surgery,
All India Institute of Medical Education and Research (AIIMS),
with complaints of left‑sided otalgia and left‑sided facial Bibinagar- 500 088, India.
weakness for a week. The ear pain was dull aching, mild to E‑mail: drbala1991@gmail.com
Submitted: 06‑Nov‑2020 Revised: 20-Jan-2021  Accepted: 22‑Mar‑2021
Published: 20-Sep-2022 This is an open access journal, and articles are distributed under the terms of the Creative
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Access this article online remix, tweak, and build upon the work non‑commercially, as long as appropriate credit
Quick Response Code: is given and the new creations are licensed under the identical terms.
Website: For reprints contact: WKHLRPMedknow_reprints@wolterskluwer.com
www.indianjotol.org

How to cite this article: Ramamourthy B, Ramavat AS, Hage N,

DOI: Govind A. Congenital mastoid cholesteatoma of adult patient masquerading
10.4103/indianjotol.indianjotol_243_20 as complicated otitis media – A case report and literature review. Indian J
Otol 2022;28:149-52.

© 2022 Indian Journal of Otology | Published by Wolters Kluwer - Medknow 149

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Ramamourthy, et al.: Congenital mastoid cholesteatoma

was congested. The tympanic membrane was intact and dull.

There was congestion over the posterosuperior quadrant of
the tympanic membrane. A working diagnosis of complicated
acute otitis media was made.
A high‑resolution computed tomography  (CT) of temporal
bone revealed a huge coalescent cavity with the destruction
of mastoid septae. The disease was also found to be eroding
the overlying cortical bone, posteriorly the sigmoid plate;
however, the tegmen appeared intact. The soft‑tissue density
also involved the attic, but no widening of the aditus was
Figure 1: Congenital cholesteatoma of mastoid showing the destruction
appreciated, and the ossicles were also unaffected. The of posterior fossa plate, sigmoid sinus, and closely abutting cerebellum
disease was in close relation with the posterior cranial
fossa [Figure 1]. Hence, contrast‑enhanced magnetic resonance
imaging (MRI) was done, which showed a T1 isointense and T2
hyperintense lesion involving the mastoid region. The lesion
encroached the cerebellum, but fat planes with the dura were
maintained [Figure 2a and b].
Pure‑tone audiometry of the patient showed moderate mixed
hearing loss in the left ear and normal hearing sensitivity
in the right. A facial nerve excitatory stimulation was done
preoperatively.
a
An emergency mastoid exploration was performed with
intraoperative findings of a huge cholesteatoma sac behind
an intact tympanic membrane which had replaced the mastoid
air cells, caused the destruction of mastoid cortical bone,
sigmoid plate, and exposure of the posterior cranial fossa
dura  [Figure  3a]. Tegmen and ossicular chain were intact.
The bony Fallopian canal was intact in its tympanic segment,
whereas it was dehiscent in its entire course in the mastoid
segment. Attic and aditus were normal except for edematous
mucosa. The semicircular canals were intact. b
Figure 2: (a) T1‑weighted image showing hypointense lesion. (b) Showing
A modified radical mastoidectomy was done, which resulted T2‑weighted image showing hyperintense lesion of the mastoid closely
in a huge cavity. The cavity was obliterated using fat and bone abutting cerebellum
pâté. Wullstein Type III tympanoplasty was performed.
Postoperatively, the patient had persistent ear discharge, which
was managed conservatively. The cavity was dry after a year
of vigilant follow‑up. The facial paresis, however, persisted.
He was last reviewed at 18 months post surgery and has been
rendered disease‑free [Figure 3b]. We intend to keep him on
a longer follow‑up.

Discussion
CC arises from an epidermoid cyst from a congenital remnant
of the keratinizing squamous epithelium of the temporal
bone.[6]
CC of mastoid origin has the following characteristics:
1. The initial presentation is usually pain in the neck and
mastoid region
2. CT scan shows an expansile, cystic lesion occupying
the mastoid process without involving the middle ear,
which may compress the cerebellum and the dural sinuses
adjacent to it and/or erode the bony labyrinth
a b
Figure 3: (a) Huge mastoid cavity after removal of cholesteatoma showing
the intact posterior fossa dura. (b) Completely healed postoperative cavity
at 18‑month follow‑up
3. MRI scanning shows little or no peripheral enhancement
on postcontrast T1‑weighted images and hyperintensity
on T2‑weighted images.[1,7,8]
Similar presenting complaints and radiological appearance
can be noted in our case as well [Figures 1 and 2]. However,

