Minimally invasive ossiculoplasty via an endoscopic transtympanic approach.

INTRODUCTION: The aim of this study was to evaluate the feasibility of ossiculoplasty via a minimally invasive endoscopic transtympanic approach (ETTA). CASE SERIES: We investigated the exposure of target structures (incus and stapes) on 4 human temporal bones by placing an endoscope into the middle ear cleft through the 4 tympanic quadrants. Then, on 3 additional specimens, we performed an incudostapedial disjunction and repaired it with a drop of hydroxyapatite cement via ETTA. We measured the size of tympanic perforation, and the acoustic transfer function of the middle ear (125-8000Hz) before and after repair by placing an insert in the external auditory canal and recording the acoustic signal in the utricle by a microphone. The acoustic signal gain was estimated in dB µV. Exposure was similar in all four quadrants but ergonomics was better with a posteroinferior myringotomy. Ossicular chain repair was conducted successfully in all cases and the acoustic transfer function of the middle ear was significantly improved. Residual tympanic perforation (n=3) was 2±0.3mm in diameter. CONCLUSION: ETTA to reconstruct incudostapedial joint with bone cement was feasible and effective. It opens perspectives for robot-based procedures guided by augmented reality.

Intraoperative imaging and navigation of the petrous apex by infralabyrinthine route.

Intraoperative imaging during skull-base surgery allows the operator to control surgical dissection and to tailor the approach, reducing morbidity due to inadequate resection or reintervention. The O-arm® (Medtronic, Fridley, MN, USA) navigation system is an intraoperative cone-beam CT device that was first designed for spinal surgery but now has applications in lateral skull-base surgery. In this technical note, we present a patient with petrous apex cholesterol granuloma located medial to the intrapetrous internal carotid artery and in the infralabyrinthine compartment. We report the possibility of natural drainage, using intraoperative imaging to guide resection, through a rapid, safe and minimally invasive approach.

The French National Cochlear Implant Registry (EPIIC): Cochlear explantation and reimplantation.

This study aims to determine the frequency and causes of cochlear explants with re-implantation (ERI) after 5 years' follow up of the patients included in the French national EPIIC (étude post-inscription des implants cochléaires) registry tracking patients with cochlear implantation. This multicenter, descriptive prospective study was conducted on 5051 patients enrolled in the EPIIC database between January 2012 and December 2016. Ninety-five patients (1.9%) received a primary implant and an ERI during the study. Of these, four benefitted from two ERIs. The number of ERIs was significantly higher in the pediatric population than among adults. The explantation and reimplantation were performed simultaneously in 86% of cases. The reasons for explantation were: in 46.4% of cases linked to a malfunction of the implant, and in 39.3% of cases for medical or surgical reasons. The number of electrodes inserted was significantly higher after the ERI than after the first implantation. There was just one post-ERI infection for these 95 explanted and re-implanted patients. As well as explantation with reimplantation rarely being necessary, it generally presents no major surgical difficulty and in most cases it allows a better integration than in the first implantation.

The French National Cochlear Implant Registry (EPIIC): Bilateral cochlear implantation.

OBJECTIVES: Assessment of the incidence and results of bilateral cochlear implantation in adults and children in France. MATERIALS AND METHODS: Multicenter retrospective study of data in the French national registry of cochlear implantations from January 1st 2012 to December 31st 2016. Functional results from CAP (Category of Auditory Performance) questionnaires and speech audiometry tests, with mono- and di-syllabic word-lists, were compared before and after implantation. Speech audiometry tests were carried out against a noisy background, except before simultaneous implantations. RESULTS: Nine hundred and forty two bilateral cochlear implantations were performed during this period, that is, 16.4% of all cochlear implantations. Five hundred and eighty eight bilateral implantations were performed sequentially. 59% of the bilateral implantations were performed in children. Bilateral implants significantly improved CAP scores in all cases (P<0.001). Auditory performance, with the two types of word-list, were significantly improved after simultaneous implantation (P<0.01). After sequential implantation, the speech discrimination score, already very good with the first implant, reached 63±26% [0-100] with monosyllabic word lists, and 72±28% [0-100] with dissyllabic words. There were more complications due to surgery in bilateral cases than in the entire population of cochlear recipients (9.1% vs 6.4%, P<0.02). CONCLUSION: Hearing is significantly improved by simultaneous cochlear implantation. For sequential implantation, at one year, when auditory results were already excellent from the first implant, in the bimodal condition CAP scores were significantly improved, although there was no further change in speech audiometry in noise.

