Temporal bone histopathology related to cochlear implantation in congenital malformation of the bony cochlea.

HYPOTHESIS: Histopathologic findings in temporal bones with congenital malformations of the bony cochlea may provide insight into cochlear implantation planning, surgical approach, and complications.

BACKGROUND

Patients with congenitally malformed cochleae account for an increasing percentage of candidates for cochlear implantation. Few studies on the relationship between histopathologic findings of temporal bones with malformation of the bony cochlea and cochlear implantation have been reported.

METHODS

We studied 21 temporal bones from 12 cases with congenital malformations of the bony cochlea. Ages ranged from stillborn to 50 years. Length of the cochleae and dimensions of facial recesses were measured with light microscopy. Other malformations associated with a shortened cochlea were determined, emphasizing how they affect cochlear implantation.

RESULTS

The average length of the malformed cochlear duct was 22.84 +/- 0.69 mm. Average dimensions of facial recesses were not significantly different between malformed cochleae and the controls. Other malformations included enlarged cochlear aqueduct (43%), abnormal facial nerve course (57%), enlarged vestibular aqueduct (52%), aplasia of the middle ear (19%), malformed ossicles (67%), abnormal oval window (57%), and abnormal round window (29%).

CONCLUSION

A shortened cochlear duct may cause an incomplete insertion of the implant electrode. Because dimensions of the facial recesses are similar to normal cases, a facial recess surgical approach is recommended. An enlarged cochlear aqueduct may cause perilymphatic oozing or gushing on fenestration of the cochlea. An anomalous course of the facial nerve is a common finding. Tympanic malformations such as round and oval window deformities and small middle ear cleft should be noted preoperatively to better guide surgery.