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Year : 2020  |  Volume : 26  |  Issue : 4  |  Page : 265-267

Peculiar presentation of congenital cholesteatoma in a young girl with congenital canal atresia

Department of Otorhinolaryngology Head and Neck Surgery, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, Kelantan, Malaysia

Date of Submission19-Jul-2020
Date of Decision03-Sep-2020
Date of Acceptance24-Sep-2020
Date of Web Publication23-Apr-2021

Correspondence Address:
Prof. Mohd Khairi Md Daud
Department of Otorhinolaryngology Head and Neck Surgery, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, 16150 Kubang Kerian, Kelantan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/indianjotol.INDIANJOTOL_153_20

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Congenital cholesteatoma is a rare entity, which traditionally is diagnosed as an incidental finding. The patient typically turns out to clinic complaining of reduced hearing and classical otoscopic finding of congenital cholesteatoma leads to the diagnosis. These may not be the case in a patient with underlying canal atresia which oftentimes may feign the actual pathology. Herein, we present a case of cholesteatoma in a teenage girl with congenital canal atresia who presented with recurrent postauricular abscess. The patient subsequently underwent modified radical mastoidectomy with meatoplasty. We would like to draw attention to the fact that early recognition and intervention are prudent as delaying appropriate management may succumb both patient and physicians to devastating outcomes and unnecessary burden.

Keywords: Congenital canal atresia, congenital cholesteatoma, microtia

How to cite this article:
Saniasiaya J, Daud MK, Mohamad H, Abdullah SN. Peculiar presentation of congenital cholesteatoma in a young girl with congenital canal atresia. Indian J Otol 2020;26:265-7

How to cite this URL:
Saniasiaya J, Daud MK, Mohamad H, Abdullah SN. Peculiar presentation of congenital cholesteatoma in a young girl with congenital canal atresia. Indian J Otol [serial online] 2020 [cited 2021 Oct 23];26:265-7. Available from: https://www.indianjotol.org/text.asp?2020/26/4/265/314339

  Introduction Top

Congenital canal atresia (CCA) is defined following the presence of hypoplasia of the external auditory canal. Over the years, CCA has been associated with other congenital abnormalities of pinna, middle as well as inner ear. The occurrence of cholesteatoma synchronously in CCA patients has been reiterated.[1] Conventionally, cholesteatoma presents with chronic otalgia and persistent fetid otorrhea along with hearing loss. Congenital cholesteatoma, on the other hand, is an incidental finding as patients are normally asymptomatic. Congenital cholesteatoma occurs following epidermoid formation in the middle ear cleft. It classically presents as a “pearl” behind the anterior superior quadrant of an intact tympanic membrane.[2] Despite its unfamiliarity, the presentation of postauricular abscess in CCA heralds toward cholesteatoma.

  Case Report Top

A 15-year-old Malay girl with right CCA and microtia presented to our clinic with a history of recurrent postauricular abscess. According to the patient's father, the first episode of postauricular abscess was when the child was 1-year old which resolved with antibiotics; subsequently, similar episode of infection was encountered when the child was 3 and 14 years old. Further history from father revealed that, the child was noted to have reduced hearing over the affected ear, however did not seek any treatment. Apart from that, there was no accompanying facial asymmetry, otalgia, otorrhea, vertigo or tinnitus, or any nasal symptoms. There were also no other notable complaints.

Her developmental milestone was normal. She had no allergy, and her immunization is complete for her age. The patient lives with her parents with all the other members of her family well, none of whom with similar symptoms. The family members had no recent contact with any tuberculous or sick contacts or any travel outside the town recently.

Upon examination, the patient was comfortable, not septic looking, and afebrile with no obvious syndromic facies features. The right pinna revealed microtia Grade 2 with stenotic ear canal [Figure 1], tympanic membrane could not be visualized due to the canal stenosis. There were no discharge or keratin flakes visible. The right postauricular region revealed healed scar from previous infection with no signs of an active infection [Figure 2]. The left pinna appeared normal with no canal stenosis. Otoscopic examination of the left ear revealed an intact tympanic membrane, patent ear canal with no evidence of keratin, or any debris. The facial nerve was bilaterally intact. Hearing assessment with a pure-tune audiometry revealed right severe conductive hearing loss and normal hearing over the left ear.
Figure 1: Microtia with canal stenosis

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Figure 2: Healed scar over the postauricular region

