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.

Lower Four Cranial Nerves in the Management of Glomus Jugulare: Anatomical Study.

Introduction The surgical management that achieves minimal morbidity and mortality for patients with glomus and non-glomus tumors involving the jugular foramen (JF) region requires a comprehensive understanding of the complex anatomy, anatomic variability, and pathological anatomy of this region. Objective The aim of this study is to propose a rational guideline to expose and preserve the lower cranial nerves (CNs) in the lateral approach of the JF. Methods The technique utilized is the gross and microdissection of 4 fixed cadaveric heads to revise the JF's surgical anatomy and high part of the carotid sheath compared with surgical cases to understand and preserve the integrity of lower CNs. The method involves radical mastoidectomy, microdissection of the JF, facial nerve, and high neck just below the carotid canal and the JF. The CNs IX, X, XI, and XII are microscopically dissected and kept in sight up to the JF. Results This study realized well the surgical and applied anatomy of the lower CNs with relation to the facial nerve and JF. Conclusions The JF anatomy is complicated, and the key to safely operate on it and preserving the lower CNs is to find the posterior belly of the digastric muscle, to skeletonize the facial nerve, to remove the mastoid tip preserving the stylomastoid foramen, to skeletonize the sigmoid sinus and posterior fossa dura not only anterior but also posteroinferior to reach and drill the jugular tubercle.

Multicenter evaluation of the digisonic SP cochlear implant fixation system with titanium screws in 156 patients.

OBJECTIVES: We describe and evaluate the process of fixation of the Digisonic SP cochlear implant with two titanium screws. METHODS: The characteristics of this implant allow cochlear implantation using a minimal incision, a subperiosteal pocket, and fixation with two titanium screws, without drilling a custom-fitted seat or creating suture-retaining holes in the skull. The fixation system relies on two tailfins for use of osseo-integratable screws, incorporated into the cochlear implant housing. The first version of this fixation system was modified after a case of device migration: the size of the titanium insert inside the silicone tailfin was increased. Data on 156 patients (8 months to 86 years of age) from a 4-year period in 6 cochlear implantation centers were retrospectively evaluated. Ten patients have undergone bilateral implantation. RESULTS: Of 166 implantations, 4 postoperative infections and 1 device failure after head trauma were reported. No cerebrospinal fluid leaks or epidural hematomas were reported. One device migration was observed in the first series; no device migrations occurred in the second series. CONCLUSIONS: The fixation system with screws embedded in the Digisonic SP involves a fast and simple surgical technique that seems to efficiently prevent implant migration.

An atypical stapedial artery.

The persistence of the stapedial artery is a rare vascular anomaly. It is mostly asymptomatic but sometimes cause conductive hearing loss, pulsatile tinnitus, or vertigo. The estimated prevalence of this rare postembryonic persistence ranged from 0.02% to 0.48%. Four different anatomical forms have been identified, and their preoperative diagnostic is essential. We report the case of an incidental discovery of pharyngo-hyo-stapedial artery, the most uncommon form of persistent stapedial artery. Its per-operative finding has become rare because tomodensitometry is performed in case of conductive hearing loss. The continuous improvement of imagery resolution will probably help to revise the incidence of this malformation.

Endoscope-assisted retrosigmoid approach in hemifacial spasm: our experience.

