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International audience; Background: To describe the characteristics of the largest European study of MEC of salivary glands and to determine the prognostic factors for overall and disease free survival.Patients and methods: Patients with MEC were prospectively included in the Réseau d'Expertise Français sur les Cancers ORL Rares (REFCOR, French Network of Rare Head and Neck Tumors) database between 2009 and 2015.Results: A total of 292 patients were included. Tumors were classified as low grade in 175 cases (60%), intermediate in 39 (13%) and high grade in 78 (27%). Median follow-up was 26 months. The 5-year OS and DFS rates were respectively 83% and 69%. In multivariate analysis, age (p = 0.004), diabetes (p = 0.02) and advanced stage (p = 0.03) were found to have a significant negative impact on OS. Diabetes (p = 0.001), alcohol consumption (p = 0.003) and advanced stage (p = 0.001) were found to have a significant negative impact on DFS. Compare to low grade, high grade tended to have a negative impact on OS (p = 0.05) and had a significant effect on DFS (0.002) while intermediate grade had no significant influence on survival. The surgical treatment had a positive impact on both OS (p = 0.00005) and DFS (p = 0.0005). Postoperative radiotherapy had no impact in multivariate analysis.Conclusion: Advanced clinical stage, high grade tumor, high age, the impossibility of carrying out a complete surgical resection, and diabetes are the main prognostic factors in this prospective series of patients with MEC. Such findings open new research perspectives on the influence of these components on initial patient care.

International audience; BACKGROUND:Head and neck mucosal melanoma (HNMM) is aggressive and rare, with a poor prognosis because of its high metastatic potential. The two main subtypes are sinonasal (sinonasal mucosal melanoma [SNMM]) and oral cavity (oral cavity mucosal melanoma [OCMM]). Consensual therapeutic guidelines considering the primary tumour site and tumour-node-metastasis (TNM) stage are not well established.MATERIAL & METHODS:Patients with HNMM from the prospective national French Rare Head and Neck Cancer Expert Network database between 2000 and 2017 were included. Clinical characteristics, treatment modalities, outcomes and prognostic factors were analysed.RESULTS:In total, 314 patients were included. The 5-year overall survival (OS) and progression-free survival (PFS) rates were 49.4% and 24.7%, respectively, in the surgery group; no long-term survivors were observed when surgery was not feasible. Moreover, even after surgery, a high recurrence rate was reported with a median PFS of 22 months. In multivariate analysis, Union for International Cancer Control (UICC) stage and tumour site correlated with PFS and OS. Postoperative radiotherapy (PORT) improved the PFS but not OS in patients with small (T3) SNMM and OCMM tumours. Nodal involvement was more frequent in patients with OCMM (p < 10-4), although, as in SNMM, it was not a significant prognostic predictor.CONCLUSION:Even early HNMM was associated with poor oncologic outcomes due to distant metastases despite surgical resection with clear margins. Lymph node metastases had no impact on the prognosis, suggesting treatment de-escalation in cervical node management. PORT might be useful for local control.

L’impianto uditivo del tronco cerebrale e una modalita di riabilitazione dell’udito le cui indicazioni sono rare. Si tratta delle situazioni dove l’impianto cocleare e impossibile o inefficace: presenza di tumori delle vie uditive, ossificazioni cocleari e malformazioni della coclea e del nervo uditivo. La principale e la neurofibromatosi di tipo 2. Le indicazioni sono poste dopo una valutazione pluridisciplinare e il posizionamento del portaelettrodi, la cui configurazione e adattata all’anatomia del tronco cerebrale, e eseguito durante un intervento da parte di un’equipe oto-neuro-chirurgica. I risultati ottenuti dal punto di vista funzionale sono variabili e possono raggiungere, nei casi migliori, quelli ottenuti con un impianto cocleare.

Cochlear implant in adults is a procedure, dedicated to rehabilitate severe to profound hearing loss. Because of technological progresses and their applications for signal strategies, new devices can improve hearing, even in noise conditions. Binaural stimulation, cochlear implant and hearing aid or bilateral cochlear implants are the best opportunities to access to better level of comprehension in all conditions and space localisation. By now minimally invasive surgery is possible to preserve residual hearing and use a double stimulation modality for the same ear: electrical for high frequencies and acoustic for low frequencies. In several conditions, cochlear implant is not possible due to cochlear nerve tumour or major malformations of the inner ear. In these cases, a brainstem implantation can be considered. Clinical data demonstrate that improvement in daily communication, for both cochlear and brainstem implants, is correlated with cerebral activation of auditory cortex.

