External Auditory Canal Cholesteatoma and Keratosis Obturans - The Role of Imagin

External auditory canal cholesteatoma and keratosis obturans: The role of imaging in preventing facial nerve injury
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December 15, 2011
External auditory canal cholesteatoma and keratosis obturans:
The role of imaging in preventing facial nerve injury
By Edward D. McCoul, MD, MPH and Matthew B. Hanson, MD
Introduction
External auditory canal cholesteatoma (EACC) is a rare entity; its estimated prevalence is 1 in 1,000 otologic patients.1 Most reported cases
have arisen spontaneously without any clear etiology, and others have occurred following surgery or trauma to the auditory canal.2,3 Ear canal
stenosis or obstruction has also been described as a causative factor.3 Spontaneous EACC has been attributed to the loss of normal migration
in the aging epithelium of the canal wall, particularly along the inferior canal wall.3 A change in cellular proliferation in the external auditory
canal as a result of an accumulation of keratin debris has also been hypothesized; such a finding may be related to local tissue hypoxia.4
Previous presentation: The information in this article has been updated from its original presentation as a poster at the Eastern Section Meeting
of the Triological Society; J an. 23-25, 2009; Boston.
Otoscopic examination of a patient with an EACC typically reveals an ulceration of the canal wall or a defect in the bony canal lateral to the
tympanic annulus.2,5 The most common sites are the posterior and inferior walls, although some reports have indicated involvement of the
anterior wall and even circumferential canal involvement.2,5,6 While some authors2 have suggested probing the area of erosion to help
determine the depth of the cholesteatoma sac, we believe that such a practice may predispose to injury of the underlying structures.
Nevertheless, the true extent of EACC may not be apparent on clinical examination alone, which means that imaging studies are essential in
establishing a diagnosis and avoiding iatrogenic complications. Facial nerve paresis has been reported as a rare manifestation of a very large
EACC.7
A more frequently encountered entity that leads to ear canal occlusion is keratosis obturans (KO). The distinction between EACC and KO has
been based largely on clinical appearance, as summarized by Piepergerdes et al2 and Naiberg et al.8 Recent reviews of the literature have
failed to find any consistent clinical symptoms to distinguish these two entities.6 This suggests that a definitive diagnosis may not be possible.
However, the erosive tendency of EACC means that it is important to make the distinction in order to ensure that vital structures are not placed
at risk during examination and management.
In this article, we describe our findings in a series of patients with EACC, and we contrast them with findings in a series of patients with KO.
Particular attention has been paid to the appearance of the external auditory canal on radiographic imaging.
Patients and methods
After obtaining Institutional Review Board approval, we conducted a retrospective search for diagnosed cases of EACC from among the records
of 3,874 patients who had presented to our academic otology practice from J uly 2005 through J une 2008. We found 6 cases; they had occurred
in 1 male and 5 females, aged 7 to 84 years (mean: 49.5). Then we identified 6 cases of KO that had been diagnosed during the same period,
and we reviewed those records for comparison purposes. The KO group consisted of 3 males and 3 females, aged 5 to 76 years (mean: 49.5).
In addition to demographic data, we compiled information on symptoms and physical examination findings for each patient. We also reviewed
computed tomography (CT) scans when available to look for evidence of bony erosion and to determine the proximity of disease to vital
structures. Both EACC and KO were defined as a localized collection of keratinaceous debris in the medial external auditory canal. The
diagnosis of EACC was favored by the presence of a localized disruption of canal wall integrity and an underlying bony cavity noted on physical
examination. The diagnosis of EACC had been confirmed by evidence of bony canal erosion on CT.
Results
EACC group. The EACC group consisted of a 7-year-old boy and 5 women aged 35 to 84 years (mean: 58.0) (table 1). Two patients with
EACC had presented with significant otalgia, 2 with otorrhea, and 3 with aural pressure. No patient complained of itching, and only 1 reported
tinnitus.
Table 1. Characteristics of patients with external auditory canal cholesteatoma
Pt Age/sex Side Otalgia Otorrhea Pressure HL Itching Tinnitus
FN
weak
Audiometry
CT
findings
Other
Key: HL = self-reported hearing loss;
FN = facial nerve; CHL = conductive
hearing loss; SNHL = sensorineural
hearing loss; MHL = mixed hearing
loss.
E1 7/M Left Yes No Yes Yes No No No
Mild to
moderate
CHL in
right ear
Inferior
and
posterior
erosion;
FN
exposed
Granulation
E2 37/F Right No No No No No No No Normal
Posterior
erosion
Right ear
polyp
removed
previously
E3 55/F Right No No Yes No No No No Normal
Superior
and
posterior
erosion;
FN
exposed

E4 79/F Right No No No Yes No No No
Profound
SNHL in
left ear;
MHL in
right ear
Inferior
and
posterior
erosion

E5 35/F Right Yes Yes Yes Yes No No No Normal
Inferior
and
posterior
erosion;
FN
exposed

E6 84/F Left No Yes No Yes No Yes No
Mild CHL
in left ear
Inferior
erosion;
FN
exposed
Diabetes;
granulation
Subjective hearing loss had been reported by 4 patients. Audiometry revealed a conductive hearing loss in 2 of these patients (mild in 1 and
mild to moderate in the other), a profound left-sided sensorineural hearing loss and a mixed right-sided hearing loss in 1 patient, and normal
hearing in the remaining patient. The results of audiometry in the 2 patients who did not report a subjective hearing loss were within normal
limits.
