Bionic ear imaging.

PURPOSE: The aim of this study was to illustrate the different imaging features of middle and inner ear implants, brainstem implants and inferior colliculus implants. MATERIALS AND METHODS: We retrospectively reviewed the computed tomography (CT) images of 468 patients with congenital or acquired transmissive or neurosensory hearing loss who underwent surgery. The implants examined were: 22 Vibrant Soundbridge implants, 5 at the long limb of the incus and 17 at the round window, 350 cochlear implants, 95 brainstem implants and 1 implant at the inferior colliculus. All patients underwent a postoperative CT scan (single or multislice scanner) and/or a Dentomaxillofacial cone-beam CT scan (CBCT) (axial and multiplanar reconstruction), and/or a plain-film radiography to visualise the correct position of the implant. RESULTS: The CBCT scan depicts Vibrant site of implant better than plain-film radiography, with a lower radiation dose compared to CT. For cochlear implants, a single plain radiograph in the Stenvers projection can directly visualise the electrodes in the cochlea. All patients with brainstem or inferior colliculus implants underwent postoperative CT to exclude complications and the assess correct implantation, but the follow-up of these implants can be performed by plain radiography alone. CONCLUSIONS: CT and CBCT scans are reliable and relatively fast methods for precisely determining the location of middle ear implants. CBCT is preferable to CT because of the lower radiation dose administered; a single plain-film radiograph is enough to visualise and follow-up cochlear, brainstem and inferior colliculus implants.

Auditory brainstem implant in posttraumatic cochlear nerve avulsion.

Patients aged over 12 years with neurofibromatosis type 2 are considered candidates for an auditory brainstem implant (ABI). This study extends the indication criteria of ABI to subjects with profound hearing loss due to damaged cochleas and/or cochlear nerves (CNs) following head injuries. In our department, over the period from April 1997 to November 2002, 32 patients, 23 adults and 9 children, were fitted with ABIs. Their ages ranged from 14 months to 70 years. These patients were suffering from a variety of tumor (13 subjects) and nontumor CN or cochlear diseases (19 subjects). Six patients, 5 adults and 1 child, had profound hearing loss following head injury. Their mean age was 25 years (range: 16-48 years). Five were male and 1 female. The retrosigmoid approach was used in all 6 patients. The electrode array was inserted into the lateral recess of the fourth ventricle and correct electrode positioning was monitored with the aid of electrically evoked auditory brainstem responses and neural response telemetry. Correct implantation was achieved in all patients. No complications were observed due to implantation surgery or related to ABI activation and stimulation of the cochlear nuclei. At activation, an average of 9.8 electrodes (range 5-13) were switched on without side effects. One to 6 electrodes were activated in the following sessions after time periods ranging from 2 to 16 months. All patients achieved auditory-alone-mode closed-set word recognition scores ranging from 40 to 100%; 3 had auditory-alone-mode open-set sentence recognition scores of 60-100%; 2 of these even had speech-tracking performance scores of 38 and 43 words, respectively, showing an ability to engage in normal conversation and converse over the phone. The present study demonstrates that the ABI is a useful rehabilitation instrument in subjects with damaged cochleas and/or CN avulsion following head injury who are unamenable or poorly responsive to auditory rehabilitation using cochlear implants.

The retrosigmoid approach for auditory brainstem implantation.