La finalidad de este articulo consiste en describir los aspectos esenciales de las tecnicas quirurgicas referentes a las protesis auditivas implantables que estan disponibles en la actualidad. Cuando se ha considerado util recordar la anatomia quirurgica, se ha hecho hincapie en los puntos fundamentales. Se revisaran los implantes cocleares, los implantes auditivos del tronco del encefalo, las protesis auditivas osteointegradas y, por ultimo, los implantes de oido medio. Se detallaran las indicaciones, los principios de funcionamiento y los resultados. Se ha previsto un apartado especial para algunas indicaciones especiales que pueden requerirse una modificacion de la tecnica estandar. Asimismo, se describen las complicaciones, que, por desgracia, son una parte integrante de las tecnicas quirurgicas.

Questo articolo ha lo scopo di descrivere l’essenziale delle tecniche chirurgiche riguardo agli ausili uditivi impiantabili attualmente disponibili. Quando e stato giudicato utile richiamare l’anatomia chirurgica, l’accento e stato posto sui punti essenziali. Saranno passati in rassegna gli impianti cocleari, gli impianti uditivi del tronco cerebrale, gli ausili uditivi ad ancoraggio osseo e, infine, gli impianti dell’orecchio medio. Sono ricordati le indicazioni, i principi di funzionamento e i risultati. Sono affrontate le varianti chirurgiche. E stato previsto uno sviluppo per le indicazioni particolari quando puo derivarne una modificazione della tecnica standard. Analogamente sono trattate le complicanze, parte purtroppo integrale delle tecniche chirurgiche.

Resume Introduction La neurofibromatose de type 2 (NF2) est une maladie genetique, a transmission autosomique dominante caracterisee par la presence de schwannomes vestibulaires bilateraux. Etat des connaissances Les autres manifestations de la NF2 incluent des meningiomes, des schwannomes, des ependymomes, localises en intracrânien ou intrarachidien, ainsi que des lesions cutanees et ophtalmologiques. En l’absence de schwannome vestibulaire bilateral, un ensemble de criteres permet neanmoins de diagnostiquer la NF2. L’expression phenotypique de la maladie est variable. Les principaux facteurs de mauvais pronostique sont un âge precoce a l’apparition des premiers symptomes et un nombre eleve de tumeurs au diagnostic. Le produit du gene NF2 , Merlin/Schwannomin est aussi implique dans le developpement de la majorite des schwannomes et meningiomes sporadiques. Perspectives et conclusion La prise en charge des patients NF2 necessite une equipe oto-neurochirurgicale experimentee, dans le cadre de « cliniques NF2 ». Une surveillance clinique et paraclinique annuelle prolongee est souhaitable. Un protocole de depistage familial est propose. Classiquement, seules les tumeurs symptomatiques sont traitees. Certains proposent une attitude active precoce sur les schwannomes vestibulaires afin de preserver l’audition. Lorsqu’un traitement est indique, la chirurgie est le traitement de reference des tumeurs. L’implant auditif du tronc cerebral a une place de choix dans la rehabilitation de l’audition des patients NF2.

Resume Objectifs Le but de cette etude est de presenter les principes de l'implant auditif du tronc cerebral (ITC) et d'evaluer le benefice apporte par cette technique innovante dans differentes indications. Materiel et methodes Les indications de l'ITC sont : la neurofibromatose de type 2 (NF2), les ossifications cochleaires totales bilaterales, les associations de schwannome vestibulaire a une surdite controlaterale et les malformations cochleaires majeures et aplasies du nerf auditif. Le bilan preoperatoire comporte une evaluation clinique, orthophonique, radiologique, et psychologique. La voie d'abord chirurgicale est retrosigmoide ou translabyrinthique. L'evaluation du benefice apporte en termes de perception auditive est realisee avec ou sans le support de la lecture labiale, pour des mots et phrases sans contexte. Resultats Les resultats montrent que, chez les patients NF2, le meilleur benefice fonctionnel auditif est obtenu dans les cas de schwannome vestibulaire de taille moyenne, avec une privation auditive du cote implantee breve ou nulle. Les facteurs de mauvais pronostic sont une privation auditive prolongee, un diametre cisternal superieur a 30 mm, une difficulte a la mise en place du porte-electrodes en peroperatoire et l'activation de moins de dix electrodes avec des sensations auditives. Dans les cas de surdite totale postmeningitique avec ossification cochleaire totale, le resultat obtenu est tout a fait comparable a celui des implants cochleaires dans cette indication (surdite postmeningitique sans ou avec ossification partielle). Il en est de meme pour les autres indications non tumorales (hors NF2). Conclusion Ces resultats demontrent que l'ITC apporte un benefice fonctionnel chez les patients NF2, ce d'autant que la privation auditive est breve et que le volume tumoral est limite. En cas d'ossification cochleaire postmeningitique, le resultat est comparable a celui d'un implant cochleaire, pour des cochlees non ou partiellement ossifiees.

