Objective According to the literature, gamma knife surgery (GKS) is a promising method for intracanalicular vestibular schwannoma (IVS) management, providing excellent tumor growth control rates (91%‐100%) and good hearing preservation rates (41%‐76%), but this evidence originates primarily from a small series of patients. The aim of this study was to present the outcomes of GKS in the largest group of patients with IVS studied to date, with particular emphasis on the long‐term outcomes of treatment. Methods The study included 136 consecutive patients with unilateral IVS, who underwent GKS in 2011‐2015. Mean age of the patients was 54 ± 12.6 years. All patients were operated on with a 192‐source cobalt‐60 gamma knife unit. All patients had complete follow‐up documentation and the mean duration of the follow‐up was 52 ± 13.8 months (6‐83 months). Neurological status (facial and trigeminal nerve), hearing and instability/dizziness presence were determined prior to GKS, immediately after the procedure, and during the follow‐up visits. Results Tumor growth control was obtained in 124/136 (~91.2%) patients. Hearing improvement was observed in 32/136 (23.5%) patients, and there was a distinct cluster of 9 patients (6.6%) regaining serviceable hearing after GKS, whereas in 36 patients (26.5%) was stable. Four patients developed facial nerve dysfunction, including 3 periodic hemifacial spasm and 1 partial paresis, which resolved spontaneously within 12 months of GKS. None of the operated patients showed new, debilitating neurological deficits, including trigeminal sensory disturbances or hydrocephalus. Conclusions GKS is a highly effective treatment for IVS, associated with low morbidity and good tumor growth control.

Fragile X Tremor Ataxia Syndrome (FXTAS) is a syndrome based on expansion of the repeats of CGG triplets. The symptoms include action tremor and cerebellar gait ataxia. Additionally symptomatology of FXTAS may be associated to parkinsonism, executive function deficits, dementia, neuropathy and dysautonomia. We present a case of a patient who after 20 year history of progressive tremor and ataxia, was diagnosed after genetic examination as mutation of FXTAS. For the treatment of tremor the patient underwent Gamma Knife (GK) thalamotomy. Reduced tremor on the right side and improvement in everyday activities were observed in the outcome of the treatment. GK thalamotomy, in the context of this patient, did not significantly affect the ataxia.

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Response to DBS may improve with the number of activated cathodal contacts within the globus pallidus internus.