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Operative management of external auditory canal cholesteatoma: Case series and literature review

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Objectives: To describe the clinical features of external auditory canal cholesteatoma, and to assess the outcome following bony meatoplasty with tragal cartilage and perichondrium graft repair. Methods: A retrospective review was carried out, comprising all patients with external auditory canal who presented between January 2007 and December 2011. Patients underwent pre-operative audiometry and computed tomography imaging of the temporal bones, before undergoing bony meatoplasty via a postauricular incision. Pre- and post-operative comparisons were made of patients’ otological symptoms and the otoscopic appearance of the external ear canal. Results and conclusion: Eight patients were included in the analyses. The median age of patients was 46.5 years (range 14–68 years), with a male to female ratio of 1:1. The median length of follow up was 16 months. The most common presenting features were unilateral otalgia and purulent otorrhoea. All patients had relatively advanced disease at presentation, with erosion of the temporal bone. All patients underwent bony meatoplasty via a postauricular approach to eradicate the disease. Bony meatoplasty was successful in the definitive management of external ear canal cholesteatoma.
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Operative management of external auditory canal
cholesteatoma: case series and literature review
M SAYLES, H A KAMEL, F F FAHMY
Department of Otorhinolaryngology - Head and Neck Surgery, West Suffolk Hospital NHS Trust, Bury
St. Edmunds, UK
Abstract
Objectives: To describe the clinical features of external auditory canal cholesteatoma, and to assess the outcome
following bony meatoplasty with tragal cartilage and perichondrium graft repair.
Methods: A retrospective review was carried out, comprising all patients with external auditory canal
cholesteatoma who presented between January 2007 and December 2011. Patients underwent pre-operative
audiometry and computed tomography imaging of the temporal bones, before undergoing bony meatoplasty via
a postauricular incision. Pre- and post-operative comparisons were made of patients otological symptoms and
the otoscopic appearance of the external ear canal.
Results and conclusion: Eight patients were included in the analyses. The median age of patients was 46.5 years
(range 14 68 years), with a male to female ratio of 1:1. The median length of follow up was 16 months. The most
common presenting features were unilateral otalgia and purulent otorrhoea. All patients had relatively advanced
disease at presentation, with erosion of the temporal bone. All patients underwent bony meatoplasty via a
postauricular approach to eradicate the diseas e. Bony meatoplasty was successful in the definitive management
of external ear canal cholesteatoma.
Key words: Cholesteatoma; External Auditory Canal; Otologic Surgical Procedures
Introduction
External auditory canal cholesteatoma is a rare disease
entity with an uncertain aetiology. Few case series have
been reported in the literature, and current management
is highly variable between centres. The locally erosive
and indolent nature of cholesteatoma in the bony exter-
nal ear canal gives rise to complications resulting from
erosion into adjacent structures, such as the mastoid
and the temporomandibular joint.
This study aimed to describe the clinical features of a
series of patients treated for cholesteatoma of the exter-
nal ear canal. We report our experience of operative
management in these patients, who underwent bony
meatoplasty.
Materials and methods
The majority of patients in the series reviewed were
referred to otolaryngology by their general practitioner
with a history of unilateral otalgia and otorrhoea, for
which they had received an (often unsuccessful) trial
of conservative antimicrobial medical therapy prior to
referral. Otoscopy most frequently showed erosion of
the inferior aspect of the bony canal wall, with accumu-
lated keratin and bony sequestrum. A clinical diagnosis
of external auditory canal cholesteatoma was made on
the basis of these findings.
Each patient underwent pre-operative investigation
with high-resolution computed tomography (CT) of
the temporal bones, audiometric assessment, and exam-
ination under anaesthesia with biopsy of the ear canal
lesion where necessary. Axial CT with coronal recon-
struction was used to assess the extent of local involve-
ment. These imaging studies confirmed the presence of
bony canal wall erosion, with or without overlying soft-
tissue attenuation material. Examination of the external
auditory canal lesion under anaesthesia, and biopsy
where appropriate, was used to exclude malignant
(necrotising) otitis externa and squamous cell carci-
noma of the external auditory canal skin. Histological
analysis of biopsy specimens showed keratin, with no
evidence of squamous malignancy.
In each case, the disease was at a relatively advanced
stage, with erosion of the temporal bone. All cases were
managed surgically in order to eradicate the disease
process, preserve normal structure and function, and
provide patients with a self-cleaning ear canal. Bony
meatoplasty, carried out via a postauricular approach,
was performed under general anaesthesia in a main
operating theatre. The postauricular and ear canal skin
Accepted for publication 23 January 2013 First published online 20 August 2013
The Journal of Laryngology & Otology (2013), 127, 859 866.
MAIN ARTICLE
© JLO (1984) Limited, 2013
doi:10.1017/S0022215113001850
was first infiltrated with 4 ml of 2 per cent lidocaine
hydrochloride with adrenaline (1:80 000). The ear
canal re-entry incision was made just lateral to the cho-
lesteatoma sac. The cholesteatoma sac was elevated and
the cholesteatoma was excised. In order to provide a
smooth ear canal contour, the bony irregularities and
the edge of the bony erosion were drilled out. The
resulting cavity was obliterated with tragal cartilage
and perichondrium grafts, with or without temporalis
fascia, to achieve a smooth, self-cleaning ear canal.
The postauricular wound was closed using layers of
subcutaneous vicryl with subcuticular Monocryl
sutures (Ethicon, Somerville, New Jersey, USA). The
ear canal was then packed with two small pieces of
ribbon gauze (approximately 2 cm in length) impreg-
nated with bismuth iodoform paraffin paste (BIPP). A
simple dry gauze dressing was applied to the ear.
Patients were reviewed and, if well, discharged home
on day one following surgery.
