Global Journal of

Otolaryngology
ISSN 2474-7556

Clinical Case

Glob J Otolaryngol

Volume 24 Issue 3- May 2021
DOI: 10.19080/GJO.2021.24.556139

Copyright © All rights are reserved by Ichiro Fukumoto

Pre-School Pediatric Cholesteatoma:
46 Cases

Ichiro Fukumoto1*, Hiroki Koizumi2, Yusuke Takata3, Seiichi Kadowaki2, Yusuke Kimura4 and Hiroshi Sunose2
1

Department of Otorhinolaryngology, Head and Neck Surgery, Chiba University Graduate School of Medicine, Japan2

Department of Otorhinolaryngology, Tokyo Women’s Medical University Medical Center East, Japan3

Department of Otorhinolaryngology, Juntendo University Graduate School of Medicine, Japan

4

Department of Otorhinolaryngology, Head and Neck Surgery, Nihon University School of Medicine, Japan

Submission: May 03, 2021; Published: May 18, 2021

*Corresponding author: Ichiro Fukumoto, Department of Otorhinolaryngology/Head and Neck Surgery, Chiba University Graduate School of
Medicine, Chiba, Japan
Abstract
Pediatric cholesteatoma is classified into congenital cholesteatoma and acquired cholesteatoma. As congenital cholesteatoma has few
symptoms, the cases are usually discovered with hearing loss during the examination during enrollment in an elementary school. Acquiredcholesteatoma is associated with inflammatory changes and eustachian tube dysfunction of the middle ear, and it is said that most cases are
more severe than congenital cholesteatoma. We analyzed 46 cases (47 ears) of pediatric cholesteatoma, diagnosed before attending school andoperated upon. We also examined the discovery mechanism, classification, progress level, operative methods of cholesteatoma, postoperative
hearing tests, and recurrences. Approximately 85% of these cases were of congenital cholesteatoma; additionally, approximately 15% had
acquired cholesteatoma. As a result, there were no significant differences between congenital cholesteatoma cases and acquired cholesteatoma
cases in terms of the degree of progression, hearing ability prognosis, and rate of recurrence. Moreover, the rate of recurrence and hearing ability
prognoses were better than those in previous reports.

In general, there are several cases in which the cholesteatoma extends to anatomical blind spots such as the tympanic sinus or facial recess
in pediatric cholesteatoma. Several papers have discussed the availability of middle ear surgeries using endoscopy (i.e., trans canal endoscopic
ear surgery [TEES]). However, we performed middle ear surgery using microscopy (i.e., microscopic ear surgery [MES]) in all cases, and we haveinvented a way to find operation fields using procedures such as drilling the anterior wall of the external auditory canals.
In conclusion, we believe that polite dissection of the cholesteatoma matrix under the microscope is an important factor in the low rate ofrecurrence of cholesteatoma..
Keywords: Congenital cholesteatoma; Acquired cholesteatoma; Pediatric cholesteatoma; MES; TEES

Introduction
Middle ear cholesteatoma is classified roughly into congenitalcholesteatoma and acquired cholesteatoma. Additionally, theacquired cholesteatoma is classified into three types: pars flaccida,pars tensa, and secondary types. As all cases of cholesteatoma areprogressive diseases and cause complications, not only otorrhea
and deafness, but also vertigo, deafness, facial nerve palsy,
sigmoid sinus thrombosis and intracranial complications suchas meningitis and epidural/subdural abscess [1], early diagnoses
and early operations are required.
Pediatric cholesteatoma is classified into congenitalcholesteatoma and acquired cholesteatoma. It is said that the

Glob J Otolaryngol 24(3): GJO.MS.ID.556139 (2021)

congenital cholesteatoma is relatively rare and accounts forapproximately 12.9% in all middle ear cholesteatomas [2].
Due of the lack of symptoms, such as otorrhea in congenitalcholesteatoma, the cases are usually discovered for hearing loss
at examination at enrollment in an elementary school. Therefore,
congenital cholesteatoma before attending school is often
confirmed accidentally by a mesotympanic white lesion in theotolaryngology outpatient department. Acquired cholesteatoma
is associated with inflammatory changes and eustachian tube
dysfunction in the middle ear. Compared with congenitalcholesteatoma, the grades of disease are often severe [3]. As mostcases of pediatric cholesteatoma are often diagnosed by a medical001

Global Journal of Otolaryngology
examination at school attendance, there are few articles about thecases that are diagnosed before attending school. We examinedcases that were diagnosed before attending school and wereoperated on at this time.