INTRODUCTION: The use of surgical decompression of facial hemispasm due to the loop in the internal auditory canal is not always accepted due to the risk related to the surgical procedure. Currently a new surgical technique allows surgeons to work in safer conditions. OBJECTIVE: To report the results with endoscope-assisted retrosigmoid approach for facial nerve microvascular decompression in hemifacial spasm due to neurovascular conflict. The surgical technique is described. METHODS: We carried out a prospective study in a tertiary referral center observing 12 (5 male, 7 female) patients, mean age 57.5 years (range 49-71) affected by hemifacial spasm, that underwent to an endoscope assisted retrosigmoid approach for microvascular decompression. We evaluated intra-operative findings, postoperative HFS resolution and complication rates. RESULTS: Hemifacial spasm resolution was noticed in 9/12 (75%) cases within 24h after surgery and in 12/12 (100%) subjects within 45 days. A significant (p<0.001) correlation between preoperative historical duration of hemifacial spasm and postoperative recovery timing was recorded. Only 1 patient had a complication (meningitis), which resolved after intravenous antibiotics with no sequelae. No cases of cerebrospinal fluid leak, facial palsy or hearing impairment were recorded. Hemifacial spasm recurrence was noticed in the only subject where the neurovascular conflict was due to a vein within the internal auditory canal. CONCLUSIONS: The endoscope assisted retrosigmoid approach technique offers an optimal visualization of the neurovascular conflict thorough a minimally invasive approach, thus allowing an accurate decompression of the facial nerve with low complication rates. Due to the less invasive nature, the procedure should be considered in functional surgery of the cerebellar pontine angle as hemifacial spasm treatment, specially when the procedure is performed by an otolaryngologist.

How body position changes visual vertical perception after unilateral vestibular loss.

Visual vertical perception, posture and equilibrium are impaired in patients with a unilateral vestibular loss. The present study was designed to investigate whether body position (standing upright, sitting on a chair and lying supine) influences the visual vertical perception in Menière's patients tested before and after a unilateral vestibular neurotomy. Data were compared with sex- and age-matched healthy participants. During the first postoperative month the body position strongly influences the visual vertical perception. The ipsilesional deviation of the visual vertical judgment gradually increased from standing upright to sitting and to lying supine. The present data indicate that visual vertical perception improves when postural control is more demanding. This suggests that postural balance is a key reference for vertical perception, at least up to one month after vestibular loss.

Lower Four Cranial Nerves in the Management of Glomus Jugulare: Anatomical Study

Abstract Introduction The surgical management that achieves minimal morbidity and mortality for patients with glomus and non-glomus tumors involving the jugular foramen (JF) region requires a comprehensive understanding of the complex anatomy, anatomic variability, and pathological anatomy of this region. Objective The aim of this study is to propose a rational guideline to expose and preserve the lower cranial nerves (CNs) in the lateral approach of the JF. Methods The technique utilized is the gross and microdissection of 4 fixed cadaveric heads to revise the JF's surgical anatomy and high part of the carotid sheath compared with surgical cases to understand and preserve the integrity of lower CNs. The method involves radical mastoidectomy, microdissection of the JF, facial nerve, and high neck just below the carotid canal and the JF. The CNs IX, X, XI, and XII are microscopically dissected and kept in sight up to the JF. Results This study realized well the surgical and applied anatomy of the lower CNs with relation to the facial nerve and JF. Conclusions The JF anatomy is complicated, and the key to safely operate on it and preserving the lower CNs is to find the posterior belly of the digastric muscle, to skeletonize the facial nerve, to remove the mastoid tip preserving the stylomastoid foramen, to skeletonize the sigmoid sinus and posterior fossa dura not only anterior but also posteroinferior to reach and drill the jugular tubercle.

European Position Statement on Diagnosis, and Treatment of Meniere's Disease.

Meniere Disease keeps challenges in its diagnosis and treatment since was defined by Prosper Meniere at the beginning of 19th Century. Several classifications and definition were made until now and speculations still exist on its etiology. As the etiology remains speculative the treatment models remain in discussion also. The European Academy of Otology and Neurotology Vertigo Guidelines Study Group intended to work on the diagnosis and treatment of Meniere's disease and created the European Positional Statement Document also by resuming the consensus studies on it. The new techniques on diagnosis are emphasized as well as the treatment models for each stage of the disease are clarified by disregarding the dilemmas on its treatment. The conservative, noninvasive and invasive therapeutic models are highlighted.

Changes of visual vertical perception: a long-term sign of unilateral and bilateral vestibular loss.

