Richard H. Lye

Type 2 neurofibromatosis: The need for supraregional care?

The results of a U.K. study of 145 cases of type 2 neurofibromatosis has shown generally very poor operative results in terms of hearing and facial nerve preservation. Only 9 out of 118 vestibular schwannoma (acoustic neuroma) operations resulted in any clinically detectable hearing preservation and only 32 left the patient with good or normal (House grade I or II) ipsilateral facial nerve function. Although operation is still the definitive treatment of vestibular schwannoma (acoustic neuroma) and may be a lifesaving procedure, it appears that the evidence in favour of early operation is only valid when carried out in highly specialized centres. The special problems of NF2 cases who may go on to develop multiple spinal and cranial tumours making them wheelchair bound and blind as well as deaf warrants a careful experienced approach. Timing of operations may be critical for the enhancement of useful years of quality life. We therefore propose the setting up of a national NF2 register, with the management of cases at a few supraregional centres.

Ionomeric bone cement in neuro-otological surgery

The use of a polymaleinate glass ionomer cement in 80 neuro-otology cases is described. It has proved of great value in translabyrinthine acoustic neuroma surgery, reducing the incidence of CSF fistula to nil. It is the method of choice for fixation of the Nucleus cochlear implant, and has many other applications in the field of otology and neuro-otology. It is easy to use and appears to have no side effects.

Acoustic neuroma (schwannoma) surgery 1978–1990

A series of 151 patients with 154 acoustic schwannomas have been operated upon in Manchester Royal Infirmary by a joint Otological and Neurosurgical team, employing either the translabyrinthine or the suboccipital approach. The perioperative mortality rate was 3 per cent. Anatomical preservation of the facial nerve was achieved in 89 per cent of tumour removals and a good to normal functional result in 79 per cent of cases. Attempts at hearing preservation have been unsuccessful, largely because of the small number of patients in the series in whom useful hearing was present preoperatively. Complications included major brain stem ischaemia (1.2 per cent), CSF fistula (5 per cent) and facial dysaesthesia (7 per cent). The incidence of mortality and morbidity is directly related to tumour size and to the experience of the surgeons. A number of patients experienced an unusual type of post-operative dreamlike state which appeared to be a form of hypnagogic hallucination, and the possible neurophysiological mechanism responsible for this phenomenon is discussed. The continuing failure to attain the ideal of early diagnosis is lamented, and the importance of a flexible bidisciplinary surgical approach emphasized.

Audio-vestibular manifestations of Chiari malformation and outcome of surgical decompression: A case report

Sensorineural hearing loss, tinnitus, dizziness and ataxia are recognised symptoms associated with Chiari malformations but they are rarely the presenting complaints. Patients with such symptoms are frequently referred to otolaryngologists and audiological physicians. We report a case of a 13-year-old girl who presented complaining of tinnitus and impaired hearing, and was subsequently diagnosed as having a type I Chiari malformation. Pure tone audiogram showed a mild hearing impairment on the left side and the speech audiogram was normal. Auditory brain stem responses and the electronystagmography were abnormal. The patient underwent posterior fossa decompression following which her tinnitus disappeared, the hearing problem recovered and some of the abnormal electrophysiological parameters were corrected.

Spontaneous dissection of the intrapetrous internal carotid artery

Two cases of cervicocephalic arterial dissection of the intrapetrous carotid artery are described. One patient presented with intolerable objective pulsatile tinnitus, the other with a cerebral infarction. Both were successfully treated with anticoagulants. The significance of minor degrees of trauma and of neck extension in the aetiology of these apparently spontaneous lesions is discussed.

Variation of expression of the gene for type 2 neurofibromatosis: absence of a gender effect on vestibular schwannomas, but confirmation of a preponderance of meningiomas in females.

Type 2 neurofibromatosis is a dominantly inherited disorder in which the great majority of sufferers develop bilateral vestibular schwannomas. In a UK study of 183 individuals from 112 families we have previously shown a fairly similar disease course within families, but quite marked inter-familial variation. We have confirmed an increase in severity when the gene is inherited from an affected mother, but evidence that women are more severely affected than men is lacking. Age at onset of symptoms, of deafness and at diagnosis are identical for the entire dataset and for a comparison of 10 male/female sibling pairs. Only three out of 42 pregnancies in symptomatic women were accompanied by a reversible worsening in symptoms due to vestibular schwannomas. Of 328 consecutive cases of unilateral vestibular schwannoma, there was no significant difference in the sex ratio or size. There now appears to be little evidence for a female hormonal effect on vestibular schwannomas. However, females with type 2 neurofibromatosis have significantly more meningiomas.

The fate of tumour rests following removal of acoustic neuromas: An MRI Gd-DTPA study

The fate of capsular fragments left attached to vital structures at the time of otherwise total tumour removal was studied in 14 of 21 such patients who underwent acoustic neuroma surgery. Imaging using magnetic resonance Gd-DTPA at post-operative intervals of 6 months-12 years (mean 70 months) showed evidence of persistent tumour in half the patients. None of the patients had developed new symptoms and computed tomography had failed to demonstrate tumour recurrence. Persistence of the tumour was more likely if the residual fragments were not cauterized at the time of operation. Four of the seven persisting tumour rests showed evidence of gradual enlargement. The implications for patient management, particularly if an attempt is made to preserve hearing, are discussed.

A clinical, genetic and audiological study of patients and families with bilateral acoustic neurofibromatosis

The neurofibromatoses consist of at least two distinct autosomal dominant hereditary disorders. Neurofibromatosis type 1 (NF1) is due to a lesion on chromosome 17q. Neurofibromatosis type 2 (NF2) is caused by a defect on chromosome 22q. The hallmark of NF2 is the development, in the second and third decades, of bilateral acoustic neuromas. NF1 is characterized by the appearance of café-au-lait spots and neurofibromas in addition to iris hamartomas, or Lisch nodules, of the eye, during the first and second decades. Ten families were personally studied. A total of 16 members were found to be affected with NF2. A protocol for evaluation and review of subjects and relatives of NF2 families is proposed. A team approach, coordinating the expertise of multiple specialties is recommended.

Haemangiopericytoma of the temporal bone.

Haemangiopericytoma is an uncommon vascular tumour with a widespread distribution. Although meningeal involvement is well recognized, only a few sporadic cases of temporal bone lesions have been documented, all with doubtful sites of origin. Late presentation together with the restrictive anatomy of this region often precludes its effective removal and even minimal residual disease may progress rapidly. A series of three such patients are presented in order to discuss the natural history, histological features and treatment of this disease.

Unruptured Aneurysms of the Intracavernous Internal Carotid Artery: Outcome following carotid ligation or conservative treatment

Although intracranial obliteration or detachable balloon occlusion of the proximal artery has been recommended for treatment of unruptured aneurysms of the cavernous sinus, carotid artery ligation continues to be used by many neurosurgeons. This study compares the long term outcome after carotid ligation with that following conservative treatment. Nine of 13 patients treated by common carotid ligation improved, two were unchanged but two patients subsequently needed internal carotid ligation (mean FU 8.2 years). Ten patients were managed conservatively. Three improved, six were unchanged and one patient died following intracranial haemorrhage (mean FU 6.9 years). The authors conclude that carotid ligation remains an acceptable method for treating these difficult lesions.