Filippo Di Lella

Auditory brainstem implants in NF2 patients: results and review of the literature

Objective Neurofibromatosis Type 2 (NF2) patients have multiple central nervous system tumors and, specifically, bilateral vestibular schwannomas (VSs) causing bilateral deafness. If the cochlear nerve is not preserved during tumor removal, the only hearing rehabilitation in these patients could be via an auditory brainstem implant (ABI). Study Design Retrospective case study and literature review. Setting Tertiary referral cranial base center. Patients In 24 NF2 patients, 25 ABIs were placed in the lateral recess of the fourth ventricle after VS surgery via a translabyrinthine approach. Results In this series, a large range of results are observed: from open speech and use of the telephone to no ABI use, because of the poor sound identification ability. Of the 24 patients, 19 use their ABI on a daily basis, 4 are nonusers, and 1 died of NF2 progression. A multivariate analysis did not reveal a good predictor for ABI outcome. In literature, the results of ABI in NF2 are difficult to compare, and the overall outcome was poor compared with cochlear implantation results. Conclusion Auditory brainstem implantation in NF2 patients directly after tumor removal is a safe procedure and the best means of hearing rehabilitation if the cochlear nerve is not preserved. The results in NF2 cases in the literature and these series are poor compared with cochlear implantation. If a cochlear implant is possible, it has the preference over an ABI, also in NF2. Nevertheless, the majority of the patients have benefit of the ABI during daily life particularly in combination with lip reading.

Cochlear implantation in children with cerebral palsy. A preliminary report

OBJECTIVES The aim of this study is to assess the post-implantation speech perception and intelligibility of speech produced by five profoundly deaf children with cerebral palsy. METHODS This study is derived by a review of a prospectively maintained data collection on all patients entering the cochlear implant program. Five children with cerebral palsy who underwent cochlear implantation participated in this study. Functional outcome was assessed using the Speech Perception Categories and the Speech Intelligibility Rating scale. The follow-up of the series ranged from 12 to 45 months. RESULTS At the last follow-up, two children who were placed into speech perception category 1 (detection of a speech signal) preoperatively progressed to category 6 (open-set word recognition with familiar words) postoperatively. Two children moved from preoperative category 2 (pattern perception) to postoperative category 6. One child placed into category 0 (no detection of speech) preoperatively progressed to category 4 (word identification) postoperatively. Before implantation, three children had connected speech unintelligible, and two subjects had connected speech intelligible to a listener who concentrates and lip-reads. At the last follow-up, one child had connected speech unintelligible, two children had connected speech intelligible to a listener who concentrate and lip-reads, one child had connected speech intelligible to a listener who has little experience of a deaf persons speech, and one child had connected speech intelligible to all listeners. CONCLUSIONS Cochlear implantation allowed these patients to dramatically improve their quality of life, increasing their self-confidence, independence and social integration.

Absence of the contralateral internal carotid artery: a challenge for management of ipsilateral glomus jugulare and glomus vagale tumors.

Objective: Extensive involvement of the internal carotid artery (ICA) by jugular and vagal paragangliomas represents a challenging problem for skull‐base surgeons: radical tumor resection and preservation of the vessel often cannot be achieved in these cases. The aim of this article was to report the management of a complex case of right ipsilateral jugular and vagal paragangliomas in a patient with absence of the contralateral ICA due to a previous removal of a left carotid body tumor. Surgical removal of the lesions was performed after stenting of the cervical and intratemporal segment of the ICA.

Intracanalicular meningioma: clinical features, radiologic findings, and surgical management.