The French Cochlear Implant Registry (EPIIC): Cochlear implant candidacy assessment of off-label indications.

OBJECTIVE: Evaluate in France the outcomes of cochlear implantation outside the selection criteria, off-label. MATERIAL AND METHODS: This is a prospective cohort study including adults and children having received a cochlear implant (CI) in an off-label indication, that is outside the criteria established by the "Haute Autorité de santé (HAS)" in 2012. The data was collected from the "EPIIC" registry on recipients who received CIs in France between 2011 and 2014. Speech audiometry was performed at 60dB preimplantation and after one year of CI use, as well as an evaluation of the scores of the quality of life with the APHAB questionnaire, the scores for CAP and the professional/academic status in pre- and post-implantation conditions. Major and minor complications at surgery have been recorded. RESULTS: In total, 590 patients (447 adults and 143 children) with an off-label indication for CIs were included in this study from the EPIIC registry (11.7% of the whole cohort of EPIIC). For adults, the median percentage of comprehension using monosyllabic word lists was 41% in preimplantation condition versus 53% after one year of CI use (P<0.001) and 60% versus 71% in dissyllabic word lists (P<0.001). The CAP scores were 5 versus 6 in pre- and post-implantation conditions respectively (P<0.001) and the APHAB scores were statistically lower after implantation (P<0.001). In the children cohort, the median percentage of comprehension using monosyllabic word lists was 51% in preimplantation condition and 65% after CI (P<0.001), and 48% versus 82% (P<0.001) for dissyllabic word lists. The CAP scores were 5 versus 7 respectively in pre- and post-CI conditions (P<0.001). Thirty-two minor complications (5.4%) and 17 major complications (2.8%) were reported in our panel of off-label indication patients. CONCLUSION: These results suggest that a revision of the cochlear implantation candidacy criteria is necessary to allow more patients with severe or asymmetric hearing loss to benefit from a CI when there is an impact on quality of life despite the use of an optimal hearing aid.

The French Cochlear Implant Registry (EPIIC): Perception and language results in infants with cochlear implantation under the age of 24 months.

OBJECTIVE: Multi-centre study of the National French Registry (EPIIC) of patients with cochlear implants, focusing on infants who were operated-on under the age of 24 months between 2012 and 2016. PATIENTS AND METHODS: A total of 615 profoundly deaf infants, who received cochlear implants (CIs) before their second birthday, were included in the registry by different CI centers. Epidemiological, surgical, speech therapy and school, follow-up data were included in the registry, 12, 24, 36 and 48 months thereafter. The following parameters were studied: type of implantation (uni- or bilateral), complications, cause of deafness, category of auditory perception (CAP), Open-set word recognition score (OSW), speech intelligibility rating, lexical comprehension with EVIP (Peabody), communication mode and type of schooling. Bilateral simultaneous CI (BiCI) and unilateral CI (UniCI) groups were compared. RESULTS: There were 744 implantations. The explantation-reimplantation rate, within the four-year follow-up, was just 3.6%. Mean implantation age was 16.0 months, and similar in the two groups (BiCI/UniCI). A total of 51% of children had their first implant between 12 and 18 months, and 15% before 12 months. Implantation was unilateral in 52% of cases. Fifty-six percent of the bilateral procedures were sequential, with a mean delay of 16.8 months for the second implantation. The cause of deafness was unknown in 52% of cases. Of the 48% (297/615) of attributed cases, 32% had clear genetic causes. The remaining deafness was due to cytomegalovirus (CMV, 8%), inner-ear malformation (5%) and meningitis (3%). The main complications were from infections (47%) and internal device failure (25%). Four years post-operation, 84% of the UniCI and 75% of BiCl groups had a CAP≥5, and 83% of UniCl and 100% BiCI had OSW≥80%. Furthermore 74% of UniCI and 77% of BiCI communicated orally and 85% of UniCI and 90% of BiCI integrated into mainstream schooling. CONCLUSION: The French Registry of cochlear implants (EPIIC) is the only such national registry in the world. Our analysis illustrates the immediate benefits of, either single or double, cochlear implantation for language, perception skills and schooling.