Follow up in the out-patient otolaryngology clinic
was arranged two weeks post-surgery for removal of
the BIPP packing. At this stage, patients were given a
one-week course of Sofradex ear drops (Sanofi-
Aventis, Guildford, UK). Patients were reviewed
again at six weeks post-surgery. Thereafter, patients
were kept under regular follow up (usually with six-
monthly intervals). The post-operative follow-up
period for this case series ranged from 6 to 36
months (median 20.5 months).
Results
Eight cases of external auditory canal cholesteatoma
were identified within the study period (Table I). The
median age of patients at presentation was 46.5 years
(range 14 68 years), and the male to female ratio was
1:1. The most common presenting features were non-
specific unilateral otalgia and purulent otorrhoea. All
patients had relatively advanced disease at presentation,
with erosion of the temporal bone (Table II). The
patients underwent bony meatoplasty via a postauricular
approach in order to eradicate the disease process. One
patient developed a second lesion post-operatively. The
median follow-up period was 16 months. Three selected
cases are described in detail below, with exemplar CT
images and intra-operative photographs.
Case one
A 68-year-old man was referred by his general prac-
titioner with a two-year history of recurrent right-
sided otitis externa. He was a smoker, with a
background of chronic obstructive pulmonary disease,
hypertension, and ischaemic heart disease with pre-
vious coronary artery bypass grafting. The otitis
externa had been managed intermittently with topical
antibiotics prescribed by his general practitioner. On
attending the senior house officers emergency otolar-
yngology clinic, the patient described right-sided
otalgia and a sense of aural fullness, with associated
purulent otorrhoea. Medical management with topical
and systemic antibiotics, in combination with aural
toilet, was unsuccessful. He was therefore referred for
a senior specialist opinion.
Otoscopy revealed two ar eas of erosion in the inferior
aspect of the ear canal, which contained bony sequestr a.
Computed tomography confirmed the pr esence of abnor-
mal soft tissue and erosion of the external auditory canal
floor (Figur e 1a). The appearance of the middle-ear cleft
on CT scans was normal. Audiometric assessment
showed moderate to severe high-frequency sensorineural
TABLE I
SUMMARY OF RESULTS
Pt sex, age
(y)
Presenting features Aetiology Risk
factors
Stage
8
Treatment
M, 68 Otalgia, purulent otorrhoea Idiopathic Smoker III Bony meatoplasty
M, 14 Otalgia, purulent otorrhoea Idiopathic III Bony meatoplasty
F, 46 Otalgia, bloody otorrhoea Idiopathic Diabetic III Bony meatoplasty, topical antibiotics for 2nd
lesion
F, 60 Otalgia, aural fullness,
progressive hearing loss
Idiopathic Smoker III Topical antibiotics, regular aural toilet, bony
meatoplasty at 3 y
F, 37 Otalgia, purulent otorrhoea Idiopathic Smoker III Bony meatoplasty
M, 58 GP referral for aural toilet for wax Idiopathic Hearing
aids
III Bony meatoplasty
M, 39 Otorrhoea Idiopathic Smoker IV (mastoid) Bony meatoplasty
F, 47 Otalgia, purulent otorrhoea Idiopathic III Bony meatoplasty
Pt = patient; y = years; M = male; F = female; GP = general practitioner
TABLE II
EXTERNAL AUDITORY CANAL CHOLESTEATOMA
STAGING CRITERIA
Stage Histologic
criteria
Macroscopic criteria
I Epithelial
hyperplasia
IIa/b Periosteitis Hyperplasia with erythema (IIa), or
denuded but not eroded bone (IIb)
III Canal wall erosion & bony
sequestration
IV Invasion into adjacent structures
Subclasses of disease stage IV include: mastoid (M), skull base
and sigmoid sinus (S), temporomandibular joint (J), and facial
nerve canal (F).
M SAYLES, H A KAMEL, F F FAHMY860
hearing loss bilaterally. Examination under general
anaesthesia revealed a 3 mm defect filled with cholestea-
toma, with no involvement of the middle ear. There were
no suspicious areas; hence biopsy was not carried out.
Bony meatoplasty was performed through a post-
auricular approach, with an ear canal skin incision
just lateral to the cholesteatoma pocket. Henles spine
was drilled out, the cholesteatoma pocket was elevated,
and the bony irregularities and ridge were drilled to
smooth the cavity. The cholesteatoma cavity was
obliterated with a tragal cartilage and perichondrium
graft (Figure 1be). The canal floor was well healed
at the six-week follow up. He remained well six
months later.
Case two
A 14-year-old boy was referred by his general prac-
titioner with an 8-week history of right-sided otalgia
and purulent otorrhoea, which was unresponsive to
topical and systemic antimicrobial therapy. Initial
FIG. 1
Case one: (a) coronal computed tomography scan, showing soft-tissue attenuation material overlying bony erosion in the right external auditory
canal floor (arrow) and normal bony appearances on the left (asterisk); (b) otoscopic appearance of ear canal cholesteatoma; and intra-operative
photographs, showing (c) cholesteatoma excised and bony irregularities drilled out, (d) cartilage graft in situ, and (e) perichondrium in situ.
EXTERNAL AUDITORY CANAL CHOLESTEATOMA MANAGEMENT 861
examination in the senior house officers emergency
otolaryngology clinic showed a large aural granulation
tissue polyp. After aural microsuction, the patient
was given a course of Gentisone HC ear drops
(Amdipharm, Basildon, UK) and followed up one
week later. At his next clinic appointment, the polyp
had receded and was cauterised with silver nitrate.
One week later, he was found to have an underlying
keratin-filled erosion of the inferior wall of the right
ear canal (Figure 2b, c).
