Sensorineural Hearing Loss in the Nonimplanted Ear Following Cochlear Implantation in a Patient With Bilateral Enlarged Vestibular Aqueducts

Abstract

Objective: 

To document the case of a patient with bilateral enlarged vestibular aqueducts who experienced sensorineural hearing loss in the nonimplanted ear following unilateral cochlear implantation complicated by perilymph gusher requiring lumbar drain insertion and to highlight the need to counsel regarding the risk of potential hearing loss to the contralateral ear when preparing for cochlear implants in the setting of inner ear malformations.

Patients: 

One patient with bilateral enlarged vestibular aqueducts in a tertiary referral center.

Intervention(s): 

Cochlear implantation complicated by perilymph gusher requiring lumbar drain insertion.

Main Outcome Measure(s): 

Bone conduction hearing thresholds, word recognition scores.

Results: 

The patient underwent unilateral cochlear implantation, which was complicated by a perilymphatic gusher and necessitated placement of an intraoperative lumbar drain. On postoperative day 1, the patient reported hearing loss in the opposite ear. The word recognition score in the contralateral ear dropped from 24% at preimplantation to 8% at 2-weeks postimplantation, and did not improve at 6 months postimplantation. Moreover, the bone conduction threshold at 1 kHz worsened from 20 dB preoperatively to no response at 75 dB (the limit of the testing equipment) at 2-weeks postoperatively and only partially improved to 40 dB at 6 months postimplantation.

Conclusion: 

As patients with inner ear malformations potentially have direct high-pressure anatomical connections between the perilymphatic spaces and the cerebrospinal fluid, they are at risk of hearing loss in the nonimplanted ear during cochlear implantation. This case highlights the need for potential additional patient counseling regarding this risk in the nonimplanted ear.