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The sinonasal tract may be involved in a wide variety of hypervascular lesions, either benign or malignant, and differential diagnosis may be challenging. We present the case of a 26-year-old man with an aggressive ethmoid hypervascular mass invading the anterior skull base and orbit. Because of massive intraoperative bleeding and difficult dissection of the lesion from the periorbita, the planned cranioendoscopic approach had to be converted into a standard craniofacial resection by a combined Lynch and coronal incision. The definitive diagnosis was consistent with lobular capillary hemangioma associated with intravascular papillary endothelial hyperplasia. Two years after surgery, the patient is free of disease. Lobular capillary hemangioma is a hypervascular lesion that may involve the sinonasal tract. The case presented herein is exceptional, both in the presentation and in the difficulties encountered in diagnosis and treatment, because of the concurrence of lobular capillary hemangioma and intravascular papillary endothelial hyperplasia.

Objective:The origin of vestibular schwannoma has always been a matter of debate. The aim of our study was to identify the nerve origin of this tumour.Study design:Prospective case review. This study was conducted at Gruppo Otologico, a private referral centre for neurotology and skull base surgery.Methods:A total of 200 cases of vestibular schwannoma were included in the study. All the tumours were removed surgically utilising the translabyrinthine approach. The origin of the tumour was sought at the fundus of the internal auditory canal.Results:A total of 200 consecutive cases was included in the study. The origin of the tumour was limited to one nerve at the fundus in 152 cases (76 per cent). Out of these cases, the tumour originated from the inferior vestibular nerve in 139 cases (91.4 per cent), from the superior vestibular nerve in nine cases (6 per cent), from the cochlear nerve in two cases (1.3 per cent) and from the facial nerve in two cases (1.3 per cent).Conclusion:The vast majority of vestibular schwannomas originate from the inferior vestibular nerve; the incidence of involvement of this nerve increases as the tumour size increases. An origin of vestibular schwannoma from the inferior vestibular nerve can be considered as one of the explanatory factors for the poor functional outcome of the extended middle cranial fossa approach, and probably accounts also for the better hearing preservation rate reported in some series for the retrosigmoid approach.

Objective: The hearing outcome after implanting a severely ossified cochlea has always been less satisfactory than implanting a patent one. The aim of our study is to present a case where brainstem implantation was successfully performed as an alternative to cochlear implantation in a child with bilateral severe ossification of the cochlea. Study Design: Case presentation. This study was conducted at Gruppo Otologico, Rome, Italy, a private referral center for neurotology and skull base surgery. Methods: The subject of our study was a 12-year-old female child with postmeningitic deafness and bilaterally ossified cochleae. This case is the first brainstem implantation performed at our center with the indication of severe ossification of the cochlea. Results: Successful brainstem implantation of a device was carried out, and the hearing of the patient was restored to the degree that she can freely use the telephone after 8 months of implantation. Conclusion: Although more cases are needed before establishing the exact outcome of brainstem implantation in cases of deafness in the presence of severe bilateral cochlear ossification, preliminary results show the superiority of brainstem implants to conventional or even customized cochlear implants.

Objectives: To study the effect of pre-operative hearing level and tumour size on the hearing outcome of hearing preservation surgery for vestibular schwannoma.Study design and setting: A review of literature conducted at Gruppo Otologico, a tertiary referral centre for neurotology and skull base surgery.Results: A total of 1993 patients in 16 publications addressing the topic of hearing preservation surgery in vestibular schwannoma were analysed. The American Academy of Otolaryngology–Head and Neck Surgery hearing classification system was the classification upon which we based our analysis.Conclusion: Defining hearing preservation as class-A hearing, there was a strong inverse relationship between pre-operative hearing and post-operative hearing levels and between tumour size and post-operative hearing levels.

Exostoses of the external auditory meatus is a well known condition which infrequently requires surgical correction. However, the stenosis caused by severe exostosis can affect quality of life considerably and may require surgical intervention. Canalplasty, in such a situation, is a valid and effective management option. In our series the commonest indication for surgery was recurrent otitis externa. The detailed surgical technique is described and a retrospective analysis of 65 such procedures is presented. There were only two significant complications, both post-operative stenosis, requiring further corrective surgery. In conclusion, canalplasty for the exostosis of the external auditory meatus is a safe surgical option.

