Purpose or Case Report: (1) To review the embryology of the temporal bone with emphasis of the inner ear.
(2) To review the detailed cross-sectional CT and MRI anatomy of the inner ear of the temporal bone.
(3) To discuss the role of CT and MR imaging in the evaluation of congenital sensorineural hearing loss.
(4) To describe the cross-sectional imaging findings of common and uncommon pathologies of congenital sensorineural hearing loss.
(5) To discuss common indications, relative as well as absolute contraindications for cochlear implantation pertinent to imaging findings.
Methods & Materials: Retrospective search of the Radiology PACS system at Tertiary Level Children Hospital was performed using keyword “Congenital”, “Sensorineural Hearing Loss”. Representative cases were identified and included in this exhibit.
The exhibit begins with a review of the embryology and detailed cross-sectional CT and MRI anatomy of the inner ear structures of the temporal bone. This is followed by a detailed discussion of imaging and clinical findings of common and uncommon pathologies of congenital sensorineural hearing loss.
Then the audience has the opportunity to apply the knowledge toward diagnosing pathologies of congenital sensorineural hearing loss via an interactive case-based approach.
Results: Various common and uncommon pathology of the inner ear structures were encountered and included in this exhibit including, not limited to Complete Labyrinthine Aplasia, Cochlear Aplasia, Common Cavity Malformation, Incomplete Partition Type I Syndrome, Incomplete Partition Type II Syndrome, Malformations of the Vestibule and Semicircular Canals, Enlarged Vestibular Aqueduct Syndrome, Cochlear Nerve Aplasia and Hypoplasia etc. Brief discuss of the implications of imaging abnormalities for cochlear implantation will be discussed.
Conclusions: Knowledge of the embryology, cross sectional anatomy and imaging characteristics of various common and uncommon inner ear malformation in setting of Congenital Sensorineural Hearing Loss will allow an accurate timely diagnosis, appropriate treatment options in terms of correctly identifying candidates for cochlear implantation for successful outcomes.