Congenital aural atresia treated with floating mass transducer on the round window: 5 years of imaging experience.

PURPOSE: The aim of this paper is to illustrate imaging features of patients affected by congenital aural atresia (CAA) before and after treatment with a Vibrant SoundBridge (VSB) device implanted on the round window. MATERIALS AND METHODS: Ten patients (5 males and 5 females; mean age 22.1 years) with CAA underwent preoperative high-resolution computed tomography (HRCT) to estimate the degree of involvement of the middle- and inner-ear structures and highlight radiological landmarks useful for surgical planning. RESULTS: Bilateral CAA, mostly of the mixed type, was present in 7 patients and ossicular chain abnormalities in 16 ears (94% of cases). The round window region was normal in all patients, whereas facial-nerve course and/or caliber abnormalities were present in 6 ears (35.3%). The tympanic cavity was small in 13 ears (76.5%), whereas the mastoid was well pneumatized in 8/17 (47%). CONCLUSIONS: HRCT provides accurate information about anatomy and malformations of the middle and inner ear and can thus assist the surgeon in planning the procedure.

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.

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.

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.

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.

Hearing habilitation with auditory brainstem implantation in two children with cochlear nerve aplasia.

Patients with aplasia and hypoplasia of the cochlear nerve have no chance of having their hearing restored by stimulating the periphery of the auditory system using the traditional cochlear implant. A possible approach to auditory rehabilitation may be direct electrical stimulation of the cochlear nuclei with an auditory brainstem implant (ABI). Recently, two children, aged 4 and 3 years, respectively, with bilateral severe cochlear malformations and cochlear nerve aplasia received an ABI. The present paper reports the technique and the preliminary results of this experience. The classic retrosigmoid approach was used. The correct position of the electrodes was estimated with the aid of EABRs and neural response telemetry (NRT). No postoperative complications were observed. High-resolution CT scans with a bone algorithm reconstruction technique were taken postoperatively to evaluate electrode placement before discharge. The ABI was activated 30 days after implantation in both patients. To date 16 and 13 electrodes, respectively, have been activated in the two children. Three months after activation the first patient had achieved good environmental sound awareness, good speech detection and some speech discrimination. The second child, 1 month after activation, had achieved good environmental sound awareness and moderate speech detection. To the best of our knowledge this is the first report of patients with hypoplasia of the cochlea and aplasia of the cochlear nerve, aged below 5 years and treated with an ABI.

[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.

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.

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.

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.

Basal turn cochleostomy via the middle fossa route for cochlear implant insertion.

OBJECTIVE: The current article describes the surgical technique and the very preliminary results of insertion of a cochlear implant, via the middle fossa (MF), in patients with middle ear disease. STUDY DESIGN: The study design was a case report and a description of surgical technique. SETTING: The study was conducted at an ENT Department, University of Verona, Verona, Italy. PATIENTS: Two subjects with profound bilateral hearing loss, the first one presenting a bilateral radical mastoidectomy cavity and the second one with fibroadhesive otitis media, were operated on via the current technique. INTERVENTION: After adequate exposure of the MF floor, a triangular bony area between the greater superficial petrous nerve and the projection of the labyrinthine portion of the facial nerve was drilled out. The basal cochlear turn facing the middle cranial fossa floor was easily encountered, a small cochleostomy measuring 1 1/2 mm in diameter was performed on the most superficial part of the basal turn, and the electrode carrier was inserted into the fenestrated cochlea. The receiver-stimulator was positioned on a bone well drilled previously in the temporal squama. MAIN OUTCOME MEASURES: The activity of the inserted electrodes was tested by means of telemetry and intraoperative recording of the electrically evoked auditory responses. Speech perception tests, performed 15 and 30 days after cochlear implant activation, showed a remarkable improvement in the outcomes versus the preoperative values that are provided for comparison. CONCLUSIONS: This new surgical approach to cochlear implant insertion via the MF route allows stimulation of part of the basal and the middle and apical areas of the cochlea, where greater survival rates of spiral ganglion cells are observed. Cochlear implant insertion via the MF approach represents a promising technique for auditory rehabilitation of subjects with a bilateral radical mastoidectomy cavity, patients suffering from middle ear malformations or chronic middle ear disease due to eustachian tube dysfunction, or subjects with doubtful responses to promontory stimulation.

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.

Myelin-containing corpora amylacea in vestibular root entry zone.

