Non-surgical management of acoustic neuromas.

Watchful waiting is one of the options available in the management of acoustic neuromas and this article deals with 13 patients who were so managed. Non-operative management was advised because of age, poor general health, small size of tumour, only hearing ear, or in patients unwilling to undergo surgery for various reasons. This group was followed up at 6-12-monthly intervals and the follow-up period ranged from 1 to 18 years (mean 5.3 years). Ten patients had small tumours and only in 2 of these was increase in tumour size demonstrated on follow-up CT scan. In one this increase was later followed by regression. Two patients required partial removal of tumour because of increasing symptoms after 3 and 7 years of follow-up; one of them died on the twelfth post-operative day. There appears to be a small group of patients for whom delay is worth while rather than to subject all patients with acoustic neuroma to surgery from which full recovery cannot be guaranteed.     

Conservative management of acoustic neuromas.

The results of this study and others document the biologic behavior of acoustic neuromas. In view of the evidence presented, which describes both variable rates of individual tumor growth and spontaneous regression in size, it would seem prudent that before selecting a nonsurgical treatment modality, the growth rate for the particular tumor in question should be established. To date, none of the literature that addresses the use of focused irradiation has attempted to do so. Our study as well as those of others suggests that the growth rate of acoustic neuromas becomes predictable over time. Based on this observation, a conservative (nontumor excision) management strategy is proposed for selected individuals. Patients to whom this management philosophy has been recommended or who themselves have chosen this option are seen twice yearly. Each visit consists of a thorough neurotologic examination as well as high-definition CT or MRI. Careful comparison of the clinical course as well as calculation of the tumor size is carried out in each instance. If the clinical course and rate of tumor growth remain unchanged over a 3-year follow-up, annual assessments are recommended. In the event of tumor enlargement, surgery may or may not be recommended, depending on the rate of growth and the age of the patient. Our experience suggests that a rate of growth equal to or exceeding 0.2 cm per year constitutes an indication for tumor removal.     

Non-surgical management of acoustic neuromas

Watchful waiting is one of the options available in the management of acoustic neuromas and this article deals with 13 patients who were so managed. Non-operative management was advised because of age, poor general health, small size of tumour, only hearing ear, or in patients unwilling to undergo surgery for various reasons. This group was followed up at 6-12-monthly intervals and the follow-up period ranged from 1 to 18 years (mean 5.3 years). Ten patients had small tumours and only in 2 of these was increase in tumour size demonstrated on follow-up CT scan. In one this increase was later followed by regression. Two patients required partial removal of tumour because of increasing symptoms after 3 and 7 years of follow-up; one of them died on the twelfth post-operative day. There appears to be a small group of patients for whom delay is worth while rather than to subject all patients with acoustic neuroma to surgery from which full recovery cannot be guaranteed.     

听觉脑干置入用于双侧听神经瘤全聋患者的听力康复(附1例报告)

目的：探讨听觉脑干置入（ABI）用于双侧听神经瘤全聋患者的听力康复。方法：对1例双侧听神经瘤全聋患者，在经乙状窦枕下径路切除第2侧听神经瘤时，同期将12道听觉脑干装置的电极阵置入第4脑室的侧隐窝内，术中行第Ⅶ、Ⅸ脑神经监测，并且记录电刺激脑干诱发电位，以确定和校正电极位置。术后2个月开通电极并作调试。结果：术后头颅X线侧位片示电极位置正确，术后开通调试发现电刺激12个电极均能引起听觉反应，无一电极引起非听觉反应。结论：多道ABI能让双侧听神经瘤全聋患者产生有意义的听觉。术中电极阵准确地置入到脑干耳蜗核是手术成功的关键。     

Conservative management of unilateral acoustic neuromas

OBJECTIVE: The aim of this study was to analyze the natural course of unilateral acoustic neuromas and to evaluate the advantages and disadvantages of an initial conservative approach. METHODS: Between 1989 and 1994, 74 consecutive patients with the diagnosis of unilateral acoustic neuroma were evaluated at the Department of Otorhinolaryngology, University of Zurich. Their charts and magnetic resonance imaging scans were retrospectively analyzed regarding duration and type of symptoms, initial tumor size, tumor growth pattern, and audiometric data. RESULTS: Of the 74 tumors, 68.9% (51/74) did not grow during a mean follow-up of 35 months. Eight (16%) of these tumors had radiologically documented tumor regression. Twenty-three tumors did grow, 16 of them (70%) with a growth rate of <2 mm/year. There was a statistically significant correlation between the first-year and the total growth rate, indicating that the tumor's behavior in the first year is highly predictive of its subsequent growth pattern. Patients with progressive hearing loss as a first symptom had a significantly lower tumor growth than those presenting with tinnitus, sudden hearing loss, or dizziness. Patients with tumor growth showed a greater tendency for hearing loss, although this trend was significant only for isolated frequencies. In 12% of the cases (9/74), the initial conservative approach had to be abandoned in favor of surgery. CONCLUSION: Watchful expectation can be regarded as a safe approach for selected cases of acoustic neuromas. Surgery or irradiation is indicated in patients with proven growth of their tumors.     

