Bilateral acoustic neuromas

Abstract

This article reviews 12 patients with bilateral acoustic neuromas. The sex incidence was equal and the mean age at diagnosis was 26.2 years. The family history was positive in nine of the patients. Five patients have had incomplete surgical removal of acoustic neuromas on both sides. Two of them are completely deaf and the other three have severe sensorineural hearing loss in one ear and no hearing in the other ear. In five patients the tumour on one side has been operated on and the other side is being observed with at least short-term preservation of good hearing. The remaining two patients died of intra-cranial complications, one of them post-operatively. Four patients developed facial palsy immediately following surgery and one developed facial weakness 6 months after surgery. Guidelines are discussed for the care of these patients including the timing of surgery and alternative treatment options (observation, radio-surgery and chemotherapy). This is essentially a group of young individuals who have had multiple operations for bilateral acoustic tumours and associated manifestations and for whom the disease and the sequelae of treatment can be tragic.

Radiology Quiz Case 1: Diagnosis

“…1 Bilateral vestibular schwannomas on MRIs suggest a diagnosis of neurofibromatosis 2 (NF-2). 3,4 There was no family history of neurofibromatosis. It is characterized by bilateral vestibular schwannomas and sometimes accompanied by schwannomas of other cranial and spinal nerves, intraspinal and intracranial meningiomas, and low-grade intracranial malignant neoplasms.…”