Computed tomography revealed bony erosion of the
inferior wall of the right external ear canal, with normal
appearances of the middle ear (Figure 2a). Audiometric
assessment showed normal hearing bilaterally. The
patient subsequently underwent bony meatoplasty. At
surgery, he was found to have two cholesteatoma
FIG. 2
Case two: (a) coronal computed tomography scan, showing bony erosion of the right external auditory canal floor (asterisk); (b) and (c) oto-
scopic appearances of the cholesteatoma eroding the external auditory canal wall; and intra-operative photographs showing (d) the middle ear
clear of disease, with the chorda tympani intact, and (e) cartilage palisade grafts in situ.
M SAYLES, H A KAMEL, F F FAHMY862
pockets adjacent to the tympanomastoid suture line,
with no extension into the middle ear. The cholestea-
toma was removed and the defects were filled with car-
tilage palisade grafts (Figure 2d, e).
Case three
A 46-year-old woman presented with a 6-month history
of intermittent right-sided otalgia and otorrhoea. She
had been treated with oral amoxicillin for otitis
externa on two occasions over the preceding four
months, as prescribed by her general practitioner. The
patient had a background of treated hypertension. In
addition, she had well controlled type II diabetes mel-
litus and was on oral hypoglycaemic medication; her
most recent HbA1c had been reported as being 7.9
per cent. She was a non-smoker.
Initial assessment revealed erosion of the floor of the
right external auditory canal, with an accumulation of
keratin debris. Following aural toilet, tri-adcortyl oint-
ment was applied to the erosion. Audiometric assess-
ment showed a high-frequency sensorineural hearing
loss bilaterally. Computed tomography confirmed the
superficial erosion of the floor of the right ear canal,
which involved the temporal bone inferiorly and pos-
teriorly (Figure 3a).
Biopsy of the ear canal lesion was carried out under
general anaesthesia to rule out malignant (necrotising)
otitis externa and squamous cell carcinoma of the
FIG. 3
Case three: (a) coronal computed tomography (CT) scan, showing soft-tissue attenuation mass in the right ear canal, with underlying erosion of
the adjacent temporal bone (arrow); intra-operative photographs demonstrating (b) abnormal bony prominence and cholesteatoma in ear canal,
and (c) a clear middle ear; and (d) coronal CT scan, showing erosion of the adjacent temporal bone anteroinferiorly (arrow) and bony erosion
adjacent to the scutum superiorly (asterisk).
EXTERNAL AUDITORY CANAL CHOLESTEATOMA MANAGEMENT 863
external auditory canal skin. Histological analysis of
the tissue specimen showed no evidence of malig-
nancy, and was consistent with a diagnosis of external
auditory canal cholesteatoma. The patient subsequently
underwent right-sided bony meatoplasty and tympanot-
omy to exclude any middle-ear disease. At surgery, the
middle ear was found to be free from cholesteatoma
(Figure 3b, c). Bony meatoplasty was carried out
with tragal cartilage and a perichondrium graft to the
external auditory canal wall.
At follow up, the external auditory canal was
initially well healed. However, one year following
surgery the patient was referred back to the otolaryn-
gology clinic by her general practitioner with increas-
ing right-sided otalgia and otorrhoea. She was
diagnosed with eczematous otitis externa. After a
further six months, examination by the senior author
revealed a small area of cavitation in the external audi-
tory canal posterosuperiorly, which contained bony
sequestrum. This was in a different location to her pre-
vious disease. Computed tomography was repeated.
This showed thickening of the ear canal soft tissues,
with bony destruction adjacent to the scutum super-
iorly. Anteriorly, there was involvement of temporal
bone at the level of the temporomandibular joint
(Figure 3d).
The patient was treated initially with oral ciproflox-
acin and topical ofloxacin ear drops. Two months
later, the ear canal lesion was found to be healing
well. She was still under regular follow up at the time
of writing.
Discussion
Aetiology
Cholesteatoma is most commonly found in the middle-
ear cavity. Rarely, cholesteatoma occurs in the external
auditory canal, with an estimated incidence rate of
1.2 3.7 per 1000 new otology patients.
1,2
The annual
incidence rate of external auditory canal cholesteatoma
in the general population is 0.15 cases per 100 000 indi-
viduals,
2
compared with 9.2 cases per 100 000 individ-
uals per year for middle-ear cholesteatoma.
3
The aetiology of external auditory canal cholestea-
toma remains unclear.
4,5
Idiopathic external auditory
canal cholesteatoma is distinguished from cholestea-
toma that arises secondary to a previous ear canal
insult. Many authorities believe that idiopathic external
auditory canal cholesteatoma results from a reduced
migratory capacity of the canal epithelium, which
leads to keratinisation in situ.
2,6 8
However, this
view has recently been challenged by a study which
showed no difference in the rate of epithelial migration
between normal ears and those affected by external
auditory canal cholesteatoma.
9
In some cases, the
occurrence of external auditory canal cholesteatoma
has been linked to branchial arch anomalies, which
result in the retention of epithelial masses and lead to
cholesteatoma formation in the external auditory
canal floor.
4,10
Epidemiologically-linked risk factors for the devel-
opment of external auditory canal cholesteatoma
include smoking, diabetes mellitus and repeated micro-
trauma (e.g. from cotton buds or hearing aids).
4,5
Smoking and diabetes are thought to result in microan-
giopathic changes in the ear canal that potentially
impair epithelial migration.
5
The majority of idiopathic
external auditory canal cholesteatoma occur in the
inferior canal wall. This is thought to be due to the rela-
tively poor blood supply to the skin along the inferior
aspect of the canal.
None of the cases presented here had any identifiable
aetiological factor; at best, they can be regarded as idio-
pathic external auditory canal cholesteatoma (Table I).
Four of the patients were smokers, one patient was dia-
betic, and one wore hearing aids; the use of hearing
aids may have predisposed the individual to external
auditory canal cholesteatoma through repeated micro-
trauma to the ear canal skin.