In addition, there are numerous reports that trans canalendoscopic ear surgery (TEES) is effective because congenitalcholesteatoma matrices often invaginate into the tympanic
sinus and facial nerve recess, which are anatomical blind spaces
in pediatric cholesteatoma [4]. Despite many reports of TEES,
we performed microscopic ear surgery (MES) in all middle ear
surgery cases in our department. Therefore, the aim of this study
was to investigate the results of our operation via microscopy,
such as the rate of recurrence and postoperative hearing levels in
pre-school pediatric cholesteatoma.

Materials and Methods

We examined 46 cases (47 ears) who were diagnosed withcholesteatoma before enrollment at school in the Department ofOtolaryngology, Tokyo Women’s Medical University East Medical
Center from June 2010 to January 2019. In total, 28 cases wereboys, 18 cases were girls, and the average age of the 46 cases
was 3.6 years old. In these cases, we examined the consultation
opportunity, classification, progress level, operative method ofcholesteatoma, postoperative hearing test, and recurrences. We

classified all cases based on the JOS staging system for middle
ear cholesteatoma (2015) [5]. Cholesteatomas that develop
congenitally in the middle ear cavity and is non-continuouswith the tympanic membrane and external auditory canal wereclassified as congenital cholesteatoma. We also classified theacquired cholesteatoma into the flaccid pars flaccida type, parstensa type, and secondary type. Cholesteatomas occurring due tosecondary development of cornified epithelium in the tympanic
membrane and behind the handle of the malleus from theperforated margin of a perforation in the pars tensa were alsoclassified as secondary cholesteatoma. Moreover, we evaluatedthe extension of cholesteatoma using the JOS staging system for
middle ear cholesteatoma (2015) [5]. The postoperative hearing
test results showed ossicular reconstruction and passed morethan one year, and audiometric test results were intended for 27
cases. We judged three patterns as successful cases as below:a.

within 15 dB of air bone gap,c.

within 30 dB of hearing level,b.

above 15 dB of hearing improvement, andbased on the Guideline for Reporting Hearing Results in
Middle Ear and Mastoid Surgery(2010). In addition, we classified
the cases of recurrence into reconstruction recurrence andpersistence recurrence as the mechanism of recurrence.

Figure 1: Forty-six cases (47 ears) diagnosed with middle ear cholesteatoma before attendance at school., A) Consultation opportunity:
Forty-two out of 46 cases (91.2%) were identified in the nearby otolaryngology outpatient department. One case was suspected to havehearing loss at a pre-school examination, and two cases were suspected of hearing loss in an examination at the age of 3 years. One case
was introduced from the pediatric department. B) Classification: Seven ears of 40 ears (85.1%) had congenital cholesteatoma. Seven ears
had acquired cholesteatoma; in these cases, four cases were Pars flaccida type (8.5%), and three cases were the secondary type(6.4%). C)
Staging of 40 patients with CC: Seven patients (17.5%) had stage Ia (in the anterior tympanium), Stage Ib (in the posterior tympanium), andStage Ic (at both sites of the tympanium) were observed in one case (2.5%). Additionally, 31 cases (77.5%) were stage II, which extended
to at least two lesions.D) Staging of 7 cases of acquired cholesteatoma: Three ears (42.9%) were stage II, three ears were stage Ib, andonly one case (14.2%) had stage III lateral semicircular canal fistula.

Result002

How to cite this article: Ichiro F, Hiroki K, Yusuke T, Seiichi K, Yusuke K, et al. Pre-School Pediatric Cholesteatoma: 46 Cases. Glob J Oto, 2021; 24(3): 556139. DOI: 10.19080/GJO.2021.24.556139

Global Journal of Otolaryngology

The opportunity to consult our department was obtainedby consultation with other otorhinolaryngologists in more than
90% of cases. In one case involving a 3-year-old child, hearing
loss was suspected at the examination; additionally, one patient
who was referred from pediatrics was noted to have hearing loss
during a pre-school medical examination (Figure 1A). In addition,
40 ears (85.1%) showed congenital cholesteatoma, and theacquired cholesteatoma was seen in 7 cases (14.9%). In acquiredcholesteatoma, three cases were secondary cholesteatoma,
and four cases were pars flaccida cholesteatoma (Figure 1B).
For congenital cholesteatoma, 9 cases (22.5%) were stage I,
one lesion was extended, and 31 cases (77.5%) were stage II,
which extended to at least two lesions (Figure 1C). As for theacquired cholesteatoma, only one case was stage III with a lateralsemicircular canal fistula; however, Stage I and Stage II wereobserved to occur in three cases (Figure 1D). There was no clear

tendency denoting that the cases of acquired cholesteatoma had amore severe extension in our cases.