Facial nerve function had been intact in all 6 patients. One patient, a 37-year-old woman, had had an ear canal polyp removed 15 years earlier
when she was a young adult; afterward, she experienced a long intervening period when she had been asymptomatic. On physical examination,
all patients had had what appeared to be cerumen in the external auditory canal. Gentle removal of the material revealed a keratin mass in an
irregular crater that extended various distances into the bony canal wall. No cases of acute otitis externa or tympanic membrane perforation
were noted.
Radiologic findings included erosion of the bony canal wall in all 6 patients. In 4 of these cases, significant erosion was seen in the vicinity of
the mastoid segment of the facial nerve. The CT of the 7-year-old boy showed an irregular bony expansion of the inferior and posterior walls of
the left external auditory canal with thinning of the bone over the mastoid segment of the fallopian canal (figure 1). Another patient, a 35-year-
old woman, had an irregular erosion of the right inferior and posterior external auditory canal walls, with a segment of facial nerve completely
dehiscent adjacent to the cholesteatoma (figure 2). CT findings in the other EACC patients included bony erosion of the posterior, inferior, and
superior canal walls and deep pockets of erosion abutting the facial nerve. None of the 6 cases involved extension into the middle ear space or
erosion of the ossicular chain.
Figure 1. A 7-year -old boy with EACC. A: Axial CTs demonstrate the extensive erosion of
the posterior bony canal wall in the left ear by the EACC. (Microscopic debridement had
already been performed in the office.) The erosion has an irregular appearance, and the
bone overlying the facial nerve (right, thick arrow) is markedly thinned. The right facial
nerve (left, thin arrow) is shown for comparison. B: Coronal CT shows the marked
erosion of the inferior wall of the left external auditory canal (arrow).
Figure 2. A 35-year -old woman with EACC. A: Axial CTs show the irregular bony erosion
of the inferior and posterior walls of the right external auditory canal and the noncritical
thinning over the right facial nerve (left, thick arrow). The left facial nerve (right, thin
arrow) is shown for comparison. B: Following debridement of the EACC, otoscopic view
of the right ear shows the deep erosion of the posterior and inferior canal walls and the
exposed facial nerve (F = facial nerve; P = posterior external auditory canal wall; T =
tympanic membrane; M = malleus).
All 6 patients were managed with microscopic de-bridement in the office setting without the need for local anesthesia. Disease recurrence was
negligible in all cases, and surgical management under general anesthesia was not required. No case of iatrogenic facial nerve injury occurred.
KO group. The KO group consisted of a 5 year-old-boy and 5 adults: 2 men and 3 women aged 21 to 76 years (mean: 58.4) (table 2). Otalgia
was uniformly present, and aural pressure or fullness was present in 5 patients. The presence of otorrhea, itching, and tinnitus was variable.
Table 2. Characteristics of patients with keratosis obturans
FN
weak
Audiometry
CT
findings
Other
K1 76/F Right Yes Yes Yes Yes No Yes No
Moderate
to severe
CHL in
right ear
N/A
Diabetes;
granular
myringitis
K2 67/M Both Yes No Yes No No No No
Mild to
moderate
SNHL in
both ears
N/A
K3 21/F Both Yes No Yes No No No No Normal N/A
Medial canal
expansion;
reaccumulation
K4 55/F Both Yes No Yes No Yes Yes No Normal N/A
Dermatophytid
reaction;
diabetes
K5 5/M Left Yes Yes No Yes No No No Normal
EAC
opacity
AOM w/TM
perforation
K6 73/M Left Yes Yes Yes Yes Yes Yes No
Mildly
sloping
MHL in left
ear
EAC
opacity
Diabetes; TMJ
osteomyelitis
Key: HL = self-reported hearing
loss; FN = facial nerve; N/A = not
applicable; CHL = conductive
hearing loss; SNHL = sensorineural
hearing loss;
EAC = external auditory canal;
AOM = acute otitis media; TM =
tympanic membrane; MHL = mixed
hearing loss; TMJ =
temporomandibular joint.
Since KO presents as a complete keratin plug filling the ear canal, all 6 patients initially reported some degree of hearing loss. Three of them
experienced a complete improvement upon the removal of the keratin plug, but the other 3 reported continued subjective hearing loss, which
was confirmed by audiometry in each; 1 patient had a moderate to severe unilateral conductive hearing loss, 1 had a mild to moderate bilateral
sensorineural hearing loss, and 1 had a mildly sloping, mixed unilateral hearing loss. Audiometric findings in the other 3 patients were within
normal limits. All 3 cases of objective hearing loss were believed to be unrelated to the external ear canal disease.
No patient exhibited any facial nerve weakness, and only 1 patient was found to have an expansion of the bony canal on physical examination;
this expansion was entirely circumferential and was not localized. There was 1 case of granular myringitis, 1 id (dermatophytid) reaction, and 1
case of acute otitis media with a tympanic membrane perforation.
CT was obtained in 2 of the 6 cases. Both scans demonstrated a soft-tissue opacity in the external auditory canal without bony erosion (figure
3).
Figure 3. A 5-year -old boy with KO. Axial CTs demonstrate the smooth bony expansion
of the external auditory canal. No significant erosion is seen over either facial nerve
canal (arrows).
Two patients experienced a rapid reaccumulation of keratin that required follow-up every 1 to 3 months. The other 4 patients were easily
managed with office cleaning every 6 months.