OBJECTIVE: To describe our experience with the retrosigmoid-transmeatal (RS-TM) approach in auditory brainstem implantation (ABI) as well as the anatomosurgical guidelines for this route. STUDY DESIGN: Retrospective case review. SETTING: Ear, Nose, and Throat Department of the University of Verona. PATIENTS: Five patients with neurofibromatosis type 2 (NF2) were operated on for vestibular schwannoma removal with ABI implantation from April 1997 to June 1999. The patients were four men and one woman, whose ages ranged from 22 to 37 years. The tumor sizes ranged from 12 to 30 mm. The records of a total of 179 patients operated on for vestibular schwannoma (VS) removal via the RS-TM approach from January 1990 to June 1999 were also evaluated. Their ages ranged from 18 to 88 years (average 54 years). The tumor sizes ranged from 4 to 50 mm. Five patients had a solitary VS in the only hearing ear. INTERVENTION: The classic RS-TM approach was used in all patients. After tumor excision, for ABI implantation, the landmarks (seventh, eighth, and ninth cranial nerves, choroid plexus) for the foramen of Luschka were carefully identified. The choroid plexus was then partially removed, and the tela choroidea was divided and bent back. The floor of the lateral recess of the fourth ventricle and the convolution of the dorsal cochlear nucleus became visible. The electrode array was then inserted into the lateral recess and correctly positioned with the aid of electrically evoked auditory brainstem responses (EABRs). MAIN OUTCOME MEASURES: Intraoperative EABR and postoperative speech perception evaluation. RESULTS: Auditory sensations were induced in all patients with various numbers of electrodes. Different pitch sensations could be identified with different electrode stimulation. CONCLUSIONS: In the authors' experience, the RS-TM approach is the route of choice for patients who are candidates for ABI when there is a chance of hearing preservation during surgery. If auditory function is lost during surgery, anatomical preservation of the cochlear nerve may allow hearing restoration with a cochlear implant. Direct intraoperative recording of cochlear nerve action potentials (CNAPs) and round window electrical stimulation are mandatory for these purposes. In addition, decompression of the intrameatal portion of the vestibular schwannoma and planned partial tumor resection with hearing preservation are also possible with the RS-TM approach.

New approach for cochlear implantation: cochleostomy through the middle fossa.

The middle fossa approach was used in 11 patients with profound bilateral hearing loss for insertion of a cochlear implant. Fibroadhesive otitis media (n = 1), bilateral cavity radical mastoidectomy (n = 1), autoimmune inner ear disease (n = 2), previous cranial trauma (n = 1), genetic prelingual deafness (n = 5), and otosclerosis (n = 1) were the causes of deafness. A cochleostomy was performed on the most superficial part of the basal turn, and the electrode array was inserted up to the cochlear apex. Speech perception tests (1-9 months after cochlear implant activation) yielded better results in these patients compared with a homogeneous group of postlingually deaf patients operated on through the traditional transmastoid route. Insertion of the implant through the middle fossa cochleostomy furnishes the possibility of stimulating areas of the cochlea (ie, the middle and apical turns) where a greater survival rate of spiral ganglion cells is known to occur, with improvement of information regarding the formants relevant for speech perception.

Tumor necrosis factor alpha and human Schwann cells: signalling and phenotype modulation without cell death.

The aim of the study was to evaluate the biological response of human Schwann cells (SC) to tumor necrosis factor alpha (TNFalpha) in vitro and to the inflammatory milieu of chronic inflammatory demyelinating polyradiculoneuritis (CIDP). By immunocytochemical and functional assays, we found that SC expressed TNF receptors and that TNFalpha promoted in SC cultures transient activation of transcription factors NFkappaB and c-jun in the absence of apoptosis. In addition, TNFalpha significantly increased the proportion of non-myelin-forming SC expressing the p75 nerve growth factor receptor. Such phenotypic effect was dose-dependent and partially mediated by NFkappaB, as assessed by functional blockage with acetylsalicylic acid. We then extended our study to a human disease in which SC are exposed to TNFalpha. Increased signals for NFkappaB, but not c-jun, molecules were observed by immunohistochemistry on SC nuclei in nerve biopsies from patients with CIDP, as compared with controls. Irrespective of the presence of nerve inflammation, SC showed no evidence of apoptosis. Taken together, our results suggested that SC are potential targets of TNFalpha and that this cytokine exerted no cytotoxic effects either in vivo or in vitro. Rather, TNFalpha may influence the fate of SC by activating transcriptional pathways and modulating their phenotype.

Retrosigmoid approach for auditory brainstem implant.

