Prevalence of vestibular disease in France: analysis of prescription data from a national health insurance database.

Vestibular problems are frequent reasons for primary care consultations. However, there is considerable uncertainty about the prevalence and cost of vestibular disorders. Despite ambiguous effectiveness data, the histamine analogue betahistine is widely and almost exclusively used for treatment of vertigo. Prescription of betahistine can, therefore, be used as a proxy estimate for prevalence. We used openly available claims data from the French health insurance data warehouse, defining annual prevalence of vestibular disease as the number of people who received at least one betahistine prescription that year. Dosage and pack size of each prescribed formulation were extracted to calculate the sum of betahistine in mg and the Defined Daily Dose (DDD) for age and sex strata and in total. To estimate the relative impact of one landmark trial, the BEMED study, we compared prescriptions from the years 2014/2015 to prescriptions in 2019/2022. A total of 735,121 (2014), 694,705 (2015), 614,431 (2019), and 562,476 (2022) persons filled in a prescription of betahistine. Patients were predominantly older and female. Average amount dispensed per year and per person increased from 4422.54 mg during the pre-BEMED period to 4736.90 mg during the post-BEMED period. DDD decreased from 130 Mio per year in 2014/2015 to 116 Mio per year in 2019/2022. Total costs for betahistine decreased by 42% from 21,615,037 Euro in 2014 to 12,894,249 Euro in 2022. Vestibular disease is frequent in France and has a relevant impact on population health. Despite conflicting clinical evidence, betahistine continues to be prescribed widely in medical practice.

Guidelines of the French Society of Otorhinolaryngology and Head and Neck Surgery (SFORL) for vestibular rehabilitation in children with vestibular dysfunction. A systematic review.

INTRODUCTION: The consequence of complete or partial uncompensated vestibular dysfunction in children is usually balance disorders, with the risk of falls and increased fatigue, particularly during tasks requiring postural control. The aim of these recommendations is to establish guidelines for vestibular rehabilitation (VR) in children with vestibular impairment. MATERIAL AND METHODS: The guidelines were developed based on a systematic review of the international literature, validated by a multidisciplinary group of French-speaking otorhinolaryngologists, scientists, and physiotherapists. They are classified as grade A, B, C, or expert opinion according to a decreasing level of scientific evidence. RESULTS: A PubMed search of studies published between January 1990 and December 2021 was carried out using the keywords "vestibular," "rehabilitation," and "children". After filtering and reviewing the articles, a total of 10 publications were included to establish the recommendations. CONCLUSION: It is recommended that a vestibular assessment be carried out before VR, including a study of vestibulo-ocular reflex, otolithic function, and postural control. In cases of vestibular dysfunction, physiotherapy treatment is recommended from an early age to train different aspects of postural control, including anticipatory and reactive postural adjustments. VR adapted to the pediatric population is recommended for children whose vestibular dysfunction leads to functional disorders or symptoms of vertigo for those who have suffered head trauma. It is recommended that children with bilateral vestibular impairment be treated using gaze stabilization exercises for adaptation and substitution. Optokinetic stimulation and virtual reality are not recommended for children and young adolescents.

Ultrarare heterozygous pathogenic variants of genes causing dominant forms of early-onset deafness underlie severe presbycusis.

