Microsurgery for patients diagnosed with neurofibromatosis type 2 complicated by vestibular schwannomas

Abstract

Most patients diagnosed with neurofibromatosis type 2 (NF2) have bilateral vestibular schwannomas (VS). Through reviewing surgical method and clinical outcomes, we tried to find out a strategy for treatments in NF2 patients with VS.We retrospectively reviewed patients diagnosed pathological NF2 and have had microsurgery (MS) for VS in the PLA Army General Hospital. Seventeen patients were included from January 2000 to December 2016. Fifteen patients had progressive hearing impairment, and 7 ears were totally deaf. Computed tomography and magnetic resonance imaging were used for preoperative and postoperative evaluation. House–Brackmann (H-B) classification was used to evaluate facial function, and the hearing outcome was classified according to American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) hearing classification system. The outcomes included functional hearing, facial function, and complications.In the 17 patients, 9 were men, and the mean age was 27.2 years old. The mean duration of disease was 38.4 months. Twenty-six VS were excised. Nine patients with bilateral VS and unilateral surgery had repeated surgery for the contralateral tumor after 3 to 12 months. The hearing preservation rate was 41.6%. In the 26 excisions for VS, 24 had intact facial nerve. In the other 2 tumor excision, damaged facial nerves had head-to-head adhesion using biological fibrin glue. The rate of facial nerve function preservation was 60%. No mortality or major complication was reported. The follow-up time ranged from 11 to 78 months with a mean value of 39 months.MS is an effective treatment for NF2 patients with VS. The operation for bilateral VS should be staged according to tumor size and bilateral hearing function. However, methods on how to preserve functional hearing and facial function remain the issue. Further randomized controlled studies are needed to find out a better treatment for NF2 patients with VS according to the overall condition.

Social Independence of Patients with Neurofibromatosis Type 2 in Japan: Analysis of a National Registry of Patients Receiving Medical Expense Subsidies, 2004–2013

“…Neurological features such as seizures or blindness were infrequently observed in previous studies. 10,11) In the present study, the most frequently observed neurological feature was hearing loss, and the number of patients with blindness or seizures was comparatively small. While direct comparisons cannot be made, our findings regarding neurological features appear to be similar to those of previous studies.…”

Surgery of the lateral skull base: a 50-year endeavour

“…As pointed out above, the preservation of hearing in NF is more challenging and less successful than in non-NF tumors 35 . Although a recent survey states that surgical resection “invariably leads to complete loss of ipsilateral hearing” 10 , some centers could prove the opposite 3517 . The reported rates of HP were 41.6% 17 , while Zhao achieved 58% of hearing of any quality 18 .…”

“…Although a recent survey states that surgical resection “invariably leads to complete loss of ipsilateral hearing” 10 , some centers could prove the opposite 3517 . The reported rates of HP were 41.6% 17 , while Zhao achieved 58% of hearing of any quality 18 . Samii et al obtained 35% average preservation rate, but in well preserved pre-operative hearing function a conservation rate up to 65% was possible 35 .…”

Long-Term Effects of Bevacizumab on Vestibular Schwannoma Volume in Neurofibromatosis Type 2 Patients

“…7 Nevertheless, NF2 patients may have worse rates of hearing preservation following surgery when compared with patients with sporadic VS. For middle cranial fossa approaches, Doyle and Shelton reported a 38% hearing preservation rate for NF2 patients in 1993, 5 compared with a hearing preservation rate in 68% of patients with sporadic VS. 4 However, Slattery et al later showed a hearing preservation rate of 65% in NF2 patients following a gross-total resection via middle cranial fossa approach, though 57.4% of patients with a minimum followup of 5 years had recurrent tumor in the surgical field. 6 With regard to the retrosigmoid approach, Chen et al reported a hearing preservation rate of 41.6% with a gross tumor resection rate of 84.6% for NF2 patients, 16 compared with hearing preservation rate of 72% for small sporadically arising VS by Yamakami et al 17 Given the bilateral nature of VS in NF2 and the possibility of worsening hearing with either surgery or radiation, conservative management of these tumors has gained favor. With regard to hearing for stable tumors in NF2 patients, a recent systematic review by Lloyd et al suggested that the best management option would be observation with surveillance MRI until the tumors are confirmed to be growing.…”