Closure of Congenital Semicircular Dehiscence in the First 10 Years of Life
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Published on: February 29, 2020
Katja Döring1, Heinrich Lanfermann1, Athanasia Warnecke2
1Hannover Medical School, Department of Diagnostic and Interventional Neuroradiology, NDS, Germany, Hanover.
Insights
Semicircular canal dehiscence (SCD) in young children often resolves with skull growth by puberty, suggesting it is acquired rather than congenital. This indicates that clinically significant SCD in adults is likely acquired, not present since childhood.
Area of Science:
- Otolaryngology
- Pediatric Radiology
- Developmental Biology
Background:
- Semicircular canal dehiscence (SCD) is a rare condition affecting the inner ear.
- Its long-term progression and developmental changes, especially in children, are not well understood.
- Understanding SCD progression is crucial for diagnosing and managing hearing loss and related symptoms.
Purpose of the Study:
- To assess the long-term progression of semicircular canal dehiscence (SCD) in young children.
- To determine if SCD size changes or ossifies during physical development.
- To evaluate the theory of acquired pathogenesis for SCD.
Main Methods:
- Retrospective study of HRCT scans from children under three years old with follow-up imaging at least two years later.
- Analysis of demographic data, clinical symptoms, and imaging findings of SCD.
- Categorization of SCD by location and severity, evaluating long-term development.
Main Results:
- Of 93 eligible children, 46 SCDs were identified at baseline (average age 11.3 months).
- Follow-up imaging (2-13 years later) showed bone coverage over the dehiscence site, with a mean thickness of 1 mm.
- SCD prevalence was highest between 0-12 months and decreased with age.
Conclusions:
- Semicircular canal dehiscence in young children is typically covered by normal bone growth by puberty.
- This supports an acquired pathogenesis, suggesting clinically significant SCD in adults is likely acquired.
- SCD is more common in younger age groups and more frequent in the superior than posterior canal.
Purpose:
To assess the long-term progression of semicircular canal dehiscence (SSCD), particularly with regard to size expansion or possible ossification during physical development.
Materials And Methods:
This retrospective study screened all patients under the age of three who underwent HRCT between 2006 and 2013 for varies indications, e.g. diagnosis of sensorineural hearing loss (SNHL), acute mastoiditis, or to evaluate for cochlear implantation. The study population was also screened for the presence of follow-up imaging. Only patients with imaging data available after at least two years or more were included in the study. Demographic data, clinical symptoms, and imaging findings were analyzed using descriptive statistics. The imaging findings of semicircular canal dehiscence were categorized by location and severity, and the long-term development was evaluated. More than 1500 HRCTs of the temporal bone taken in children ages 0 to 10 years old from 2006 to 2025 were additionally examined for the presence of SCC dehiscence.
Results:
Of the 586 HRCT scans performed on young patients under three years of age, 93 examinations for which follow-up examinations were available two or more years later could be recorded. These were distributed among 32 children with an average age of 11.3 ± 7.4 months (range 3-24 months). At the time of initial imaging (baseline examination), 46 SCDs of the superior or posterior semicircular canal were identified. A total of 36 ears were affected. In the subsequent HRCT examination, conducted 2-13 years later, the extent of bone coverage was consistently quantifiable. The mean thickness of the bone over the site of the previous dehiscence was 1 mm (range 0.2-3.3 mm).
Conclusion:
In the vast majority of cases, the SCC dehiscence observed in studies of the temporal bones of young children will be covered by normal bone growth by puberty. This suggests that SCC dehiscence does not persist from birth into adulthood, and instead it supports the theory of an acquired pathogenesis of SCC dehiscence.
Key Points:
· In children, SSCD is most likely to be completely covered by skull growth.. · Clinically significant SSCD in adults is most likely to be acquired (not present since childhood).. · There is a high prevalence of SCCD between the ages of 0-6 and 6-12 months.. · SSCD occurs more frequently than PSCD overall.. · The prevalence of SCCD decreases as age increases..
Citation Format:
· Döring K, Lanfermann H, Warnecke A et al. Closure of Congenital Semicircular Dehiscence in the First 10 Years of Life. Rofo 2025; DOI 10.1055/a-2725-8648.
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