Facial Nerve Outcomes Following Total Excision of Vestibular Schwannoma by the Enlarged Translabyrinthine Approach.

OBJECTIVE: To study the early and late facial nerve (FN) outcomes in different tumor classes in addition to determining the predictive factors for the same. STUDY DESIGN: A retrospective clinical study. SETTING: A quaternary referral otology and skull base center. PATIENTS AND METHODS: A retrospective study of 1983 cases of vestibular schwannomas (VSs) with preoperative normal FN function, undergoing total excision with anatomical preservation of the nerve by enlarged translabyrinthine approach (ETLA) were included. FN status was recorded postoperatively at day 1, at discharge, and at 1-year follow-up and were analyzed in different tumor sizes. RESULTS: At 1 year, 988 patients with House-Brackmann (H-B) grade I and II FN at day 1 after surgery, 958 (96.9%) maintained their status up-to 1 year. Of the 216 patients with H-B grade III at day 1 after surgery, 113 (52.3%) improved to H-B grade I and II. Similarly, of the 779 patients with H-B grade IV and VI FN function at day 1 after surgery, improvement to H-B III and H-B I and II were noted in 442 (56.7%) and 80 (10.3%) of patients, respectively. Intrameatal and extrameatal tumors upto 2 cm showed better recovery from H-B grade III to H-B I and II and from H-B grade IV and VI to H-B I and III when compared with extrameatal tumors >2 cm (p = 0.001). CONCLUSION: Tumors of smaller sizes have good immediate postoperative FN results and recover well at the end of 1 year while more than 3 cm have poor outcomes and recover poorly at the end of 1 year. When the VSs reaches more than 1 cm, the HB I and II outcomes drop significantly.

Endolymphatic sac tumors in von Hippel-Lindau disease: report of three cases.

OBJECTIVES: To illustrate the diagnostic and pathophysiologic issues of endolymphatic sac tumors (ELSTs) and its clinical association with von Hippel-Lindau (VHL) disease and to demonstrate the interest of genetic testing in such cases. STUDY DESIGN: Retrospective analysis of 3 cases of ELST with VHL in 3 different clinical and prognostic situations. MAIN OUTCOMES: The first case was diagnosed on the concomitant finding of a central nervous system lesion. The familial genetic testing revealed the presence of the VHL mutation in an asymptomatic daughter, which justified regular follow-up. In the second case of known VHL syndromic disease, the pathophysiologic role of the endolymphatic sac was questioned, with vertigo as the initial, unique symptom of ELST. In the third case, a giant hypervascularized ELST, initially diagnosed as jugular paraganglioma, was the unique manifestation of VHL disease. The young age of the patient could explain the absence of other tumors. CONCLUSION: Diagnosis of ELSTs may be difficult especially when the original site of the lesion cannot be clearly identified. Such tumors may be revealed or may develop with VHL disease. On presentation of a lytic, vascularized tumor of the posterior face of the petrous bone, clinicians should systematically search for other manifestations of VHL disease and propose a VHL genetic testing for patients, and relatives in case of a positive test, to detect early asymptomatic other tumors. The type of VHL mutation might predict the aggressiveness.