Factors affecting final facial nerve outcome following vestibular schwannoma surgery.

OBJECTIVE: To determine factors affecting facial nerve outcome of vestibular schwannoma surgery. METHODS: This retrospective cohort study comprised 652 patients. The outcome measure was House-Brackmann classification at two years post-operatively. Univariate and multivariate analyses were carried out to determine the factors affecting facial nerve outcome. The incidence rates of hemifacial spasm, metallic taste and crocodile tear syndrome were recorded. RESULTS: For tumours less than 1.5 cm, 95 per cent of outcomes were normal, 100 per cent were satisfactory (House-Brackmann grades I-III) and 0 per cent were unsatisfactory (grades IV-VI). For tumours 1.5-2.4 cm, 83 per cent of outcomes were normal, 99 per cent were satisfactory and 1 per cent were unsatisfactory. For tumours 2.5-3.4 cm, 68 per cent of outcomes were normal, 96 per cent were satisfactory and 4 per cent were unsatisfactory. For tumours 3.5-4.4 cm, 52 per cent of outcomes were normal, 80 per cent were satisfactory and 20 per cent were unsatisfactory. For tumours larger than 4.4 cm, 50 per cent of outcomes were normal, 72 per cent were satisfactory and 28 per cent were unsatisfactory. CONCLUSION: Tumour size and operation year were significant predictors of facial nerve outcome. The surgical learning curve was steepest for the first 50 patients.

Outcome following retrosigmoid microvascular decompression of the facial nerve for hemifacial spasm.

This paper evaluates the outcome of retrosigmoid microvascular decompression of the facial nerve in a series of patients suffering from hemifacial spasm who had been referred to the skull-base team (comprising senior authors DAM and DGH). The paper is a retrospective review of 15 patients who underwent retrosigmoid microvascular decompression of the facial nerve at Addenbrooke's Hospital between 1985 and 1995. In this series it was possible to obtain complete resolution of hemifacial spasm in 93.3 per cent of cases in the short term and in 80 per cent in the long term. Twelve patients (80 per cent) were symptom-free post-operatively. Two patients had minor recurrence of symptoms occurring within six months of the procedure. One patient with no identifiable vascular impingement of the facial nerve had no improvement following surgery. Three patients suffered sensorineural hearing loss. Two patients complained of post-operative tinnitus, and transient facial palsy was noted in one patient. Retrosigmoid microvascular decompression of the facial nerve provides excellent long-term symptom control in a high percentage of patients with hemifacial spasm.

Team working to improve outcome in vestibular schwannoma surgery.

The concept of the 'learning curve' in the early years of consultant appointment is no longer acceptable and yet, for most complex operations, learning is a career-long process. Sub-specialization offers part of the solution to this problem by concentrating expertise, but there remains the question of how to introduce new team members without adversely affecting patient outcome. Shortened training and an increasingly aware public, who are more questioning of the experience and outcomes of those treating them, has required the surgical profession to move away from individual consultant practice and embrace team-working. When, in 1997, a second neurosurgeon joined the skull base team at Addenbrooke's Hospital, his introduction to acoustic neuroma surgery was mentored and operative cases were carefully selected at first. In this article, we discuss our experience with consultant mentoring, and compare the results of the first 100 translabyrinthine acoustic neuroma operations performed under this system with the first cohort of a similar number of patients published from Cambridge in 1989.

New proposals for training in neurosurgery.

The rapidly evolving changes in working patterns, career structure and the regulation of training of doctors have provided an ideal opportunity for proposals to improve the programme for the training of neurosurgeons. The Education and Training Committee of the Society of British Neurological Surgeons (D.G. Hardy, A. J. W. Steers, N. T. Gurusinghe, P. M. Foy, P. van Hille, R. A. Cowie, H. A. Crockard, O. Sparrow and S. Burn) has, in recent months, worked closely with the Specialist Advisory Committee (SAC) in neurosurgery, Department of Health (Modernizing Medical Careers Group, H. A. Crockard, A. Havers, T. Hobbs) and colleagues from the major neuroscience specialties to develop a new programme based on a 'Common Stem' approach. This article describes the principles of the programme. The proposals have received approval by the Council of the SBNS, the Presidents of the four Surgical Royal Colleges and the Senate of Surgery.

