Surgical Treatment of Middle Ear Cholesteatoma in Children with Down Syndrome

Abstract

To report our personal experience in the surgical treatment of cholesteatoma in children with Down syndrome. Retrospective study. Tertiary care otology and skull base centers. Nine patients with Down syndrome were surgically treated for cholesteatoma. Two patients had bilateral disease, resulting in a total of 11 ears surgically treated. A canal-wall-up mastoidectomy was performed in two ears; in eight of the ears, a canal-wall-down mastoidectomy was carried out and a modified Bondy procedure was performed in one ear. Residual cholesteatoma was found in one ear after the canal-wall-up mastoidectomy and recurrent cholesteatoma developed in another ear, also after canal-wall-up mastoidectomy. The recurrence required conversion to canal-wall-down mastoidectomy. One patient developed a perforation of the neotympanic membrane that had to be revised. Cholesteatoma in children with Down syndrome is a challenging entity for the otologic surgeon. Otolaryngologists should always suspect a cholesteatoma in each child with Down syndrome presenting warning symptoms such as otorrhea and hearing loss. If there is any doubt on inspection, further imaging studies (high-resolution computed tomography) are necessary. To the best of our knowledge, the current study is the first report to document the surgical treatment of cholesteatoma in subjects with Down syndrome.