Table 2:
Patients with cochlear hypoplasiaa
| Patient | Age (yr) Sex | L CH (mm) | R CH (mm) | TYPE HL | Diagnosis | CT Findings |
|---|---|---|---|---|---|---|
| 1 | 6.3 Female | 2.8 | 2.5 | SNHL, bilaterally | Bilateral vestibulocochlear dysplasia, L Mondini malformation | Bilateral vestibulocochlear dysplasia |
| R common chamber malformation, absent vestibular aqueduct, aplastic modiolus | ||||||
| L Mondini malformation (hypoplastic cochlea with partition defect) | ||||||
| 2 | 1.9 Male | 3.4 | 3.6 | Mixed HL | BOR syndrome | Bilateral dilated vestibular aqueduct with small modiolus, trumpet-shaped IAC, small mass in R middle ear (possible congenital cholesteatoma) |
| 3 | 0.8 Female | 3.7 | 3.5 | Mixed HL | BOR syndrome | Bilateral tympanostomy tubes, hypoplasia of modiolus, vestibular ectasia, dilated vestibular aqueducts, Mondini deformities (cochlear ectasia with partition defects), question of ossicular fusion |
| R middle ear and mastoid air cell opacification; absent vs hypoplastic stapes | ||||||
| L hypoplastic mastoid with soft tissue thickening at L mesotympanum | ||||||
| 4 | 6.7 Female | 3.6 | 4.3 | Mixed HL, bilaterally | CHARGE syndrome | Bilateral hypoplastic SCCs, prominent EAC, vestibular dysplasia, cochlear ectasia with partition defects, hypoplastic stapes |
| R sclerosis of ossicles, poorly visualized oval window, L poorly defined modiolus | ||||||
| 5 | 0.1 Male | 4.2 | 3.8 | Mixed HL, bilaterally | CHARGE syndrome | Bilateral absent SCCs, middle ear/mastoid air cell congestion or inflammation, normal middle ear morphology |
| 6 | 6.6 Male | 4.0 | 4.5 | SNHL, bilaterally | EVA syndrome | Bilateral enlarged vestibular aqueducts, R cochlear implant, L hypoplastic modiolus |
| 7 | 7.1 Male | 4.2 | 4.3 | CHL, bilaterally | Bilateral class II microtia and EAC atresia | R malformed IAC, partially formed middle ear cavity, poorly defined ossicles, narrow EAC, normal SCCs/vestibule |
| L absent lateral SCC, vestibular dysplasia, ossicular dysplasia, absent membranous EAC, normal cochlea |
Note:—R indicates right; L, left; IAC, internal auditory canal; SCC, semicircular canals; BOR, branchio-oto-renal; EVA, enlarged vestibular aqueduct; EAC, external auditory canal..
a
CHs >2 SDs below the mean CH.