Presenting features
A recent meta-analysis of published case series con-
cluded that the most common presenting symptoms
of idiopathic external auditory canal cholesteatoma
are unilateral otorrhoea with mild to moderate
otalgia.
5
A minority of patients complained of unilat-
eral hearing loss; or were asymptomatic, with the cho-
lesteatoma being discovered on routine otoscopy
performed for a separate indication. These findings
are consistent with our experience. Six of the eight
patients presented with unilateral otalgia, five of
which presented with associated otorrhoea. One
patient had painless otorrhoea; one patient mentioned
progressive unilateral hearing loss; and one was asymp-
tomatic, with cholesteatoma being discovered inciden-
tally on otoscopy.
Staging and complications
The indolent and locally erosive nature of external
auditory canal cholesteatoma can result in late presen-
tation, with severe complications due to the involve-
ment of neighbouring structures. If the cholesteatoma
invades into the mastoid, it may erode the facial
nerve canal, sigmoid sinus and semicircular
canals.
2,7,11 17
Erosion through the anterior wall of
the canal may affect the temporomandibular
joint.
2,13,18
Rarely, in advanced cases, extension into
the posterior fossa has been reported, with resulting
intracranial abscess.
15
Naim et al.
8
developed a (I IV) staging system for
external auditory canal cholesteatoma based on the
extent of erosion into nearby structures (Table II).
Seven of the cases reported here presented with stage
III disease, in which there was erosion of the adjacent
temporal bone in the inferior aspect of the external
auditory canal. In one patient, the disease involved
the adjacent mastoid air cells and was therefore
M SAYLES, H A KAMEL, F F FAHMY864
graded as stage IV (mastoid subclass).
8
The majority of
cases reported in the literature are of at least stage IIb.
Reports of stage I external auditory canal cholestea-
toma are very rare.
5,8
This is probably because the ear-
liest stages of the disease are usually asymptomatic. As
the disease progresses to involve the bony canal walls,
the accumulated keratin debris and bony sequestrum
become a focus for infection, which results in the
typical presentation with recurrent otalgia and
otorrhoea.
Computed tomography of the temporal bones has
become accepted as the gold standard for staging and
pre-operative planning in external auditory canal cho-
lesteatoma.
19
This method allows accurate evaluation
of the extent of local bone erosion and examination
for the involvement of adjacent structures.
Treatment
As with middle-ear cholesteatoma, the aims of treatment
in e xternal auditory canal cholestea toma cases are the
eradication of the disease with pr eservation of normal
structure and function, and the restoration of normal epi-
thelial migra tion. C onservative therapy, with fr equent
debridement of the keratin debris and sequestered
bone, is fa v oured in early stage disease.
5,11
If these
simple measures are inadequate to control otalgia and
otorrhoea, or in more advanced disease (stages
IIbIV), surgical intervention is necessary. The aim of
surgery is to excise the cholesteatoma and to restore a
smooth, self-cleaning canal wall epithelium, usually
with the aid of cartilage and fascial grafts to pr otect
denuded structures, and fill any canal wall defect.
When the mastoid air cells are invaded, a modified
radical mastoidectomy may be indicated, with the tym-
panic membrane and ossicles left intact.
20
External auditory canal cholesteatoma is rare,
with potentially serious complications
Computed tomography is the investigation of
choice for disease staging and pre-operative
planning
Most patients present with non-specific
symptoms of unilateral otalgia and otorrhoea
Bony meatoplasty with cartilage graft repair
is effective in the definitive management of
this disease
All our patients underwent bony meatoplasty via a
postauricular approach. One patient was initially reluc-
tant to pursue operative management as her symptoms
were mild. She was therefore initially managed with
topical antibiotic preparations and regular debridement
in the clinic. After three years of follow up, she under-
went successful curative surgery. One patient suffered
further external auditory canal cholesteatoma at a sep-
arate location in the same ear canal 18 months after
bony meatoplasty. This was being managed conserva-
tively at the time of writing.
Conclusion
External auditory canal cholesteatoma is a poorly
understood and rare disease entity, which presents
diagnostic and therapeutic challenges. Computed tom-
ography is the most useful investigation for disease
staging and pre-operative planning. Of the eight
patients reviewed, seven underwent meatoplasty for
stage III external auditory canal cholesteatoma and
one for stage IV (mastoid subclass) disease. In the
absence of surgical intervention, close clinical monitor-
ing with regular follow up is required to treat symptoms
and monitor patients for potentially serious
complications.
Acknowledgement
We would like to thank Mr Patrick Axon (consultant ENT
surgeon at Addenbrookes Hospital, Cambridge, UK) for
his assistance in the management of case seven.
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Address for correspondence:
Mr M Sayles,
Department of Otorhinolaryngology - Head and Neck Surgery,
West Suffolk Hospital NHS Trust,
Hardwick Lane,
Bury St. Edmunds IP33 2QZ, UK
E-mail: sayles.m@gmail.com
Mr M Sayles takes responsibility for the integrity
of the content of the paper
Competing interests: None declared
M SAYLES, H A KAMEL, F F FAHMY866
... CT allows accurate evaluation of the extent of local bone erosion and examination for the involvement of adjacent landmark structures, namely the middle ear, tegmen tympani, mastoid, skull base, and fallopian canal. The involvement of these structures may determine management [13,38]. ...
... The current management of EACC is highly variable between centres [38]. Holt suggested dividing the treatment options for EACC into conservative and surgical, based on the pathological extent of the disease [23]. ...
... Holt suggested dividing the treatment options for EACC into conservative and surgical, based on the pathological extent of the disease [23]. Treatment aims are disease eradication, preservation of normal structure and function, and the restoration of normal epithelial migration and thus a self-cleaning EAC [38]. ...