The French Cochlear Implant Registry (EPIIC): General indicators.

Cochlear and brainstem implants have been included on the list of reimbursable products (LPPR) in France since March of 2009. The implants were initially inscribed for 5 years, after which an application for renewal with the French National Commission for the Evaluation of Medical Devices and Health Technologies (Commission Nationale d'évaluation des dispositifs médicaux et des technologies de santé - CNEDiMTS) was required [Haute Autorité de santé, 2009]. Upon registration to the list of reimbursable products, the companies and the reference centers for cochlear and brainstem implants were asked to set up a post-registration registry called EPIIC. This article reports the evolution in the EPIIC registry of the general indicators for 5051 patients over the five years from 2012-2016.

The French Cochlear Implant Registry (EPIIC): Cochlear implantation complications.

OBJECTIVES: The aim of this study was to evaluate peri- and post-operative complications related to cochlear implantations. We searched for risk factors predicting these complications and analyzed the complications in the youngest and most elderly. STUDY DESIGN: Retrospective analysis of cochlear implant patients. MATERIALS AND METHODS: All patients who underwent cochlear implantation in France between January 2012 and December 2016 were anonymized and registered in the EPIIC database. This population included 3483 adults and 2245 children. Their demographic and surgical data and their incidence of peri- or post-operative complications, including their severity, whether major or minor, were all indicated. RESULTS: The global complication rate was 6.84%. The risk of complication was higher in initial implantation versus reimplantation (P<0.0001). The risk was also higher for bilateral implantation versus unilateral (P<0.0001). Complications were more frequent for patients with cochlear malformation (P=0.002). There was no difference in complication rates across age groups; babies under 1 year old, and the elderly over 80 and even over 90, did not have more complications than the rest of the population. Patients treated in the daily care unit had no more complications than those who were hospitalized for one night or more (P=0.64). CONCLUSION: Cochlear implantation is a safe technique with a low incidence of complications. The absence of increased risk in patients at the extremes of the age spectrum justifies offering this solution to all, without age limitation.

The French National Cochlear Implant Registry (EPIIC): Results, quality of life, questionnaires, academic and professional life.

This study concerns the results of cochlear implantation in children and adults from French cochlear implantation centers, monitored at one, two and three years by the Cochlear Implant French Registry EPIIC. This multicenter study enrolled 2603 subjects (1667 adults and 936 children) implanted in one ear. The following parameters were studied: hearing overall performances, monosyllabic or dissyllabic word perception, speech intelligibility, self-assessment questionnaire of Cochlear Implant (CI) benefits (Abbreviated profile of Hearing aid Benefit); professional activity and schooling. This study confirms the ceiling effect in adults' performances after the 1st year and the progressive growth in children's performances. It also shows that the contralateral hearing aid enhances performances compared to the CI alone condition, in all follow-up sessions. The French register of CIs is the only worldwide register of systematic follow-up on a period of three years and more of all adults and children implanted in a country.

The French National Cochlear Implant Registry (EPIIC): Cochlear implantation in adults over 65years old.

OBJECTIVES: To analyze the performance of cochlear implants in French patients aged 65 and over, implanted between 2012 and 2016, using data from the French national registry for cochlear implants (EPIIC). MATERIALS AND METHODS: The French national registry incorporates patient data from before implantation and for three years after implantation, stratified in different age groups (18-39, 40-64years, 65-74years and>75years). Here, we assessed the latter two categories. Hearing was assessed using mono- and disyllabic words in a silent background. The Category of Auditory Performance (CAP) scale was also implemented and subjects took the Abbreviated Profile of Hearing Aid Benefit (Aphab) questionnaire. RESULTS: The population aged over 65 accounted for 38% (n=1193) of the 3178 adult implanted patients. The performance for mono- and disyllabic words in silence, the CAP scores and the APHAB questionnaire answers for ease of communication, background noise and reverberation were dramatically improved at one year post-implantation (P<0.0001 for each score) and remained stable between one and three years thereafter. The percentage improvement was similar across all age groups. The scores for loud-noise intolerance did not change after cochlear implantation in any age group. CONCLUSION: Cochlear implants improve hearing and communication in subjects aged 65 and over, with comparable efficiency to that achieved in younger subjects. Cochlear implantation should thus be proposed whenever hearing aids provide only limited benefit. However, between 2012 and 2016, cochlear implantation was given to less than 1% of the French population aged 65 and over with profound deafness.