Several cases in the KO group exhibited some unusual clinical features:
A 76-year-old woman with diabetes mellitus was found to have KO incidentally after she presented with bleeding from the ear canal. An
underlying granular myringitis was found, and she was treated with a topical antibiotic cream and acetic acid solution.
A 55-year-old woman with diabetes and multiple allergies presented with a history of tinea pedis that had arisen while she had been
taking systemic steroids for the treatment of bunions. During that time, she noticed a worsening of her long-standing aural fullness and
itching in both ears, as well as scaling of the scalp. She sought an otologic evaluation and was found to have dense KO in both ears
that reaccumulated rapidly and was resistant to numerous topical preparations. She was diagnosed with a dermatophytid reaction that
ultimately required systemic antifungal therapy to control. Treatment of the distal dermatophyte infection resulted in resolution of the ear
canal disease.
A 73-year-old man with diabetes was being treated for acute otitis externa when he was diagnosed with KO of the left ear. He returned
several months later with increasing pain over the pretragal area that did not improve during treatment with an ototopical preparation. CT
revealed a narrowing of the temporomandibular joint (TMJ ), and subsequent magnetic resonance imaging demonstrated a dysplastic
mandibular condyle suggestive of osteomyelitis. It was felt that the osteomyelitis began in the temporal bone and then spread to the
TMJ and mandible. Treatment with an oral fluoroquinolone was instituted for several weeks, and the patient improved. Follow-up
imaging confirmed resolution of the infection.
Discussion
Cholesteatoma is an uncommon inflammatory condition that typically affects the middle ear and mastoid air-cell system. It appears
histologically as a cystic structure lined with keratinizing stratified squamous epithelium. Cholesteatoma of the external auditory canal was first
described by Altmann and Waltner9 in 1943, and it was later distinguished from KO in a series of 6 patients by Piepergerdes et al2 in 1980.
Presentation of EACC. The classic clinical presentation of EACC includes otorrhea and chronic dull pain.2 The debris accumulation is believed
to be copious enough to cause hearing loss only in rare cases.2 This was not the case in our own series, but it was felt that the audiometric
abnormalities found in 3 of the 6 EACC cases were unrelated to the canal disease.
In the largest series of EACC cases published to date, Owen et al analyzed 48 consecutively presenting patients over a 26-year period and
found that more than half of them had no identifiable inciting event.6 The presence of otalgia and otorrhea was variable, and aural occlusion
and fullness occurred only occasionally. Moreover, 24% of patients were asymptomatic. These findings underscore the need for additional
diagnostic criteria when EACC is suspected.
In 2005, Naim et al4 proposed a four-stage classification system for EACC based on its histopathologic appearance. Based on this system, all
of our patients would have had stage III disease, and surgical excision would be recommended. However, surgery was not required for any of
our patients for disease control. The Naim staging system, with its reliance on histologic findings, is of uncertain value for clinical decision
making in EACC.
Presentation of KO. KO is closely related to EACC; indeed, these two entities were once believed to represent the same disease process.2
KO generally occurs in younger patients, it is often bilateral, and it has a reported association with bronchiectasis and sinusitis.2
KO is described as an occluding plug of keratin without significant otorrhea and with a thickened but intact tympanic membrane.2 The original
description of KO was published by Toynbee10 in 1850, and the term keratosis obturans was coined by Wreden11 in 1874. The pathogenesis
of KO has been most often described as a chronic desquamative process of the ear canal. However, the initiating event remains unknown.2
Some authors2 have associated KO with bronchiectasis by way of a sympathetic stimulation of the ceruminous glands and hyperemia; we
found no known pulmonary comorbidity in any of the patients in our study. Corbridge et al documented faulty epithelial migration patterns of the
external auditory canal epithelium in patients with KO.12
CT imaging of KO demonstrates a soft-tissue plug in the external auditory canal, often with generalized canal widening.8,13 The distinguishing
feature is the absence of focal bone erosion without formation of bony spicules. Because KO produces a pattern of bony expansion rather than
erosion, the occurrence of invasive complications is unlikely.
Disease course. Extension of EACC into adjacent structures has been described.5,13 The middle ear space may be involved, typically by
disruption of the tympanic annulus. Invasion into the mastoid air-cell system represents a more complicated entity that poses a greater risk to
the facial nerve. In either case, more extensive surgical debridement is required. As with other cholesteatomas, EACC may result in dehiscence
of the tegmen tympani or tegmen mastoideum and a subsequent need for surgical reconstruction to prevent complications such as
cerebrospinal fluid otorrhea and meningitis. Semicircular canal erosion may also be possible, although this has not been reported with EACC.
Treatment. The treatment of cholesteatoma of the middle ear and mastoid is focused on the complete surgical removal of all keratin-bearing
tissue from the middle ear space. In contrast, in both EACC and KO that is not advised because this is the normal epithelium in this location.
Therefore, treatment of these disorders is focused on (1) removing keratin debris to provide an open, clean canal and (2) preventing extension
of the disease into other areas, such as the middle ear. Usually this will require nothing more than frequent cleaning and debridement of keratin
debris in the outpatient setting.2
If chronic pain is present or if the lesion is more extensive, surgical management may become necessary. Surgical options include debridement
via a postauricular approach, canaloplasty, and split-thickness skin grafting to cover the canal defect. Erosion into the mastoid air cells may
necessitate a mastoidectomy.
In an effort to guide therapy, Holt3 described three stages of EACC and their recommended treatments:
For superficial, localized, saucerized defects, frequent cleaning with debridement as an office procedure is sufficient.