Article
Full-text available
Background External auditory canal cholesteatoma (EACC) is a rare pathological condition representing 0.1% of all new otologic cases. Bilaterality manifests in 10% of EACC cases. Similar disease processes include keratosis obturans (KO) and benign necrotizing otitis externa (BNOE). Diagnostic differentiation may not always be unexacting but does however influence management and eventual outcome. This study aims to briefly review the literature, describe an unusual case of synchronous bilateral primary EACC, and simplify the diagnostic challenges surrounding this disease. Case presentation A 66-year-old man with no relevant family history, no concurrent or intercurrent illnesses, having undergone no relevant surgery, and had visited the Department of Otorhinolaryngology regarding right-sided otorrhoea and aural pruritis 9 years ago. At the time, he was diagnosed clinically with KO. Nine years later after having defaulted follow-up, he presented again with the same symptoms. Oto-microscopy now revealed excavation of the posterior canal wall, keratinous desquamation, and an intact tympanic membrane. Oto-endoscopy demonstrated extension posteriorly into the mastoid segment of the temporal bone. Computed tomography evinced a soft tissue attenuating mass within the excavated sub-adjacent bone, with extension into the mastoid air cell system. The contralateral ear had a smaller epithelial defect of the canal floor with underlying tympanic plate erosion. A closed mastoidectomy with the reconstruction of the posterior canal wall was performed on the right ear. The left ear was managed conservatively with micro-suctioning and aural toilette. Six months post-surgery, however, the reconstructed posterior canal wall underwent necrotic breakdown. The contralateral lesion gradually progressed into a Naim et al. (2005) Stage III EACC. Conclusion This case illustrates the diagnostic challenge presented by EACC and is unusual regarding its bilaterality. The importance of diagnostic differentiation, both in the management of this disease and in the prevention of its progression, is highlighted. The idea of oto-endoscopy as a routine clinical tool is introduced, and endoscopic images are compared to high-definition computed tomography scans of the affected temporal bones.
... In contrast to growing data of the effect of middle ear surgery on postoperative taste function [15], there is little information about taste alteration for specific otologic diseases prior to surgery [16,17,[33][34][35]. For EACC, none of the 19 more extensive case series published in the English literature [1,2,4,5,[18][19][20][21][36][37][38][39][40][41][42][43][44][45][46][47] systematically described the symptoms and signs of taste dysfunction. All of these studies focus on the cardinal symptoms, i.e., frequent unilateral otorrhea or otalgia, or they discuss symptoms of potential distinctive features of EACC caused by keratosis obturans (KO) [36]. ...
Article
Introduction: External auditory canal cholesteatoma (EACC) is a rare disease, with an estimated incidence of approximately 1:1,000 adult and 1.6:1,000 pediatric otologic patients. Systematic studies of chronic ear disease and taste alteration prior to surgery are rare; in fact, there are no such studies for EACCs. Therefore, we describe chorda tympani nerve (CTN) dysfunction and the related clinical consequences in EACC patients. Methods/study design: Between 1992 and 2021, we retrospectively analyzed the symptoms, signs, and radiological and intraoperative descriptions of CTN involvement in 73 patients. Liquid taste tests and, since 2009, Taste StripsTM as well as an olfactory screening test (Smell DiskettesTM) have been performed for all symptomatic patients and, when feasible, all other EACC patients. Results: Ten of 73 patients complained subjectively of dysfunction, and 8 showed abnormal taste test results. Four patients complained of olfactory dysfunction (3 cases with pathological taste tests). Gustatory dysfunction was most frequent in radiogenic EACC cases (n = 4), followed by postoperative EACC (n = 3). Two postoperative patients were asymptomatic despite abnormal test results. Rarely, patients with idiopathic (n = 2) and posttraumatic (n = 1) EACC showed acute taste dysfunction that was confirmed in each with abnormal test results. Discussion/conclusion: CTN dysfunction often developed asymptomatically in chronic ears, except for idiopathic and posttraumatic EACCs under previous healthy middle ear conditions. Taste disturbance is not a cardinal symptom of EACC, but objective testing suggests that up to one out of 10 EACC patients with advanced disease may experience regional gustatory dysfunction prior to surgery. Especially in context of a new and acute presentation, regional taste dysfunction may alert the clinician of potential progressive EACC invasion and danger to the facial nerve.
... Patients with a stenosis of 2 mm or less are at a high risk of developing cholesteatoma and should be operated on [8]. There have been no large-scale investigations of the clinical aspects and longterm consequences of CAS to yet, and no research have detailed the critical parameter of determining the diameter of the EAC [2,[8][9][10][11][12][13][14]. ...
Article
The absence of a patent ear canal, which can be acquired or congenital, is known as Aural Atresia. The most common cause of acquired Aural Atresia is an inflammatory response to trauma or otologic surgery. Although rare, acquired Aural Atresia is most commonly found after external ear trauma, such as car accidents, gunshot wounds, or recent otologic surgery. The main treatment of Aural Atresia is surgical, also the invention of bone anchored hearing devices provide greater alternative solution, each methods has its advantages of disadvantages, we hope In the future of developing more effective treatment options.