Objective: Intracanalicular meningiomas are extremely rare, and only 36 cases have been reported to date. The aim of this study is to describe the clinical, radiologic, and histopathologic features of 13 intracanalicular meningiomas highlighting important aspects of tumor diagnosis and treatment. Study Design: Retrospective study. Setting: Quaternary referral otology and cranial base private center. Patients: Thirteen consecutive patients with pathologically confirmed intracanalicular meningioma surgically treated between December 1988 and July 2006. The follow-up of the series ranged from 13 to 60 months. Interventions: Nine patients with unserviceable hearing were operated on through the translabyrinthine approach. Four patients underwent enlarged middle cranial fossa approach with the intention of preserving their hearing. Results: Total tumor removal was achieved in all cases. The postoperative facial nerve function was either excellent or good (House-Brackmann Grade I or II) in 10 cases (77%) and acceptable (Grade III) in one case. Two patients with preoperative facial paresis showed no improvement in their facial nerve function. In patients who underwent surgery via the enlarged middle cranial fossa, hearing was preserved at the preoperative level in two cases, but serviceable hearing was lost in the remaining two. Conclusion: Primary meningiomas of the internal auditory canal are extremely rare. On account of a lack of specific symptoms and the limited diagnostic findings, preoperative diagnosis of intracanalicular meningioma still represents a diagnostic challenge. Intraoperative findings may help in differentiating meningiomas from other intracanalicular lesions.

Intracranial facial nerve grafting after removal of vestibular schwannoma

OBJECTIVE The objectives of this study were to evaluate outcomes from facial nerve (FN) cable grafting in patients who experienced FN transection during vestibular schwannoma removal and to compare the FN outcomes of patients who underwent FN grafting by using fibrin glue with those of patients who underwent FN grafting by using microsuture. MATERIAL AND METHODS We retrospectively evaluated a series of 33 patients in whom FN grafting was achieved either by using microsuture (8 cases) or fibrin glue (25 cases). Immediate repair of the FN was performed in all cases at the time of initial resection. The patients FN function was assessed preoperatively, in the immediate postoperative period, and at 3, 6, 9, and 12 months or more postoperatively using the House-Brackmann grading system. All patients had at least 1-year follow-up. RESULTS At 12 months, a House-Brackmann grade III was achieved in 75% of those who underwent cable nerve graft interposition by using microsuture and in 76% of those who underwent cable nerve graft interposition by using fibrin glue. Analysis of final FN function outcomes demonstrated no statistically significant difference in FN outcomes between the 2 groups (P = .891, Mann-Whitney U test; P = .1, Fisher exact test). CONCLUSIONS The functional results after FN cable grafting by using fibrin glue exclusively were equivalent to those obtained with microsuture. However, the technique of FN repair by means of fibrin glue is technically simple, less time-consuming, and imparts less trauma on the nerve than does the traditional suture method.

Main peak interleaved sampling (MPIS) strategy: Effect of stimulation rate variations on speech perception in adult cochlear implant recipients using the Digisonic SP cochlear implant

Conclusion: Data obtained from a cohort of 10 post-lingually deaf adult patients indicated that use of a higher stimulation rate, in the setting of the main peak interleaved sampling (MPIS) strategy coupled with the Neurelec-MXM Digisonic SP cochlear implant system, gives a significant advantage in terms of speech perception if compared with a lower rate, especially in a noisy environment. Objectives: To evaluate speech recognition performances in post-lingually deaf adult cochlear implant recipients using two different stimulation rates (260 pps/e and 600 pps/e) in the setting of the MPIS strategy combined with the MXM-Neurelec Digisonic SP cochlear implant system. Patients and methods: Ten post-lingually deaf adults who consecutively received a Neurelec-MXM Digisonic SP device at the CI Center of the University of Parma participated in the study. The study was conducted prospectively as a within-subject repeated measures (ABA protocol) between January 2007 and January 2008. Each subject was exposed to and sequentially tested with two different rates of stimulation (260 pps/e and 600 pps/e), in quiet and in a noisy environment. Statistical analysis was performed on the data obtained. Results: Subjects using the MPIS strategy with a stimulation rate of 600 pps/e performed significantly better in words and phrases recognition tests in both a noisy and a quiet environment.