For deeper localized pockets of external auditory canal erosion, canaloplasty is recommended.
Lesions that extend into the mastoid require mastoid surgery.
Since both EACC and KO may appear at presentation as nothing more than a dense wax impaction, the removal of these lesions should
always be done in a careful, painless manner. Attempts to remove dense cerumen impaction should always be attempted under microscopic
visualization. Instrumentation should begin laterally, and the physician must take great care to avoid tugging or pulling if debris is not easily
extracted. Irrigation should be avoided because water might introduce bacteria into an epithelial pocket, resulting in a wet, infected
cholesteatoma that might require additional medical or surgical management.
An important guiding factor in the treatment of EACC and KO is the presence of pain. Removal of a large EACC may be precluded by severe
pain, which may be a sign of deeper penetration of the disease process. In this scenario, no further office cleaning should be attempted, and
the patient should be sent for CT to better define the depth of invasion and the extent of bony erosion.
If the use of instrumentation does not produce excessive pain, complete removal may be possible in the office. Once the ear canal is
adequately cleared, any expansion of the canal wall should be evaluated further with CT. It is important to refrain from anesthetizing the ear to
facilitate patient comfort during cleaning because this may mask an important sign of deep tissue invasion by an EACC.
If an EACC or KO is large or extends into areas inaccessible through the meatus, a surgical meatoplasty should be considered. This will smooth
the contour of the canal wall and prevent keratin reaccumulation or progression.
In the present series, all patients had been treated with serial microscopic debridement in the office without the need for debridement under
anesthesia. Reaccumulation was minimal and amenable to further office debridement, suggesting a potentially effective alternative to traditional
surgical management. As previously noted, CT is an invaluable resource in such cases to facilitate safe debridement and to avoid iatrogenic
injury.
Special attention must be paid when CT demonstrates extensive dehiscence of the facial nerve in its course through the fallopian canal.
Although such a circumstance was not seen in our series, we believe that such a case warrants debridement in the operating room. Surgical
debridement allows for identification and safe dissection of the facial nerve in an uninvolved segment. Additional study is needed to support this
recommendation.
In conclusion, EACC is an uncommon entity that may be mistaken for KO, cerumen impaction, or another disease process. Because it is
typically indolent and longstanding, significant bony erosion may be present at the time of diagnosis. Exposure of vital structures, including the
facial nerve, must be taken into consideration when initial cleaning of the ear canal is attempted. KO generally produces a distinctive smooth
erosion of the medial canal, which poses minimal danger to deeper structures. Pain may be an important indicator of deep infiltration, and it
should prompt acquisition of CT prior to complete debridement. EACC may be managed effectively in the outpatient setting, with surgery
reserved for appropriately selected high-risk cases.
From ENT and Allergy Associates, LLP (Westchester County), Tuckahoe, N.Y. (Dr. McCoul); and the Department of Otolaryngology, SUNY
Downstate Medical Center, Brooklyn, N.Y., and the Department of Otolaryngology, Long Island College Hospital, Brooklyn (Dr. Hanson). The
study described in this article was performed at the SUNY Downstate Medical Center. Corresponding author: Matthew B. Hanson, MD,
Department of Otolaryngology, SUNY Downstate Medical Center, 450 Clarkson Ave., Box 126, Brooklyn, NY 11203. Email:
matthew.hanson@downstate.edu
References
1. Anthony PF, Anthony WP. Surgical treatment of external auditory canal cholesteatoma. Laryngoscope 1982; 92 (1): 70-5.
2. Piepergerdes MC, Kramer BM, Behnke EE. Keratosis obturans and external auditory canal cholesteatoma. Laryngoscope 1980; 90 (3):
383-91.
3. Holt J J . Ear canal cholesteatoma. Laryngoscope 1992; 102 (6): 608-13.
4. Naim R, Linthicum F J r., Shen T, et al. Classification of the external auditory canal cholesteatoma. Laryngoscope 2005; 115 (3): 455-60.
5. Heilbrun ME, Salzman KL, Glastonbury CM, et al. External auditory canal cholesteatoma: Clinical and imaging spectrum. AJ NR Am J
Neuroradiol 2003; 24 (4): 751-6.
6. Owen HH, Rosborg J , Gaihede M. Cholesteatoma of the external ear canal: Etiological factors, symptoms and clinical findings in a
series of 48 cases. BMC Ear Nose Throat Disord 2006; 6:16.
7. Sapi T, U&gbreve;ur G, Karavus A, et al. Giant cholesteatoma of the external auditory canal. Ann Otol Rhinol Laryngol 1997; 106 (6):
471-3.
8. Naiberg J , Berger G, Hawke M. The pathologic features of keratosis obturans and cholesteatoma of the external auditory canal. Arch
Otolaryngol 1984; 110 (10): 690-3.
9. Altmann F, Waltner J G. Cholesteatoma of the external auditory meatus. Arch Otolaryngol 1943; 38 (3): 236-40.
10. Toynbee J . Specimen of molluscum contagiosum developed in the external auditory meatus. Lond Med Gazette 1850; 46:811.
11. Wreden R. Keratosis obturans. Arch Ophthalmol Otol 1874; 4:261.
12. Corbridge RJ , Michaels L, Wright T. Epithelial migration in keratosis obturans. Am J Otolaryngol 1996; 17 (6): 411-14.
