CT and MRI in congenital sensorineural hearing loss

Objective: To determine the value of CT and MRI in children with congenital sensorineural hearing loss (SNHL). Methods: The CT and MRI of 205 patients with congenital SNHL were analyzed before cochlear implantation. Results: White matter lesions (10/205, 4.9%), bony labyrinth malformation (59/205, 28.8%), internal auditory canal malformation (5/205, 2.4%), cochlear nerve hypoplasia (19/205, 9.3%), and cochlear nerve aplasia (5/205, 2.4%) were identified on CT and/or MRI. The labyrinth malformation in 109 ears included dilated vestibular aqueduct (63), classic Mondini's dysplasia (18), incomplete partition (10), common cavity deformity (5), vestibular-semicircular tube dysplasia (4), and immature cochlea (2). Conclusions: CT and MRI can demonstrate labyrinth malformation and development of the cochlear nerve and central nervous system. Both are valuable for assessing sensorineural hearing loss before cochlear implantation. (authors)