Presbycusis, or age-related hearing loss (ARHL), is a major public health issue. About half the phenotypic variance has been attributed to genetic factors. Here, we assessed the contribution to presbycusis of ultrarare pathogenic variants, considered indicative of Mendelian forms. We focused on severe presbycusis without environmental or comorbidity risk factors and studied multiplex family age-related hearing loss (mARHL) and simplex/sporadic age-related hearing loss (sARHL) cases and controls with normal hearing by whole-exome sequencing. Ultrarare variants (allele frequency [AF] < 0.0001) of 35 genes responsible for autosomal dominant early-onset forms of deafness, predicted to be pathogenic, were detected in 25.7% of mARHL and 22.7% of sARHL cases vs. 7.5% of controls (P = 0.001); half were previously unknown (AF < 0.000002). MYO6, MYO7A, PTPRQ, and TECTA variants were present in 8.9% of ARHL cases but less than 1% of controls. Evidence for a causal role of variants in presbycusis was provided by pathogenicity prediction programs, documented haploinsufficiency, three-dimensional structure/function analyses, cell biology experiments, and reported early effects. We also established Tmc1N321I/+ mice, carrying the TMC1:p.(Asn327Ile) variant detected in an mARHL case, as a mouse model for a monogenic form of presbycusis. Deafness gene variants can thus result in a continuum of auditory phenotypes. Our findings demonstrate that the genetics of presbycusis is shaped by not only well-studied polygenic risk factors of small effect size revealed by common variants but also, ultrarare variants likely resulting in monogenic forms, thereby paving the way for treatment with emerging inner ear gene therapy.

Intratemporal facial nerve schwannomas: multicenter experience of 80 cases.

PURPOSE: To provide more data on the clinical presentation and natural evolution of facial nerve schwannomas and to provide guidance for therapeutic decision making. METHODS: A retrospective case review of eighty patients diagnosed with a facial nerve schwannoma between 1990 and 2018 in ten tertiary referral centers in Europe was performed. Patients' demographics, symptomatology, audiometry, anatomical site (segments involved), size and whenever possible volume measurement were registered. RESULTS: At presentation, transient or persistent facial palsy was the most common symptom, followed by hearing loss. The schwannoma involved more than one segment in the majority of the patients with the geniculate ganglion being most commonly involved. Initial treatment consisted of a wait and scan approach in 67.5%, surgery in 30% and radiation therapy in 2.5% of the patients. Tympanic segment schwannomas caused mainly conductive hearing loss and were more prone to develop facial palsy at follow-up. Internal auditory canal or cerebellopontine angle schwannomas presented with significantly more sensorineural hearing loss. CONCLUSIONS: Although modern imaging has improved diagnosis of this tumor, choosing the best treatment modality remains a real challenge. Based on the literature review and current findings, more insights into the clinical course and the management of facial nerve schwannomas are provided.

Positive Impact of Genetic Test on the Management and Outcome of Patients With Paraganglioma and/or Pheochromocytoma.

CONTEXT: Pheochromocytomas and paragangliomas (PPGLs) are characterized by a strong genetic component, with up to 40% of patients carrying a germline mutation in a PPGL susceptibility gene. International guidelines recommend that genetic screening be proposed to all patients with PPGL. OBJECTIVE: Our objective was to evaluate how a positive genetic test impacts the management and outcome of patients with SDHx or VHL-related PPGL. DESIGN: We performed a multicentric retrospective study involving 221 propositi carrying an SDHB, SDHD, SDHC, or VHL germline mutation. Patients were divided into two groups: genetic patients, who were informed of their genetic status within the year following the first PPGL diagnosis, and historic patients, who only benefited from the genetic test several years after initial PPGL diagnosis. RESULTS: Genetic patients had better follow-up than historic patients, with a greater number of examinations and a reduced number of patients lost to follow-up (9.6% vs 72%, respectively). During follow-up, smaller (18.7 vs 27.6 mm; P = 0.0128) new PPGLs and metastases as well as lower metastatic spread were observed in genetic patients. Of note, these differences were reversed in the historic cohort after genetic testing. Genetic patients who developed metachronous metastases had a better 5-year survival rate than historic patients (P = 0.0127). CONCLUSION: Altogether, our data suggest that early knowledge of genetic status had a positive impact on the management and clinical outcome of patients with a germline SDHx or VHL mutation.

Middle Ear Osteoma Causing Mixed Hearing Loss: A Case Report.

Osteomas of the middle ear are rare benign tumors. Their consequences and symptoms are due to their specific location, such as the promontory or the epitympanum and their contact with the facial nerve, the semicircular canal, the ossicles, and the oval or round windows. We report a very unusual case of middle ear osteoma (MEO) in a 23-year-old male patient causing a right mixed hearing loss by contacting and overwhelming the incus and stapes. The lesion was also closely attached to the tympanic portion of the fallopian canal. Since the stapes was not clearly visible behind the lesion, careful observation was preferred to surgery owing to the high risk of inner ear damage and facial palsy with removal of the lesion. MEOs are rarely situated at this critical site. Regular clinical and computerized tomography monitoring is warranted to check their growth. This case also supports the etiological theory of chronic middle ear inflammation causing osteomas.

