Neurofibromatosis II

ABSTRACT Neurofibromatosis type II is an inherited autosomal dominant syndrome, characterized by multiple neoplasms of the central and peripheral nervous system associated with ocular abnormalities. The most common tumor associated with the disease is the vestibulocochlear schwannoma, and as many as 10% of patients with this tumor have neurofibromatosis type 2. In this report, we aim to present a 36-year-old female who presented with chief complaints of unilateral tinnitus, and, during her workup, which included cranial and whole spine magnetic resonance imaging, we found bilateral acoustic neuroma with multiple meningiomas. Based on clinical and imaging findings, the diagnosis of neurofibromatosis type 2 was made.

Bilateral Acoustic Neuroma with underlying Neurofibromatosis Type 2: A Therapeutic Challenge

The presence of bilateral acoustic neuroma may require modification of the usual therapeutic protocol. Preservation of hearing and total removal of the tumour is much more difficult than in sporadic unilateral lesion. This case illustrates the management of a girl with bilateral acoustic neuroma with underlying neurofibromatosis type 2. DOI: http://dx.doi.org/10.3329/bjms.v11i4.12609 Bangladesh Journal of Medical Science Vol. 11 No. 04 Oct’12

Cochlear implantation in an intralabyrinthine acoustic neuroma patient after resection of an intracanalicular tumour

This case study describes a therapeutic strategy using a cochlear implant for a bilateral acoustic neuroma deafened patient. The cochlear nerve had previously been sacrificed on one side during tumour removal, but on the remaining side a functioning cochlear nerve was assessed by electric promontory stimulation in spite of a neuroma extending into the vestibular labyrinth. The patient was successfully stimulated with a Nucleus 22- channel implant after removal of the intracanalicular portion of the neuroma via a middle fossa approach.

Bilateral acoustic schwannoma: post-operative hearing in the contralateral ear

A patient with neurofibromatosis two (NF2) presented with bilateral acoustic neuroma. Pre-operative audiometry revealed a dead right ear and severe left-sided sensorineural hearing loss. Following surgical removal of the larger right acoustic neuroma we have documented a sensorineural improvement of 45 dB in the contralateral ear on pure tone audiometry, which as far as the authors are aware has not previously been described.