Article Figures & Data
Figures
- FIG 1.
A, Axial CT image of a normal inner ear shows the normal shape of the internal auditory canal, cochlea, modiolus (arrow), and otic capsule. CT and MR images of a 7-month-old male patient’s right ear. B, Axial CT image shows a bulbous dilated internal auditory canal and the absence of the modiolus with the interscalar septa present (black arrows) and thinning of the otic capsule (arrowheads), with mild pericochlear hypodensity. The middle turn width of the cochlea is shorter than normal (white arrow). C, Axial SPACE image shows the CN, VN, and the hypointense structure in the middle turn of the cochlea (short arrows). The branches of the CN are also shown. D, Oblique sagittal CISS image perpendicular to the internal auditory canal shows the facial nerve (FN), CN, and VN. There is a spiral structure (black arrows) extending from the fundus of internal auditory canal to the cochlea, which is connected to the cochlear nerve.
- FIG 2.
CT and MR images of a 3-year-old male patient’s right ear. A, Axial CT image shows an enlarged labyrinthine facial nerve canal (short white arrow), enlarged superior vestibular nerve canal (long white arrow), enlargement of the vestibular aqueduct with a cystic appearance at the part close to the vestibule (arrowhead), and the normal vestibular aqueduct orifice (black arrow). B, Axial CT image shows the enlarged singular nerve canal (arrow) and absence of the modiolus. C, Axial SPACE image shows fluid signal in the enlarged labyrinthine facial nerve canal (short white arrow), superior vestibular nerve canal (long white arrow), and the vestibular aqueduct (arrowhead). D, Axial SPACE image shows fluid signal in the singular nerve canal (arrow).
- FIG 3.
CT images of a 4-year-old male patient’s right ear. A, Axial CT image shows a cystic bulge between the superior semicircular canal feet (arrow). B, Axial CT image shows the irregularly shaped vestibule (arrow) and small cystic bulges at the lateral semicircular canal (arrowhead). C and D, MPR CT image of the superior semicircular canal and coronal CT image show the cystic bulge of the vestibule protruding upward (arrow).
- FIG 4.
A–C, Axial CT images of a 3-year-old male patient’s right ear show multicystic appearance at the superior semicircular canal (SSCC), posterior semicircular canal (PSCC), and lateral semicircular canal (LSCC). Bulbous dilation of the IAC and absence of modiolus are also demonstrated. D, A 3D MR hydrography image shows the cystic appearance of the semicircular canals and dilation of the IAC.
- FIG 5.
CT images of a 19-month-old male patient’s right ear. A, Axial CT image shows thickness of the stapedial footplate and atresia of the oval window (arrow) and absence of the modiolus. B, Coronal CT image shows atresia of the oval window (arrow).
Tables
Imaging findings of the patients
Imaging Findings Cases First Reported, Year Dilated internal auditory canal 19 Phelps et al,3 1991 Hypoplasia at the cochlear base 19 Phelps et al,3 1991 Absence of the bony modiolus 19 Talbot and Wilson,5 1994 Thin otic capsule 19 Sennaroglu,13 2016 Enlarged superior vestibular nerve canal 16 Saylisoy et al,6 2014 Enlarged labyrinthine facial nerve canal 11 Phelps et al,3 1991 Enlarged singular nerve canal 8 Talbot and Wilson,5 1994 Vestibule with cystic appearance 17 Gong et al,7 2014 Semicircular canal with cystic appearance 9 Anderson et al,14 2020 Dilated vestibular aqueduct 7 Talbot and Wilson,5 1994 Dysplasia of oval window 12 Saylisoy et al,6 2014 Dysplasia of round window 11 Saylisoy et al,6 2014 Abnormal stapes 5 Saylisoy et al,6 2014
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