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Ramamourthy, et al.: Congenital mastoid cholesteatoma

facial nerve palsy as a presenting complaint with features of
acute mastoiditis is rare.
Hidaka et al. reported a similar case where the patient presented
with postauricular swelling and pain as the presenting
complaint. Along with mastoid abscess, the patient also had an
isolated mastoid CC with the erosion of posterior fossa bony
plate and sigmoid plate similar to the findings in our patient.[8]
As in most patients of CC, mastoid was well pneumatized in
our patient. Pnematization is not affected because inflammation
is absent in CC. Whereas, in acquired cholesteatoma
inflammation and pathologic changes of mucosa are more
evident leading to sclerotic mastoid. Kojima et al. analyzed
temporal bone pneumatization in 53 patients of CC, 27 (50.9%)
and 16  (30.2%) had very good and good pneumatization,
respectively. They also found that otorrhea or otalgia was an
initial symptom in 17.5% of the patients.[9]
Potsic et  al. described a staging system for CC, which are
defined as follows:
• Stage I – Single quadrant: No ossicular involvement or
mastoid extension
• Stage II – Multiple quadrants: No ossicular involvement
or mastoid extension
• Stage III – Ossicular involvement: It includes erosion of
ossicles and surgical removal for eradication of disease;
no mastoid extension
• Stage IV  –  Mastoid extension  (regardless of findings
elsewhere).
Their study showed a strong association between stage and
residual disease, ranging from a 13% risk in Stage I to 67%
in Stage IV.[10] Our patient belongs to Stage IV according to
their staging system, emphasizing the need for a regular long
time follow‑up.
Warren et al. presented a case series of nine patients who they
divided into mastoid‑only cholesteatoma and mastoid and
middle ear cholesteatoma groups. The former group tends to
be clinically silent as the lesion does not impinge upon any
critical neurovascular structures and shows a higher incidence
of dural exposure, facial nerve exposure, and auditory canal
erosion.[11] Our patient fits into the mastoid‑only group and had
both dural exposure and facial nerve exposure.
Giannuzzi et al., in their study, concluded that many cases,
which were reported as mastoid cholesteatoma in the past,
were not true mastoid cholesteatoma and thereby detailed the
following characteristics of mastoid CC which differentiated
it from middle ear CC.
1. Presenting complaint – Neck and temporal region pain
2. Age at the presentation – Adulthood
3. Findings at otoscopy – No abnormalities
4. Findings of imaging restricted to the mastoid process.
Based on their literature review, only 12 of the 20 reported
cases were true mastoid cholesteatomas. Our case fits the
description of true mastoid cholesteatoma in all aspects defined
by Giannuzzi et al.[12]
Our literature review on patients of CC who presented as
complicated otitis media is summarized in Table 1. We were
able to find four such cases in the literature; however, one was
deliberately omitted, as it was not in English.
Informed consent
Informed consent was obtained from the patient included in
the study.
Declaration of patient consent
The authors certify that they have obtained all appropriate
patient consent forms. In the form, the patient has given his
consent for his images and other clinical information to be

Table 1: Reported cases of congenital cholesteatoma presented as complicated otitis media

Authors Year Age (years) Presentation Hearing loss Imaging Facial nerve status Surgery
Hidaka et al.[8] 2010 65 Postauricular Mixed Postauricular abscess Intact Initially, abscess
swelling and pain hearing loss with a cystic lesion in drainage followed
mastoid causing erosion by intact canal wall
of postfossa bone and mastoidectomy
sigmoid plate
Santhi et al.[13] 2012 5 Progressive Failed Soft‑tissue mass in middle NA Modified radical
supra‑auricular auditory brain ear cleft and ossicular mastoidectomy
swelling stem response destruction along with
and Type B Luc’s abscess
tympanogram
Tabook et al.[14] 2015 21 Right‑side The tuning NA NA Intact canal wall
mastoid pain and fork test was mastoidectomy
postauricular normal
swelling
Present case 50 Ear pain and tragal Mixed Large cystic lesion Grade V facial Canal wall down
tenderness with hearing loss confined to mastoid paresis mastoidectomy
sudden‑onset facial causing erosion of with Type III
paresis with no ear posterior fossa bone, tympanoplasty and
complaints in past sigmoid plate, and closely cavity obliteration
abutting cerebellum
NA: Not applicable

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Ramamourthy, et al.: Congenital mastoid cholesteatoma
reported in the journal. The patient understands that name
and initials will not be published, and due efforts will be made
to conceal the identity, but anonymity cannot be guaranteed.
Conclusion
• CC of mastoid origin is very rare and has specific
characteristics like late age of presentation, destruction
of adjoining structures like posterior fossa bone, facial
nerve canal, sigmoid plate, tegmen, and intact ossicular
chain
• CC presenting as mastoiditis, though a rare phenomenon,
has to be kept in mind as these cases pose special surgical
challenges to the surgeon
• Imaging of the temporal region helps the surgeon (a) to
arrive at the diagnosis preoperatively, (b) to see the extent
of disease, and (c) to do surgical planning.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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