Cochlear Implantation in a Human Immunodeficiency Virus-Infected Patient

Objectives/Hypothesis: Patients infected with HIV have an increased risk of developing sensorineural hearing loss (SNHL), yet pathogenesis of SNHL in HIV infection is still poorly understood. In subjects affected by bilateral profound or total SNHL, cochlear implantation may be the only possibility to restore a hearing level that allows them to have an acceptable quality of life.

Vestibular Schwannoma in the Only Hearing Ear: Role of Cochlear Implants

Objectives: We sought to delineate the role of cochlear implantation in the management of vestibular schwannoma or other cerebellopontine angle tumors in the only hearing ear. Methods: We performed a retrospective analysis in a quaternary referral skull base center of all patients who were affected by vestibular schwannoma (or other lesions of the cerebellopontine angle) in the only hearing ear and received a cochlear implant before or after tumor treatment (surgery or radiotherapy) or during the wait-and-scan follow-up. We also performed a systematic review of the English-language literature. Results: The clinical and audiological results of 10 patients are reported. All patients were managed with contralateral cochlear implantation. In 7 patients, cochlear implantation was performed before tumor removal, while hearing in the ear with the tumor was still present. In 3 patients, the implant was placed after curative surgery. Nine of the 10 patients routinely use their implant with subjective benefit and fairly good auditory performance (median disyllabic word recognition, 90%; median sentence comprehension, 75%). The literature search retrieved no major series with assessment of the long-term efficacy of cochlear implantation in this rare clinical scenario. Conclusions: Patients affected by vestibular schwannoma in their only hearing ear may significantly benefit from a cochlear implant on the contralateral side prior to tumor removal. Recent and significant hearing deterioration and tumor growth represent the main indications for cochlear implantation.

Nonvascular Lesions of the Jugular Foramen: The Gruppo Otologico Experience

Tumors other than paragangliomas in the jugular foramen are uncommon. Of these, schwannomas and meningiomas predominate. Little clinical data are available in the literature on these tumors at this site. The purpose of this article is to review our experience at the Gruppo Otologico of the management of these tumors. A retrospective series is presented of 32 consecutive patients affected by jugular foramen schwannomas and meningiomas in which their clinical and radiological signs, together with surgical techniques and outcomes, were reviewed. A single-stage resection was possible for the majority of patients when the petro-occipital trans-sigmoid (POTS) approach was used. This allowed resection of both intra- and extradural components of the tumor with hearing preservation and avoidance of facial nerve transposition. No deaths occurred. Lower cranial nerve palsies constituted the major cause of morbidity, but none of the patients required an adjunctive procedure such as vocal cord medialization, tracheostomy, or percutaneous gastrostomy.

Lipomas of the internal auditory canal and cerebellopontine angle.

Objectives: Lipomas of the internal auditory canal (IAC) and cerebellopontine angle (CPA) are exceedingly rare lesions. The purpose of this report was to describe our experience with lipomas of the IAC and CPA and perform a review of the literature. Methods: We report 8 cases of lipomas involving the IAC and/or the CPA that were managed at Gruppo Otologico between April 1987 and October 2012. Results: Four cases of entirely intracanalicular lipomas were radiologically misinterpreted as vestibular schwannomas and underwent tumor removal by a translabyrinthine approach. Two of these patients experienced postoperative facial nerve palsy. Lipomas were suspected in 4 patients on the basis of imaging findings and were managed conservatively. Of these 4 cases, 3 did not show any growth after an average period of 28 months, and 1 case demonstrated tumor growth on follow-up imaging. Conclusions: Neuroimaging represents an extremely important tool for this diagnosis. Attempts to achieve complete resection may result in severe neurologic sequelae, especially in large lesions. Observation with repeated imaging in order to detect growth of the lesion is usually recommended. Debulking of the tumor, mainly aimed at brain stem and cranial nerve decompression, should be considered in cases of disabling and uncontrolled neurologic symptoms and signs.