Transtympanic pseudoaneurysm of the internal carotid artery complicating a myringotomy in a four-year old child: Case report and literature review.

We report the first case of a transtympanic iatrogenic internal carotid artery (ICA) pseudoaneurysm diagnosed in a 4-year-old child following a myringotomy. An endovascular treatment with a covered-stent was decided; spontaneous thrombosis was found during the therapeutic arteriography, and the procedure was aborted. Otoscopy and computed tomography (CT) scan monitoring showed a prolonged thrombosis and the disappearance of the pseudoaneurysm 18months after the diagnostic arteriography. Based on literature review, endovascular techniques seem to be preferred to the surgical approach for treatment of intrapetrous ICA pseudoaneurysm, however clinical and CT scan monitoring may also be a valid option.

Long-term results of middle fossa plugging of superior semicircular canal dehiscences: clinically and instrumentally demonstrated efficiency in a retrospective series of 16 ears.

The objective of this study is to report the surgical outcome after middle fossa approach (MFA) plugging in patients suffering from a superior semi-circular canal dehiscence (SCD) syndrome. This is a retrospective case review. Tertiary referral center. Sixteen ears in 13 patients with a SCD syndrome suffering from severe and disabling vestibular symptoms with a bony dehiscence on CT scan >3 mm and decreased threshold of cervical vestibular evoked potentials (cVEMPs). We assessed preoperatively: clinical symptoms, hearing, cVEMPs threshold, size of dehiscence and videonystagmography (VNG) with caloric and 100 Hz vibratory tests. Postoperatively, we noted occurrences of neurosurgical complication, evolution of audiological and vestibular symptoms, and evaluation of cVEMP data. Tullio's phenomenon was observed in 13 cases (81.3 %) and subjectively reported hearing loss in seven (43.7 %). All patients were so disabled that they had to stop working. No neurosurgical complications were observed in the postoperative course. In three cases (16.6 %), an ipsilateral and transitory immediate postoperative vestibular deficit associated with a sensorineural hearing loss (SNHL) was noted, which totally resolved with steroids and bed rest. All patients were relieved of audiological and vestibular symptoms and could return to normal activity with a mean follow-up of 31.1 months (range 3-95). No patient had residual SNHL. cVEMPs were performed in 14 ears postoperatively and were normalized in 12 (85.7 %). Two of the three patients operated on both sides kept some degree of unsteadiness and oscillopsia. MFA plugging of the superior semi-circular canal is an efficient and non-hearing deteriorating procedure.

Effect of corticosteroids on facial function after cerebellopontine angle tumor removal: a double-blind study versus placebo.

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.

Prognostic Factors of Balance Quality After Transpetrosal Vestibular Schwannoma Microsurgery: An Instrumentally and DHI-based Prospective Cohort Study of 48 Patients.

