Cochlear implant and congenital cholesteatoma

Abstract

BackgroundThe 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 presentationWe 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.ConclusionWe 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.

Periotic congenital cholesteatoma – A rare case report

“…Levenson, later in 1989, updated the criteria as the presence of uncomplicated acute otitis media does not exclude congenital cholesteatoma. 4 Congenital cholesteatoma is said to be due to inadequate folding of the epidermoid inside the middle ear cleft and accumulation of stratified squamous epithelium through the third and fifth weeks of gestation and slowly grows over time.…”

“…According to Nager congenital cholesteatoma is least common in mastoid process. 4 Congenital cholesteatoma of the temporal bone is rare while the involvement of petrous apex is very rare. A search conducted on 19 th March 2020 in google scholar with keywords as "congenital cholesteatoma" and "India.…”

Congenital Cholesteatoma: The Silent Pathology

“…In some patients with no labyrinthine destruction or intracranial extension, a canal wall down mastoidectomy was performed. In patients with middle ear lesions or ossicular destruction without extramastoid extensions or infection, an ossiculoplasty was performed in the same stage [3,4,6,[12][13][14][15][16][19][20][21][22][23][24][25].…”