13. Remley KB, Swartz J D, Harnsberger HR. The external auditory canal. In: Swartz J D, Harnsberger HR, eds. Imaging of the Temporal
Bone. 3rd ed. New York:Thieme; 1998:16-46.
Ear Nose Throat J . 2011 December;90(12):E1
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REPRINTS
December 15, 2011 by Edward D. McCoul, MD, MPH and Matthew B. Hanson, MD
External auditory canal cholesteatoma and keratosis
obturans: The role of imaging in preventing facial
nerve injury
Abstract
We conducted a retrospective study to compare the clinical characteristics of external auditory
canal cholesteatoma (EACC) with those of a similar entity, keratosis obturans (KO). We also
sought to identify those aspects of each disease that may lead to complications. We identified 6
patients in each group. Imaging studies were reviewed for evidence of bony erosion and the
proximity of disease to vital structures. All 6 patients in the EACC group had their diagnosis
confirmed by computed tomography (CT), which demonstrated widening of the bony external
auditory canal; 4 of these patients had critical erosion of bone adjacent to the facial nerve. Of the
6 patients with KO, only 2 had undergone CT, and neither exhibited any significant bony erosion
or expansion; 1 of them developed osteomyelitis of the temporal bone and adjacent
temporomandibular joint. Another patient manifested KO as part of a dermatophytid reaction. The
essential component of treatment in all cases of EACC was microscopic debridement of the ear
canal. We conclude that EACC may produce significant erosion of bone with exposure of vital
structures, including the facial nerve. Because of the clinical similarity of EACC to KO,
misdiagnosis is possible. Temporal bone imaging should be obtained prior to attempts at
debridement of suspected EACC. Increased awareness of these uncommon conditions is
warranted to prompt appropriate investigation and prevent iatrogenic complications such as facial
nerve injury.
Introduction
External auditory canal cholesteatoma (EACC) is a rare entity; its estimated prevalence is 1 in 1,000 otologic
patients. Most reported cases have arisen spontaneously without any clear etiology, and others have
occurred following surgery or trauma to the auditory canal. Ear canal stenosis or obstruction has also
been described as a causative factor. Spontaneous EACC has been attributed to the loss of normal
migration in the aging epithelium of the canal wall, particularly along the inferior canal wall. A change in
cellular proliferation in the external auditory canal as a result of an accumulation of keratin debris has also
been hypothesized; such a finding may be related to local tissue hypoxia.
Previous presentation: The information in this article has been updated from its original presentation as a
poster at the Eastern Section Meeting of the Triological Society; J an. 23-25, 2009; Boston.
Otoscopic examination of a patient with an EACC typically reveals an ulceration of the canal wall or a defect
in the bony canal lateral to the tympanic annulus. The most common sites are the posterior and inferior
walls, although some reports have indicated involvement of the anterior wall and even circumferential canal
involvement. While some authors have suggested probing the area of erosion to help determine the
depth of the cholesteatoma sac, we believe that such a practice may predispose to injury of the underlying
structures. Nevertheless, the true extent of EACC may not be apparent on clinical examination alone, which
1
2,3
3
3
4
2,5
2,5,6 2
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means that imaging studies are essential in establishing a diagnosis and avoiding iatrogenic complications.
Facial nerve paresis has been reported as a rare manifestation of a very large EACC.
A more frequently encountered entity that leads to ear canal occlusion is keratosis obturans (KO). The
distinction between EACC and KO has been based largely on clinical appearance, as summarized by
Piepergerdes et al and Naiberg et al. Recent reviews of the literature have failed to find any consistent
clinical symptoms to distinguish these two entities. This suggests that a definitive diagnosis may not be
possible. However, the erosive tendency of EACC means that it is important to make the distinction in order
to ensure that vital structures are not placed at risk during examination and management.
In this article, we describe our findings in a series of patients with EACC, and we contrast them with findings
in a series of patients with KO. Particular attention has been paid to the appearance of the external auditory
canal on radiographic imaging.
Patients and methods
After obtaining Institutional Review Board approval, we conducted a retrospective search for diagnosed
cases of EACC from among the records of 3,874 patients who had presented to our academic otology
practice from J uly 2005 through J une 2008. We found 6 cases; they had occurred in 1 male and 5 females,
aged 7 to 84 years (mean: 49.5). Then we identified 6 cases of KO that had been diagnosed during the
same period, and we reviewed those records for comparison purposes. The KO group consisted of 3 males
and 3 females, aged 5 to 76 years (mean: 49.5).
In addition to demographic data, we compiled information on symptoms and physical examination findings
for each patient. We also reviewed computed tomography (CT) scans when available to look for evidence of
bony erosion and to determine the proximity of disease to vital structures. Both EACC and KO were defined
as a localized collection of keratinaceous debris in the medial external auditory canal. The diagnosis of
EACC was favored by the presence of a localized disruption of canal wall integrity and an underlying bony
cavity noted on physical examination. The diagnosis of EACC had been confirmed by evidence of bony
canal erosion on CT.
Results
EACC group. The EACC group consisted of a 7-year-old boy and 5 women aged 35 to 84 years (mean:
58.0) (table 1). Two patients with EACC had presented with significant otalgia, 2 with otorrhea, and 3 with
aural pressure. No patient complained of itching, and only 1 reported tinnitus.
Table 1. Characteristics of patients with external auditory canal
cholesteatoma
FN
weak
Audiometry
C
f
E1 7/M Left Yes No Yes Yes No No No
Mild to
moderate
CHL in
right ear
I
a
p
e
F
e
E2 37/F Right No No No No No No No Normal
P
e
E3 55/F Right No No Yes No No No No Normal
S
a
p
e
F
e
7
2 8
6
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Figure 1. A 7-year-old boy with EACC.