Cavernous haemangioma of the internal auditory canal.

Cavernous haemangiomas are rare lesions of the cerebello-pontine angle that can mimic the more commonly occurring vestibular schwannoma. A case report involving a patient with a cavernous haemangioma of the internal auditory canal (IAC) highlights this as a diagnostic possibility for lesions of the IAC by comparing and contrasting the clinical and radiological findings with the more commonly occurring vestibular nerve and facial schwannomas. Symptoms such as hearing loss and facial paralysis that are disproportionate to the size of the lesion or fluctuate with hormonal changes such as those seen in pregnancy are suggestive of haemangioma. Radiological imaging demonstrating a lesion enhancing with gadolinium and containing areas of calcification is also suggestive of haemangioma. It is important to consider the possible diagnosis of haemangioma as early recognition of this entity may improve the chances of preserving the functional integrity of the facial nerve.

Clinical presentation of a group of NF2 patients to a tertiary referral skull base unit.

The objective of the investigation was to document the clinical presentations of a group of patients with neurofibromatosis type two syndrome (NF2), and took the form of a retrospective case note review. The investigation took place in the tertiary referral skull base unit. The dominant presentation of patients with NF2 is with auditory/vestibular symptoms (56%), with a smaller proportion of presentations being due to non-acoustic intracranial and spinal tumours (44%). At the time of presentation the majority of clinically significant tumours could be demonstrated in each patient with MRI of the head and spine, with few new tumours arising during the subsequent follow-up period. The range of presentation of patients with NF2 results in their management being fragmented into a variety of clinical setting, diluting the experience amassed in any one centre.

Postoperative quality of life in vestibular schwannoma patients measured by the SF36 Health Questionnaire.

OBJECTIVE: To quantify the postoperative quality of life in patients following surgical treatment for vestibular schwannoma. STUDY DESIGN: Patient self-assessment using the short form 36 (SF36) multidimensional quality of life health questionnaire. Sex- and age-matched normalized scores were calculated using a standardized process and accepted normative data. SETTING: Tertiary referral skull base unit. RESULTS: An 80% response rate (90 patients) was achieved. The postoperative quality of life in vestibular schwannoma patients, as quantified by seven of the eight SF36 health scales was less than the appropriate matched healthy standard. Comparison of a variety of preoperative patients and tumor factors-different operative approaches (translabyrinthine and retrosigmoid), tumor size (group cut of points of tumor diameter 1.5 mm and 2.5 mm), patient sex, and ranking of patient age-showed no statistically significant difference in measured quality of life outcomes for each of these traditional predictors. CONCLUSION: Reduced quality of life in patients after surgical treatment for vestibular schwannoma, coupled with the low tumor growth rates and minimal preoperative symptoms, supports a conservative approach to patient management. The advantages and disadvantages of a variety of approaches used to measure the quality of life after surgical treatment of vestibular schwannoma and their impact on clinical decision making for patients, are discussed.

Rupture of an internal carotid artery aneurysm in the petrous temporal bone.

A case of otorrhagia following the rupture of an aneurysm of the intrapetrous portion of the internal carotid artery is presented. The aneurysm was successfully treated by balloon occlusion of the parent artery. The problems associated with diagnosis and clinical management of these lesions is discussed and possible solutions suggested.

Hearing preservation in solitary vestibular schwannoma surgery using the retrosigmoid approach.

The results of 50 cases of vestibular schwannoma surgery with hearing preservation performed by the retrosigmoid approach at Addenbrooke's Hospital, Cambridge, during a 10-year period are presented. The hearing-preservation rate, using audiometric criteria set by others as "serviceable hearing" (Wade PJ, House W. Otolaryngol Head Neck Surg 1984;92:1184-93; Silverstein H, et al. Otolaryngol Head Neck Surg 1986;95:285-91; Cohen NL, et al. Am J Otol 1993;14:423-33) was 8% (4 of 50 cases). When the more stringent selection criteria of near-normal hearing and reporting criteria of socially useful hearing preservation (pure-tone average < 30 dB/speech discrimination score > 70%) is used, the hearing-preservation rate is 4.8% (1 of 21 cases). The only preoperative factor that may predict a favorable hearing-preservation outcome is normal auditory brain stem response morphology (Fisher's exact 2-tailed test, P < 0.001). The number of suitable candidates for hearing-preservation surgery are few. Reasonable indications for attempted vestibular schwannoma surgery with hearing preservation are discussed.

