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Cholesteatoma in patients with congenital external auditory canal anomalies: retrospective review

Published online by Cambridge University Press:  16 August 2011

A Mazita ,
M Zabri ,
W H Aneeza ,
A Asma  and
L Saim
A Mazita*
Affiliation:
Department of Otorhinolaryngology-Head and Neck Surgery, Universiti Kebangsaan Malaysia Medical Centre, Kuala Lumpur, Malaysia
M Zabri
Affiliation:
Department of Otorhinolaryngology-Head and Neck Surgery, Universiti Kebangsaan Malaysia Medical Centre, Kuala Lumpur, Malaysia
W H Aneeza
Affiliation:
Department of Otorhinolaryngology-Head and Neck Surgery, Universiti Kebangsaan Malaysia Medical Centre, Kuala Lumpur, Malaysia
A Asma
Affiliation:
Department of Otorhinolaryngology-Head and Neck Surgery, Universiti Kebangsaan Malaysia Medical Centre, Kuala Lumpur, Malaysia
L Saim
Affiliation:
Department of Otorhinolaryngology-Head and Neck Surgery, Universiti Kebangsaan Malaysia Medical Centre, Kuala Lumpur, Malaysia
*
Address for correspondence: Dr A Mazita, Senior Lecturer and ORL Surgeon, Department of Otorhinolaryngology-Head and Neck Surgery, Universiti Kebangsaan Malaysia Medical Centre, Cheras 56000, Kuala Lumpur, Malaysia Fax: +603 91456675 E-mail: mazitaami@yahoo.com
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Abstract

Objective:

To review cases of congenital external auditory canal anomaly with cholesteatoma, documenting clinical presentation, cholesteatoma site and extent, complications, and surgery.

Method:

Retrospective review of all cases of congenital canal anomaly with cholesteatoma treated between 1998 and 2009.

Results:

Of 41 cases with canal anomalies, 17 (43.9 per cent) had associated cholesteatoma. Medical records were unretrievable for four cases. Of the remaining 13 patients (five females and eight males, age range four to 73 years, mean 21 years), 10 presented chiefly with recurrent otorrhoea, two with postauricular discharge from mastoid abscess, and one with otalgia, postauricular tenderness and neck stiffness. Hearing loss was conductive in 10 patients (76.9 per cent) and sensorineural (severe to profound) in three. No facial nerve palsy was documented. Cholesteatoma was seen in all cases on high resolution computed tomography, and confirmed intra-operatively. Six patients underwent canalplasty with split skin grafting, and seven modified radical mastoidectomy. Six patients recovered well, two needed repeated canalplasty for soft tissue restenosis, and five needed cautery and split skin grafting for mastoid cavity granulation tissue.

Conclusion:

Congenital canal anomaly is uncommon. Canal cholesteatoma should be suspected in all cases, and high resolution temporal bone computed tomography undertaken in all patients aged four years or more. In patients with otorrhoea, the risk of cholesteatoma is greater. Treatment is generally surgery; the type depends on the disease extent.

Keywords

AbnormalitiesEarExternalAtresiaStenosisCholesteatomaMeatoplasty

Information

Type
Main Articles
Information
The Journal of Laryngology & Otology , Volume 125 , Issue 11 , November 2011 , pp. 1116 - 1120
Copyright
Copyright © JLO (1984) Limited 2011

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References

1De la Cruz, A, Fayad, JN. Congenital aural atresia. In: Nadol, JB, McKenna, MJ, eds. Surgery of the Ear and Temporal Bone. Philadelphia: Lippincott Williams & Wilkins, 2005;325–35Google Scholar
2Cole, RR, Jahrsdoerfer, RA. The risk of cholesteatoma in congenital aural stenosis. Laryngoscope 1990;100:576–8CrossRefGoogle ScholarPubMed
3Jafek, BW, Nager, GT, Strife, J, Gayler, RW. Congenital aural atresia: an analysis of 311 cases. Trans Sect Otolaryngol Am Acad Ophthalmol Otolaryngol 1975;80:588–95Google ScholarPubMed
4Jahrsdoefer, RA. Congenital atresia of the ear. Laryngoscope 1978;88:1–48Google Scholar
5Ghosh, A, Saha, S, Sandhu, A, Saha, PV. Imaging of congenital cholesteatoma with atretic ear – a rare case report. Ind J Radiol Imag 2006;4:673–5Google Scholar
6Caughney, RJ, Jahrsdoefer, RA, Kesser, BW. Congenital cholesteatoma in a case of congenital aural atresia. Otol Neurotol 2006;27:934–6CrossRefGoogle Scholar
7Glasscock, ME 3rd, Schwaber, MK, Nissen, AJ, Jackson, CG. Management of congenital ear malformations. Ann Otol Rhinol Laryngol 1983;92:504–9CrossRefGoogle ScholarPubMed
8De la Cruz, A, Linthicum, FH Jr, Luxford, WM. Congenital atresia of the external auditory canal. Laryngoscope 1985;95:421–7CrossRefGoogle ScholarPubMed
9Altmann, F. Congenital atresia of the ear in man and animals. Ann Otol Rhinol Laryngol 1955;64:824–58CrossRefGoogle Scholar
10Vrabec, JT, Chaljub, G. External canal cholesteatoma. Am J Otolaryngol 2000;21:608–14Google ScholarPubMed
11Michaels, L. An epidermoid formation in the developing middle ear: possible source of cholesteatoma. J Otolaryngol 1986;15:169–74Google ScholarPubMed
12Sade, J, Babiacki, A, Pinkus, G. The metaplastic and congenital origin of cholesteatoma. Acta Otolaryngol 1983;96:119–29CrossRefGoogle ScholarPubMed
13Tasar, M, Yetiser, S, Yildirim, D, Bozlar, U, Tasar, MA, Saglam, M et al. Preoperative evaluation of the congenital aural atresia on computed tomography: an analysis of the severity of the deformity of the middle ear and mastoid. E J Rad 2007;62:97–105CrossRefGoogle ScholarPubMed
14Griffin, C, Delapaz, R, Euzmann, E. MR and CT correlation of cholesterol cysts of the petrous bone. AJNR Am J Neuroradiol 1987;8:825–9Google ScholarPubMed
15Aarts, MCJ, Rogers, MM, van der Veen, EL, Schilder, AGM, van der Heijden, GJM, Grolman, W. The diagnostic value of diffusion-weighted MRI in detecting a residual cholesteatoma. Otolaryngol Head Neck Surg 2010;143:12–16CrossRefGoogle ScholarPubMed
16Declau, F, Cremers, C, Van de Heyning, P. Diagnosis and management strategies in congenital atresia of the external auditory canal. Study Group on Otological Malformations and Hearing Impairment. Br J Audiol 1999;33:313–27CrossRefGoogle Scholar
17Jahrsdoerfer, RA, Yeakley, JW, Aguilar, EA, Cole, RR, Gray, LC. Grading system for the selection of patients with congenital aural atresia. Am J Otol 1992;13:6–12Google ScholarPubMed