Neurofibromatosis type 2 (NF2) is known to result in bilateral hearing loss, even when there is no significant tumour growth. The cause is postulated to be multifactorial: stretching and compression of the cochlear nerve by the tumour, impairment of labyrinthine vascular supply, intra-labyrinthine haemorrhage, endolymphatic hydrops, cochlear aperture block with subsequent intracochlear accumulation of proteins and the disruption of CSF-blood and labyrinthine barrier. In this paper the authors report their experience with internal auditory canal (IAC) decompression in an attempt to preserve hearing in a cohort of NF2 patients with contralateral hearing loss. They recommend IAC decompression in patients with the hearing ear and objective hearing modification (progressive, fluctuating or sudden hearing loss). They suggest that the surgery can also be combined with chemotherapy in an attempt to preserve hearing as long as possible. It is worthwhile considering whether IAC decompression will benefit all patients with NFs or only those who have cochlear nerve compression by the tumour.