OBJECTIVES: To evaluate short- and mid-term level of imbalance after vestibular schwannoma (VS) microsurgery by the transpetrosal approach, to search for factors predictive of vestibular compensation, and to determine which patient categories need a postoperative vestibular rehabilitation program. STUDY DESIGN: Prospective cohort study at a tertiary referral center. METHODS: Between 2010 and 2011, patients aged 18 to 75 operated on for VS by transpetrosal approaches were included. VS was characterized by its size (Koos classification) and the presence or not of a cystic component. Hearing was classified according to the Gardner Robertson grading. The preoperative workup included an audiogram, computerized video nystagmography (VNG) with caloric testing, gaze study, rotatory tests, click-evoked cervical vestibular evoked myogenic potential measurements (cVEMPs), and subjective visual vertical test. Patients were asked to complete a Dizziness Handicap Inventory (DHI). Postoperatively, patients were reevaluated on D7 (clinical status), D90 (VNG and DHI), and D180 (DHI). Timing and duration of vestibular rehabilitation were also recorded. RESULTS: Forty-eight patients were included. Preoperatively, 77% experienced mild instability problems with a mean DHI score of 14.1 (range 4-32). Postoperatively, 71% reported stable or even improved perceived stability. Mean DHI scores were 28.1 on D90 and 19.8 on D180. Serviceable hearing, cystic transformation, normal cVEMPs, diplopia, and vestibular syndrome on D7 were found to be predictive of worse equilibrium outcome than when absent. A preoperative caloric deficit greater than 75% seemed to be a good prognostic factor. Vestibular rehabilitation was conducted in 56% of patients. Starting it early (<1 mo) seemed to be beneficial for final equilibrium outcome. CONCLUSION: VS microsurgery provides good stability results. Some preoperative parameters may be predictive of worse or improved balance recovery, as is clinical status on D7.

Evolution of the reliability of the fully implantable middle ear transducer over successive generations.

OBJECTIVE: To describe the reliability of the fully implantable middle ear transducer after successive technological changes. STUDY DESIGN: Prospective, observational, multicenter study. SETTING: Eight tertiary referral centers. PATIENTS: One hundred twenty-three adults were implanted with one of the five successive versions of the fully implantable middle ear implant between September 2005 and July 2012. METHODS: The reliability of each generation 2 years after implantation or at the maximal lifetime was reported by means of survival curves. Only technological failures were considered; non-technological failures were excluded from the analysis of reliability. RESULTS: One hundred fifty-seven devices were implanted during the period of study. Fifteen explantations were related to non-technological problems (e.g., infections, extrusions, etc.). One hundred forty-two implants were followed in the analysis of reliability. We observed 32 technical failures at 2 years. At the maximal lifetime of follow-up, 46 implants failed. The end of the follow-up was the first of November 2012. Survival rates at 2 years were 0%, 76.1%, 84.2%, 81.8%, and 100% for each of the successive available versions, respectively. CONCLUSION: The reliability of the fully implantable middle ear implant improved over generations thanks to successive technological modifications that corrected the observed failures. The latest generation seems to be a reliable fully implantable middle ear implant system up to 22 months after implantation. The need to know the reliability of these active middle ear implants incites the creation of a follow-up register including patient's data and device failures to improve patient management.

Effects of body tilt on multifrequency admittance tympanometry.

INTRODUCTION: Hydrops and abnormalities of inner fluid pressure are involved in some otologic diseases such as Ménière's disease (MD). However, demonstrating abnormal perilymphatic or endolymphatic pressure is challenging. Multifrequency tympanometry studies in MD patients demonstrated an increase of the width of conductance tympanograms (outside an attack) compared with controls. To confirm that the increase in conductance width is caused by hyperpressure and not hypopressure in these patients tested outside an attack, we assessed the effect of changes in inner ear fluid pressure caused by body tilt on the results of multifrequency admittancemetry tympanograms. MATERIALS AND METHODS: A multifrequency tympanometry including conductance (G) tympanogram at 2 kHz and resonance frequency measurements were performed in 20 volunteers (40 ears) free of otologic or neurologic disease. The measures were collected in three different positions: vertical, supine, and Trendelenburg positions. RESULTS: Changes in inner ear fluid pressure caused by body tilt induced an increase in the width of G tympanograms. In the vertical position, the mean value was 141.7 ± 56.5 daPa; in the supine position, it increased to 158 ± 58.3 daPa; and increased even more in the Trendelenburg position (20 degrees), with a mean of 184 ± 69.6 daPa (p < 0.01). Resonance frequency also increased in the Trendelenburg position.We conclude that the increased width of G tympanograms in MD patients outside an attack may be caused by an increase in inner ear fluid pressure.

Stitchless fibrin glue-aided facial nerve grafting after cerebellopontine angle schwannoma removal: technique and results in 15 cases.