The present paper reports our experience with the surgical retrosigmoid-transmastoid (RS-TM) technique for implanting auditory brainstem implants (ABIs). From April 1997 to August 1998, four patients with neurofibromatosis type 2 (NF2) were operated on for vestibular schwannoma removal with ABI implantation. The subjects (three men and one women) ranged in age from 22 to 31 years. Tumour size ranged from 12 to 30 mm. A classical RS-TM approach was performed. After tumour excision, identification of landmarks (VIIth, VIIIth and IXth cranial nerves, choroid plexus) to the foramen of Luschka was carefully carried out. The choroid plexus was partially removed and the tela choroidea divided and deflected. The floor of the lateral recess of the fourth ventricle and the convolution of the dorsal cochlear nucleus became visible. The electrode array was then inserted into the lateral recess and placed in the correct position with the help of electrically-evoked auditory brain stem responses. Auditory sensations were induced in all patients with various numbers of electrodes. Different pitch sensations could be identified with different electrode stimulation. Details of the results are presented. In our series, the RS-TM approach represents the elective route for ABI insertion.

Advantages of the retrosigmoid approach in auditory brain stem implantation.

From April 1997 to December 1999, six patients (five men and one woman), ranging in age from 22 to 37 years with neurofibromatosis type 2 (NF2) were operated on via the classic retrosigmoid-transmental (RS-TM) approach for removal of a vestibular schwannoma (VS) (tumor size from 12 to 40 mm) and for auditory brain stem implantation (ABI). After tumor removal, the floor of the lateral recess of the fourth ventricle and the convolution of the dorsal cochlear nucleos were reached, and the ABI was inserted. More recently, an ABI was implanted via the retrosigmoid approach in a 4-year-old boy with a cochlear malformation (common cavity) associated with cochlear nerve aplasia. Electrically evoked auditory brain stem responses (EABRs) and neural response telemetry (NRT) were performed to verify the correct positioning of the inserted electrodes. No major complications related to ABI were observed. ABI has been activated to date in five of the NF2 patients. Auditory sensations with various numbers of electrodes were evoked in all patients. We consider the RS-TM approach the route of choice for ABI insertion in patients with NF2 and good hearing, offering a chance of hearing preservation, and in patients with complete cochlear ossification, severe head trauma and cochlear fracture, or nerve disruption, or a combination of these. A new indication for ABI implantation via the RS approach is presented by patients with bilateral cochlear nerve aplasia.

Intraoperative monitoring for hearing preservation and restoration in acoustic neuroma surgery.

The present article reports on our experience with hearing preservation during 158 acoustic neuroma (AN) operations via the retrosigmoid-transmeatal (RS-TM) approach with the aid of intraoperative auditory monitoring. Several auditory monitoring methods are described. Of these, the bipolar cochlear nerve action potential (CNAP) was found to be the most helpful in preserving hearing. Of 106 patients with useful hearing preoperatively, more than 50% had useful hearing after surgery. Electrical auditory brainstem responses were useful in the placement of an auditory brain stem implant (ABI) in 4 patients with neurofibromatosis type 2 (NF2). All 4 reported speech perception benefit and use their ABIs regularly in their lives. It is our firm belief that intraoperative auditory monitoring has a pivotal role in the preservation and restoration of hearing in AN surgery.

Human neoplastic Schwann cells: changes in the expression of neurotrophins and their low-affinity receptor p75.