[Neurofibromatosis type 2]. / La neurofibromatose de type 2.

INTRODUCTION: Neurofibromatosis 2 (NF2) is a rare autosomal dominant disease whose hallmark is the development of bilateral vestibular schwannomas. STATE OF THE ART: 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. PERSPECTIVES AND CONCLUSIONS: 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.

[Preoperative mixed embolization of a paraganglioma using Onyx]. / Embolisation préopératoire par abord mixte d'un paragangliome à l'aide d'Onyx.

Paragangliomas, or glomus tumors, are highly vascular benign tumors of the head and neck. Clinical symptoms are essentially progressive and neurological, involving infiltration of the regional cranial nerves. The usual treatment is surgery, which itself is a challenge because of the close proximity of vital structures and the considerable blood loss. Preoperative embolization can reduce morbidity, and several techniques have been described using arterial injection of particles or of cyanoacrylate directly into the tumor. This case report is of a patient treated by surgery using a new technique-preoperative embolization involving both the arteries and veins, and injection of Onyx, resulting in complete devascularization of the tumor's arteriovenous network.

[Auditory brainstem implant: indications and results]. / Implant auditif du tronc cérébral: indications et résultats.

OBJECTIVES: 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. MATERIAL AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.

[Performances and complications of cochlear implant in 134 adult patients implanted since 1990]. / Performances et complications de l'implant cochléaire chez 134 patients adultes implantés depuis 1990.

OBJECTIVE: The aim of this study was to analyse performance of cochlear implants in a retrospective series of adults with postlingually in order to search for predictive factors and identify complications. METHODS: Between 1990 and 2003, 134 adult patients were implanted: 129 patients on one side and 5 patients on both sides. Hearing benefit at 6, 12, 24 and 36 months and performance at 12 months were analyzed as function of the etiology, age at implantation, lipreading and speech coding strategy. RESULTS: Compared to performances before implantation, cochlear implants provided significant and rapid improvement at 6 months (p<0.0001). The results were not correlated with age at implantation, etiology or lipreading ability before implantation. Performances were worse in patients with meningitis (p<0.01), but the percentage of improvement was similar between the different etiologies. Major complications were rare: one case of regressive postoperative facial nerve weakness. CONCLUSION: Cochlear implants provide significant and rapid improvement, with low morbidity. Results are not correlated with age at implantation, etiology or lipreading ability.

Local anaesthesia and conscious sedation for cochlear implantation: experience with 20 patients.

OBJECTIVE: Cochlear implantation is mostly performed under general anaesthesia. This study aimed to evaluate cochlear implantation performed under local anaesthesia and sedation. METHOD: Twenty patients had a cochlear implant fitted under combined local anaesthesia (local anaesthesia group) and 41 patients had one fitted under general anaesthesia (general anaesthesia group) for bilateral profound hearing loss, from 2011 to 2014. Surgical duration, period of post-operative hospitalisation and early post-operative symptoms were compared. In the local anaesthesia group, operative symptoms reported during the surgery and by questionnaire were analysed. RESULTS: Mean surgical duration was significantly shorter in the local anaesthesia group: 87 versus 122 minutes (p < 0.001). No significant difference was observed between the local anaesthesia and general anaesthesia groups regarding mean post-operative hospitalisation and early post-operative symptoms. Under local anaesthesia, patients had no particular symptoms in 60 per cent of cases. The remaining patients in this group experienced vertigo (10 per cent), pain (20 per cent) and pain-related movement (10 per cent) during the intervention. These symptoms can be controlled with symptomatic treatment. CONCLUSION: Combined local anaesthesia for cochlear implantation is a good alternative to general anaesthesia for co-operating patients.