This study investigates how unilateral and bilateral vestibular deafferentation modifies visual vertical perception in the presence of dynamic and static visual cues. We tested 40 Menière's patients before and after (from 1 week to 1 year) a curative unilateral vestibular neurotomy (UVN), and 4 patients with bilateral vestibular loss. Patients' performances were compared with those of 24 healthy subjects. The perception of the dynamic visual vertical (DVV) was investigated during optokinetic stimulations around the line of sight at various angular velocities. The static visual vertical (SVV) was recorded with a stationary visual pattern. In the acute stage after UVN, Menière's patients exhibited drastic impairment of DVV, which was tilted towards the lesioned side, whatever the direction of the optokinetic stimulation. In addition, the SVV was systematically tilted towards the lesioned side. The optokinetic-induced tilt of the vertical was asymmetrically organized around the new SVV with a significant decrease for contralesional stimulations and no change for ipsilesional stimulations, whatever the postoperative time. The SVV regained normal values 1 year postoperatively. For the patients with bilateral vestibular loss, the optokinetic-induced tilt of the visual vertical was drastically increased and symmetrically organized around an unmodified SVV aligned with the gravitational vertical. This study constitutes the first description of the recovery time-course of DVV perception after unilateral vestibular loss. Data reveal a long-term impairment of the DVV perception after unilateral vestibular loss, suggesting an asymmetrical processing of visual information and a permanent increased weight of dynamic visual cues after bilateral vestibular loss.

Age-related hearing loss in CD/1 mice is associated to ROS formation and HIF target proteins up-regulation in the cochlea.

Pathologies of senescence, in particular those of neurosensory organs represent an important health problem. The improvement of the life expectation entails the fast increase of the frequency of the age-related hearing loss (ARHL) in the population. There are numerous factors that contribute to this process, which include altered vascular characteristics, hypoxia/ischemia, genetic mutations and production of reactive oxygen species. We were interested in understanding the mechanisms involved in the cochlear degeneration in a mouse model of ARHL, the cd/1 mice. Since in human, hypoxia/ischemia is an important pathogenetic factor for inner ear disease, the regulation of HIF-1 activity in the cochlea, the presence of radical oxygen species in the cochlea and its subsequent disturbances of cellular signaling cascades were investigated. In this study, we explored auditory function of cd/1 mice at the age of 4, 12 and 24 weeks and correlated it with the presence of oxidative damage in the cochlea, and cochlear HIF-1 responsive target genes regulation, involved in pathways promoting inflammation such as tumor necrosis factor (TNF-alpha), or cell death with the p53 protein, Bax protein and surviving factors with insulin-like growth factor-1 (IGF-1). After implantation of electrodes for auditory nerve acoustic thresholds measurements, we analyzed every cochlea. First, we confirmed that the cd/1 mice presented a characteristic profile of ARHL starting at 12 weeks of age. Then, according to our previous report [Riva, C., Longuet, M., Lucciano, M., Magnan, J., Lavieille, J.P., 2005. Implication of mitochondrial apoptosis in neural degeneration in a murin model for presbyacusis. Rev. Laryngol. Otol. Rhinol. 126 (2), 67-74], we noticed many alterations in the cochlea. Histologically, at 4 weeks, intensive HIF-1alpha expression was detected in the cochlea followed by ROS formation at 12 weeks, which may lead to cochlear degeneration and induction the onset of ARHL in the cd/1 mice model. In the cochlea, while the inner and the outer hair cells remained intact at 4 and 12 weeks, the spiral ganglion was more altered. Moreover, the Schwann cells of the spiral ganglion seemed to be more vulnerable to free radical damage than the neurons and degenerated more rapidly. The mechanisms of degeneration in the spiral ganglion involved a caspase-3 and Bax mediated-apoptosis via p53 protein accumulation. Since oxygen radicals are required for the post-translational stabilization of HIF-1alpha during hypoxia, the tandem " HIF-ROS " induced multiple reactions within the cochlea, like a strong inflammatory response with increased expression of TNF-alpha, and inhibition of neuronal protection mechanisms with repression of IGF-1.

New perspectives for middle ear implants: first results in otosclerosis with mixed hearing loss.