A: Axial CTs demonstrate the
Key: HL =
self-reported
hearing loss;
FN = facial
nerve; CHL =
conductive
hearing loss;
SNHL =
sensorineural
hearing loss;
MHL = mixed
hearing loss.
E4 79/F Right No No No Yes No No No
Profound
SNHL in
left ear;
MHL in
right ear
I
a
p
e
E5 35/F Right Yes Yes Yes Yes No No No Normal
I
a
p
e
F
e
E6 84/F Left No Yes No Yes No Yes No
Mild CHL
in left ear
I
e
F
e
Subjective hearing loss had been reported by 4 patients. Audiometry revealed a conductive hearing loss in 2
of these patients (mild in 1 and mild to moderate in the other), a profound left-sided sensorineural hearing
loss and a mixed right-sided hearing loss in 1 patient, and normal hearing in the remaining patient. The
results of audiometry in the 2 patients who did not report a subjective hearing loss were within normal limits.
Facial nerve function had been intact in all 6 patients. One patient, a 37-year-old woman, had had an ear
canal polyp removed 15 years earlier when she was a young adult; afterward, she experienced a long
intervening period when she had been asymptomatic. On physical examination, all patients had had what
appeared to be cerumen in the external auditory canal. Gentle removal of the material revealed a keratin
mass in an irregular crater that extended various distances into the bony canal wall. No cases of acute otitis
externa or tympanic membrane perforation were noted.
Radiologic findings included erosion of the bony canal wall in all 6 patients. In 4 of these cases, significant
erosion was seen in the vicinity of the mastoid segment of the facial nerve. The CT of the 7-year-old boy
showed an irregular bony expansion of the inferior and posterior walls of the left external auditory canal with
thinning of the bone over the mastoid segment of the fallopian canal (figure 1). Another patient, a 35-year-
old woman, had an irregular erosion of the right inferior and posterior external auditory canal walls, with a
segment of facial nerve completely dehiscent adjacent to the cholesteatoma (figure 2). CT findings in the
other EACC patients included bony erosion of the posterior, inferior, and superior canal walls and deep
pockets of erosion abutting the facial nerve. None of the 6 cases involved extension into the middle ear
space or erosion of the ossicular chain.
All 6 patients were managed with
microscopic de-bridement in the office
extensive erosion of the posterior
bony canal wall in the left ear by the
EACC. (Microscopic debridement had
already been performed in the office.)
The erosion has an irregular
appearance, and the bone overlying
the facial nerve (right, thick arrow) is
markedly thinned. The right facial
nerve (left, thin arrow) is shown for
comparison. B: Coronal CT shows the
marked erosion of the inferior wall of
the left external auditory canal (arrow).
Figure 2. A 35-year-old woman with
EACC. A: Axial CTs show the irregular
bony erosion of the inferior and
posterior walls of the right external
auditory canal and the noncritical
thinning over the right facial nerve
(left, thick arrow). The left facial nerve
(right, thin arrow) is shown for
comparison. B: Following debridement
of the EACC, otoscopic view of the
right ear shows the deep erosion of
the posterior and inferior canal walls
and the exposed facial nerve (F =
facial nerve; P = posterior external
auditory canal wall; T = tympanic
membrane; M = malleus).
setting without the need for local anesthesia.
Disease recurrence was negligible in all
cases, and surgical management under
general anesthesia was not required. No
case of iatrogenic facial nerve injury
occurred.
KO group. The KO group consisted of a 5
year-old-boy and 5 adults: 2 men and 3
women aged 21 to 76 years (mean: 58.4)
(table 2). Otalgia was uniformly present, and
aural pressure or fullness was present in 5
patients. The presence of otorrhea, itching,
and tinnitus was variable.
Table 2. Characteristics of
patients with keratosis
obturans
Pt Age/sex Side Otalgia
Key: HL = self-
reported hearing
loss; FN = facial
nerve; N/A = not
applicable; CHL =
conductive hearing
K1 76/F Right Yes
K2 67/M Both Yes
K3 21/F Both Yes
K4 55/F Both Yes
K5 5/M Left Yes
K6 73/M Left Yes
Figure 3. A 5-year-old boy with KO.
Axial CTs demonstrate the smooth
bony expansion of the external
auditory canal. No significant erosion
is seen over either facial nerve canal
(arrows).
loss; SNHL =
sensorineural
hearing loss;
EAC = external
auditory canal;
AOM = acute otitis
media; TM =
tympanic
membrane; MHL =
mixed hearing
loss; TMJ =
temporomandibular
joint.
Since KO presents as a complete keratin plug filling the ear canal, all 6 patients initially reported some
degree of hearing loss. Three of them experienced a complete improvement upon the removal of the keratin
plug, but the other 3 reported continued subjective hearing loss, which was confirmed by audiometry in
each; 1 patient had a moderate to severe unilateral conductive hearing loss, 1 had a mild to moderate
bilateral sensorineural hearing loss, and 1 had a mildly sloping, mixed unilateral hearing loss. Audiometric
findings in the other 3 patients were within normal limits. All 3 cases of objective hearing loss were believed
to be unrelated to the external ear canal disease.
No patient exhibited any facial nerve weakness, and only 1 patient was found to have an expansion of the
bony canal on physical examination; this expansion was entirely circumferential and was not localized.
