Giuseppe De Donato

Otoneurological management of petrous apex cholesterol granuloma.

OBJECTIVE The aim of the study is to review the management of petrous apex cholesterol granuloma. The surgical approaches for drainage or total removal and the wait and see policy were analyzed, and outcomes were evaluated. METHODS Retrospective charts of 27 patients managed for petrous apex cholesterol granuloma with a minimum follow-up of 12 months were analyzed in a quartenary skull base center. Presenting symptoms and signs were recorded, and radiologic imaging was evaluated. Management options included wait and see policy and surgery by several approaches. RESULTS The mean age of patients affected by the lesion was 38.8 years. The mean follow-up was 56.7 months. Patients complained of hearing loss, vertigo, tinnitus, diplopia, hemifacial spasm, trigeminal neuralgia, and facial paresthesia. Twelve patients were managed by wait and see policy, and in this category, only one lesion showed growth during the follow-up. Depending upon size and location, 15 patients were surgically treated by infralabyrinthine approach (9 patients), infratemporal type B approach (3 patients), combined infratemporal type B transotic approach (2 patients), and transotic approach (1 patient). One recurrence was recorded during the follow-up. CONCLUSIONS Radiologic evaluation is required for diagnosis and management. Patients with good hearing can be treated by infralabyrinthine approach. Infratemporal fossa type B approach is advocated in patients with extensive disease and internal carotid artery involvement. Wait and see policy is recommended for asymptomatic cases. Drainage and permanent ventilation are the goals of treatment. Complete removal is indicated in selected cases where placement of drainage tube is not feasible.

Combined endovascular-surgical management of the internal carotid artery in complex tympanojugular paragangliomas.

The infratemporal fossa approach described by Fisch overcame most of the factors that had previously prevented the total removal of tympanojugular paragangliomas (TJP). The remaining problem has been infiltration of the internal carotid artery (ICA) for which there has been no entirely satisfactory solution. At the least, severe encasement risks the possibility of an arterial rupture at surgery. In order to reduce this risk, preoperative endovascular interventions have been employed-mainly balloon occlusion, with or without arterial bypass. Recently, intra-arterial stents to reinforce the encased segment of the ICA have been introduced. This study evaluates our experience with 20 patients affected by TJP in which the ICA has been subjected to preoperative interventions. Ten patients underwent a preoperative balloon occlusion and the other 10 patients had their ICAs reinforced with stents. Problems that arose during embolization necessitated that one patient with a stent required ligation of their ICA. No other problems were encountered during endovascular treatment or surgical resection. In one patient with a stent, it was impossible to establish a cleavage plane between their recurrent tumour and the ICA. These early results are encouraging and suggest that intra-arterial stents have a part to play in the surgical management of large TJPs.

Management of complex tympanojugular paragangliomas including endovascular intervention.

To identify complex cases of tympanojugular paraganglioma (TJP) and to analyze their management and surgical outcome. To present strategy and guidelines on how to deal with the perioperative complicating factors.

Titration Streptomycin Therapy in Meniere's Disease: Long-Term Results

As soon as the use of streptomycin was started for the treatment of tuberculosis, its ototoxic effect was discovered. Initial reports demonstrated that streptomycin was successful in reducing vestibular responses while preserving cochlear activity. Streptomycin sulfate has been used for almost half a century for the treatment of vertigo in patients with Menieres disease. At the Gruppo Otologico, Piacenza, Italy, between 1987 and 1995 we treated 16 patients with either bilateral Menieres disease or with Menieres disease in their only hearing ear, adopting the titration streptomycin therapy protocol of Graham et al. Three patients with follow-up periods of less than 2 years were not included in this study. The ages of the patients ranged from 36 to 64 years with a mean of 49.2 years. The patients were observed for 2 to 8.8 years. The dose of administered streptomycin varied from 14 to 49 gm. Episodic vertigo was totally relieved in all of the patients after initial treatment, whereas hearing remained the same or was improved when compared with the pretreatment levels. Five of the patients experienced recurring vertigo during a period of 4 to 24 months after initial treatment. Oscillopsia persisted in two patients and ataxia in darkness persisted in another two patients. Hearing was unchanged in 14 ears, improved in 2 ears, and worsened in 4 ears at the last follow-up evaluation. The overall outcome indicated that intramuscular titration streptomycin therapy seems to be one of the most important therapeutic options in the treatment of disabling vertigo in patients with either bilateral Menieres disease or Menieres disease in an only hearing ear.

