Abstract | INTRODUCTION: METHODS: 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. 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.
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Authors | Janet Chung, Sharon L Cushing, Adrian L James, Karen A Gordon, Blake C Papsin |
Journal | Cochlear implants international
(Cochlear Implants Int)
Vol. 14
Issue 1
Pg. 32-5
(Jan 2013)
ISSN: 1754-7628 [Electronic] England |
PMID | 22450542
(Publication Type: Journal Article)
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Chemical References |
- Anion Transport Proteins
- SLC26A1 protein, human
- Sulfate Transporters
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Topics |
- Anion Transport Proteins
(genetics)
- Child, Preschool
- Cholesteatoma
(diagnostic imaging, epidemiology, genetics, rehabilitation)
- Cochlear Implantation
- Comorbidity
- Cross-Sectional Studies
- DNA Mutational Analysis
- Deafness
(diagnostic imaging, epidemiology, genetics, rehabilitation)
- Ear, Inner
(abnormalities)
- Ear, Middle
(abnormalities)
- Follow-Up Studies
- Genetic Testing
- Goiter, Nodular
(diagnostic imaging, epidemiology, genetics, rehabilitation)
- Hearing Loss, Sensorineural
(diagnostic imaging, epidemiology, genetics, rehabilitation)
- Humans
- Incidental Findings
- Male
- Multidetector Computed Tomography
- Retrospective Studies
- Sulfate Transporters
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