Does choice of hearing selection criterion and reporting criteria affect the hearing preservation rate in vestibular schwannoma surgery?

With increasing refinement in the surgery of vestibular schwannoma the aims of complete tumor removal and facial nerve preservation have been largely fulfilled. However, the reputation of and place for hearing-preservation surgery still remain uncertain. A major part of this uncertainty is the result of difficulties in interpretation of the various reported results of hearing-preservation surgery. Meaningful comparison between series is difficult because of the varied number of postoperative reporting criteria commonly in use today. Although it is acknowledged that the postoperative reporting criteria affect the hearing-preservation rates, what is not readily appreciated is that preoperative selection criteria for hearing-preservation cases can also significantly affect the success rate of hearing-preservation acoustic neuroma surgery. This article models the many possible outcomes of hearing-preservation schwannoma surgery by use of the previously reported Cambridge series as an illustrative example. With these models some understanding can be gained of the effect of choosing various preoperative and postoperative hearing criteria on the overall hearing-preservation success rate.

An aneurysm of the petrous internal carotid artery.

Internal carotid artery aneurysms are a rare cause of pulsatile tinnitus and conductive hearing loss but should be borne in mind when there is a suspected diagnosis of glomus jugulare or high-riding jugular bulb. Most cases are congenital. We present a case of otorrhagia which was initially thought to be a glomus jugulare, the diagnosis of internal carotid artery aneurysm was made at angiography and treated by balloon embolization.

Use of autologous osteocyte containing bone pate for closure of tegmental defects.

OBJECTIVE: This article aimed to describe a new technique for the reconstruction of middle fossa tegmental defects using autologous osteocyte containing bone pate. STUDY DESIGN: The technique is demonstrated using clinical photographs and x-ray examples of tegmental defects. TECHNIQUE: Pliable bone pate graft is manufactured using autologous bone dust, blood, iodine solution, and tissue glue (Tisseel; Immuno, Austria). The graft is placed accurately via a combined mini-middle fossa craniotomy and middle mastoidectomy approach. CONCLUSION: This new technique of middle fossa bony tegmental repair has considerable advantages over previously described methods that use rigid or synthetic materials.

Somatic neurofibromatosis type 2 gene mutations and growth characteristics in vestibular schwannoma.

BACKGROUND: The growth of hereditary and sporadic vestibular schwannomas shows wide variation, but what determines this is poorly understood. HYPOTHESIS: In neurofibromatosis type 2 (NF2), there is some correlation between the nature of the germline NF2 gene mutation and phenotype. Somatic mutations in the NF2 gene occur in sporadic tumors, but their relation to tumor behavior is unknown. METHODS: This study has investigated the molecular pathogenesis of vestibular schwannoma by looking for NF2 gene mutations. The authors have screened 17 exons of the NF2 gene in 91 sporadic vestibular schwannomas, 2 NF2, and 1 vagal schwannoma. These data have been correlated with a clinical growth index and a tumor cell proliferation index, determined using a monoclonal antibody to the proliferating cell nuclear antigen. RESULTS: Of the 94 tumors studied, 40 somatic gene mutations (38%) have been sequenced in 36 tumors. The mutations included 36 protein truncating mutations, 1 in-frame deletion, 2 splice site mutations, and 1 missense mutation. Regression analysis showed no correlation between the nature of the NF2 gene mutation and either the clinical (R2 = 0.006) or the proliferative index (R2 = 4 x 10(-8). CONCLUSION: The results of this study show no association between the nature of the intragenic NF2 gene mutation and tumor behavior. It is likely therefore that NF2 gene inactivation is not the only determinant of tumor behavior in vestibular schwannoma.

Extended temporal bone resection for squamous cell carcinoma.

