Cochlear implantation in irradiated tympanojugular paraganglioma.

OBJECTIVE: Indications for cochlear implantation have extended progressively over the years both from an audiological and clinical point of view, including the introduction of cochlear implants (CI) for hearing rehabilitation following skull base surgeries and neurotological procedures. In the past, cochlear implantation has been used for hearing rehabilitation in radiation-induced sensorineural hearing loss in nasopharyngeal and tonsillar carcinoma with successful outcomes. Here we describe a similar outcome following total deafness after tympanojugular paraganglioma irradiation, which is also the first such report in literature. DESIGN: The irradiated ear of this patient was implanted with a CI through standard posterior tympanotomy. STUDY SAMPLE: A 26 year old male with multiple paragangliomas with bilateral deafness, secondary to surgery on one ear and radiotherapy for the opposite ear. RESULTS: After a follow-up of 48 months, no local complications have occurred and the hearing results have remained stable with 100% sentence recognition. CONCLUSIONS: Bilateral and multiple paragangliomas are rare tumors. Despite the modality of treatment, the hearing is almost always compromised. In these patients, cochlear implantation offers a new perspective for hearing restoration. This report demonstrates that cochlear implantation can be effectively performed after tympanojugular paraganglioma irradiation with long-lasting, satisfactory results, even in the presence of residual tumor.

Lower cranial nerves function after surgical treatment of Fisch Class C and D tympanojugular paragangliomas.

The aim of this study was to report the postoperative lower cranial nerves (LCNs) function in patients undergoing surgery for tympanojugular paraganglioma (TJP) and to evaluate risk factors for postoperative LCN dysfunction. A retrospective case review of 122 patients having Fisch class C or D TJP, surgically treated from 1988 to 2012, was performed. The follow-up of the series ranged from 12 to 156 months (mean, 39.4 ± 32.6 months). The infratemporal type A approach was the most common surgical procedure. Gross total tumor removal was achieved in 86% of cases. Seventy-two percent of the 54 patients with preoperative LCN deficit had intracranial tumor extension. Intraoperatively, LCNs had to be sacrificed in 63 cases (51.6%) due to tumor infiltration. Sixty-six patients (54.09%) developed a new deficit of one or more of the LCNs. Of those patients who developed new LCN deficits, 23 of them had intradural extension. Postoperative follow-up of at least 1 year showed that the LCN most commonly affected was the CN IX (50%). Logistic regression analysis showed that intracranial transdural tumor extension was correlated with the higher risk of LCN sacrifice (p < 0.05). Despite the advances in skull base surgery, new postoperative LCN deficits still represent a challenge. The morbidity associated with resection of the LCNs is dependent on the tumor's size and intradural tumor extension. Though no recovery of LCN deficits may be expected, on long-term follow-up, patients usually compensate well for their LCNs loss.

The role of wait-and-scan and the efficacy of radiotherapy in the treatment of temporal bone paragangliomas.

OBJECTIVE: To define the role of wait-and-scan and radiotherapy (RT) in the treatment of temporal bone paragangliomas and to review the literature on the outcomes of RT as a treatment modality. MATERIALS AND METHODS: This was a retrospective and literature review. The records of 381 patients with 382 tumors between 1988 and 2012 were analyzed. Patients who were subjected to initial wait-and-scan or primary RT were analyzed for tumor response, facial, and lower cranial nerve status on follow-up (FU). RESULTS AND OBSERVATIONS: None of the 145 patients (38%) with Class A and B tumors were subjected to wait-and-scan or RT and all were operated on. Of the 237 patients (62%) with Class C and D tumors, 47 (19.8%) were subjected to an initial wait-and-scan policy and 1 (0.4%) underwent primary RT. In 24 patients subjected to wait-and-scan with a FU of less than 3 years, tumor remained stable in 22 patients (92%). In 12 patients with a FU between 3 and 5 years, the tumor remained stable or regressed in 10 patients (83%). In 11 patients with a FU of more than 5 years, the tumor remained stable or regressed in 5 patients (45%). A literature review of the results of RT did not conclusively prove that it was effective as a primary modality of treatment for temporal bone paragangliomas. CONCLUSION: In elderly patients with advanced tumors, a wait-and-scan approach is recommended, and RT is initiated only when fast-growing tumors are detected. There is insufficient evidence in literature to suggest that RT could be an effective alternative to surgery in Class C and D tumors.

