Congenital cholesteatoma and cochlear implantation: Implications for management
Bibliographic record
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. 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. 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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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.002 | 0.009 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.002 | 0.002 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.002 | 0.003 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.003 | 0.002 |
| Insufficient payload (model declined to judge) | 0.003 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".