Summary of inner ear findings in patients with branchio-oto-renal syndrome
| Patients with BOR | Hearing Loss | Unwound Cochlea (Reader 1) | Unwound Cochlea (Reader 2) | Other Inner Ear Abnormalities |
|---|---|---|---|---|
| 1 | B Mixed | Bilateral | Bilateral | R vestibular aqueduct enlargement; B medialized facial nerve; B funnel IAC |
| 2 | B Mixed | Bilateral | Bilateral | B hypoplastic apical turn/modiolus; right hypoplastic posterior semicircular canal; B funnel IAC |
| 3 | B Conductive | Bilateral | Bilateral | B medialized facial nerve; B funnel IAC |
| 4 | B Conductive | Bilateral | Bilateral | B vestibular aqueduct enlargement; B funnel IAC |
| 5 | B Conductive | Bilateral | Bilateral | L vestibular aqueduct enlargement; B hypoplastic lateral semicircular canal; L medialized facial nerve; B funnel IAC |
| 6 | B Sensorineural | Bilateral | Bilateral | B hypoplastic apical turn/modiolus; B cochlear aperture stenosis; B vestibular aqueduct enlargement; B medialized facial nerve; L funnel IAC |
| 7 | B Sensorineural | No | No | B cochlear hypoplasia with absent middle and apical turns; B medialized facial nerve |
| 8 | B Mixed | Bilateral | No | B medialized facial nerve |
| 9 | B Sensorineural | Bilateral | Bilateral | B hypoplastic apical turn/modiolus; B vestibular aqueduct enlargement; B medialized facial nerve; B funnel IAC |
Note:—IAC indicates internal auditory canal; R, right side; L, left side; B, bilateral.