Table 1.
Inner-ear defects in Eya1bor/+, Eya1+/−, Eya1bor/bor and Eya1bor/− hypomorphic mutant embryos at E18.5
| Abnormalities | bor/+, n = 30 (15) | +/−, n = 44 (22) | bor/bor, n = 40 (20) | bor/−, n = 20 (10) | |
|---|---|---|---|---|---|
| Endolymphatic duct/sac | 0 | 3a (4) | 20a (16) | 7b (5), 7c (7) | |
| Semicircular canals | |||||
| Anterior | 0 | 2a (2) | 22a (13) | Formed one canal | |
| Posterior | 0 | 2a (2) | 40b (20) | ||
| Lateral | 0 | 0 | 0 | ||
| Ampulla | |||||
| Anterior | 0 | 1a (1) | 22b (13), 18c (11) | 20b (10) | |
| Posterior | 0 | 1a (1) | 40b (20) | 20b (10) | |
| Lateral | 0 | 40c (20) | 20c (10) | ||
| Saccule | 0 | 5c (5) | 40c (20) | 16b (9), 4c (3) | |
| Utricle | 0 | 0 | 40c (20) | 20c (10) | |
| Cochlea | 0 | 15d (9) | 40d (20) | 20b (10) |
n, number of ears (number of embryos).
Fifteen ears of 44 Eya1+/− ears (nine of 22 mice) in C3HeB/FeJ strain showed shortened or/and malformed cochlea at E18.5. Among them, two ears (from two embryos) had most severe phenotype: one ear retained only about one-half turn and another ear retained about three-quarter turn. Majority of the affected ears lost around one-half turn of the cochlea and exhibited variable malformations of inner-ear structures. In contrast, all Eya1bor/bor and Eya1bor/− embryos in the same strain showed more severe inner-ear defects.
aTruncated.
bAbsent.
cMalformed.
dShortened.