Vestibular schwannomas are histopathologically benign tumors arising from the Schwann cell sheath surrounding the vestibular branch of cranial nerve VIII and are related to the NF2 gene and its product merlin. Merlin acts as a tumor suppressor and as a mediator of contact inhibition. Thus, deficiencies in both NF2 genes lead to vestibular schwannoma development. Recently, there have been major advances in our knowledge of the molecular biology of vestibular schwannomas as well as the development of novel therapies for its treatment. In this article the authors comprehensively review the recent advances in the molecular biology and characterization of vestibular schwannomas as well as the development of modern treatments for vestibular schwannoma. For instance, merlin is involved with a number of receptors including the CD44 receptor, EGFR, and signaling pathways, such as the Ras/raf pathway and the canonical Wnt pathway. Recently, merlin was also shown to interact in the nucleus with E3 ubiquitin ligase CRL4(DCAF1). A greater understanding of the molecular mechanisms behind vestibular schwannoma tumorigenesis has begun to yield novel therapies. Some authors have shown that Avastin induces regression of progressive schwannomas by over 40% and improves hearing. An inhibitor of VEGF synthesis, PTC299, is currently in Phase II trials as a potential agent to treat vestibular schwannoma. Furthermore, in vitro studies have shown that trastuzumab (an ERBB2 inhibitor) reduces vestibular schwannoma cell proliferation. With further research it may be possible to significantly reduce morbidity and mortality rates by decreasing tumor burden, tumor volume, hearing loss, and cranial nerve deficits seen in vestibular schwannomas.

The presentation of spontaneous intracranial hypotension (SIH) can be associated with various clinical and neuroimaging features that may impede a rapid diagnosis of this entity. The authors report the case of a patient who presented with bilateral third cranial nerve palsies and bilateral subdural hematomas. Intracranial pressure monitoring proved to be useful in the diagnosis and management of SIH in this patient.

Postimplantation DBS lead localization and therefore targeting accuracy was not significantly different between frame-based stereotactic 1.5T-MRI and frame-based stereotactic head CT fused with recent 3T-MRI.

Background:Tinnitus, a profoundly widespread auditory disorder, is characterized by the perception of sound in the absence of external stimulation. The aim of this work is to review the various surgical treatment options for tinnitus, targeting the various disruption sites along the auditory pathway, as well as to indicate novel neuromodulatory techniques as a mode of tinnitus control.Methods:A comprehensive analysis was conducted on published clinical and basic neuroscience research examining the pathophysiology and treatment options of tinnitus.Results:Stereotactic radiosurgery methods and microvascular decompressions are indicated for tinnitus caused by underlying pathologies such as vestibular schwannomas or neurovascular conflicts of the vestibulocochlear nerve at the level of the brainstem. However, subsequent hearing loss and secondary tinnitus may occur. In patients with subjective tinnitus and concomitant sensorineural hearing loss, cochlear implantation is indicated. Surgical ablation of the cochlea, vestibulocochlear nerve, or dorsal cochlear nucleus, though previously suggested in earlier literature as viable treatment options for tinnitus, has been shown to be ineffective and contraindicated. Recently, emerging research has shown the neuromodulatory capacity of the somatosensory system at the level of the trigeminal nerve on the auditory pathway through its inputs at various nuclei in the central auditory pathway.Conclusion:Tinnitus remains to be a difficult disorder to treat despite the many surgical interventions aimed at eliminating the aberrant neuronal activity in the auditory system. A promising novel neuromodulatory approach using the trigeminal system to control such a bothersome and difficult-to-treat disorder deserves further investigation and controlled clinical trials.