Table 2:

Patients with cochlear hypoplasiaa

PatientAge (yr) SexL CH (mm)R CH (mm)TYPE HLDiagnosisCT Findings
16.3 Female2.82.5SNHL, bilaterallyBilateral vestibulocochlear dysplasia, L Mondini malformationBilateral vestibulocochlear dysplasia
R common chamber malformation, absent vestibular aqueduct, aplastic modiolus
L Mondini malformation (hypoplastic cochlea with partition defect)
21.9 Male3.43.6Mixed HLBOR syndromeBilateral dilated vestibular aqueduct with small modiolus, trumpet-shaped IAC, small mass in R middle ear (possible congenital cholesteatoma)
30.8 Female3.73.5Mixed HLBOR syndromeBilateral 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
46.7 Female3.64.3Mixed HL, bilaterallyCHARGE syndromeBilateral 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
50.1 Male4.23.8Mixed HL, bilaterallyCHARGE syndromeBilateral absent SCCs, middle ear/mastoid air cell congestion or inflammation, normal middle ear morphology
66.6 Male4.04.5SNHL, bilaterallyEVA syndromeBilateral enlarged vestibular aqueducts, R cochlear implant, L hypoplastic modiolus
77.1 Male4.24.3CHL, bilaterallyBilateral class II microtia and EAC atresiaR 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.