Bilateral congenital cholesteatoma: Surgical treatment and considerations

To describe a multicenter study regarding surgical management of bilateral congenital cholesteatoma (BCC) and underline the importance of endoscopes in the management of this condition. In BCC, hearing preservation is more crucial than in unilateral cases. The endoscopic approach allows complete removal of cholesteatoma via a minimally invasive technique offering low residual disease rates while preserving the normal physiology of the middle ear and possibly the ossicular chain.

Retrospective chart and surgical video review of patients with BCC who underwent surgery at Otolaryngology Department of Modena and Verona University Hospitals and the Hospital for Sick Children, Toronto.

From 2002 to November 2016, six patients were identified with bilateral congenital cholesteatoma and included in this study. Pre-operative assessments, surgical treatments and outcomes were collected and described.

The median age at presentation was 4 years (range 2-7 years). A microscopic post auricular tympanoplasty was performed in two ears, four underwent a canal wall up mastoidectomy procedure and in the other six a transcanal endoscopic approach (TEA) was used. No intra- or post-operative complications were observed in any patients. The mean follow up period was 54.5 months.

When both ears are involved with congenital cholesteatoma, it is particularly important to use a minimally invasive technique that preserves normal ossicular and mastoid structure and function whenever possible. In many cases this can be achieved with TEA, even in young children. In addition the endoscope allows good surgical control of cholesteatoma removal from hidden recesses.