The vestibular nerve of patients with Meniere's disease and vascular cross-compression syndrome of the root entry zone due to the antero-inferior cerebellar artery was studied. All patients underwent vestibular neurectomy using the retrosigmoid approach, which permits the removal of a long nerve segment. CA were found in the cytoplasm of astrocytes that had not shown signs of degeneration at the central portion of the vestibular root entry zone. No membrane intervened between CA and the surrounding cytoplasm, which was rich in filaments, in particular near the CA, and poorly equipped with other organelles. CA were round or oval inclusions measuring 10-12 microns in diameter. The matrix of the CA was composed of low-density amorphous material, with irregular masses displaying a medium density. A network of randomly oriented filaments and bilaminar, osmiophilic lipid fragments with the same structure and thickness of myelin layers were embedded in the matrix. The CA rich in bilaminar fragments were recognizable also at low magnification for their high electron density. In the astrocytic cytoplasm, near the CA, round or ovalshaped, electron-dense bodies with a multilamellar structure were often visible. These results confirm the hypothesis that CA may contain degenerating myelin embedded in a microenvironment rich in glucose polymers and that CA could be an indicator of neurodegeneration.

Investigation of the long-term effects of unilateral hearing loss in adults.

The recent audiological literature has put forward the hypothesis that children with unilateral hearing loss (UHL) show delays in educational achievement and academic progress and some behavioural difficulties. This motivated us to investigate the long-term effects of monaural auditory deprivation in a group of adults who had suffered from UHL since childhood. A group of subjects, ranging in age from 30 to 55 years, suffering from sensorineural UHL since early childhood, has been examined for psychosocial and psychoacoustical effects and statistically compared with a control group matched for age and sex. We prepared a questionnaire directed to provide some objective and subjective indices of psychosocial disability and handicap. Some questions were directed towards specific aspects of auditory function; others assessed the degree of education and the type of working performed. The results of the investigation confirmed the superiority of binaural v. monaural hearing. This was clearly demonstrated in psycho-acoustical performance in sound localisation, speech recognition in noise, together with the appreciation of music. On the other hand, the parameters concerned with educational, social and employment achievement did not support the existence of any significant difference between binaurally and monaurally hearing subjects. The data obtained in the present study thus do not support the existence of non-auditory, long-term effects of monaural hearing loss.

Improved auditory performance of cochlear implant patients using the middle fossa approach.

The preliminary results of insertion of a cochlear implant via the middle fossa in nine patients with profound bilateral hearing loss are described. Aetiologies included a bilateral radical mastoidectomy cavity, adhesive otitis media, autoimmune inner ear disease, previous cranial trauma, genetic pre-lingual deafness, and otosclerosis. A classic middle fossa approach was adopted. A small cochleostomy measuring 1.5 mm in diameter was performed on the most superficial part of the basal turn. A Nucleus 24M cochlear implant system (Cochlear Corporation) was inserted in four patients, a Lauraflex implant (Philips Hearing Implants) was used in three patients and a Combi 40+ (Med-el) with a double electrode array in two. Single electrode arrays were inserted from the cochleostomy to the cochlear apex and occupied a portion of the basal turn, as well as the middle and apical turns. Double electrode arrays were inserted, one towards the apex and one into the basal turn of the cochlea towards the round window. The receiver stimulator was positioned in a bone well previously drilled in the temporal squama and the electrode carrier was inserted in the fenestrated cochlea. The activity of the inserted electrodes was tested by means of telemetry and intraoperative recording of electrically evoked auditory brainstem responses (EABR). Speech recognition tests, performed over a period of time ranging from one to six months after cochlear implant activation, yielded better results in these patients compared with those obtained in postlingually deaf patients operated on via the traditional transmastoid route. Cochlear implant insertion via the middle fossa approach is a technique which is suitable for the implantation of patients with bilateral radical mastoidectomy cavities, chronic middle ear disease, middle ear malformations, or with partial obliteration of the cochlea in the basal turn. However, the main advantage of inserting the implant through the middle fossa cochleostomy consists in the possibility of stimulating, with the single array, areas of the cochlea, i.e. part of the basal, middle and apical turns, where a greater survival rate of spiral ganglion cells is known to occur. In addition, with the double array total occupation of the cochlea is possible, providing the possibility of replicating the tonotopic organization of the cochlea. This new approach has led to major improvements in speech recognition in all patients compared with patients operated on via the transmastoid approach and, given the present state of the art, may be the elective approach for optimal implantation outcomes.

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.

"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.