We performed single-stage operations in approximately
90% of cases (Figure 2A). In the cases in which cholesteatomas
extended to the mastoid, only one patient underwent canal wall
down (CWD) mastoidectomy (i.e., drilling of the posterior wallof the external auditory canal). Notably, other cases underwent
canal wall up (CWU) mastoidectomy (i.e., preserving the posteriorwall of the external auditory canal). In total, 12.8% of cases ofextension of the cholesteatoma confined by the attic were treatedwith trans canal atticotomy (TCA). Cholesteatoma was confinedto the tympanum in 42.6% of cases, and mastoidectomy was not
performed (Figure 2B). Approximately 80% of the cases required
sound conduction reconstruction, and type III reconstruction
was required in 21 cases(44.6%) and type IV in 17 cases(36.1%)(Figure 2c).

Figure 2: Surgeries of 47 ears; A) Staging: Single-stage surgery was performed in 42 out of 47 ears(89.4%), and 5 ears (10.6%) underwent
double-staged surgery. B) Mastoidectomy: In 42.6% of all cases, cholesteatomas extended to the mastoid, and only one case was CWD
mastoidectomy, while other cases were CWU mastoidectomy. The cases of extension of the cholesteatoma confined by attic were 12.8%
and were treated with TCA. Cholesteatoma was confined to the tympanum in 42.6% of cases, and mastoidectomy was not performed. C)
Rebuilding of Sound: Sound conduction reconstruction was not required in 19.2% of the ears. Type IIIi-M reconstruction was performed
in five ears (10.6%), and type IIIc reconstruction was performed in 16 ears (34.0%). Type IVi-M reconstruction was performed in five ears
(10.6%), and type IVc reconstruction was performed in 12 ears (25.5%).

We evaluated hearing tests in 27 cases one year after soundconduction reconstruction; additionally, the success rate was
81.5% in 22 cases (Table 1A). The success rate was 81.8%(18/22
cases) for congenital cholesteatoma and 80.0%(4/5 cases) of
acquired cholesteatoma. However, the evaluation was difficult
because the number of acquired cholesteatoma cases were few.
There was no significant difference in the hearing prognoses
between congenital cholesteatoma and acquired cholesteatoma(Table 1B).
Recurrences were found in six cases in 47 cases(12.8%).
Six cases were all advanced cases of stage II or stage III, and003

there were no recurrence cases in the case of Stage I (Table 2).
The recurrences seemed to have several persistence-related
recurrences in the previous report [6]; however, persistencerelated, and reconstitution-related recurrences occurred inthree cases. Additionally, the recurrent style did not show any
apparent difference (Table 2). Recurrences were observed in 5ears out of 40(12.5%) of congenital cholesteatoma and in one
case (14.3%)of acquired cholesteatoma (Table 3). As there were
few cases of acquired cholesteatoma, the evaluation was difficult;no major differences in the recurrence rate between congenitalcholesteatoma and acquired cholesteatoma were found.

How to cite this article: Ichiro F, Hiroki K, Yusuke T, Seiichi K, Yusuke K, et al. Pre-School Pediatric Cholesteatoma: 46 Cases. Glob J Oto, 2021; 24(3): 556139. DOI: 10.19080/GJO.2021.24.556139

Global Journal of Otolaryngology
Table 1: Hearing tests in 27 cases one year after sound conduction reconstruction.
Table 1A
Hearing Test after one year from operation

Total 27 ears

Above 15dB of improvement from pre-operation

6 earsWithin 15dB Air-Bone gap

9 earsWithin 30dB of hearing test

22 ears

Correspond to either (Success)Correspond to non (Failure)

22 ears(81.5%)5 ears(18.5%)

Table 1B

Successful rate

Total 27 ears

congenital cholesteatoma
Table 2: Cases of Recurrence.