I fluidi del labirinto, perilinfa ed endolinfa, hanno un doppio ruolo fisiologico: concorrono all’attivazione delle cellule ciliate cocleari e vestibolari attraverso la trasmissione del segnale meccanico e partecipano alla trasformazione del segnale in un messaggio nervoso attivando fenomeni molecolari tra i liquidi e le cellule ciliate. Grazie alle osservazioni, tanto istologiche quanto terapeutiche, che hanno suggerito l’esistenza di una relazione tra udito e omeostasi dei fluidi dell’orecchio interno, molti studi hanno permesso di precisare i caratteri fisicochimici dei fluidi del labirinto. Alla fine degli anni Cinquanta due scoperte hanno segnato una svolta decisiva nello studio di questi fluidi: la misura di un potenziale di riposo elevato e positivo nell’endolinfa cocleare (+80 mV rispetto al sangue o alla perilinfa) da parte di Von Bekesy e la dimostrazione di concentrazioni elevate di potassio e basse di sodio nell’endolinfa cocleare e vestibolare da parte di Smith et al. Questi risultati hanno guidato le ricerche, in particolare nella coclea, per i trenta anni successivi, ricerche sviluppate a partire da concetti e tecniche elaborati per la fisiologia cellulare e per la fisiologia dei trasporti transmembranari. Nei successivi venti anni sono state applicate le tecniche di biologia cellulare e molecolare allo studio dell’orecchio interno. La tappa seguente e stata superata con l’identificazione di numerose mutazioni coinvolte nella comparsa di sordita sindromiche o non sindromiche. Attualmente, la composizione dei fluidi del labirinto e ben stabilita, sono state precisate le origini rispettive di perilinfa ed endolinfa, sono stati chiariti i meccanismi cellulari implicati nella secrezione di ciascuno di questi liquidi e sono stati identificati alcuni dei sistemi regolatori di tali fluidi. I progressi realizzati nella comprensione dei meccanismi fisiopatologici implicati nel mantenimento dell’omeostasi dei fluidi dell’orecchio interno dovrebbero permettere, in un prossimo futuro, di aprire nuove strade per la terapia delle sordita e delle vertigini legate direttamente o indirettamente alle anomalie di tali fluidi.

The aim of this study was to assess the effect of corticosteroids administered intra- and postoperatively on the occurrence of facial palsy after a cerebellopontine angle (CPA) tumor resection, and to investigate pre- and intraoperative prognostic factors. A multicenter, prospective, randomized, double-blind and versus-placebo study was conducted between 2006 and 2010. Three hundred and ten patients operated on for a CPA tumor (96% vestibular schwannomas, 4% miscellaneous) were included by five participating centers. The population was stratified into patients with small (≤15 mm CPA on axial MRI views) and large tumors. In each group, patients were randomized to receive corticosteroid (1 mg/kg/day i.v. methylprednisolone intraoperatively and at postoperative days 1-5) or placebo. Steroids did not affect the facial function at postoperative days 1, 8 and 30 in patients with small or large tumors as evaluated by House and Brackmann grading.

We report the MRI findings of six unusual lesions of the internal auditory canal: three haemangiomas, one lipoma, one metastasis and one traumatic neuroma. We compare the findings to those of 20 intracanalicular schwannomas. We noted the site and size of the tumour, its signal intensity, borders and the homogeneity of enhancement were studied on T1-weighted images before and after intravenous contrast medium and T2-weighted images. Most schwannomas were homogeneous lesions, isointense on T1- and T2-weighted images, and strongly enhancing. Spontaneous high signal on T1-weighted images, heterogeneous contrast enhancement and extranodular enhancement were helpful for recognising lesions other then schwannomas; site, size and signal on T2-weighted images were not. All the haemangiomas had a specific pattern of contrast enhancement, with an anterior core intensely enhancing portion and a posterior portion which enhanced moderately or not at all.

