Introduction: The association hydrocephalus and vestibular schwannoma (VS) has been known for a long time. However, there is no therapeutic consensus, especially the place of drainage of cerebrospinal fluid (CSF). We report the result of our experience on the management of this pathology from a group of patients with a high volume VS (Koos IV) and operated consecutively. After reflections based on current literature data, we propose a therapeutic decisional algorithm. Materials and Methods: This is an analytical, retrospective study of 171 patients operated on KOOS IV vestibular schwannoma from January 2003 to December 2016 at the Marseille University Hospital Center. Of these, 32 patients with hydrocephalus and stage IV vestibular schwannoma were included. Radio-diagnostic criteria for hydrocephalus were based on Evans’ index, cortical furrow status, and the presence of trans-ependymal resorption. Our sample was divided into 2 groups. The first consisted of patients first operated on their hydrocephalus and secondarily treated with schwannoma surgery (group I);patients who underwent surgical resection of their first-line tumor were group II. Epidemiological, clinical, radiological, therapeutic and monitoring data were analyzed. The comparison of the quantitative variables was made by Fisher’s test. Results: During our study period (13 years), 171 cases of stage IV SV had been operated. The association between hydrocephalus and SV stage IV of Koos accounted for 18.7%. The average age of our patients was 53 years with a sex ratio of 0.7. The clinical picture was primarily composed of otological signs (90.6%), headache (56.3%) and cerebellar involvement (43.8%). The average diameter of VS inponto-cerebellar angle (PCA) was 31.5 mm. The treatment consisted of placing a first shunt of the CSF in 34.4% (group I). The ventriculoperitoneal shunt (VPS) was performed in 90.9% of cases. The first surgical removal of the tumor (group II) involved 65.6% of the patients. The postoperative tumor residue averaged 0.76 cc. The Evans index was evaluated on average at 0.33 in each of the 2 groups postoperatively. The average follow-up time for patients was 51 months. Eight cases of complications were recorded during the study. Secondly, in group II, VPS was performed in 9.5% (2 cases). Conclusion: Hydrocephalus is a condition commonly associated with stage IV vestibular schwannoma. The first optimal surgical excision of the tumor seems to be the treatment of choice for this pathological association. The success of the surgery is very often related to the management of hydrocephalus pre, per and post operative.
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