Cochlear implant and congenital cholesteatoma

BACKGROUND: The occurence of cholesteatoma and cochlear implant is rare. Secondary cholesteatomas may develop as a result of cochlear implant surgery. Primarily acquired cholesteatoma is not typically associated with congenital sensorineural hearing loss or cochlear implant in children. The occurrence of congenital cholesteatoma during cochlear implant surgery has never been reported before, partly because all patients are preoperatively submitted to imaging studies which can theoretically exclude the disease. CASE PRESENTATION: We have reported a rare case of congenital cholesteatoma, found during sequential second side cochlear implantation in a 3-year-old child. The child underwent a computed tomography (CT) scan and magnetic resonance imaging (MRI) at 12 months of age, before the first cochlear implant surgery, which excluded middle ear pathology. The mass was removed as an intact pearl, without visible or microscopic violation of the cholesteatoma capsule. All the areas where middle ear structures were touching the cholesteatoma were vaporized with a laser and the cochlear implant was inserted uneventfully. Further follow-up excluded residual disease. CONCLUSION: We believe that primary, single stage placement of a cochlear implant (CI) with simultaneous removal of the congenital cholesteatoma can be performed safely. However, to prevent recurrence, the capsule of the cholesteatoma must not be damaged and complete laser ablation of the surface, where suspicious epithelial cells could remain, is recommended. In our opinion, cholesteatoma removal and cochlear implantation should be staged if these conditions are not met, and/or the disease is at a more advanced stage. It is suspected, that the incidence of congenital cholesteatoma in pediatric CI candidates is much higher that in average pediatric population.