title: Congenital cholesteatoma and cochlear implantation: Implications for management.
abstract: BACKGROUND
  Cholesteatoma is an uncommon condition that has occasionally been associated with cochlear implantation (CI). Cases of secondary acquired cholesteatoma have been described, in which intra-operative breech of the posterior canal wall is thought to be a contributing factor. Primary acquired cholesteatoma is not typically associated with congenital sensorineural hearing loss (SNHL) or CI in children. Congenital cholesteatoma is a rarer entity yet with an incidence in the literature of 2‐4% of all cholesteatomas. We present lessons learned from our experience of congenital cholesteatoma in CI candidates.
  
  
  METHODS
  Retrospective reviews of departmental CI and cholesteatoma databases in a tertiary/quaternary pediatric center were conducted. Cases of congenital cholesteatoma were identified. The proportion of congenital cholesteatoma cases in CI candidates was compared with number of acquired cholesteatoma. Optimum management of congenital cholesteatoma in CI candidates was reviewed.
  
  
  RESULTS
  In our pediatric CI population, 2/794 patients (0.25%) were recognized as having a congenital cholesteatoma during their evaluation for CI. No cases of primary acquired cholesteatoma were identified in this population at presentation or at follow up to 18 years.
  
  
  CONCLUSIONS
  The 0.25% incidence of congenital cholesteatoma in our population of CI patients is higher than expected of this rare condition. It is surprisingly common given the absence of any cases of primary acquired cholesteatoma, which is considerably more common even in the pediatric population. Both patients likely had an inherited form of hearing loss and a genetic contribution to the presence of congenital cholesteatoma cannot be excluded. The presence of congenital cholesteatoma has implications for the algorithm currently employed for the assessment of CI. We consider that surgery should be staged to ensure complete removal of the cholesteatoma before implantation. Thus bilateral CI should be provided sequentially rather than simultaneously in the presence of unilateral cholesteatoma.
authors.affiliation: Department of Otolaryngology–Head and Neck Surgery, University of Toronto, Toronto, Ontario, Canada. janchung@gmail.com
authors.forename: Janet
authors.initials: J
authors.lastname: Chung
authors.forename: Sharon L
authors.initials: SL
authors.lastname: Cushing
authors.forename: Adrian L
authors.initials: AL
authors.lastname: James
authors.forename: Karen A
authors.initials: KA
authors.lastname: Gordon
authors.forename: Blake C
authors.initials: BC
authors.lastname: Papsin
mesh_terms: D027321:Anion Transport Proteins / Q000235:genetics; D002675:Child, Preschool; D002781:Cholesteatoma / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000235:genetics* / Q000534:rehabilitation; D019929:Cochlear Implantation; D015897:Comorbidity; D003430:Cross-Sectional Studies; D004252:DNA Mutational Analysis; D003638:Deafness / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000235:genetics* / Q000534:rehabilitation*; D007758:Ear, Inner / Q000002:abnormalities; D004432:Ear, Middle / Q000002:abnormalities; D005500:Follow-Up Studies; D005820:Genetic Testing; D006044:Goiter, Nodular / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000235:genetics / Q000534:rehabilitation; D006319:Hearing Loss, Sensorineural / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000235:genetics / Q000534:rehabilitation; D006801:Humans; D033162:Incidental Findings; D008297:Male; D061330:Multidetector Computed Tomography; D012189:Retrospective Studies; D000076988:Sulfate Transporters
chemical_list: D027321:Anion Transport Proteins; C473560:SLC26A1 protein, human; D000076988:Sulfate Transporters