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We proceeded with high-resolution computer tomography (HRCT) of the temporal bone which revealed an atretic right ear canal. There was a soft-tissue mass occupying the right mastoid and middle ear cavity with abnormal configuration of ossicles along with evidence of thinning of the tegmen tympani [Figure 3]. In light of patient's history, clinical and imaging, provisional diagnosis of cholesteatoma with underlying right canal atresia was made. The parents were notified on this and were counseled at once for right modified radical mastoidectomy which they agreed upon. Thus, the patient underwent right modified radical mastoidectomy under microscope guidance, upon which intraoperatively keratin flakes were noted occupying mastoid cavity with evidence of automastoidectomy. Keratin flakes were removed from the mastoid and middle ear cavity. There was a rudimentary handle of malleus seen without normal tympanic membrane. Other ossicles were absent. The tympanic and mastoid segment of the facial nerve was intact. The tegmen tympani were eroded. Endoscope was performed to look for residue over the facial recess and sinus tympani. Postoperatively, the patient was well and was discharged home the subsequent day with a 1-week course on antibiotics. Upon follow-up, the patient was well with no new complaints.
Figure 3: Computed tomography scan showing a soft-tissue mass over the right middle ear cavity

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  Discussion Top

Cholesteatoma is a cystic mass lined by stratified squamous epithelium causing bony erosion and periosteitis found mostly in the middle ear cavity. Congenital cholesteatoma is formed secondary to retained epithelial remnants in the temporal bone can present at any age ranging from infancy to adolescence with a mean age of 16.7 years.[3] Histologically congenital cholesteatoma and acquired cholesteatoma is similar.

It is noteworthy that, congenital cholesteatoma is present in 4%–7% of CCA.[4] In a case with underlying canal atresia, embryonic cellular development arrest has been postulated as a contributing factor of this entity. Trapping of the epithelium lateral to the atretic plate upon embryological arrest of auditory canal formation leads to the formation of cholesteatomas.[5]

Commonly, congenital cholesteatoma is a silent entity yet may present with otitis media, facial paralysis as well as hearing loss.[4] In our patient, her only presentation was recurrent postauricular abscess with no episodes of otorrhea. These may be the case as cholesteatoma located behind the stenotic canal segment are hindered by severely narrowed canal as a result of which it manifests as extra-temporal complications such as mastoiditis, cutaneous fistula, and even intracranial complications.[6]

Ideal imaging modality in managing this entity is HRCT which can be performed after the age of 4–5 as temporal bone growth is adequate and ossicles mineralisation can be visualized.[7] Apart from that, HRCT temporal aids in detecting cholesteatoma, extent of cholesteatoma, and for surgical mapping. In our case, HRCT temporal was done for the patient, delineated the cholesteatoma along with abnormal configuration of ossicles and erosion of tegmen tympani.

As for the surgical treatment, in patients with CCA with congenital cholesteatoma, the main objective is for the complete removal of cholesteatoma and to prevent recurrence. Although being secondary, hearing improvement can also be achieved through canalplasty which can be done concurrently with the cholesteatoma removal. In spite of that, cases involving infants and toddler, Schucknecht proposed watchful waiting until the child is more co-operative, especially for postoperative aural toileting.[8]

  Conclusion Top

Cholesteatoma in CCA albeit rare warrants a high clinical suspicion and prompt management among clinicians as the absence of typical clinical features usually lands both the attending physician and patient in dilemma. We would like to highlight a high-index suspicion of cholesteatoma in all cases with CCA.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Abdel-Aziz M. Congenital aural atresia. J Craniofac Surg 2013;24:e418-22.  Back to cited text no. 1
Solmaz F, Akduman D, Haksever M, Gündoğdu E, Mescioğlu A. Atypical presentation of congenital cholesteatoma in an adult case with good hearing result. Ann Med Surg (Lond) 2015;4:26-9.  Back to cited text no. 2
Kojima H, Miyazaki H, Tanaka Y, Shiwa M, Honda Y, Moriyama H. Congenital middle ear cholesteatoma: Experience in 48 cases. Nihon Jibiinkoka Gakkai Kaiho 2003;106:856-65.  Back to cited text no. 3
Sohal BS, Aggarwal P, Goyal JP, Gupta A. Congenital cholesteatoma with canal atresia: Three case reports. Indian J Otol 2013;19:146-8.  Back to cited text no. 4
  [Full text]  
Caughney RJ, Jahrsdoefer RA, Kesser BW. Congenital cholesteatoma in a case of congenital aural atresia. Otol Neurotol 2006;27:934-6.  Back to cited text no. 5
Yildiri N, Sahan M, Akkaya A. Primary cholesteatoma within stenotic segment ear canal: Report of two cases, one with extra-temporal complications. Int Adv Otol 2009;5:281-5.  Back to cited text no. 6
Tasar M, Yetiser S, Yildirim D, Bozlar U, Tasar MA, Saglam M, et al. Preoperative evaluation of the congenital aural atresia on computer tomography: An analysis of the severity of the deformity of the middle ear and mastoid. E J Rad 2007;62:97-105.  Back to cited text no. 7
Schuknecht HF. Congenital aural atresia. Laryngoscope 1989;99:908-17.  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3]


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