Cerebellopontine angle tumours, other than acoustic neuromas. A report on 34 cases. A presentation of 7 bilateral acoustic neuromas.

The symptoms, preoperative diagnostic results and histological findings in 34 cerebellopontine angle tumour cases, other than acoustic neuromas, are presented. The meningeomas dominate by far this group of tumours. The symptomatology is much less uniform than that of the acoustic neuromas, where the VIII nerve is dominant. The facial nerve is less involved, whereas the other cranial nerves give symptoms more frequently. One remarkable sign is the presence of severe trigeminal neuralgia, which we have not encountered in acoustic neuromas. Only 35% of the 'non-neuroma' patients had elevated spinal fluid protein, compared with 100% in our acoustic neuroma cases. Furthermore, 7 patients with bilateral acoustic neuromas are presented. The connection with von Recklinghausens disease and multiple meningeomas is discussed.     

Effectiveness of conservative management of acoustic neuromas

OBJECTIVE: The goal of this study was to assess the effectiveness of the conservative management in patients with acoustic neuroma (vestibular schwannoma). STUDY DESIGN: This retrospective study was performed in a university hospital. PATIENTS: Patients were selected for this wait-and-see policy on the basis of age, general condition, audiometric results, tumor size, and patient preference. The study group included 97 patients, 87 of whom had at least two neuroradiologic examinations. The mean age of this population was 63 years (29 to 89 years). The mean length of follow-up of this population was 31 months. Eighty-seven of these patients had at least two radiologic examinations (magnetic resonance imaging or computed tomography). The mean interval between the initial and follow-up radiologic examinations was 15 months. MAIN OUTCOME MEASURES: Tumor size was measured by use of two-dimensional data in all patients. The mean tumor size was 12 mm. The growth rate of the tumor was estimated by comparison of the results of the measurements from the initial and follow-up neuroradiologic examinations. RESULTS: Of the 97 patients studied, 6 patients required surgery and 6 required radiotherapy. Sixty patients (62%) were still being treated conservatively at the end of the study period. Three patients of 28 who were classified as candidates for hearing preservation surgery lost their candidacy during the observation period. The mean annual tumor growth rate was 1.52 mm/year. The tumor was stable in size in 36% of patients, regressed in 11% of patients, or grew in 53% of patients. The growth patterns of the acoustic neuroma fell into five categories: continuous growth in 15% of patients, negative growth in 5%, growth followed by negative growth in 40%, negative growth followed by growth in 20%, and no variation of tumor size in 20%. CONCLUSION: Conservative management of acoustic neuromas carries difficulties: long-term follow-up of the patients and unpredictability of the tumor growth pattern. A reliable and reproducible radiologic method for evaluating tumor size is of great importance.     

Cochlear implants.

All presently devised single channel devices generate a primitive sensation of hearing by the mechanism of 'periodicity pitch'. No 'place pitch' encoding is possible. Although some enhancement of communicative skills with lip reading results, unaided speech discrimination is not possible. Definite psychological advantages for the totally deaf have been observed with these simple devices. Multiple segments of auditory nerve must be stimulated in a manner which will simulate the complex patterns of neural activity necessary for speech discrimination. Electrode optimization and the pathophysiological consequences of electrical stimulation of the auditory nerve can best be determined in animals. The perceptual consequences of electrical stimulation of the auditory nerve, however, can best be determined in man. How much we will have to innovate the methods of aural rehabilitation will depend upon how well we can generate perceptual speech patterns by electrical excitation of the auditory nerve.     

