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Introduction: Vestibular schwannomas (VSs) are benign tumors, developing from neoplastic Schwann cells, that produce myelin sheath covering vestibular nerve fibers. The majority of these tumors are sporadic VS and very rarely they can be associated with neurofibromatosis type 2 (NF2). VSs are usually characterized with slow growth rate, however some of them appear to have a cystic structure, grow more rapidly, can cause facial nerve palsy and brainstem compression. The molecular hallmark of both sporadic and NF-2 associated VS is the inactivation of supresor gene NF-2, also called merlin gene. Aim: In this review we summarize the current knowledge on the molecular biology of VS, including genetic and epigenetic aberrations, altered gene expression and specific microRNA expression profiles.

In recent years a significant progress has been made in the field of molecular background of vestibular schwannoma (VS) emergence and growth. Main genetic and epigenetic aberrations, as well as gene expression changes and specific signaling pathways have been described, that contribute to the development of sporadic VS, and these connected with type II neurofibromatosis (NF2). These findings led to search for potential prognostic markers and biological treatment. In this article we summarize main research directions in the field of molecular diagnostics and biological therapies for VS.