The cause of sudden sensorineural hearing loss (SHL) remains unknown, even though studies have evidenced two main hypotheses: a latent virus infection due to herpesvirus and a local decrease in cochlear blood flow caused by vasospasm and/or hyperviscosity. Clinical evaluation and diagnostic tests should always be performed, to rule out an acoustic neuroma, revealed in 5–26% of cases by a sudden deafness. The two main major prognostic factors are the severity of hearing loss and the time from onset to treatment, suggesting that two groups of patients are susceptible to benefit from therapy: patients with a hearing loss of less than 40 dB, managed within 2 months from the onset, and patients with a hearing loss of more than 40 dB, treated within 1 month after the onset of deafness. Steroids appear to be the only treatment available with a significant beneficial effect on SHL.

The efficacy and tolerability of ceftibuten 400 mg o.d. or 200 mg b.i.d. was compared with amoxicillin/clavulanate (AMX/CA, 500 mg/125 mg) t.i.d. in the treatment of acute sinusitis. This was a multicenter, open, randomized, parallel-group 8-day study. In total, 450 patients were evaluable for safety and 400 patients for efficacy. At day 10, clinical cure rates were 83% in the ceftibuten 400 mg o.d. group, 87% in the ceftibuten 200 mg b.i.d. group and 89% in the AMX/CA t.i.d. group; the clinical/radiological cure rates were 52, 44 and 56%, respectively. Clinical and clinical/radiological cure rates at day 40 for the ceftibuten 400 mg o.d. group, ceftibuten 200 mg b.i.d. group and the AMX/CA t.i.d. group were similar, with clinical cure rates of 81, 82 and 87%, and clinical/radiological cure rates of 62, 58 and 63%, respectively. Most adverse events were mild. Ceftibuten, 400 mg o.d. or 200 mg b.i.d., was equally effective and well tolerated as AMX/CA t.i.d. in the treatment of acute sinusitis.

To present three pediatric cases of auditory brainstem implantation (ABI) and review literature data concerning this topic.The first two children had a neurofibromatosis type II with bilateral sensorineural deafness; in both cases, the implant was inserted during the surgical removal of a vestibular schwannoma; the third patient had profound deafness due to bilateral cochlear nerve insufficiency associated with inner ear malformation.Two postoperative complications were observed: patient 1 had a persistent fever which required the replacement of the fat graft used to seal the translabyrinthine approach; patient 3 had a CSF leakage requiring additional surgery and lumbar external drainage. In our three patients, the numbers of active electrodes were 6/22 (Cochlear ABI 24M ABI), 11/12 (Medel Opus II ABI) and 11/12 (implant Medel), respectively. Due to additional major surgical procedures and to disappointing functional results of the ABI, patient 1 stopped wearing her implant 18 months after implantation. Nine months after surgery, patient 2 achieved open-set speech recognition and was very satisfied with the implant. Six months after implantation, patient 3 (cochlear nerve deficiency), who was 3.5 years-old at the time, clearly reacted to some environmental sounds but was not yet able to achieve speech recognition.ABI has now entered the list of treatments that can be proposed in pediatric profound sensorineural deafness. Its major risks of complications are CSF leakage and non-auditory side effects. Its outcomes are worse and less predictable than cochlear implants. Thus, its indications must remain restricted to cases meeting the following conditions: absence of alternative option to restore hearing, patients and parents high level of motivation and realistic expectations.