There was 1 case of granular myringitis, 1 id (dermatophytid) reaction, and 1 case of acute otitis media with
a tympanic membrane perforation.
CT was obtained in 2 of the 6 cases. Both scans demonstrated a soft-tissue opacity in the external auditory
canal without bony erosion (figure 3).
Two patients experienced a rapid
reaccumulation of keratin that required
follow-up every 1 to 3 months. The other 4
patients were easily managed with office
cleaning every 6 months.
Several cases in the KO group exhibited
some unusual clinical features:
A 76-year-old woman with diabetes mellitus
was found to have KO incidentally after she
presented with bleeding from the ear canal.
An underlying granular myringitis was found,
and she was treated with a topical antibiotic
cream and acetic acid solution.
A 55-year-old woman with diabetes and
multiple allergies presented with a history of
tinea pedis that had arisen while she had
been taking systemic steroids for the treatment of bunions. During that time, she noticed a worsening of
her long-standing aural fullness and itching in both ears, as well as scaling of the scalp. She sought an
otologic evaluation and was found to have dense KO in both ears that reaccumulated rapidly and was
resistant to numerous topical preparations. She was diagnosed with a dermatophytid reaction that
ultimately required systemic antifungal therapy to control. Treatment of the distal dermatophyte infection
resulted in resolution of the ear canal disease.
A 73-year-old man with diabetes was being treated for acute otitis externa when he was diagnosed with
KO of the left ear. He returned several months later with increasing pain over the pretragal area that did
not improve during treatment with an ototopical preparation. CT revealed a narrowing of the
temporomandibular joint (TMJ ), and subsequent magnetic resonance imaging demonstrated a dysplastic
mandibular condyle suggestive of osteomyelitis. It was felt that the osteomyelitis began in the temporal
bone and then spread to the TMJ and mandible. Treatment with an oral fluoroquinolone was instituted
for several weeks, and the patient improved. Follow-up imaging confirmed resolution of the infection.
Discussion
Cholesteatoma is an uncommon inflammatory condition that typically affects the middle ear and mastoid air-
cell system. It appears histologically as a cystic structure lined with keratinizing stratified squamous
epithelium. Cholesteatoma of the external auditory canal was first described by Altmann and Waltner in
1943, and it was later distinguished from KO in a series of 6 patients by Piepergerdes et al in 1980.
Presentation of EACC. The classic clinical presentation of EACC includes otorrhea and chronic dull pain.
The debris accumulation is believed to be copious enough to cause hearing loss only in rare cases. This
was not the case in our own series, but it was felt that the audiometric abnormalities found in 3 of the 6
EACC cases were unrelated to the canal disease.
In the largest series of EACC cases published to date, Owen et al analyzed 48 consecutively presenting
patients over a 26-year period and found that more than half of them had no identifiable inciting event.
The presence of otalgia and otorrhea was variable, and aural occlusion and fullness occurred only
occasionally. Moreover, 24% of patients were asymptomatic. These findings underscore the need for
additional diagnostic criteria when EACC is suspected.
In 2005, Naim et al proposed a four-stage classification system for EACC based on its histopathologic
appearance. Based on this system, all of our patients would have had stage III disease, and surgical
excision would be recommended. However, surgery was not required for any of our patients for disease
control. The Naim staging system, with its reliance on histologic findings, is of uncertain value for clinical
decision making in EACC.
Presentation of KO. KO is closely related to EACC; indeed, these two entities were once believed to
represent the same disease process. KO generally occurs in younger patients, it is often bilateral, and it
has a reported association with bronchiectasis and sinusitis.
KO is described as an occluding plug of keratin without significant otorrhea and with a thickened but intact
tympanic membrane. The original description of KO was published by Toynbee in 1850, and the term
keratosis obturans was coined by Wreden in 1874. The pathogenesis of KO has been most often
described as a chronic desquamative process of the ear canal. However, the initiating event remains
unknown. Some authors have associated KO with bronchiectasis by way of a sympathetic stimulation of
the ceruminous glands and hyperemia; we found no known pulmonary comorbidity in any of the patients in
our study. Corbridge et al documented faulty epithelial migration patterns of the external auditory canal
epithelium in patients with KO.
CT imaging of KO demonstrates a soft-tissue plug in the external auditory canal, often with generalized
canal widening. The distinguishing feature is the absence of focal bone erosion without formation of
bony spicules. Because KO produces a pattern of bony expansion rather than erosion, the occurrence of
invasive complications is unlikely.
Disease course. Extension of EACC into adjacent structures has been described. The middle ear
space may be involved, typically by disruption of the tympanic annulus. Invasion into the mastoid air-cell
system represents a more complicated entity that poses a greater risk to the facial nerve. In either case,
more extensive surgical debridement is required. As with other cholesteatomas, EACC may result in
dehiscence of the tegmen tympani or tegmen mastoideum and a subsequent need for surgical
reconstruction to prevent complications such as cerebrospinal fluid otorrhea and meningitis. Semicircular
canal erosion may also be possible, although this has not been reported with EACC.
Treatment. The treatment of cholesteatoma of the middle ear and mastoid is focused on the complete
surgical removal of all keratin-bearing tissue from the middle ear space. In contrast, in both EACC and KO
that is not advised because this is the normal epithelium in this location. Therefore, treatment of these
disorders is focused on (1) removing keratin debris to provide an open, clean canal and (2) preventing
extension of the disease into other areas, such as the middle ear. Usually this will require nothing more than
frequent cleaning and debridement of keratin debris in the outpatient setting.
