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

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.