Resume Cette etude multicentrique evalue la diffusion de la ciprofloxacine dans les structures tissulaires de l'oreille moyenne apres des administrations reiterees de 500 mg par voie orale toutes les 12 heures. Les prelevements ont ete effectues en phase per-operatoire chez des patients adultes devant subir une intervention motivee par une otite chronique. L'administration de ciprofloxacine a debute 9 jours avant l'intervention. Les prelevements ont ete realises a differents intervalles apres la derniere prise afin d'evaluer l'evolution des concentrations en fonction du temps. Les concentrations maximales moyennes observees et les delais d'apparition de celles-ci ont ete les suivants : muqueuse de l'oreille moyenne (n = 16) : 5,54 ± 3,46 μg/g (3–4 heures), os cortical mastoidien (n = 21) : 1,07 ± 1,29 μg/g (4 heures). Les mesures effectuees 12 heures apres la derniere prise montrent que les concentrations de la ciprofloxacine dans la muqueuse de l'oreille moyenne sont encore au moins egales aux CMI de cet antibiotique vis-a-vis de la plupart des germes responsables des surinfections d'otites chroniques. Ces resultats permettent de penser que la ciprofloxacine administree a la posologie de 500 mg PO toutes les 12 heures peut constituer un traitement efficace des otites chroniques suppuratives. Ils devront etre confirmes par des etudes cliniques.

Four cases of facial nerve neuroma were evaluated by computed tomographic (CT) scan and magnetic resonance imaging (MRI). The extension of the tumor in the petrous bone or the parotid gland was well defined by MRI in all cases. CT scan was useful to demonstrate bone erosions and the relation of the tumor to inner ear structures. In cases of progressive facial palsy, CT and MRI should be combined to detect a facial neuroma and to plan the surgical approach for tumor removal and nerve grafting.

Cochlear implant in adults is a procedure, dedicated to rehabilitate severe to profound hearing loss. Because of technological progresses and their applications for signal strategies, new devices can improve hearing, even in noise conditions. Binaural stimulation, cochlear implant and hearing aid or bilateral cochlear implants are the best opportunities to access to better level of comprehension in all conditions and space localisation. By now minimally invasive surgery is possible to preserve residual hearing and use a double stimulation modality for the same ear: electrical for high frequencies and acoustic for low frequencies. In several conditions, cochlear implant is not possible due to cochlear nerve tumour or major malformations of the inner ear. In these cases, a brainstem implantation can be considered. Clinical data demonstrate that improvement in daily communication, for both cochlear and brainstem implants, is correlated with cerebral activation of auditory cortex.

The tele-operated system with three arms for the microsurgery of the middle ear is composed of an operator console from where the surgeon tele-operates three robotized arms that hold surgery tools with a high level of accuracy. The main difference between these micromanipulators and the conventional minimal-invasive surgery robots is the increased field of vision capacity to carry out complex operational gestures without using dextral tool with intra-body mobility. The method used to design the micromanipulator tool holder is described. A first task consists of analyzing functional specifications. The next step is to define and select a kinematic structure adapted to the task. Finally, a dimensional optimization is carried out by using Pareto front method.

Twenty-seven patients with chronic middle ear infection were prospectively studied with pre- and post-contrast magnetic resonance (MR) images to assess the role of MR imaging in the recognition of middle ear tissue abnormalities. The findings were correlated with computed tomographic (CT) scans and surgical and pathologic data. Granulation tissue constantly appeared enhanced on studies done with gadolinium diethylenetriaminepentaacetic acid (DTPA), unlike cholesteatoma, cholesterol granuloma, or brain herniation into the middle ear cavities. Evaluation of extension and thickness of the inflammatory tissue with MR imaging was in accordance with surgical findings. In six cases, isolated granulation tissue misdiagnosed as either a cholesteatoma or herniated brain on CT scans was accurately evaluated on postcontrast MR images. When granulation tissue was associated with other soft-tissue masses, Gd-DTPA-enhanced MR images allowed accurate definition of the site and the extension of each lesion. Furthermore, abnormal meningeal enhancement was precisely depicted by MR images in two cases.

Neurofibromatosis 2 (NF2) is a rare autosomal dominant disease whose hallmark is the development of bilateral vestibular schwannomas.Other features of NF2 include schwannomas, meningiomas, ependymomas, localized along the central nervous system, schwannomas of the peripheral nerves, cutaneous and ophthalmological manifestations. NF2 can be diagnosed in patients without bilateral vestibular schwannoma with sets of diagnostic criteria. Disease phenotype is variable among patients. Main negative prognostic factors are a young age at onset of symptoms and a high number of tumors at diagnosis. NF2 tumor suppressor gene encodes Merlin/Schwannomin, and is also involved in most sporadic schwannomas and meningiomas. Its functions remains largely unknown.Treatment and follow of NF2 patients up require oto-neurosurgical teams experienced in NF2. Yearly and life time surveillance is mandatory. A clinical screening protocol is suggested. Classically, only symptomatic lesions are to be treated. Some authors advocate an early proactive strategy against vestibular schwannoma in order to preserve hearing. When a treatment is advisable, surgery remains the treatment of choice for tumors. Auditory brainstem implant must be taken into account in hearing rehabilitation.

