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Congenital cholesteatoma and cochlear implantation: Implications for management.
Congenital cholesteatoma and cochlear implantation: Implications for management.
- Source :
-
Cochlear implants international [Cochlear Implants Int] 2013 Jan; Vol. 14 (1), pp. 32-5. - Publication Year :
- 2013
-
Abstract
- Introduction: 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.<br />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.<br />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.<br />Discussion: 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.
- Subjects :
- Anion Transport Proteins genetics
Child, Preschool
Cholesteatoma diagnostic imaging
Cholesteatoma epidemiology
Cholesteatoma rehabilitation
Comorbidity
Cross-Sectional Studies
DNA Mutational Analysis
Deafness diagnostic imaging
Deafness epidemiology
Ear, Inner abnormalities
Ear, Middle abnormalities
Follow-Up Studies
Genetic Testing
Goiter, Nodular diagnostic imaging
Goiter, Nodular epidemiology
Goiter, Nodular genetics
Goiter, Nodular rehabilitation
Hearing Loss, Sensorineural diagnostic imaging
Hearing Loss, Sensorineural epidemiology
Hearing Loss, Sensorineural genetics
Hearing Loss, Sensorineural rehabilitation
Humans
Incidental Findings
Male
Multidetector Computed Tomography
Retrospective Studies
Sulfate Transporters
Cholesteatoma genetics
Cochlear Implantation
Deafness genetics
Deafness rehabilitation
Subjects
Details
- Language :
- English
- ISSN :
- 1754-7628
- Volume :
- 14
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Cochlear implants international
- Publication Type :
- Academic Journal
- Accession number :
- 22450542
- Full Text :
- https://doi.org/10.1179/1754762812Y.0000000001