NF2 is an uncommon genetic disorder, which is characterized by an increased risk of benign nervous system tumour development [
8]. The diagnostic criteria for neurofibromatosis type 2 is bilateral vestibular schwannomas (VS) or family history of NF2 according to first degree family relative, unilateral vestbular schwannomas under 30 years old, or any two of the following: meningioma, glioma, schwannoma, juvenile posterior subcapsular lenticular opacities/juvenile cortical cataract [
9‐
12]. Nunes et al. [
13], presented clinical data on 12 patients with NF2 at age before 18 years and in large number with positive family history. One third of the children had hearing impairments and two thirds presented with dysfunctions of the cranial nerve. Radiological examinations revealed cranial meningiomas in 75% of cases, cranial schwannoms in 83%, and spinal cord tumours in 75%. Evans et al. [
14] showed that at least 18% of NF2 sufferers presented in childhood with isolated features of the disease had no family history. These paediatric patients presented with a more severe course of the disease with multiple tumours. In the study of Bosch et al. [
15], children with early onset of the disease presented with ophthalmologic symptoms and lower motor neuron extremity weakness and in those with late disease onset, eight nerve impairment was observed. MacCollin and Mautner [
16] showed that the first ocular manifestation of NF2 in the paediatric population was visual loss or diplopia. Our patient had an atypical onset of this autosomal-dominant disorder. After a few months, she presented with hearing loss, most often related to the development of vestibular schwannomas. Hearing loss, often accompanied by tinnitus, occurs in around 60% of adults and up to 30% of children [
17,
18]. The MRI scan showed the presence of tumours in the central nervous system and spinal canal. In the literature, there are a great many reports confirming the variety of spinal tumours associated with NF2 [
2,
19,
20]. Bilateral vestibular schwannomas are found in 90–95% of patients with NF2. It is reported that more than 99% of VS in NF2 are benign, but they remain an important cause of mortality due to their location [
1]. Schwannomas can develop along the course of cranial, spinal and peripheral nerves, differently than vestibular. Most often it arises from the oculomotor, trigeminal and facial nerves [
21]. Schwannomas of spinal nerves may result in discrete peripheral neuropathies. In our patient, the histopathology examination showed meningioma in the posterior cranial fossa, which is the second most common tumour connected with NF2. Intracranial meningiomas appear in 45–58% of patients with this disorder and intradural extramedullary spinal meningiomas in 20% [
2]. Although meningiomas are benign, they may give clinical symptoms, the nature of which are related to their size and anatomical location. It is proven that meningiomas associated with NF2 more frequently have higher proliferative activity and a tendency to form more atypical and anaplastic grades than sporadic meningiomas [
22].
The typical treatment strategy for patients with VS is “watch and wait then rescan”, complete with surgical resection and stereotactic radiotherapy. The authors noted that more than 50% of VS were stable in size, so in this situation the “watch and wait then rescan” program could be deemed the proper course [
7]. Of contrary opinion is Brackmann et al. [
23], regarding situations in which tumours are of less than 3 cm in diameter when early surgical management is recommended. It can preserve normal hearing in 30–65% and the functioning of facial nerves in 75–92% of patients. The role of stereotactic radiotherapy is not yet clearly determined [
7].
The majority of meningiomas located in hemispheres and spinal canal can be resected safely and radically. In our patient, the location of the meningioma in the posterior cranial fossa made it impossible to remove completely. In this situation, we agreed that multi-variety protocols should be used. Due to chemotherapy, her overall survival was prolonged.