Article
Objective We evaluated the outcomes of canalplasty for external auditory canal cholesteatoma (EACC) using an inferior-pedicled periosteal flap to cover the eradicated diseased bone. Study Design Retrospective chart review. Setting Tertiary referral center. Patients Thirty-one ears in 30 patients surgically treated for stages III and IV primary EACC. Intervention Canalplasty using an inferior-pedicled periosteal flap with or without tympanoplasty. Main Outcome Measures Disease recurrence, hearing, and clinical factors influencing the time taken to achieve complete epithelization of the external auditory canal. Results The 31 ears with EACC required 2 to 40 weeks (mean, 7.8 wk; median, 4 wk) to achieve complete epithelization. After epithelization, 84% of the ears became self-cleaning ear canals without any local treatment. Two patients with renal dysfunction experienced recurrence of EACC in the anterior-superior bony canal wall and needed additional treatment. Younger patients took a significantly shorter time to achieve epithelization than older patients ( p < 0.001). Patients with renal dysfunction tended to need longer periods for epithelization than those without renal dysfunction ( p = 0.092). Conclusion Canalplasty for stages III and IV EACC using an inferior-pedicled periosteal flap achieved good postoperative results, with 86% of ears becoming self-cleaning ear canals in a short healing time. Special attention must be paid to the patients with renal dysfunction to create a pedicled flap of sufficient size to cover the bone and maintain a good blood supply.
Article
Objective This study aims to analyze disease and treatment patterns at a tertiary care center and propose guidelines for the management of canal cholesteatomas. Study Design A retrospective cohort study. Setting This study involves patients diagnosed and treated with external auditory canal cholesteatomas (EACC) from January 1, 2010, to January 1, 2021 at the University of Florida, a tertiary care center. Methods Inclusion criteria included a diagnosis of EACC with at least 1 follow‐up visit after diagnosis. Patient demographics, risk factors, etiology if acquired, EACC characteristics such as location and staging, and treatment modality were recorded and analyzed. The primary outcome was a recurrence of disease. Results Out of the 175 patients reviewed, 90 patients and 100 ears met the criteria. Most patients were treated with surgery (81%, N = 81) as opposed to medical management (ie, serial debridement). While there were no factors that were significant for higher odds of surgical intervention, all Stage IV (n = 9) and superior canal wall locations (n = 8) were treated with surgery. Mastoidectomy was more likely to be performed in higher staged cholesteatomas ( χ ² = 13.41, P = .0012) and posterior canal cholesteatoma location (odds ratio [OR] = 11.20, P = .0001), whereas anterior (OR = 0.11, P = .0390) and inferiorly located EACCs (OR = 0.22, P = .0169) had more odds of being treated with canalplasty/tympanoplasty. Recurrence was seen up to 4.78 years after surgery. Conclusion Disease location and staging and patient factors should be considered in the shared management decision‐making of EACC treatment. Surveillance duration should be up to 5 years.
Article
Objective: To report a common site of external ear canal erosion in multiple pathologies, located inferiorly at 6 o'clock. Patients: Otology patients who came in 2023 for treatment of external auditory canal erosions. Intervention: This clinical capsule is an observational report of the external canal's propensity to erosion at the 6 o'clock location. Patient treatments were canalplasty, mastoidectomy, and medical management. Main outcome measure: Documentation of the propensity to erosion at the 6 o'clock location in the external auditory canal. Locations of the niduses of prior series of external auditory canal pathologies are documented. Results: Eight patients are presented with external auditory canal erosion in 10 ears originating at the 6 o'clock position medial to the bony-cartilaginous junction. No other patient with spontaneous canal erosion presented with their nidus of pathology in another canal location. (A review of 42 case series of 291 patients found that keratosis obturans and bisphosphonate-induced osteonecrosis tended to arise from the same 6 o'clock lateral bony canal location, while 26% of necrotizing otitis externa cases arose there.). Conclusions: The "6 o'clock spot" in the external canal is a common location of canal erosion for spontaneous wax and keratin collections and may be the precursor to keratosis obturans, bisphosphonate-induced osteonecrosis of the ear canal, and necrotizing otitis externa.
Article
External auditory canal (EAC) cholesteatoma is characterized by a bony defect in the auditory canal with a cystic structure lined by keratinizing stratified squamous epithelium and retention of keratin debris. Clinically, patients present with chronic dull pain, otorrhea, or conductive hearing loss. Lamellar keratin debris in a bony defect in the case of an intact tympanic membrane is highly suggestive of external ear canal cholesteatoma. It must be differentiated from keratosis obturans. The locally erosive and indolent nature of cholesteatoma in the bony external ear canal may result in complications due to erosion into adjacent structures such as mastoid cells, semicircular canals, fallopian canal, and temporomandibular joint. The treatment options include conservative therapy and surgery. In this case report, we presented a 75-year-old male with a primary left EAC cholesteatoma underlining the diagnostic and therapeutic features of this rare disease.
Article
External auditory canal cholesteatoma (EACC) is a rare disease, and the pathogenesis and optimal treatment method are not yet established. From January 2018 to July 2019, we performed transcanal endoscopic ear surgery (TEES) on 6 ears with EACC. The median age of the patients was 64 years, and the site of involvement was the inferior wall in 4 ears and both the inferior and anterior walls in 2 ears. According to Naim’s classification, 4 of the affected ears were classified as Stage III and the remaining 2 were classified as Stage IV. Of the 2 Stage IV cases, the disease extended to the temporomandibular joint in 1 case, and to the temporomandibular joint, eardrum, and tympanic cavity in the other. Underlying disease with microangiopathy was noted in 66% of the cases (4/6 ears) had (myocardial infarction, n = 1; dialysis for chronic renal failure, n = 1; administration of bisphosphonate, n = 1; subarachnoid hemorrhage and hypertension, n = 1). Therefore, from the perspective of wound healing, EACC requires minimally invasive surgery. Even in cases where the temporomandibular joint capsule was exposed, the anterior wall of the external auditory canal could be visualized because the endoscope had a wide field of view. In all patients, a dry and self-cleaning external auditory canal was found to be maintained at the time of the follow-up at 12 months. Our results suggest that TEES is useful for EACC.