Anatomical considerations of high jugular bulb in lateral skull base surgery

In order to study high jugular bulb management in lateral skull base surgery, an anatomical study was conducted on 30 temporal bones by examining the relationship between the internal auditory canal (IAC) and the jugular bulb. The following parameters were measured: 1) Height of the jugular bulb (H)... distance between the level of jugular bulb dome and the line passing through the confluence of the sigmoid sinus with the jugular bulb (SS-JB), 2) Mastoid length (ML)... distance between the mastoid process and middle cranial fossa dura, 3) Distance between the most inferior part of the porus acousticus and jugular bulb dome (A), 4) Distance between the porus acousticus and SS-JB (B). The jugular bulb was defined as high when it occupied more than two thirds of (B). The incidence of a high jugular bulb was 23 per cent in this study. When the jugular bulb was high, the mean (H) and (A) were 9.4 +/- 1.9 mm and 2.7 +/- 0.5 mm, respectively. (H) was higher on the right side than on the left side. No statistically significant difference was found between small and large mastoids (t-test: p > 0.05). It was concluded that when a high jugular bulb was encountered during lateral skull base surgery, the jugular bulb position allows a very small working area inferior to the IAC. In these cases, a 3 or 4 mm depression of the jugular bulb is necessary in order to expose the lower cranial nerves. This can be accomplished by lowering the jugular bulb with the technique already described.

Clinical Observations on Coexistence of Sudden Hearing Loss and Vestibular Schwannoma

It has long been recognized that sudden hearing loss (SHL) may be a harbinger of vestibular schwannoma (VS). Among 192 VS patients who underwent operation in the Gruppo Otologico, Piacenza, Italy, from April 1987 to October 1995, the charts of 14 (7.3%) cases with a history of SHL were examined. SHL was the first symptom in 8 (4.2%) patients. Eight (57.1%) of 14 VS cases with SHL anamnesis had reported recovery of their previous hearing either totally or partially before establishment of tumor diagnosis. Five (35.7%) cases had recurrent bouts of SHL. SHL was observed less frequently in cases with large tumors (>3 cm). However, the frequency of SHL in patients with small tumors did not differ from that of medium-sized tumors. Awareness about coexistence of SHL and VS, as well as concomitant use of auditory brain stem response and magnetic resonance imaging, is crucial to rule out the diagnosis of VS in a patient with SHL.

Oncologic outcome in surgical management of jugular paraganglioma and factors influencing outcomes

The purpose of this study was to identify the factors that may influence the management outcome in patients with jugular paragangliomas.

Giant Cell Tumors of the Skull Base: Case Series and Current Concepts

Objective: To study the clinical features, tumor characteristics and outcomes of giant cell tumors (GCTs) in the skull base based on long-term follow-up. We also report the largest series of GCTs in the temporal bone and the lateral skull base. Materials and Methods: A retrospective study was conducted of all GCTs managed at the Gruppo Otologico, a quaternary referral skull base institute, in Italy from 1993 to 2013. The clinical features, investigations, surgical management and follow-up were recorded. The surgical approaches used were infratemporal fossa approach (ITFA) type B and D and middle cranial fossa (MCF) approaches. Results and Observations: A total of 7 patients with GCTs of the skull base were treated at our institution. The principal complaints were hearing loss reported in 6 (85.71%) patients, tinnitus in 5 (71.43%) and swelling in 3 (42.9%). Pure-tone audiometry showed conductive hearing loss in 5 (71.43%) patients. High-resolution CT scan and MRI with gadolinium enhancement were done in all patients. Radiology showed involvement of the ITF and middle ear in 6 (85.71%) patients each, temporomandibular joint in 4 (57.14%) patients, invasions of the squamous part of the temporal bone, mastoid, MCF and greater wing of sphenoid in 3 (42.9%) patients each and the petrous bone in 2 (28.6%) patients. ITFA type B was applied as an approach for tumor removal in 5 (71.43%) patients, including a case where an additional MCF approach was employed, and ITFA type D and the transmastoid approach were applied in 1 (14.3%) patient each. Total tumor removal and successful cure was achieved in 6 (85.71%) patients. Subtotal removal leading to recurrence and eventual mortality was the result in 1 (14.3%) patient. Conclusions: A thorough knowledge of the anatomy of the skull base and the various skull base approaches is necessary to tackle GCTs. ITFA type B and D combined with MCF approaches provide good exposure of the tumor with minimal postoperative sequelae and good locoregional control. Recurrence due to either subtotal removal or suboptimal treatment may have disastrous consequences for the patient.