Middle ear implantation is an efficient procedure to restore moderate to severe sensorineural hearing loss (HL) in selected patients. Implantation of such devices requires ossicular chain integrity. Patients suffering from otosclerosis with mixed HL should be eligible for this treatment after stapes surgery with air-bone gap closure. To address this issue, we report four cases of middle ear implantation after or during stapes surgery. Results and complications obtained with Vibrant SoundBridge, MedEl and Middle Ear Transducer, Otologics are reported. Audiologic results were similar to those obtained in cases of sensorineural HL. One case of postoperative labyrinthitis was observed.

Surgical management of lesions of the internal carotid artery using a modified Fisch type A infratemporal approach.

OBJECTIVE: To report seven cases of vascular repair of the internal carotid artery (ICA) using a modified Fisch type A infratemporal approach and a venous grafting. STUDY DESIGN: Retrospective case review. SETTING: Tertiary care center. PATIENTS: We have analyzed the clinical presentation, paraclinical assessment, and postoperative results regarding the vascular repair and the facial and auditory function from seven consecutive patients. All patients have been operated on by a multidisciplinary team of ENT and vascular surgeons. RESULTS: The study includes four men and three women, aged from 21 to 62 years old. Six patients suffered from vascular traumatic injury after motor vehicle accident (n = 5) or cervical manipulation (n = 1) and one patient presented an atheromatous stenosis. All benefited from a vascular repair with a venous grafting through a modified Fisch Type A infratemporal approach. No death and no new stroke were noted (mean follow-up, 34 mo). The postoperative angiographies showed six functional grafts and one asymptomatic thrombosis. Six immediate postoperative facial palsy occurred but recovered to Grade I or II within 6 months. There was one traumatic injury of the facial nerve and one postoperative anacusis. For the six other patients, the reconstitution of the external auditory canal and ossicular chain allowed to limit the hearing loss to a mean air-bone gap of 22.5 dB (range, 15-35 dB). CONCLUSION: The lesions of the intrapetrous aspects of the ICA remain the subject of debates regarding the indication for a vascular repair. For young or in good health patients, the infratemporal approach provides a safe and reliable access to the horizontal segment of the ICA, offering to the vascular surgeons optimal conditions for the vascular repair.

Ear canal keratinocyte culture: clinical perspective.

HYPOTHESIS: Autologous epidermal sheets obtained by cultivating keratinocytes of the external auditory meatus can be used to repair cutaneous defects of the ear canal. The Rheinwald and Green method has been used to know whether the produced epidermal layer preserves its specificities after the culture. BACKGROUND: Using a split-thickness skin graft during a functional ear atresia surgery does not allow for the restitution of external auditory canal self-cleaning. Some authors cultivated external auditory meatus keratinocytes and showed migration capacities of these colonies. METHODS: Samples of preauricular skin and of the bony part of the external auditory canal were harvested from 10 patients. Keratinocytes were extracted and cultured until an epidermal sheet was obtained. The output, the keratinocyte plating efficiency, and the production delay were measured during the culture. Culture product sections and biopsy sections were examined using optical microscopy after standard coloration and indirect immunohistochemistry. RESULTS: Nine epidermal layers from 10 biopsies were obtained in each group. A significant difference between external auditory meatus and preauricular keratinocyte plating efficiency was highlighted. The average production delay of 23 cm2 external auditory canal and preauricular epidermal layers was 21 days. There was no difference in the cytokeratine expression between external auditory canal and preauricular skin, nor between external auditory canal and preauricular culture products. All cultures expressed the cytokeratine 5 characteristic of stratifying epithelium. CONCLUSION: The Rheinwald and Green keratinocyte culture method allows the production of ear canal-stratified epidermal sheets, which can be used for external ear reconstruction.

Improved cryosections and specific immunohistochemical methods for detecting hypoxia in mouse and rat cochleae.