To evaluate results of cochlear implantation in patients with far-advanced otosclerosis.Sixteen patients with far-advanced otosclerosis had undergone unilateral (n = 13) or bilateral (n = 3) cochlear implantation. Surgical difficulties, incidence of complications and postoperative benefit were analyzed.A full electrode insertion was achieved in all patients without surgical difficulties. All patients demonstrated excellent benefit of cochlear implantation. Binaural implantation still improves speech performances, compared to unilateral implantation. In case of residual cochlear function of one nonoperated side, a stapes surgery, performed during the same surgical time as cochlear implantation, can improve speech scores and restore bilateral hearing. Facial nerve stimulation occurred only in 1 patient.Cochlear implantation is the method of choice for rehabilitation of patients with otosclerosis, presenting profound or total hearing loss. Patients obtain excellent benefit with a low rate of complications.

To summarize the indications and evaluate the Auditory Brainstem Implant (ABI) performances in neurofibromatosis type 2 (NF2) and other otologics indications, as postmeningitis ossified cochlea.Main and first indication of ABI is NF2. Emergent indications are bilateral total ossified cochlea, vestibular schwannoma with controlateral lesions, cochlear nerve aplasia or inner ear's malformations. The pre-operative evaluation includes clinical, radiological, lipreading, and psychological status. A translabyrinthine or retrosigmoid approach is performed, depending on tumoral or not tumoral status. The auditory perception with the ABI is evaluated by testing, the words recognition in open-set lists, and the speech understanding with usual sentences.In NF2 patients, best results are obtained in cases of smaller vestibular schwannoma and none, or short term, auditory deprivation. Negative prognostic factors are duration of total hearing loss (10 years), tumor size (30 mm), difficulties in electrode array placement, complications during post-operative course and number of active electrodes (10). In cases of postmeningitis total deafness with totally ossified cochlea, results demonstrate a good benefit reaching these obtained with cochlear implant in post-meningitis deafness.These results show a clear benefit of ABI in NF2 patients, with or without previous tumor removal, in case of small tumor with a short duration of hearing loss. In case of postmeningitis ossified cochlea, results potentially reach those of cochlear implants.

Vertigo and dizziness are the most common complications of cochlear implantation. Data of a multicentric study about clinical aspects and cost utility were collected for vertigo and dizziness: incidence, clinical and treatment were analyzed among 469 adults and children. Results demonstrated that 16% of adults and 3% of children experienced dizziness postoperatively. In a few cases a specific mechanism as perilymphatic fistula was identified. In other cases the mechanism underlying delayed vertigo remains speculative and endolymphatic hydrops was suggested.

Skull base osteomyelitis is an uncommon but severe condition generally secondary to necrotizing otitis externa. The aim of this study was to determine the patients demographics, clinical and radiological findings, and outcomes of this condition.We prospectively follow 5 patients with a diagnosis of skull base osteomyelitis between 2004 and 2005. Clinical, biologic and imaging data were collected, and also follow-up during treatment.The five patients were men and the average age at presentation was 75 years (63-89). All except one had a previous diabetes mellitus, with a more or less recent clinical story of otitis externa. All of them suffered of headaches and cranial nerve(s) deficits, particularly facial nerve. Diagnosis was made on MRI with gadolinium injection and fat saturation, and Pseudomonas aeruginosa identified as pathogen in all cases. One patient deceased of cranial nerves palsy complication. In 4 cases we found previously a too short or non adapted treatment of necrotizing otitis externa.In the setting of headache, cranial nerves deficit and abnormal skull base imaging on MRI with Fat sat, osteomyelitis should be consider as the likely diagnosis. This complication should be avoided by optimal management of necrotizing otitis externa.

* orthophonistes, centre referent implant cochleaire adulte d'Ile de France et surdite genetique de l'adulte du Pr O.Sterkers, groupe hospitalier Pitie-Salpetriere bâtiment Paul Castaigne 50-52 bd Vincent Auriol 75013 Paris ** orthophoniste, Dr en neurosciences, service ORL du Pr G. Lamas, groupe hospitalier PitieSalpetriere 50-52 bd Vincent Auriol 75013 Paris ***Docteur, Unite d’Otologie, Implants Auditifs et Chirurgie de la Base du Crâne, Hopital PitieSalpetriere, AP-HP, Paris. **** Professeur, Unite d’Otologie, Implants Auditifs et Chirurgie de la Base du Crâne, Hopital PitieSalpetriere, AP-HP, Paris.

