Table 2.
Distribution of SLC26A4 mutations in the different groups
| Inner ear image | No mutation | Monoallelic mutation | Biallelic mutation | Total |
|---|---|---|---|---|
| MD | 27 (96.4%) | 1 (3.6%) | 0 | 28 (100%) |
| EVA with MD | 1 (2.0%) | 3 (6.0%) | 46 (92.0%) | 50 (100%) |
| EVA | 1 (2.0%) | 3 (6.0%) | 46 (92.0%) | 50 (100%) |
| IEM | 14 (87.5%) | 2 (12.5%) | 0 | 16 (100%) |
| Total | 43 | 9 | 92 | 144 |
MD: Isolated Mondini dysplasia, EVA with MD: Enlarged vestibular aqueduct with Mondini dysplasia, EVA: enlarged vestibular aqueduct without Mondini dysplasia, IEM: Other types of inner ear malformations