TYPE-2 NEUROFIBROMATOSIS - THE NEED FOR SUPRAREGIONAL CARE

The results of a U.K. study of 145 cases of type 2 neurofibromatosis h as shown generally very poor operative results in terms of hearing and facial nerve preservation. Only 9 out of 118 vestibular schwannoma (a coustic neuroma) operations resulted in any clinically detectable hear ing preservation and only 32 left the patient with good or normal (Hou se grade I or II) ipsilateral facial nerve function. Although operatio n is still the definitive treatment of vestibular schwannoma (acoustic neuroma) and may be a lifesaving procedure, it appears that the evide nce in favour of early operation is only valid when carried out in hig hly specialized centres. The special problems of NF2 cases who may go on to develop multiple spinal and cranial tumours making them wheelcha ir bound and blind as well as deaf warrants a careful experienced appr oach. Timing of operations may be critical for the enhancement of usef ul years of quality life. We therefore propose the setting up of a nat ional NF2 register, with the management of cases at a few supraregiona l centres.