Table 2 Hearing tests and CT data of selected subjects from the 2011 and 2014 Alagille Alliance meetings.
From: Requirement for Jagged1-Notch2 signaling in patterning the bones of the mouse and human middle ear
Subject | Age | Mutation | Ear | Audiologic Data | CT Data | ||
|---|---|---|---|---|---|---|---|
Type of Hearing Loss | Degree of Hearing Loss | Tympanogram | Affected Structures | ||||
1 | 16 | JAG1 | R | — | — | normal | PSCC, CoA |
L | — | — | normal | OW, PSCC, CoA | |||
2a | 9 | JAG1 | R | sens | mild | normal | SSCC, PSCC |
L | — | — | normal | incus | |||
3 | 8 | JAG1 | R | mixed | mild | negative ME pressure/normal compliance | OW, RW, SSCC, PSCC |
L | mixed | mild-severe | negative ME pressure/normal compliance | OW, RW, SSCC, PSCC | |||
4 | 7 | N.D. | R | — | — | normal | PSCC, CoA |
L | cond | mild | normal | PSCC | |||
5b | 49 | JAG1 | R | sens | normal-mild | normal | stapes, SSCC, PSCC, CoA |
L | mixed | mild-profound | normal | stapes, SSCC, PSCC, CoA | |||
