The objective of the investigation was to document the clinical presentatio
ns of a group of patients with neurofibromatosis type two syndrome (NF2), a
nd took the form of a retrospective case note review. The investigation too
k place in the tertiary referral skull base unit. The dominant presentation
of patients with NF2 is with auditory/vestibular symptoms (56%), with a sm
aller proportion of presentations being due to non-acoustic intracranial an
d spinal tumours (44%). At the time of presentation the majority of clinica
lly significant rumours could be demonstrated in each patient with MRI of t
he head and spine, with few new rumours arising during the subsequent follo
w-up period. The range of presentation of patients with NF2 results in thei
r management being fragmented into a variety of clinical settings, diluting
the experience amassed in any one centre.