The present study was undertaken to develop an improved cryoembedding method for analysis of mice and rat cochleae, which permits high-quality cryosections and preserves overall structure and cellular resolution as shown by hematoxylin/eosin staining. The preservation of morphology and antigenicity is mandatory to achieve optimal results. A total of 20 male cd/1 mice and 14 male Sprague-Dawley rats were used in experiments for optimization of preservation, fixative, decalcification, embedding and cryosectioning of cochleae from adult and aged rodents. In addition, a novel immunohistochemical procedure (using Hydroxyprobe-1 kit) was developed for detecting regions of hypoxia in mice and rat cochlea. This method employs a primary fluorescent-conjugated monoclonal antibody directed against pimonidazole protein adducts that are created in hypoxic tissues. Subsequent studies of hypoxia inducible factor-1alpha (HIF-1alpha) by immunofluorescence in the cochlea of these animals were performed in order to confirm that immunochemical detection of pimonidazole protein is representative of a hypoxic environment. We conclude that the present method results in high-quality cryosections of cochlear tissues presenting good anatomical and histological preservation. Furthermore, our optimized procedures provide novel tools for the investigation of neuro-sensory-epithelium in physio-pathological situations associated with hypoxia and/or ischemia, such as inner ear development, plasticity, regeneration and senescence.

How minimally invasive vestibular neurotomy for incapacitating Meniere's disease improves dizziness and anxiety.

CONCLUSION: Patients with incapacitating Meniere Disease (MD) suffer in their daily lives and activities because of the dizziness and anxiety induced by MD. Minimally Invasive Vestibular Neurotomy (MIVN) is a safe and effective surgical treatment for these individuals, and improved their dizziness and anxiety. OBJECTIVES: This study aimed to assess the state of dizziness and anxiety of patients with incapacitating MD and its improvement through MIVN. METHOD: A total of 118 patients with incapacitating MD who underwent MIVN in France and Japan were evaluated. The DHI (Dizziness Handicap Inventory), SAST (Short Anxiety Screening Test), and STAI (State Trait Anxiety Index) questionnaires were used to evaluate disequilibrium and anxiety. RESULTS: The MIVN method appears safe and effective for patients with incapacitating MD. Pre-operative assessment results by DHI and SAST were significantly related to each other, and were influenced by lifestyle and profession. This prospective study showed that MIVN improved dizziness and anxiety in these patients.

Visual field dependence-independence before and after unilateral vestibular loss.

To investigate whether visual field dependence-independence changed after unilateral vestibular loss, Menière's patients were tested before and after unilateral vestibular neurotomy and compared with controls. Using the rod and frame test, visual vertical perception was tested under four visual contexts (with a frame tilted either clockwise or counterclockwise, with a vertical frame, and without visual reference). Both controls and Menière's patients before unilateral vestibular loss split into visual field dependent and independent subpopulations. Unilateral vestibular loss did not change this partition but, for both subpopulations, it induced asymmetrical visual field dependence with a reduced or abolished contralesional dependence. Finally, when vertical/horizontal references were provided, visual vertical perception was improved in both subpopulations, suggesting that all patients relied on the same allocentric strategy.

Torsional optokinetic nystagmus after unilateral vestibular loss: asymmetry and compensation.

The aim of this study was to analyse torsional optokinetic nystagmus (tOKN) in 17 patients with Menière's disease before and after (1 week, 1 month and 3 months) a curative unilateral vestibular neurotomy (UVN). The tOKN was investigated during optokinetic stimulations around the line of sight directed towards either the lesioned or the healthy side, at various constant angular velocities. Dynamic properties of tOKN and static ocular cyclotorsion were analysed using videonystagmography. Patients' performances were compared with those of 10 healthy subjects. The results indicate that, in the acute stage after UVN, patients exhibited drastic impairment of tOKN velocity that depended on the direction of stimulation: tOKN velocity increased for ipsilesional stimulations and decreased for contralesional stimulations. These changes were responsible for a dramatic tOKN asymmetry, with ipsilesional directional preponderance of torsional slow-phase eye velocity. The changes were associated with static ocular cyclotorsion towards the operated side. Despite progressive compensation of tOKN deficits over time, tOKN velocity still differed from that recorded preoperatively, and tOKN asymmetry remained uncompensated 3 months after UVN. A static ocular cyclotorsion remained up to 3 months after lesion. These results are the first description of tOKN deficits and recovery after unilateral vestibular loss. They show that vestibular cues contribute to gaze stabilization during optokinetic stimulation around the line of sight. They also strongly suggest that tOKN impairment could be part of the long-term asymmetrical functions reported after unilateral loss of vestibular functions.