Vertebral Artery Involvement by Tympanojugular Paragangliomas: Management and Outcomes with a Proposed Addition to the Fisch Classification

Objectives: To present the management and outcomes of cases of tympanojugular paraganglioma (TJP) with vertebral artery (VA) involvement, and to propose the addition of the new ‘V’ category to the Fisch classification. Materials and Methods: Retrospective analysis of data from a quaternary neuro-otologic and skull base referral center. We studied 230 TJP patients to identify the cases with VA involvement and present their management, surgical findings and outcome. Results: Out of 230 patients, 8 patients were found to have VA involvement by TJP. The extradural artery was involved in 1 patient and the intradural VA was involved in 6 patients. The intradural and extradural VA were simultaneously involved in 1 patient. Seven of the 8 patients underwent surgery. In 2 patients, preoperative occlusion was performed (1 with balloon, 1 with coils). In 5 of these 7 patients, the tumor was successfully separated from the VA by microdissection with or without endovascular intervention. In another patient, the tumor together with the involved VA was removed. Of 7 patients who had surgery, 1 patient did not undergo endovascular intervention and had subtotal tumor removal from the VA. There were no serious complications in removing the tumor from around the VA. Conclusion: Although uncommon, the intradural or extradural VA can be involved by TJP. To avoid vascular accidents, the VA should be thoroughly evaluated at the planning stage. We advocate that the addition of a ‘V’ category would help to identify patients who need individualized VA management as part of a complete surgical evaluation.

Management of Vagal Paragangliomas Including Application of Internal Carotid Artery Stenting

Background: The primary treatment of vagal paraganglioma (VP) includes ‘wait and scan’, surgery and radiotherapy. Objectives: To present the clinical findings, surgical treatment including application of internal carotid artery (ICA) stenting to facilitate surgery, and complications, as well as to review the literature and to discuss the decision-making process in the management of VP cases based on our experience and the literature. Design: A retrospective case review of 22 cases with VP. Setting: Quaternary neurotologic and skull base referral center. Material and Methods: The retrospective chart review identified 22 patients presenting with VP. Our indication for surgery was VP in younger patients, irrespective of the existence of vocal cord paralysis. Preoperative endovascular management of the ICA included permanent balloon occlusion (PBO) and stenting. The transcervical approach and the infratemporal fossa approach type A (ITFA) were used. Results: Fifteen cases had multicentric paragangliomas, 5 cases bilateral tumors, 3 cases a genetic mutation, and 2 cases a positive family history. The most common symptoms were hoarseness, tinnitus and hearing loss. The surgical approaches commonly employed for excision were the transcervical approach (9 cases) and the ITFA (12 cases), whereas 1 case did not have surgery. Three cases had PBO and 7 had intracarotid stent insertion. Gross total removal was achieved in 19 cases, and 1 case had a recurrence. Eighteen cases had no dysphagia or were well compensated after surgery. There were no significant complications noted in our series. Conclusions: In younger patients with VP, surgery should be recommended. The proper preoperative endovascular intervention and surgical approach facilitates gross total tumor removal. In the management of bilateral or familial paragangliomas, careful and appropriate decision making is essential.