18/22ears(81.8%)

acquired cholesteatoma

4/5ears(80.0%)

Case

Age

Sex

Classification

Extension

Staging

Recurrent Style

Recurrent Part

1

3

F

Congenital

Stage Ⅱ

SinglePersistence

Mesotympanic

3

5

F

AcquiredStage Ⅲ

SinglePersistence

attic and mastoid

Congenital

Stage Ⅱ

Reconstitution

attic and mastoid

2
4
5
6

6
2
4
6

F
F

Congenital

M

CongenitalF

Table 3: Recurrent rate.
congenital

Discussion

CongenitalacquiredStage Ⅱ

DoubleStage Ⅱ

DoubleReconstitution

Stage Ⅱ

SingleReconstitution

5/40ears(12.5%)
1/7ears(14.3%)

There have been several discussions about the surgicalmethod for middle ear surgery. There are numerous papers
comparing the methods of mastoidectomy to determine thesuperior method: CWD or CWU [7]. In our department, we oftenchoose CWD mastoidectomy based on the rate of recurrences inmost adult cases, except in cases where pneumatization of the
mastoid is highly developed. Past reports concluded that CWD
mastoidectomy can obtain a low recurrence rate and a high rateof trouble-free ear in the long term in pediatric cholesteatoma[8]. However, in pediatric cases, we choose CWU mastoidectomy
because of the undeveloped pediatric temporal bone; this method
helps to maintain the form of the external auditory canal and thepatients can enjoy swimming. In this study, we performed CWU
mastoidectomy, except in one case. One case involved severe casesof stage III with lateral semicircular canal fistula, which recurred
several times. About the number of operations, there are many
advantages to performing a double-staged operation.
Silicon sheets can be inserted to prevent tympanic adhesion
during the first surgery and confirm the presence of recurrence
004

SinglePersistence

Mesotympanic
attic
attic

at the second stage of surgery. Nevertheless, we performed singlestaged operations in 89.4% of cases, and the rate of recurrence
was only 12.8% in pediatric cholesteatoma cases. This rate
was better than that in previous reports [6]. In general, thepostoperative prognosis of hearing ability was good in pediatriccholesteatoma, and we were able to obtain a success rate of 81.5%.
We performed all the middle ear operations in our department
via microscopy using the MES approach. However, in the recent
years, the number of operations performed using endoscopy (i.e.,
TEES) has increased. There are many reports of TEES [9,10], andthe effectiveness of MES and TEES has been discussed [11].

In congenital cholesteatoma cases, the matrix of thecholesteatoma may extend to the anatomical blind spaces,
including the tympanic sinus or facial recess. In these cases, TEES
may be effective [12]. Moreover, TEES should remain in effect in anarrow external auditory canal, such as pediatric cases [13]. The
field of TEES is good; however, the surgical procedure is not at
all brief, and adverse events increase in inexperienced operators[14]. We use microscopy techniques to open the external auditory
canal, such as drilling the anterior wall and finding a good fieldof anatomical blind spaces. We used an endoscope to confirm
whether the anatomical blind spot had persistence of thecholesteatoma matrix. We concluded that a polite dissection of thecholesteatoma matrix under microscopy was the key factor in the
low recurrence rate.

How to cite this article: Ichiro F, Hiroki K, Yusuke T, Seiichi K, Yusuke K, et al. Pre-School Pediatric Cholesteatoma: 46 Cases. Glob J Oto, 2021; 24(3): 556139. DOI: 10.19080/GJO.2021.24.556139

Global Journal of Otolaryngology

Conclusion
We examined 46 cases (47 ears) with cholesteatomas who
were diagnosed and operated upon before attending elementary
school. In most cases, we could preserve the posterior walls of theexternal auditory canals and perform single-staged operations. In
all cases, we performed the surgeries using microscopy, and we
had no cases that required endoscopy. However, we were able toobtain a low rate of recurrence and a good hearing prognosis.

Acknowledgement

This study was supported by the KAKENHI, 18K16866.

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How to cite this article: Ichiro F, Hiroki K, Yusuke T, Seiichi K, Yusuke K, et al. Pre-School Pediatric Cholesteatoma: 46 Cases. Glob J Oto, 2021; 24(3): 556139. DOI: 10.19080/GJO.2021.24.556139