Direction and distance deficits in path integration after unilateral vestibular loss depend on task complexity.

The effects of peripheral vestibular disorders on the direction and distance components of the internal spatial representation were investigated. The ability of Menière's patients to perform path integration was assessed in different situations aimed at differentiating the level of spatial processing (simple versus complex tasks), the available sensory cues (proprioceptive, vestibular, or visual conditions), and the side of the path (towards the healthy versus the lesioned side). After exploring two legs of a triangle, participants were required either to reproduce the exploration path, to follow the reverse path, or to take a shortcut to the starting point of the path (triangle completion). Patients' performances were recorded before unilateral vestibular neurotomy (UVN) and during the time-course of recovery (1 week and 1 month) and were compared to those of matched control subjects tested at similar time intervals. Both the angular and linear path components of the trajectory were impaired for patients compared to controls. However, deficits were restricted to the complex tasks, which required a higher level of spatial processing. Most deficits were maximal 1 week after UVN, and some remained up to the first post-operative month. Spatial representation was differentially impaired according to the available sensory cues: deficits were absent in active locomotor blindfolded condition, appeared in conditions involving visual and vestibular information, and were maximal when visual cues alone were available. Finally, concerning the side of the path, unilateral vestibular loss led to global impairment of the internal spatial representation, yet some asymmetrical spatial performances were observed 1 week after UVN. On the whole, results suggest that the environment experienced by the patients is different after UVN and that a different internal spatial representation is constructed, especially for tasks requiring high levels of spatial processing.

Neuro-otologic surgery through minimally invasive retrosigmoid approach: endoscope assisted microvascular decompression, vestibular neurotomy, and tumor removal.

OBJECTIVES: The objective of this study was to describe and evaluate the efficacy of the endoscope assisted minimally invasive retrosigmoid approach. STUDY DESIGN: Retrospective study and literature review. METHODS: From December 1993 to December 2004, a total of 1,177 cases of endoscope assisted minimally invasive retrosigmoid approach were performed at the Otorhinolaryngology unit of Hôpital Nord in Marseille. By using this approach, we performed microvascular decompression for hemifacial spasm and trigeminal neuralgia, vestibular neurotomy for refractory Ménière's disease with repeated attacks of dizziness, and tumor removal of acoustic neurinoma. We examined the results and postoperative complications. RESULTS: All the results were positive, and we did not experience any mortal complications. The most common complication was cerebrospinal fluid leakage, encountered in 42 (3.6%) cases. CONCLUSIONS: We believe that the combination of an endoscope and microscope that provides accurate information with low invasion is becoming indispensable for these types of operations, which are in the category of functional surgery. We report the merits and significance of the approach of combining the endoscope and microscope and discuss the operational technique to perform a minimally invasive surgery as an oto-neurosurgeon.

Prolactinoma in some Ménière's patients--is stress involved?

Dizziness is a common complaint in primary care clinics and can enter the diagnostic profile of different pathologies spanning from psychiatric problems to vestibular dysfunction. Episodes of vertigo in Ménière's patients are often reported to be triggered by stress but no physiological data are available to account for the subjective link. The study involved 42 Ménière's patients hospitalized for neurectomy of the vestibular nerve for relief of incapacitating vertigo. In addition 18 patients with neurinoma of the vestibular nerve and 12 patients with facial spasm, who underwent surgery, served as controls. A blood sample was taken on the day of surgery in order to determine the level of battery of different stress hormones. The most striking observation was the presence of hyperprolactinemia (above 20 microg/l) in 14 Ménière's patients. The presence of prolactinoma was confirmed by MRI in six cases out of six investigated and the others have not yet been followed up in this retrospective study. These observations are clearly indicative for systematic determination of prolactin levels before opting for surgery in Ménière's patients.