Neurotrophins are known to influence Schwann cells during development and to promote peripheral nerve regeneration after axonal damage. In neoplastic conditions. Schwann cells from experimentally-induced schwannomas appear to retain their responsiveness to nerve growth factor (NGF), although the role of neurotrophins in the neoplastic process in poorly understood. In this study, human neoplastic Schwann cells (five cases of acoustic schwannoma and two cases of malignant peripheral nerve sheath tumours [MPNST]) were investigated for the expression in situ of molecules of the neurotrophin system. In particular, we studied the 75 kDa low-affinity receptor (p75) and the mRNA for its ligands, NGF and neurotrophin-3 (NT-3). By immunohistochemistry, the p75 receptor was found to be the present at high levels in Schwann cells from acoustic schwannomas, whereas it was very weak or absent in MPNST. Messenger RNA for NGF and NT-3 was detected by reverse transcriptase in situ polymerase chain reaction technique and showed the same fluctuation of p75, being up-regulated in acoustic schwannomas and very weak or absent in MPNST. In normal non-neoplastic tissue, no detectable amounts of either ligand or receptor were observed. Our results indicate that changes in the expression of neurotrophins and their p75 receptor occurred during the neoplastic transformation of Schwann cells. In benign schwannomas, such changes are likely to reflect the loss of axonal contact, while in MPNST they may be related to a complete derangement of cell machinery in the tumour cells.

"En-bloc" removal of small- to medium-sized acoustic neuromas with retrosigmoid-transmeatal approach.

Surgery of acoustic neuroma (AN) has significantly refined over the past years due to a series of advances in diagnostics and surgical technique. Electrophysiologic investigation performed during surgery has greatly contributed to this progress, increasing the surgeon's understanding of the mechanism of damage and suggesting various changes in his or her surgical strategy.In this context, the advantages of the retrosigmoid "en-bloc" removal of small to medium size ANs have been examined in the present study. At the ENT Department of the University of Verona, 103 subjects with AN were operated on, from January 1990 to December 1995, with a retrosigmoid-transmeatal approach. Eighteen subjects (17.4%) presented pure a intracanalar (IC) tumor and 85 (82.6%) had both IC and extracanalar (EC) involvement. All the IC tumors (n = 18) and 70 of the IC-EC neuromas with an EC size less than 25 mm are reported in this paper for a total of 88 patients. The first 48 patients were operated on via the classic procedures described in the literature, characterized by removal of the tumor after "debulking" and limited exposure of the internal auditory canal (IAC). The following 40 subjects were operated on according to the technique of "en-bloc" removal of the tumor and wide exposure of the IAC.In the "en-bloc" group the tumor was first detached from the cerebellar flocculus and the pons, when necessary. The tumor was not debulked to preserve the anatomic relationship with the nerves and to facilitate identification, cleavage and dissection of the tumor from the neural structures. Thereafter, the posterior wall of the IAC was drilled out and opened in a circumferential range from 180 to 270 degrees . The IAC dura was subsequently opened, and the distal end of the AN along with the vestibular nerves were identified. The vestibular nerves were sectioned in the distal portion of the IAC and dissected with the tumor from the underlying facial and cochlear nerves. Dissection continued medially to the IAC porus. The AN was progressively dissected from the cochlear and facial nerves in the cerebellopontine angle (CPA) with multiple direction maneuvers, as required by the characteristics and degree of adherence to the neural structures.The anatomic and functional results obtained with this new procedure ("en-bloc" removal) were compared with the classic "debulking" technique. The statistical analysis shows an improvement in postoperative outcome for both auditory and facial nerve function. The "en-bloc" removal procedure along with the wide exposure of the content of the IAC and electrophysiologic monitoring of the seventh and eighth cranial nerves are, in our experience, the recommended strategies for improving outcomes in small to medium size ANs.

Mechanisms of auditory impairment during acoustic neuroma surgery.