Conservative management of sporadic unilateral acoustic neuromas

Our objective was to review retrospectively patients with a unilateral acoustic neuroma managed by observation. One hundred patients with tumours (<24 mm) were followed a median 25.5 months. Thirty-six acoustic neuromas grew with four growth patterns. No factors were associated with growth. Eighty percent of growing tumours grew in the first year. Eleven patients proceeded to surgery. Twenty-two patients were eligible for hearing preservation surgery; five of the 15 available for analysis subsequently lost eligibility. In conclusion, selected patients can be safely observed with serial imaging and follow up. Size increase in the first year may predict future growth. Delaying surgery until required by symptoms or tumour growth does not result in more morbidity for the patient. Some may lose the opportunity for hearing preservation surgery but operating on all would result in more sustaining a loss of hearing in the first few years after diagnosis.     

Incidence of acoustic neuromas.

The annual incidence of diagnosed acoustic neuromas in Denmark during two 7-year periods from June 1976 to June 1990 was compared, collecting all operated and non-operated tumors from the entire country. During the first period 278 tumors were diagnosed with an annual incidence of 38 tumors or 7.8 tumors per million per year. During the second period the annual incidence rose to 48 tumors and 9.4 tumors per million per year. There was a significant decrease of extrameatal tumor size from 33 to 26 mm and diminishing of giant tumors.     

Imaging of acoustic neuromas.

A negative high-quality, high-resolution, contrast-enhanced MRI scan is excellent evidence that a patient does not have an AN. Most nerve sheath tumors have a characteristic appearance, and when a tumor is detected there is seldom any doubt as to the identity of the lesion. There are other causes of enhancement, however, or of high signal that can be mistaken for an AN, and these must be kept in mind when a case is considered positive. In some cases, it may be appropriate to defer surgery to clarify a questionable finding by obtaining a follow-up scan. CT is still a reliable examination. In addition to evaluating the IAC, valuable information about the architecture of the petrous bone and labyrinth is provided. Improvements in imaging technology are occurring at a rapid rate. Thinner slices and more rapid scan techniques will make MRI even more useful in evaluation of the IAC.     

Cochlear implants in the management of hearing loss in Neurofibromatosis Type 2

Objective: Review of cochlear implant (CI) outcomes in patients with Neurofibromatosis Type 2 (NF2), implanted in the presence of an ipsilateral vestibular schwannoma (VS). Hearing restoration was combined in some cases with a Bevacizumab regime.

Method: Retrospective review of 12 patients, managed over the period 2009–2016, at a tertiary referral multidisciplinary NF2 clinic. The patients are grouped by hearing outcomes to explore likely protective factors, and to generate a proposed decision-making tool for the selection of either CI or Auditory Brainstem Implant (ABI).

Results: Four of the 12 patients achieved speech discrimination without lip-reading. In these individuals there is reason to think that the mechanism of their hearing loss was cochlear dysfunction. A further four patients received benefit to lip-reading and awareness of environmental sound. For such patients their hearing loss may have been due to both cochlear and neural dysfunction. Two patients gained access to environmental sound only from their CI. Two patients derived no benefit from their CIs, which were subsequently explanted. Both these latter patients had had prior ipsilateral tumour surgery, one just before the CI insertion.

Conclusion: Cochlear implantation can lead to open set speech discrimination in patients with NF2 in the presence of a stable VS. Use of promontory stimulation and intraoperative electrically evoked auditory brainstem response testing, along with case history, can inform the decision whether to implant an ABI or CI.     

Epidemiology of acoustic neuromas

In Denmark one surgical team, during the last 7 years, has performed about 80 per cent of all acoustic neuroma surgery. Because of this centralization, in such a limited population as that of Denmark, we have attempted to make a epidemiological survey of all diagnosed tumours in the period from 1976 to 1983. Systematic and prospective records were made by the authors of all patients with translabyrinthine operations, and data on patients with suboccipital operations were collected retrospectively from the six neurosurgical departments in Denmark. The average annual incidence was 8 tumours per million inhabitants, with the highest incidence of approximately 13 tumours per million occurring in Copenhagen County. The incidence reported in previously published autopsy series is 800-900 times higher and the following may serve as an explanation for this enormous difference: Autopsy series are in all probability based on highly selected cases; they are predominantly based on elderly people and the incidence is not directly applicable to the population at large. Several of the silent tumours from the autopsy series were located in the cochlea or in the labyrinth and not in the internal ear canal. The conclusion from our study is that both the knowledge and data available at present are insufficient to serve as a basis for an actual calculation of incidence and prevalence of acoustic neuromas.(ABSTRACT TRUNCATED AT 250 WORDS)