To evaluate the benefit of cochlear implantation in older adults aged 60 Years and over.Fifty-six profoundly or totally hearing-impaired patients, aged 60 Years and over, were studied retrospectively. At the end of the preoperative evaluation, 28 patients received a cochlear implant. The mean age was 66 Years and the median follow-up was 22.5 Months. Speech perception scores before and after implantation were analyzed in order to evaluate the benefit of cochlear implantation. The speech perception score before implantation was compared to that of the non-implanted patients.There was a significant improvement of the dissyllabic words and sentences scores after implantation. The patients who are over 70 Years performed as well as those who are younger (between 60 and 70 Years). One patient developed a postoperative vertigo due a perilymphatic fistula. There was no flap-related problems. In the non-implanted group (mean age: 68 Years), 18 patients declined the cochlear device because they thought the subjective benefit of their hearing aid was sufficient and 5 patients declined because of surgical risk. The mean age, the cause and the duration of the deafness, and the speech perception scores were similar between implanted and non-implanted patients.This study demonstrates the beneficial effect of cochlear implantation in the elderly. These results suggest that a similar benefit could have been obtained in the patients who declined surgery. An early implantation could reduce the duration of the deafness and preserve binaural sound perception allowing increased performance in older people.

Two cases of progressive bilateral hearing loss associated with superficial hemosiderosis of the central nervous system are reported. This is a rare disease caused by repetitive hemorrhage in subarachnoid spaces with hemosiderin deposits on the brain surface and cranial nerves. MRI provided the etiologic diagnosis in both cases based on typical low-density signals from the brain, the brainstem, and the cerebellar surfaces on T1 and T2 sequences. In one case a fourth ventricle ependymoma, which was the probable cause of hemosiderosis, was also discovered. Based on a literature review, we discuss the pathophysiological hypotheses, the modalities for treating hearing loss including cochlear implantation despite retrocochlear sensorineural hearing loss.

To investigate the surgical techniques and speech performance of multichannel auditory brainstem implant (ABI) in patients with bilateral acoustic neuromas (neurofibromatosis type 2).The nucleus 21 channel auditory brainstem implant was implanted into the lateral recess of the fourth ventricle through the translabyrinthine approach in 7 patients after removal of the tumor. The accurate placement of electrode array was ensured by the electromyogram monitoring of the 7th and 9th nerves and the electrically evoked auditory brainstem responses (EABR). Initial switch-on occurred six weeks postoperatively. Speech evaluation was performed every 3 months for the first year and annually thereafter.During the surgery, the lateral recess could be found and the typical EABR could be recorded in 6 cases. They later reported a significant benefit from the device. Two of the cases have achieved functional open-set speech understanding. In contrast, one patient with no EABR because of difficulty of the anatomic location during the surgery had no sensations postoperatively.The multichannel ABI could effectively restore auditory sensations in patients deafened by bilateral acoustic neuromas. The accurate location of the cochlear nucleus complex during surgery was the key factor for the success of the operation.

Otogenic pneumocephalus is uncommon. Typical causes include trauma, tumor, infection, and nasosinusal or mastoid surgery but spontaneous otogenic pneumocephalus is very exceptional. We report a case of spontaneous otogenic pneumocephalus located in the left temporal lobe revealed by sudden onset aphasia. The temporal pneumatocele was cured through an epidural subtemporal approach with needle puncture of the aeroma and duroplasty. Two years after surgery, no recurrence was observed and the patient remained symptom free.

