title: Cochlear implant and congenital cholesteatoma.
abstract: 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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Otolaryngology, Audiology and Phoniatrics, Children's Hospital of Bydgoszcz, Chodkiewicza 44, 85-667, Bydgoszcz, Poland. jmierzw@gmail.com.
authors.forename: J
authors.initials: J
authors.lastname: Mierzwinski
authors.affiliation: Department of Otolaryngology, Audiology and Phoniatrics, Children's Hospital of Bydgoszcz, Chodkiewicza 44, 85-667, Bydgoszcz, Poland. ajfishman@mac.com.
authors.forename: A J
authors.initials: AJ
authors.lastname: Fishman
authors.affiliation: Department of Otolaryngology, Audiology and Phoniatrics, Children's Hospital of Bydgoszcz, Chodkiewicza 44, 85-667, Bydgoszcz, Poland. tomek.grochowski.q45@gmail.com.
authors.forename: T
authors.initials: T
authors.lastname: Grochowski
authors.affiliation: Department of Otolaryngology, Audiology and Phoniatrics, Children's Hospital of Bydgoszcz, Chodkiewicza 44, 85-667, Bydgoszcz, Poland. sylwiadrewa@o2.pl.
authors.forename: S
authors.initials: S
authors.lastname: Drewa
authors.affiliation: Department of Otolaryngology, Audiology and Phoniatrics, Children's Hospital of Bydgoszcz, Chodkiewicza 44, 85-667, Bydgoszcz, Poland. maria.drela@gmail.com.
authors.forename: M
authors.initials: M
authors.lastname: Drela
authors.affiliation: Department of Otolaryngology, Head and Neck Surgery, University Hospital of Bydgoszcz, Ujejskiego 52, 85-168, Bydgoszcz, Poland. piotrwiniarski26@wp.pl.
authors.forename: P
authors.initials: P
authors.lastname: Winiarski
authors.affiliation: Department of Pediatric Otolaryngology, University Children's Hospital of Katowice, ul Medyków 16, 40-752, Katowice, Poland. irekbess@o2.pl.
authors.forename: I
authors.initials: I
authors.lastname: Bielecki
mesh_terms: D018424:Cholesteatoma, Middle Ear / Q000151:congenital / Q000175:diagnosis / Q000601:surgery*; D003054:Cochlear Implants; D005260:Female; D006309:Hearing / Q000502:physiology*; D006801:Humans; D007223:Infant; D053685:Laser Therapy / Q000379:methods*; D008279:Magnetic Resonance Imaging
references.citation: Laryngoscope. 1994 Mar;104(3 Pt 2):1-24
references.pmid: 8127199
references.citation: Otolaryngol Clin North Am. 1989 Oct;22(5):941-54
references.pmid: 2694070
references.citation: Arch Otolaryngol. 1961 Mar;73:252-61
references.pmid: 13691781
references.citation: Otol Neurotol. 2005 Mar;26(2):135-9
references.pmid: 15793394
references.citation: Laryngoscope. 2008 Mar;118(3):517-21
references.pmid: 18091330
references.citation: Otol Neurotol. 2009 Jan;30(1):54-8
references.pmid: 19092558
references.citation: B-ENT. 2009;5(4):233-40
references.pmid: 20163049
references.citation: Eur Ann Otorhinolaryngol Head Neck Dis. 2010 Sep;127(4):143-50
references.pmid: 20860924
references.citation: Otolaryngol Clin North Am. 2012 Feb;45(1):1-24
references.pmid: 22115679
references.citation: Cochlear Implants Int. 2013 Jan;14(1):32-5
references.pmid: 22450542
references.citation: Curr Opin Otolaryngol Head Neck Surg. 2013 Oct;21(5):431-9
references.pmid: 23995327
references.citation: Laryngoscope. 2014 Jul;124(7):1694-9
references.pmid: 24390811
references.citation: Int J Pediatr Otorhinolaryngol. 1986 Apr;11(2):113-27
references.pmid: 2427466
references.citation: Otol Neurotol. 2004 Sep;25(5):730-9
references.pmid: 15354004
references.citation: Arch Otolaryngol Head Neck Surg. 2002 Jul;128(7):804-9
references.pmid: 12117340
references.citation: Laryngoscope. 2000 Nov;110(11):1890-7
references.pmid: 11081605
references.citation: Zhonghua Er Bi Yan Hou Ke Za Zhi. 1993;28(4):228-30, 252-3
references.pmid: 8167044
references.citation: Laryngoscope. 1987 Mar;97(3 Pt 2 Suppl 40):2-14
references.pmid: 3821363
references.citation: Ann Otol Rhinol Laryngol. 1965 Sep;74(3):706-27
references.pmid: 5846535
references.citation: Otolaryngol Head Neck Surg. 1986 Jun;94(5):560-7
references.pmid: 3088516