Surgical Treatment of Middle Ear Cholesteatoma in Children with Down Syndrome

OBJECTIVES: To report our personal experience in the surgical treatment of cholesteatoma in children with Down syndrome. STUDY DESIGN: Retrospective study. SETTING: Tertiary care otology and skull base centers. PATIENTS: Nine patients with Down syndrome were surgically treated for cholesteatoma. Two patients had bilateral disease, resulting in a total of 11 ears surgically treated. INTERVENTION: 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. RESULTS: 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. CONCLUSIONS: 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.