The principal objectives of acoustic neuroma surgery in the order of priority are absence of mortality and neurological morbidity, total removal of the lesion, preservation of facial function, and preservation of hearing. The aim of this work was to evaluate the selection criteria and the therapeutic strategy for hearing preservation in acoustic neuroma management. This retrospective study included 436 consecutive patients referred to our department for an acoustic neuroma. The population comprised 365 patients (84%) treated surgically, 66 patients (15%) managed conservatively, and 5 irradiated patients (1%). The mean age was 54 years (range: 13-87). The mean follow-up period was 24 months (range: 1-120). The surgical approaches were translabyrinthine in 294 (81%), retrosigmoid in 37 (10%), and through the middle fossa in 34 cases (9%). Approaches preserving the labyrinth were employed in patients aged less than 60 years with lesions measuringor=2 cm and a serviceable hearing (pure tone average50 dB and speech discrimination scoreor=50%). A serviceable hearing was preserved in 53% of the cases with a serviceable hearing in 44% of the cases. Postoperative normal or subnormal facial function was obtained in 72 to 98% of cases depending on tumor size. Two cases (0.5%) of recurrence were reported. In conclusion, our surgical strategy based on age, tumor size and pre-operative hearing function permitted hearing preservation in 50% of selected cases, a high rate of facial function preservation and a low risk of recurrence.

Middle ear adenoma is an uncommon tumor. We report two cases and review the relevant literature concerning this rare entity.Two patients with a middle ear adenoma were operated on in our department. Pre- and postoperative data concerning clinical examination, audiometry, and radiology were obtained. Information concerning intraoperative observations and the pathological examination were also gathered.The diagnosis of middle ear adenomas is based on clinical, radiological, and pathological confrontation. The microscopic examination should be combined with histochemical and immuno-histochemical methods in order to evidence the glandular and neuroendocrin components of these lesions.Today, classified as a benign epithelial tumor, the middle ear adenoma is associated with an excellent prognosis provided total excision.

To analyze outcome after otosclerosis surgery with stamedeotomy with blood clot sealing.Otosclerosis surgery was performed in 150 adult patients between 1997 and 1999 by five surgical teams (70% of the procedures were performed by senior surgeons) and followed for 18 months. Stapedotomy was carried out under general anesthesia with an intrameatal approach in 96% of the cases. Stapedotomy (n=120, 80%) was performed with a drill in 141 cases and by laser in 9 (6%). Ninety percent of the Teflon prostheses had a 0.4 mm diameter and a 4.5 mm length. The footplate opening was sealed with blood clots. Venous interposition (n=30, 20%) was performed in the event of partial or total stapedectomy which occurred in spite of an initial stapedotomy attempt.The preoperative air-bone gap (ABG) was 32 +/- 10.3 dB. The gain in air conduction was 25 +/- 11.7 dB with 75% of the patients having more than 15 dB gain. The ABG was 10 +/- 5.4 dB with 73% of the patients having less than 5 dB gain. The interaural difference was 0.5 +/- 14.1 dB and the bone conduction (BC) variation was 1 +/- 7.5 dB. Functional failures were related to significant intralabyrinthine bleeding and revision procedure. The following factors had not effect on outcome: i) stapedotomy versus partial or total stapedectomy, footplate opening sealed by clots or vein, ii) diameter of the stapedotomy and/or the prosthesis, iii) surgical procedure performed by a junior surgeon.Sealing the stapedotomy opening with blood clots appears to provide reliable and reproducible functional outcome that remains stable over time. In this study, changing from partial to total stapedectomy with vein interposition did not modify the functional outcome.

Round window (r.w.) administration of drugs involves the delivery of medication directly into the inner ear via the r.w. membrane, avoiding a systemic effect of the therapy. Earlier experimental studies suggest that a number of antioxidants and scavengers hold promise for ameliorating the tissue damaging capacity of reactive oxygen species in some acquired cochlear disorders. D-Methionine and thiourea are two small sulfur-containing molecules with an antioxidative and scavenging effect. The passage through the r.w. of radioactive D-methionine and thiourea administered by 1 h infusion to the r.w. was studied in a rat model. Levels of the tracers were measured in scala tympani perilymph (PLT) 17-254 min after r.w. administration. Both tracers pass the r.w. membrane readily. Peak levels were found in the earliest taken samples after the administration. The radioactivity in PLT of the basal turn reached a peak to about 1.5-1.9% of the irrigating medium radioactivity. Following the r.w. administration, the concentration of radioactive D-methionine and thiourea declined with a terminal half-life of 0.57 and 0.77 h, respectively. The distribution of the tracers at the cellular level was analyzed by autoradiography. The most intense expression was found in the lateral wall of the cochlea. It can be postulated that local delivery to the cochlea of D-methionine and thiourea via the r.w. gives high local concentrations of the substances in PLT in the basal turn.