Objective Inner ear extension of vestibular schwannomas (VSs) is a rare finding but has important clinical implications. This report reviews the treatment options and presents the experience of the Gruppo Otologico, Piacenza, Italy, in this field. Study Design Case report and literature review. Methods Five cases of VSs with inner ear extension were surgically removed. In all of them, the cochlea was partially or completely invaded by the lesion. Results In 4 cases, the inner ear extension was preoperatively identified on magnetic resonance imaging, and the surgical removal was planned through a transotic approach. In the last case, the cochlear invasion was not detected preoperatively, and the lesion was removed during a second surgery performed to seal a cerebrospinal fluid fistula. Conclusions VSs with inner ear extension should be distinguished from pure intralabyrinthine schwannomas because of differences in clinical significance. Cochlear involvement is more frequent than vestibular involvement and is often accompanied by a dead ear. Dead ear caused by small VSs should alert the surgeon to the possibility of a cochlear extension. The presence of an intracochlear involvement requires the adoption of an approach that allows control of the cochlear turns, and we found the transotic approach to be the most suitable. Undetected cochlear extensions that are left in place may grow with time.

Background The increasing expertise in the field of transnasal endoscopic surgery recently has expanded its indications to include the management of sinonasal malignancies. We report our experience with the endoscopic management of nasoethmoidal malignancies possibly involving the adjacent skull base. Methods A retrospective analysis was performed of patients treated by an exclusive endoscopic approach (EEA) or a cranioendoscopic approach (CEA) from 1996 to 2006 managed by two surgical teams at the Departments of Otorhinolaryngology of the University of Brescia, and the University of Pavia/Insubria-Varese, Italy. Results One-hundred eighty-four patients were considered eligible for the present analysis. An EEA was performed in 134 patients and the remaining 50 patients underwent the CEA. The most frequent histotypes encountered were adenocarcinoma (37%), squamous cell carcinoma (13.6%), olfactory neuroblastoma (12%), mucosal melanoma (9.2%), and adenoid cystic carcinoma (7.1%). Overall, 86 (46.7%) patients received some form of adjuvant treatment. The patients were followed up for a mean of 34.1 months (range, 2-123 months). The 5-year disease-specific survival was 91.4 ± 3.9% and 58.8 ± 8.6% (p = 0.0004) for the EEA and CEA group, respectively. Conclusion To the best of our knowledge, this is the largest series reported to date of malignant tumors of the sinonasal tract and adjacent skull base treated with pure endoscopic or cranioendoscopic techniques. A 5-year disease-specific survival of 91.4% and 58.8% for the EEA and the CEA groups, respectively, seem to indicate that endoscopic surgery, when properly planned and in expert hands, may be a valid alternative to standard surgical approaches for the management of malignancies of the sinonasal tract.

Objective: Jugular paragangliomas with extensive involvement of the internal carotid artery (ICA) represent a true challenge for surgeons, especially in the presence of inadequate collateral circulation through the circle of Willis. The aim of our study is to present a preliminary report of our experience with the surgical removal of three such cases using the stenting of the ICA as the method of choice for protecting and preserving the integrity of the artery. Methods: This retrospective study was conducted at Gruppo Otologico, a private referral center for neurotology and skull base surgery. The subjects of our study are three cases of jugular paragangliomas with extensive involvement of the ICA and inadequate collateral circulation. These cases are the first three cases operated at our center after stenting of the intratemporal portion of the ICA. Results: Complete surgical removal of the tumor, including the part involving the ICA, was achieved in all cases. Over a follow-up period of 22 to 30 months, no complications occurred and the patency of the stented arteries was preserved. Conclusion: Although more follow-up is still needed before establishing the exact long-term outcome of stenting the intratemporal ICA, our preliminary report shows that the stent has facilitated the complete surgical removal of the tumor, preserving the integrity of the ICA.