Article
Objective: To report the clinical characteristics and treatment outcomes, as well as endoscopic-assisted ear surgery techniques used in patients with advanced external auditory canal cholesteatoma (EACC). Study design: Retrospective case series. Setting: University hospital. Methods: From October 2014 to September 2017, adult patients (age > 18) with advanced EACC (Naim's classification: stage III or IV) who underwent transcanal endoscopic ear surgery (TEES) were enrolled. The presenting features, extent of the lesion, and reconstruction techniques used were assessed. The healing time which was defined as the time required to develop a dry, re-epithelialized, and self-cleaning external auditory canal, was compared between stage III and IV. Results: Twenty-three patients were included. EACC was categorized as stage III in 11 ears and stage IV in 12 ears. Cholesteatoma involved the mastoid (30%), middle ear (26%), chorda tympani (22%), temporomandibular joint, antrum, and facial nerve (17% for each). In 96% of patients, a dry and self-cleaning external auditory canal (EAC) was maintained after a mean follow-up of 15 months. The median healing time was 8 weeks in stage III, which was significantly shorter than the 12 weeks required for stage IV (p < 0.05). There was no significant difference in the median healing time between TEES and the canal wall up mastoidectomy for stage IV EACC (14 weeks) performed by the same surgeon over the same period (p > 0.05). Conclusions: TEES is a feasible and safe technique for the exposure and eradication of advanced EACC. Some critical endoscopic techniques for resecting disease and reconstructing the defect in the EAC and middle ear should be mastered before performing this operation.
Article
Full-text available
We propose here a classification system for external auditory canal cholesteatoma (EACC). We classified the EACC by the computed tomography findings and clinical findings of the patients, and we evaluated the EACC characteristics by the proposed staging system. Stage classification was done according to the results of temporal bone computed tomography and the clinical findings of the patients. Stage I indicates that the EACC lesion is limited to the external auditory canal. Stage II indicates that the EACC lesion invades the tympanic membrane and middle ear. Stage III indicates that the EACC lesion creates a defect of the external auditory canal and it involves the air cells in the mastoid bone. Stage IV indicates that the EACC lesion is beyond the temporal bone. Between 1996 and 2006, 29 patients with EACC and who underwent surgery were prospectively collected. This study was comprised of 16 males and 13 females with a mean age of 22.8+/-15.0 yr. We reviewed the characteristics and results of surgery by our proposed staging system. A total of 29 patients who underwent operation due to EACC were classified by this system, and the number of stage I, II, III, and IV cases was 14, 3, 10, and 2, respectively. Symptoms such as otorrhea, hearing impairment and otalgia occurred in 12, 17, and 17 cases, respectively. The most common wall invaded by EACC was the inferior wall. The number of cases that had a spontaneous, congenital, post-traumatic, post-inflammatory or tumorous origin was 14, 9, 2, 2, and 1, respectively. Cholesteatoma recurred in 2 patients after surgery. Both cases were stage 1 and both were caused by congenital disease. There were 3 cases with meatal stenosis after surgery, and their primary disease was congenital. This proposed staging is simple and easily applicable for use when deciding the treatment plan for patients with EACC.
Article
Objective: Primary cholesteatoma of the external auditory canal (EACC) is a rare disease, characterized by osteonecrosis with formation of sequesters and ingrowth of keratinizing squamous epithelium in the bony EAC. The aetiology and pathogenesis are unknown, but an earlier study has demonstrated abnormal epithelial migration in such ears. The present study explored whether this interesting result can be reproduced. Study Design: The epithelial migration in 10 ears with EACC was studied using the ink-dot method. Two ears with minor lesions were studied before treatment, and 8 ears were studied after operation or conservative treatment. The results were compared with the migration in 15 normal cars examined in a previous study using the same method. Setting: Tertiary referral center. Main Outcome Measures: 1) Presence or absence of epithelial migration; 2) change of the normal centrifugal pattern; and 3) estimated migration speed compared with normal cars. Results: A qualitatively normal centrifugal migration was present on intact (unaffected or healed) skin in all 10 ears with EACC and was only missing directly on crust-covered lesions. The estimated migration speed was similar to the speed in normal ears, that is, approximately 150 mu m/d. Conclusion: Ears with EACC seem to have qualitatively and quantitatively normal epithelial migration except directly on crust-covered lesions. It is unlikely that an abnormal epithelial migration is involved in the pathogenesis of this disease. These observations have implications for EAC disorders with similar clinical features that involve bony invasion, including osteoradionecrosis.
Article
A total of 500 patients with cholesteatoma diagnosed and operated during 1982-91 in the region of Tampere University Hospital and Mikkeli Central Hospital in Finland were analysed retrospectively. The mean annual incidence was 9.2 per 100,000 inhabitants (range 3.7-13.9) and during the study period the annual incidence decreased significantly. The incidence was higher among males under the age of 50 years. There was no accumulation of cholesteatoma diseases in lower social groups. The majority (72.4%) of cholesteatoma patients had suffered from otitis media episodes. Tympanostomy was carried out in 10.2% and adenoidectomy or adenotonsillectomy in 15.9% of all cholesteatoma ears prior to cholesteatoma surgery. Cholesteatoma behind an intact tympanic membrane with no history of otitis media was verified in 0.6% of patients and in cleft palate patients in 8%. In this study, 13.2% of patients had ear trauma or ear operation in anamnes.
Article
Keratosis obturans and external auditory canal cholesteatoma (EACC) have previously been considered to represent the same disease process. However, review of the literature and our cases reveal these to be two different clinical and pathological processes. Keratosis obturans presents as hearing loss and usually acute, severe pain secondary to the accumulation of large plugs of desquamated keratin in the ear canal. External auditory canal cholesteatoma presents as otorrhea with a chronic, dull pain secondary to an invasion of squamous tissue into a localized area of periosteitis in the canal wall. The treatment previously recommended for both of these conditions has been conservative debridement of the external canal and application of topical medication. While this remains the treatment of choice for keratosis obturans, surgery may be required to eradicate EACC.
