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Unexpected cholesteatoma in a very young child with a congenital aural duplication anomaly.


Congenital aural stenosis is defined as an external auditory canal less than 4 mm across. (1) Cholesteatoma is far more likely in a bony ear canal opening of 2 mm or less, but regardless of the degree of stenosis, the incidence of cholesteatoma is very low in patients younger than 3 years. Therefore, surgery is usually delayed until later in childhood, (1) and high-resolution computed tomography (CT) is not recommended until children reach 4 to 5 years of age. (1,2)

A 2-year-old (25 months) girl presented at our clinic with left otalgia and otorrhea of 3 days' duration. She had a bifid external auditory canal and two skin tags in front of the external auditory canal. The right ear was normal (figure 1, A), but the upper external auditory canal of the left ear was only 2 mm across, and the lower external auditory canal was only 1 mm across (figure 1, B). The right tympanic membrane was intact, but we were unable to visualize the left tympanic membrane due to left congenital aural stenosis, which had been diagnosed 2 years previously.

Auditory brain response testing revealed right hearing of 30 dB above normal adult hearing level (nHL) and left hearing of 70 dB above nHL. The patients hearing had been checked regularly at 6 to 12 months of age. Auditory impedance showed type A in the right ear and type B in the left ear.

The patient was initially diagnosed with acute otitis media, and appropriate antibiotic and analgesic treatment was started. However, her otalgia and otorrhea were unresolved after 5 days of treatment, at which point a temporal bone CT scan was performed, revealing destruction of the left external auditory canal at the inferior and posterior wall, as well as a blunted scutum (figure 2 A and B). The external auditory canal and middle ear cavity were filled with a soft-tissue density, and the ossicles were eroded.

We suspected cholesteatoma in the left external auditory canal and middle ear, although initially the patient was considered too young to have developed this lesion. However, diffusion-weighted magnetic resonance imaging (DW-MRI) and T2-weighted MRI confirmed cholesteatoma in the external auditory canal and middle ear (figure 2, C and D). The upper external auditory canal extended to the tympanic membrane, but the lower external auditory canal ended with a blunted canal.

The patient underwent a canal-wall-down mastoidectomy since the canal wall was already destroyed, and the cholesteatoma was found to extend into the sinus tympani and facial recess. There were no postoperative complications, and the wound was completely healed 2 months after surgery. During 6 months of follow-up, no recurrence was noted.

We are unsure of the cause of such rapid development of the cholesteatoma in this case, but we believe the lesion originated from the external auditory canal rather than the middle ear or mastoid, since the destruction of the external auditory canal was more severe than that of the middle ear or antrum. In addition, the attic area was relatively preserved although the scutum was blunted. Development of cholesteatoma normally requires a certain amount of time in congenital aural stenosis; therefore, it is possible that an epithelial plug that already existed during the developmental stage at failure of recanalization might have caused the cholesteatoma. (3) In addition, chronic infection might make a cholesteatoma more destructive. (4)

CT imaging is not usually performed in children younger than 4 years because of an increased risk of cancer in younger patients. A recent study showed that DW-MRI is a reliable diagnostic tool for identifying residual or recurrent cholesteatoma. (5)

This case demonstrates that cholesteatoma can develop in very young children with congenital aural stenosis and a duplication anomaly, and physicians should consider this condition in affected children with otalgia and otorrhea. Finally, DW-MRI is a useful screening tool to confirm this diagnosis in young children.



(1.) Cole RR, Jahrsdoerfer RA. The risk of cholesteatoma in congenital aural stenosis. Laryngoscope 1990;100(6):576-8.

(2.) Zhao S, Han D, Wang D, et al. The formation of sinus in congenital stenosis of external auditory canal with cholesteatoma. Acta Otolaryngol 2008;128(8):866-70.

(3.) Levine JL, Wright CG, Pawlowski KS, Meyerhoff WL. Postnatal persistence of epidermoid rests in the human middle ear. Laryngoscope 1998;108(1 Pt l):70-3.

(4.) Yamane H, Takayama M, Sunami K, et al. Disregard of cholesteatoma in congenital aural stenosis. Acta Otolaryngol 2007;127(2): 221-4.

(5.) Jindal M, Riskalla A, Jiang D, et al. A systematic review of diffusion-weighted magnetic resonance imaging in the assessment of postoperative cholesteatoma. Otol Neurotol 2011;32(8):1243-49.

From the Department of Otolaryngology-Head and Neck Surgery, Seoul National University College of Medicine, Seoul, Korea.

Funding: This research was supported by the Basic Science Research Program through the National Research Foundation of Korea (NRF), funded by the Ministry of Science, ICT and Future Planning (NRF-2012R1A1A1013003).
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Author:Park, Moo Kyun
Publication:Ear, Nose and Throat Journal
Article Type:Report
Geographic Code:9SOUT
Date:Apr 1, 2015
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