[Labyrinthine hemorrhage caused by anticoagulant therapy]. / Hémorragie labyrinthique liée aux anticoagulants.

OBJECTIVE: To report a case of labyrinthine hemorrhage with a sudden sensorineural hearing loss and vertigo. This is a rare mechanism of inner ear damage, which may be considered in patients with anticoagulant therapy. MATERIAL AND METHODS: We report a case of sudden sensorineural hearing loss and vertigo in a 73-year-old male on anticoagulant therapy for arrhythmia. An audiovestibular and imaging work-up was performed. RESULTS: Audiometry showed a left profound hearing loss. Videonystagmography demonstrated a left labyrinthine deficit. Hemostasis assessment showed an INR at 7.5 MR imaging evidenced a high intralabyrinthine signal on T1-weighted images without gadolinium and on FLAIR T2-weighted images, suggesting labyrinthine hemorrhage. At clinical follow-up the vertigo regressed but the hearing function was not recovered. CONCLUSION: Labyrinthine hemorrhage should be evoked in the event of sudden sensorineural hearing loss in patients with anticoagulant therapy. MRI is a useful diagnostic tool in this case.

The pathophysiology of otosclerosis: Review of current research.

Otosclerosis is a complex disease of the human otic capsule with highest incidence in adult Caucasians. So far, many possible etiological factors like genetics, HLA, autoimmunity, viruses, inflammation, and hormones have been investigated but still the development of the disease remains unclear. Currently, the surgical replacement of stapes (stapedotomy) remains the best possible treatment option. In this review, we analyze different etiological factors studied so far in otosclerosis pathophysiology and discuss most recent findings and possible new research pathways.

Langerhans' cell histiocytosis of the labyrinth in adults.

OBJECTIVE: To describe and analyze three unusual cases of Langerhans' cell histiocytosis of the temporal bone in adults. STUDY DESIGN: Retrospective case review. SETTING: A tertiary referral center. PATIENTS: Three adult patients with progressive sensorineural or mixed hearing loss, vertigo, and tinnitus as presenting symptoms of Langerhans' cell histiocytosis of the temporal bone. INTERVENTION: Patients were evaluated by means of computed tomography and magnetic resonance imaging. All patients underwent complete surgical excision of the lesion via a transmastoid approach, extended to a translabyrinthine approach in one case. One patient with a multifocal disease underwent excision of a mandibular lesion 1 year later, followed-up by chemotherapy. RESULTS: The Langerhans' cell histiocytosis was located adjacent to or within the area of the endolymphatic sac region in all cases. The middle ear was spared. Hearing function was preserved in the two patients who had serviceable hearing preoperatively. No complication occurred. CONCLUSION: Langerhans' cell histiocytosis of the temporal bone is centered on or adjacent to the endolymphatic sac. The fact that the endolymphatic sac plays an immunologic role in the inner ear suggests that the infiltration of the Langerhans' cell may derive from it.

H+,K+-ATPase is not involved in endolymph pH homeostasis.

In endolymph, the calculated electrochemical gradients suggest the presence of active transport of H+ and K+. The presence of H+,K+-ATPase, an active transporter that mediates the exchange of intracellular H+ for extracellular K+, was investigated using reverse transcription-polymerase chain reaction on rat microdissected inner ear tissues. Distal colon and kidney were used as positive controls. The two H+,K+-ATPase colonic or gastric isoforms were not detected in the rat cochlea and endolymphatic sac. This result suggests that H+,K+-ATPase is not involved in H+ and K+ endolymph homeostasis.

[Lingual rhabdomyosarcoma in an adult patient]. / Rhabdomyosarcome lingual chez un adulte.

A case of rhabdomyosarcoma of the tongue in an adult male is reported. The diagnosis of this rare lesion is difficult and should be considered in every growing mass of the head and neck region. It is based on the pathologic examination of tissue samples guided by immunohistological methods. In this case the examination revealed an embryonal type. The tumoral progression is rapidly fatal unless an aggressive therapeutic regimen including surgery and radiotherapy is instituted. In this manner dramatic improvement in survival rate has been achieved in recent years.