Intraoperatively diagnosed cerebellopontine angle facial nerve schwannoma: how to deal with it.

OBJECTIVE: This study aimed to report our experience in the management of patients with intraoperatively diagnosed intracranial facial nerve schwannomas (FNSs) and propose a decision-making strategy. METHODS: Twenty-three patients with FNS of the internal auditory canal and/or cerebellopontine angle operated on between 1992 and 2012 were identified. RESULTS: Preoperatively, all cases have been radiographically diagnosed as vestibular schwannomas. Operative procedures consisted of total tumor resection with grafting in 43.4% of patients, near-total resection leaving behind the tumor capsule overlying the facial nerve in 21.7%, total tumor resection with preservation of anatomic continuity of the facial nerve in 13%, and subtotal resection in 4.3%. Four patients (17.4%) underwent bony decompression with no tumor removal. CONCLUSION: Management of FNS diagnosed at surgery represents a significant clinical challenge. We considered total tumor resection with grafting when patients presented with preoperative facial nerve palsy (≥ grade III). Both subtotal and near-total tumor removal can be performed in patients with preoperative good facial function and/or large tumors with brainstem compression. Patients with small tumors who were selected for hearing preservation surgery can be considered for bony decompression. Fascicle preservation surgery may be an option when a clear cleavage plane between the tumor and the facial nerve is found.

State of the art in temporal bone malignancies.

PURPOSE OF REVIEW: To discuss the histological variants, tumor staging, work up and the latest trends in the treatment of malignancies of the temporal bone. RECENT FINDINGS: Because of the rarity of this subset of tumors, there has been no serious attempt to study tumor histologies of the temporal bone other than the squamous cell carcinomas (SCCs). The modified Pittsburgh tumor staging, though popularly used, was primarily developed only for SCC of the external auditory canal. Recent studies have shown that this staging is not without faults. There is also divergence of opinions regarding the surgical procedures to be adopted in treating temporal bone carcinomas. Moreover, the role of radiotherapy and chemotherapy has not been clearly defined. SUMMARY: In this review, we analyzed all the histological varieties of tumors that can arise from the temporal bone and classified them. The merits and demerits of the modified Pittsburgh tumor staging has been discussed outlining the need for further refining this system. The surgical approaches and their applications with respect to the extent of the tumor have been defined. The role of parotidectomy, neck dissection and adjuvant radiotherapy has been discussed.

Management of facial nerve in surgical treatment of previously untreated fisch class C tympanojugular paragangliomas: long-term results.

The aim of this study was to evaluate the long-term facial nerve outcome according to management of the facial nerve in patients undergoing surgery for Fisch class C tympanojugular paragangliomas. The study population consisted of 122 patients. The infratemporal type A approach was the most common surgical procedure. The facial nerve was left in place in 2 (1.6%) of the 122 patients, anteriorly rerouted in 97 (79.5%), anteriorly rerouted with segmental resection of the epineurium in 7 (5.7%), and sacrificed and reconstructed in 15 (12.3%). One patient underwent cross-face nerve grafting. At last follow-up, House-Brackmann grade I to II was achieved in 51.5% of patients who underwent anterior rerouting and in 28.5% of those who underwent anterior rerouting with resection of the epineurium. A House-Brackmann grade III was achieved in 73.3% of patients who underwent cable nerve graft interposition. The two patients in whom the facial nerve was left in place experienced grade I and grade III, respectively. The patient who underwent cross-face nerve grafting had grade III. Gross total resection was achieved in 105 cases (86%). Management of the facial nerve in tympanojugular paraganglioma surgery can be expected to ensure satisfactory facial function long-term outcome.