Article
An ink dot staining method was used to examine epithelial migration in normal ears, in pathologic tympanic membranes, and in external auditory canal cholesteatoma. The direction of the epithelial migration was found to coincide with that of the vessels in normal ears. This suggested that epithelial migration was controlled by the vessels which supply the epidermal layer of the tympanic membrane. In pathologic tympanic membranes and external auditory canal cholesteatomas, epithelial migration was disturbed to various degrees and depended on the local conditions. In these pathologic conditions, the tympanic membrane showed less vascularization. Based on our findings, we believe that a poor blood supply is the major etiologic factor for the epithelial migratory disturbances of the tympanic membrane.
Article
Spontaneous cholesteatoma of the external auditory canal (EAC) is an uncommon condition that is difficult to diagnose. In a patient with such a possibility, serious clinical investigation along with radiologic and histologic exploration should be performed early on because a delay in treatment can lead to severe complications. Given the rarity of EAC cholesteatoma, no therapeutic consensus has emerged. The type of management depends on the extensiveness of invasion and bone erosion and the status of the neighboring structures. The primary therapeutic objectives are to eradicate the cholesteatoma and then to fill in the residual cavity, which in our opinion can be best accomplished with a muscle flap and EAC reconstruction. Postoperative follow-up should be carried out to look for infections, stenosis, and recurrence. We report a new case of spontaneous EAC cholesteatoma, and we review its diagnostic and therapeutic challenges.
Article
We present an update on clinical evaluation, staging, classification and treatment of canal cholesteatoma, including a meta-analysis of clinical data of the last 30 years. Ear canal cholesteatoma is frequently associated secondarily to other canal pathologies. The cause for the rare idiopathic form of the disease remains enigmatic. Epidemiologic and experimental studies of its pathogenesis have increased; however, the main explanatory theory of a deficient migratory capacity of the canal epithelium affected has been falsified only recently. Therefore, the debate on the pathogenesis has gained additional impetus and more data is needed. Canal cholesteatoma is a rarity in otologic pathology, often leading to misdiagnosis as external otitis or otomycosis by physicians unfamiliar with the disease. It presents typically with otorrhea, focal erosion and keratin accumulation in the osseous ear canal and has to be distinguished from keratosis obturans, which leads to otalgia and bilateral conductive hearing loss by ceruminal plugs, with circumferential distention of the ear canal. Treatment by canaloplasty is curative and highly successful. Alternative conservative treatment is feasible, however, requiring long-term follow up, with often painful cleaning of the lesion.
Article
To investigate the spectrum of disease presentation and clinical management of primary external auditory canal cholesteatoma (EACC). Case series with chart review. Specialty teaching hospital. Ten cases of primary EACC were identified in nine patients treated over 14 years (1995-2009). Cases were reviewed with regard to demographics, presentation, physical examination, CT findings, and clinical management. The most common symptoms were otalgia and hearing loss, followed by otorrhea and tinnitus. Erosion was present in the mastoid air cells in seven patients, middle ear in six, temporomandibular joint in two, otic capsule in two, and fallopian canal in one patient. Eight of nine patients were managed with serial debridement. EACC is associated with adjacent bony erosion, most often involving the inferior EAC. Despite the potentially destructive nature of these lesions, most cases can be successfully managed with serial debridement.
Article
External auditory canal cholesteatoma (EACC) is a rarity. Although there have been numerous case reports, there are only few systematic analyses of case series, and the pathogenesis of idiopathic EACC remains enigmatic. In a tertiary referral center for a population of 1.5 million inhabitants, 34 patients with 35 EACC (13 idiopathic [1 bilateral] and 22 secondary) who were treated between 1994 and 2006 were included in the study. EACC cardinal symptoms were longstanding otorrhea (65%) and dull otalgia (12%). Focal bone destruction in the external auditory canal with retained squamous debris and an intact tympanic membrane were characteristic. Only 27% of the patients showed conductive hearing loss exceeding 20 dB. Patients with idiopathic EACC had lesions typically located on the floor of the external auditory canal and were older, and the mean smoking intensity was also greater (p < 0.05) compared with patients with secondary EACC. The secondary lesions were assigned to categories (poststenotic [n = 6], postoperative [n = 6], and posttraumatic EACC [n = 4]) and rare categories (radiogenic [n = 2], postinflammatory [n = 1], and postobstructive EACC [n = 1]). In addition, we describe 2 patients with EACC secondary to the complete remission of a Langerhans cell histiocytosis of the external auditory canal. Thirty of 34 patients were treated surgically and became all free of recurrence, even after extensive disease. For the development of idiopathic EACC, repeated microtrauma (e.g., microtrauma resulting from cotton-tipped applicator abuse or from hearing aids) and diminished microcirculation (e.g., from smoking) might be risk factors. A location other than in the inferior portion of the external auditory canal indicates a secondary form of the disease, as in the case of 2 patients with atypically located EACC after years of complete remission of Langerhans cell histiocytosis, which we consider as a new posttumorous category and specific late complication of this rare disease.
Article
An ink dot staining method was used to examine epithelial migration in normal ears, in pathologic tympanic membranes, and in external auditory canal cholesteatoma. The direction of the epithelial migration was found to coincide with that of the vessels in normal ears. This suggested that epithelial migration was controlled by the vessels which supply the epidermal layer of the tympanic membrane. In pathologic tympanic membranes and external auditory canal cholesteatomas, epithelial migration was disturbed to various degrees and depended on the local conditions. In these pathologic conditions, the tympanic membrane showed less vascularization. Based on our findings, we believe that a poor blood supply is the major etiologic factor for the epithelial migratory disturbances of the tympanic membrane.