Hearing loss during removal of acoustic neuroma (AN) may be due to labyrinthine and/or neural and/or vascular damage. Surgical maneuvers relating to perioperative and postoperative hearing may give rise to mechanisms of auditory impairment. Recording action potentials from the intracranial portion of the cochlear nerve (CN) has proven particularly useful for identifying the mechanisms of iatrogenic auditory injury. In this paper intraoperative and postoperative auditory impairments are investigated in relation to surgical steps in a group of 47 subjects with AN (size ranging from 5 to 25 mm) undergoing removal by a retrosigmoid-transmeatal approach. Drilling of the internal auditory canal (IAC), removal of the AN from the IAC fundus, coagulation close to the CN, lateral to medial tumor traction, separation of the CN from the facial nerve, and stretching of the CN have proven to be the most critical surgical steps in hearing preservation. On the other hand, maneuvers such as intracapsular tumor removal, vestibular neurectomy, suction close to the AN, and closure of the IAC defect did not correlate with changes in auditory potentials. Predisposing factors to postoperative hearing deterioration were IAC enlargement greater than 3 mm, IAC tumor size greater than 7 mm, extracanalar tumor size greater than 20 mm, labyrinth medial to the IAC fundus, severe involvement of the CN in the IAC, preoperative abnormal auditory brainstem responses, and normal vestibular reflectivity. Age and preoperative hearing did not prove to be statistically related to postoperative hearing. The variations in morphology and latency of CNAPs are discussed in relation to the mechanisms of iatrogenic injury.

Extrusion of corpora amylacea from the marginal gila at the vestibular root entry zone.

We have studied the vestibular nerve in patents suffering from Meniere' s disease and vascular cross-compression syndrome of the root entry zone due to close contact with the nerve of the antero-inferior cerebellar artery or one of its branches. All patients underwent vestibular neurectomy using the restrosigmoid approach which allows the resection of a relatively long nerve segment. In all the studied vestibular nerves a central and a peripheral zone could be distinguished. In the central zone, a massive accumulation of corpora amylacea (CA) was detected in the cytoplasm of astrocytes. Many CA were seen to protrude from the central nervous system into the pial connective tissue. These structures resembled sessile or predunculated polyps, with a complex system of scissurae at their bases. CA were also found in extracellular location in the pial connective tissue near capillaries, and not wrapped by membranes. Our findings suggest that after their production in astrocytes, CA are transferred into a pial connective tissue across the glial-limiting lamina. Thus, the present results indicate that CA do not merely represent an accumulation of abnormal material, but they could instead be part of a glio-pial system devoted to the clearance of substances from the nervous system.

Vestibular Neurectomy and Microvascular Decompression of the Cochlear Nerve in Meniere's Disease.

Vestibular neurectomy (VN) results in a high success rate in the control of vertigo in Meniere's disease, although the subsequent fate of auditory function is fairly unpredictable. The present investigation reports the postoperative results obtained in a group of 30 subjects with a clinical diagnosis of Meniere's disease and vascular cross-compression of cranial nerve VIII. All subjects underwent VN using a retrosigmoid approach, and in half of them microvascular decompression (MVD) of the cochlear nerve with interposition of autogenous muscle was performed at the same time. All patients had complete relief from vertigo. Hearing was significantly improved in the VN-MVD group (46.7% of subjects). In this group tinnitus and aural fullness also improved significantly, with values of 62.6% and 66.6%, respectively.

Investigations in electrophysiology and morphology of the VIII cranial nerve.

The morphological and functional changes of the eighth nerve in patients undergoing cerebello-pontine angle (CPA) surgery are under investigation in the ENT Department of the University of Verona. The main aspects of the research are: ultrastructural examination of the eighth nerve in normals and subjects operated on for vestibular or cochleo-vestibular neurectomy and for acoustic neuroma; monitoring cochlear nerve function during CPA surgery by monopolar and bipolar recording of cochlear nerve action potential (CNAPs). The specific aims of this research are: to acquire information on the pathophysiology of a number of peripheral cochleo-vestibular disorders; to evaluate the role of intraoperative recording of the eighth nerve in preserving hearing.

[A case of brown tumor of the vomer]. / Osservazione di un caso di tumore bruno del vomere.

A case of brown-tumor of the vomer in a 21-year-old woman is described. The term giant cell lesions encompasses a variety of conditions. Three distinct entities are found in the facial area i.e. giant cell reparative granuloma, true giant cell tumor and brown-tumor. After having described the clinical, radiological and histopathological characteristics of the lesion, emphasis is placed on the importance of clinical, anamnestic, laboratory, radiological and histological findings in accurately differentiating the various types of giant cell lesions of the facial skeleton.