If chronic pain is present or if the lesion is more extensive, surgical management may become necessary.
Surgical options include debridement via a postauricular approach, canaloplasty, and split-thickness skin
grafting to cover the canal defect. Erosion into the mastoid air cells may necessitate a mastoidectomy.
In an effort to guide therapy, Holt described three stages of EACC and their recommended treatments:
9
2
2
2
6
4
2
2
2 10
11
2 2
12
8,13
5,13
2
3
For superficial, localized, saucerized defects, frequent cleaning with debridement as an office procedure
is sufficient.
For deeper localized pockets of external auditory canal erosion, canaloplasty is recommended.
Lesions that extend into the mastoid require mastoid surgery.
Since both EACC and KO may appear at presentation as nothing more than a dense wax impaction, the
removal of these lesions should always be done in a careful, painless manner. Attempts to remove dense
cerumen impaction should always be attempted under microscopic visualization. Instrumentation should
begin laterally, and the physician must take great care to avoid tugging or pulling if debris is not easily
extracted. Irrigation should be avoided because water might introduce bacteria into an epithelial pocket,
resulting in a wet, infected cholesteatoma that might require additional medical or surgical management.
An important guiding factor in the treatment of EACC and KO is the presence of pain. Removal of a large
EACC may be precluded by severe pain, which may be a sign of deeper penetration of the disease process.
In this scenario, no further office cleaning should be attempted, and the patient should be sent for CT to
better define the depth of invasion and the extent of bony erosion.
If the use of instrumentation does not produce excessive pain, complete removal may be possible in the
office. Once the ear canal is adequately cleared, any expansion of the canal wall should be evaluated further
with CT. It is important to refrain from anesthetizing the ear to facilitate patient comfort during cleaning
because this may mask an important sign of deep tissue invasion by an EACC.
If an EACC or KO is large or extends into areas inaccessible through the meatus, a surgical meatoplasty
should be considered. This will smooth the contour of the canal wall and prevent keratin reaccumulation or
progression.
In the present series, all patients had been treated with serial microscopic debridement in the office without
the need for debridement under anesthesia. Reaccumulation was minimal and amenable to further office
debridement, suggesting a potentially effective alternative to traditional surgical management. As previously
noted, CT is an invaluable resource in such cases to facilitate safe debridement and to avoid iatrogenic
injury.
Special attention must be paid when CT demonstrates extensive dehiscence of the facial nerve in its course
through the fallopian canal. Although such a circumstance was not seen in our series, we believe that such
a case warrants debridement in the operating room. Surgical debridement allows for identification and safe
dissection of the facial nerve in an uninvolved segment. Additional study is needed to support this
recommendation.
In conclusion, EACC is an uncommon entity that may be mistaken for KO, cerumen impaction, or another
disease process. Because it is typically indolent and longstanding, significant bony erosion may be present
at the time of diagnosis. Exposure of vital structures, including the facial nerve, must be taken into
consideration when initial cleaning of the ear canal is attempted. KO generally produces a distinctive smooth
erosion of the medial canal, which poses minimal danger to deeper structures. Pain may be an important
indicator of deep infiltration, and it should prompt acquisition of CT prior to complete debridement. EACC
may be managed effectively in the outpatient setting, with surgery reserved for appropriately selected high-
risk cases.
From ENT and Allergy Associates, LLP (Westchester County), Tuckahoe, N.Y. (Dr. McCoul); and the
Department of Otolaryngology, SUNY Downstate Medical Center, Brooklyn, N.Y., and the Department of
Otolaryngology, Long Island College Hospital, Brooklyn (Dr. Hanson). The study described in this article was
performed at the SUNY Downstate Medical Center. Corresponding author: Matthew B. Hanson, MD,
Department of Otolaryngology, SUNY Downstate Medical Center, 450 Clarkson Ave., Box 126, Brooklyn,
NY 11203. Email: matthew.hanson@downstate.edu
References
1. Anthony PF, Anthony WP. Surgical treatment of external auditory canal cholesteatoma. Laryngoscope
1982; 92 (1): 70-5.
2. Piepergerdes MC, Kramer BM, Behnke EE. Keratosis obturans and external auditory canal
cholesteatoma. Laryngoscope 1980; 90 (3): 383-91.
3. Holt J J . Ear canal cholesteatoma. Laryngoscope 1992; 102 (6): 608-13.
4. Naim R, Linthicum F J r., Shen T, et al. Classification of the external auditory canal cholesteatoma.
Laryngoscope 2005; 115 (3): 455-60.
5. Heilbrun ME, Salzman KL, Glastonbury CM, et al. External auditory canal cholesteatoma: Clinical and
imaging spectrum. AJ NR Am J Neuroradiol 2003; 24 (4): 751-6.
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6. Owen HH, Rosborg J , Gaihede M. Cholesteatoma of the external ear canal: Etiological factors,
symptoms and clinical findings in a series of 48 cases. BMC Ear Nose Throat Disord 2006; 6:16.
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Ear Nose Throat J . 2011 December;90(12):E1
Therapeutic approaches to
complicated cholesteatoma of
the external auditory canal: A
case of associated facial
paresis
Seborrheic keratosis of the
external auditory canal
Cholesteatoma of the external
auditory canal in an
immunocompromised patient
Extensive external auditory
canal cholesteatoma in the
infratemporal area without
mastoid involvement: Use of a
new surgical technique
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