Utility of preoperative computed tomography and magnetic resonance imaging in adult and pediatric cochlear implant candidates.

OBJECTIVES/HYPOTHESIS: Determine the utility of preoperative imaging in adult and pediatric cochlear implant candidates. STUDY DESIGN: Retrospective chart review. METHODS: Medical records of 101 consecutive adult and 20 consecutive pediatric patients who underwent 137 cochlear implantation (CI) procedures at a single institution were reviewed. RESULTS: Computed tomography (CT) was obtained preoperatively in 110 (90.9%) patients, preoperative magnetic resonance imaging (MRI) was obtained in 102 (84.3%) patients, and both were obtained in 94 (77.7%) patients. MRI revealed one acoustic neuroma and two meningiomas, which affected surgical planning for three (2.2%) procedures. MRI identified enlarged vestibular aqueduct (EVA) in 2.0% of adult patients. CT demonstrated middle ear disease in four (3.3%) patients. CT was useful in indicating round window and cochlear patency in three (2.2%) patients with cochlear otosclerosis. Twenty pediatric patients underwent 27 CI procedures. Preoperative CT in the pediatric cohort demonstrated five (25%) dysplastic cochleae, three (15%) dysplastic vestibules and/or semicircular canals, and three (15%) EVAs. In one patient, CT demonstrated a duplicated right internal auditory canal (IAC) and hypoplastic left IAC; MRI confirmed hypoplastic cochlear nerves. CONCLUSIONS: Preoperative MRI can demonstrate retrocochlear pathology, cochlear patency, and EVA in adults being evaluated for cochlear implantation. CT may provide additional information in patients with chronic otitis media or otosclerosis. However, in postlingually deafened adults without conductive or asymmetrical hearing loss, imaging is unlikely to affect surgical decision making. Both CT and MRI can identify anomalies in pediatric patients. MRI does not offer substantial benefit over CT for routine evaluation of pediatric inner ear and temporal bone anatomy. LEVEL OF EVIDENCE: 4 Laryngoscope, 126:1440-1445, 2016.

Restoration of hearing in the VGLUT3 knockout mouse using virally mediated gene therapy.

Mice lacking the vesicular glutamate transporter-3 (VGLUT3) are congenitally deaf due to loss of glutamate release at the inner hair cell afferent synapse. Cochlear delivery of VGLUT3 using adeno-associated virus type 1 (AAV1) leads to transgene expression in only inner hair cells (IHCs), despite broader viral uptake. Within 2 weeks of AAV1-VGLUT3 delivery, auditory brainstem response (ABR) thresholds normalize, along with partial rescue of the startle response. Lastly, we demonstrate partial reversal of the morphologic changes seen within the afferent IHC ribbon synapse. These findings represent a successful restoration of hearing by gene replacement in mice, which is a significant advance toward gene therapy of human deafness.