AIM OF THE STUDY: To evaluate the results of facial nerve (FN) grafting using great auricular cable graft and fibrin glue without suturing to palliate FN disruption after removal of large cerebellopontine angle (CPA) vestibular schwannoma (VS) or facial nerve schwannoma (FNS). To assess whether tumor size and origin influenced the results. STUDY DESIGN AND SETTING: Retrospective review of all patients having undergone removal of FNS/VS and needing intraoperative FN repair between 2001 and 2011. INTERVENTION: FN was rehabilitated using great auricular nerve cable graft and fibrin glue (Tisseal) without stitching suture. MAIN OUTCOME MEASURES: All data recorded were reviewed to access age, sex, tumor type, and tumor size according to the Koos classification and presenting symptoms. FN function was evaluated preoperatively and at 18 months using the House-Brackmann (HB) grading system. RESULTS: Among the 595 patients operated for CPA schwannomas in this period, 15 patients (2.5%) underwent FN repair, including 7 cases of FNS and 8 cases of VS. Tumor removal was total in all cases. FN recovery was HB3 in 13 cases (86.7%) and HB4 in 2. The mean time to the first clinical signs of facial reinnervation was 10 months (6-12 mo). No significant relation was found between postoperative facial function and tumor size or type, even if all cases of preoperative FP were noted in FNS. CONCLUSION: Immediate FN reconstruction with fibrin glue-aided greater auricular nerve graft can effectively restore FN function with excellent outcomes. The results seem better than those observed by other authors using sutured grafts or delayed hypoglossal-facial nerve anastomosis.

New outcomes with auditory brainstem implants in NF2 patients.

OBJECTIVE: To determine factors related to high levels of speech recognition in patients with the auditory brainstem implant (ABI). STUDY DESIGN: Retrospective case review. SETTING: International multicenter data from hospitals and tertiary referral facilities. PATIENTS: Patients with neurofibromatosis type 2 (NF2) and bilateral vestibular schwannomas. INTERVENTION: ABIs were placed after the removal of vestibular schwannomas. MAIN OUTCOME MEASURES: Demographic and surgical data were collected from 26 patients with ABIs who achieved scores of better than 30% correct identification of sentences presented in quiet listening conditions and without lipreading cues. RESULTS: Scores better than 30% speech recognition of standard sentence test materials (HINT or equivalent) in quiet listening conditions were obtained in 26 of the 84 NF2 patients (31%). ABI speech recognition was correlated with surgical position, length of deafness, the number of distinct pitch electrodes, perceptual levels, and ABI stimulation rate, but not correlated with tumor size, tumor stage, the number of electrodes used, or electrophysiological recordings. This paper presents the consensus opinion from a meeting of surgeons to compare outcomes across ABI surgical centers. CONCLUSIONS: The consensus opinion was that brainstem trauma is a primary factor in the variability of outcomes in NF2 patients. The significant co-factors in outcomes implied that ABI surgery should be accomplished with great care to minimize physical and venous trauma to the brainstem. It is clear that high levels of speech recognition, including high levels of open-set speech recognition, are possible with the ABI even in patients with NF2 and large tumors.

Clinical presentation of intralabyrinthine schwannomas: a multicenter study of 110 cases.

