Bilateral otoneurological pathology: To operate or not?

OBJECTIVE

To illustrate our experience when managing a complex patient with potentially life-threatening bilateral otological disease facing multisensory compromise including complete loss of audiovestibular function and visual disturbance Clinical presentation: A 67 year old lady, presented with a large left vestibular schwannoma and extensive right cholesteatoma encircling the otic capsule. She underwent translabyrinthine resection of the vestibular schwannoma, resulting in profound sensorineural hearing loss, vestibular hypofunction and corneal scarring following an initial temporary facial palsy. Due to the extent of the disease and good right-sided bone conduction thresholds, the cholesteatoma was managed conservatively utilising a bone-anchored-hearing-aid with regular review by the Skull-Base team. However, following acute deterioration in hearing and disease extension threatening right facial nerve function, the decision was taken for surgical intervention with a view to staged cochlear implantation (CI).

INTERVENTION

Our patient underwent right lateral petrousectomy, total osseous labyrinthectomy, and implantation of an intracochlear 'dummy' with blind sac closure of the external auditory canal. Recovery was excellent with no further deterioration in balance and no loss of facial nerve function. CI occurred 3 months post-operatively with good functional outcome.

CONCLUSION

We present the management of bilateral complex otoneurological disease with significant risk of multisensory compromise. Size of the left vestibular schwannoma necessitated surgical resection with resultant loss of ipsilateral audiovestibular function. Due to residual right audiovestibular function and the risks of surgery, the extensive right cholesteatoma was managed conservatively until disease progression necessitated surgical intervention. Subsequently it was possible to restore access to open-set speech with a right CI.