OBJECTIVE: The aim of this study was to assess the surgical results of a series of patients from this unit who underwent extended temporal bone resection for recurrent squamous cell carcinoma as a salvage procedure. DESIGN: The surgical records of 15 patients were analyzed in detail. Each patient had salvage surgery in the form of an extended temporal bone resection with supraomohyoid block dissection, dural grafting, and free microvascular forearm or scalp rotation flap repair for recurrent squamous cell carcinoma in a radical mastoid cavity. RESULTS: Radical surgery yielded a 47% 5-year survival. Twenty-nine percent of the survivors had temporal lobe involvement that necessitated a partial excision of the temporal lobe of the brain. Histologic evidence of local lymph node involvement in the supraomohyoid neck dissection was present in 13% of cases. Those who died did so in the first postoperative year. All those with poorly differentiated tumors died. The survivors had well or moderately differentiated tumors. CONCLUSIONS: Radiotherapy alone or partial temporal bone resection, most commonly a radical mastoidectomy with or without preoperative or postoperative radiotherapy is used by the majority of otolaryngologists in treating squamous cell carcinoma of the temporal bone. The 5-year survival rate after this treatment remains depressingly low and the prognosis gloomy, particularly for advanced tumors. The findings in this series of extended temporal bone resections as salvage surgery in recurrent disease is encouraging, and radical surgery combined with radiotherapy from the outset may give much better 5-year survival figures in the future than the conventional partial temporal bone resection and radiotherapy.

A molecular, clinical, and immunohistochemical study of vestibular schwannoma.

The molecular pathogenesis of vestibular schwannoma has been investigated by determining the extent of chromosome 22 loss of heterozygosity in 77 tumors and relating these findings to clinical and immunohistochemical indexes of tumor behavior. Loss of heterozygosity was looked for at eight chromosome 22q loci. Clinical details were obtained in all 77 cases, and a clinical growth index was calculated for each tumor. The proliferative index was estimated in all tumors by using a monoclonal antibody to the proliferating cell nuclear antigen and by calculating the labeling index. Forty percent (31 of 77) of the tumors showed allele loss, and in each case this loss involved the region of the neurofibromatosis type 2 gene. No evidence was found that the presence of chromosome 22 allele loss was associated with the clinical growth index. On the log scale, however, an association was seen between the clinical growth index and the proliferating cell nuclear antigen labeling index p = 0.001). These results suggest that chromsome 22 allele loss is a frequent event in vestibular schwannoma. Tumor behavior, however, appears to be independent of the chromosome 22 mutation. It is proposed that chromosome 22 allele loss and neurofibromatosis type 2 gene inactivation is an early event, possibly involved in the initiation of tumorigenesis in vestibular schwannoma. Tumor growth appears to be independent of this mutation and is likely to be determined by other as yet undefined factors.

Audio-vestibular findings in meningioma of the cerebello-pontine angle: a retrospective review.

The aim of this study was the determination of the incidence of symptoms of audio-vestibular dysfunction and of abnormalities on audio-vestibular testing in patients found to have a unilateral meningioma of the cerebello-pontine angle (CPA). The case notes of 25 patients diagnosed with unilateral, sporadic and histologically proven CPA meningioma were retrospectively reviewed. The age range of this series was 31-71 years, with a mean age of 50 years. Two patients were male (eight per cent) and 23 were female (92 per cent). The mean length of history was 44.7 months. The distribution of tumour size was skewed toward larger tumours, with 15 cases (60 per cent) having tumours with a maximum diameter greater than 3.5 cm on imaging. Pure tone audiometry was normal in five cases (20 per cent), and no patients exhibited the high frequency sensorineural hearing loss that is characteristic of vestibular schwannoma. Speech audiometry was normal in 50 per cent of cases. Caloric testing was abnormal in 77 per cent of the 18 cases tested, whilst auditory brainstem responses (ABR) were abnormal in 100 per cent of the 18 cases who had sufficient hearing for this test to be possible. The presence of normal audiometry in patients with a proven CPA lesion indicates that, if in a protocol for investigation, asymmetry of hearing is mandatory then some pathology will be missed. Any suspicion of a CPA lesion warrants investigation even in the absence of hearing loss. The investigation of choice for the identification of CPA lesions has become magnetic resonance imaging (MRI). If this technique is not available then this study indicates that ABR is a suitable and sensitive investigation. It should be borne in mind however that the data in this study has been derived from a series of predominantly large tumours, and the sensitivity of ABR to smaller CPA meningiomata may fall, as is the case for vestibular schwannoma.