Bilateral acoustic neurinomas

Abstract

A 20-year-old girl with family history of neurofibromatosis presented with complaints of decreased hearing in both ears, change in voice, headaches and progressive ataxia for the past 1 year. On admission, she was alert, active but dysarthric. There was bilateral horizontal gaze evoked nystagmus. Fundus examination revealed bilateral papilledema. The right corneal reflex was depressed and the sensations over the right side of face were marginally decreased. There was right facial paresis, bilateral sensorineural deafness and severe truncal ataxia. There were marked bilateral cerebellar signs. MR imaging showed large bilateral acoustic neurinomas severely compressing the brainstem [Figures 1 and 2]. It was difficult even to identify the brainstem in certain sequential images. A radical resection of both acoustic neurinomas was performed through bilateral retromastoid craniectomies in two stages at an interval of 3 months. On both sides, a thin sheath of tumor was left behind adjacent to the facial nerves in an attempt to save their function. The patient developed right facial palsy following the first surgery and needed tarsorrhaphy. However, her ataxia improved remarkably. Post-operative MR imaging showed considerable reformation of the brainstem [Figure 3].