OBJECTIVE: To describe the presentation of intralabyrinthine schwannomas (ILSs). STUDY DESIGN AND SETTING: Retrospective multicenter study involving 12 European skull base surgery tertiary referral centers. PATIENTS: One hundred ten patients with the diagnosis of ILS, either labyrinth confined or extending into the internal auditory meatus for less than 50% of their volume. MAIN OUTCOME MEASURES: Data collected were age, sex, nature and timing of presenting symptoms, hearing (according to the AAO-HNS grading system), results of vestibular tests (caloric tests and cervical vestibular-evoked myogenic potentials [c-VEMPs]), and tumor localization. Presenting symptoms and laboratory test results were studied according to the extension of the lesion into the cochlea (C) and vestibule (V), on one hand, and according to unifocal (L1) or plurifocal (L2) extension into the labyrinth, on the other. RESULTS: Intracochlear type was more common (50%) than vestibular (19.1%) and more diffuse forms (30.9%). The mean delay for diagnosis was long (72.5 mo; SD, 76.6). Mean age was 53.9 years (SD, 13.2). Deafness was the most common symptom (77.8 dB HL [SD, 33.6], with only 24.6% of patients keeping viable hearing. Caloric tests (65.5% of patients) were abnormal in 77.8% of cases. c-VEMPs were abnormal in 65.7% of the 36 cases analyzed. In V forms, hearing was significantly better (class A + B in 21.1% in C and 45.8% in V forms) (p = 0.03), and vestibular function was more altered (C: 57%, V: 100%, p = 0.0009*). L2 forms were diagnosed later (L1: 59.1 mo, L2: 104.5 mo; p = 0.004*) and were associated more frequently with a dead ear (L1: 13.1%, L2: 41.2%, p = 0.002*) than L1 forms. CONCLUSIONS: This series, which is the largest in the literature, demonstrates that even very small and localized ILSs heavily compromise labyrinthine functions.

The role of radiation therapy in the management of head and neck paragangliomas: impact of quality of life versus treatment response.

PURPOSE: To assess the impact of radiotherapy in paragangliomas (PGLs) with regard to overall survival, local control, volumetric response and particularly quality of life (QoL). MATERIALS AND METHODS: From 1985 to 2010, 130 cases of head and neck (H&N) PGLs were managed at Bordeaux University Hospital. With a median follow-up of 7.6 years, we retrospectively present a cohort of 30 consecutive patients treated with radiation therapy for H&N PGLs. QoL was evaluated for 20 patients by the EORTC QLQ-C30 and H&N35 questionnaires through a cross-sectional study. RESULTS: The 5-year overall survival and local control were 95% and 96% respectively. QoL is altered following management of PGLs. The H&N35 score is lower after combined modality therapy (surgery±embolization and radiation therapy) for speech and hearing (p=0.004), trismus (p=0.003) and total score (p=0.01) than after radiotherapy alone. Tumor shrinkage was significant at 2 and 3 years after radiotherapy (p=0.018; p=0.043). CONCLUSION: Ultimate QoL should be a major goal of any treatment strategy for this benign disease. Definitive radiotherapy should be considered as a reasonable alternative to multimodality treatment as it provides comparable disease control with an apparent improvement in QoL.

Meningeal carcinomatosis of the internal auditory meatus: clinical and imagery-aided differentiation.

OBJECTIVES: To describe difficulties in diagnosing meningeal carcinomatosis of the internal auditory meatus (IAM-MC). STUDY DESIGN: Retrospective case review. SETTING: Tertiary skull base surgery referral center. PATIENTS: A series of six cases of patients diagnosed with IAM-CM over 14 years. RESULTS: The primary cancer was known before diagnosis in three cases (adenocarcinoma of lung, breast, and melanoma). In two others, it was discovered at workup (lung, ethmoid adenocarcinoma). In the last case, no primary cancer was identified. Vestibular symptoms were the first complaint in five of the six cases. Referral symptom was facial paralysis in five cases and rapid-onset total deafness associated with severe unsteadiness in one. Rapid hearing decrease and symptoms of bilateralization were observed in five cases. Diagnosis was ascertained by discovery of malignant cells in the CSF in three cases and at tumor biopsy in one case. In the two other cases, the association of brain metastases and increased dura mater infiltration was convincing. Treatment consisted in radiation therapy to the whole brain in five cases and intrathecal chemotherapy in one case. Evolution was rapidly lethal in five cases. The last died free of disease 4 years after treatment. CONCLUSION: IAM-MC is difficult to demonstrate. Progressive facial paralysis associated with aggressive and rapidly bilateral cochlear and vestibular symptoms are highly evocative in the event of cancer in the patient's history. If there is no history of cancer, lumbar puncture is to be repeated until malignant cells are discovered in the CSF before beginning radiotherapy.

Tolerance and osteointegration of TricOs(TM)/MBCP(®) in association with fibrin sealant in mastoid obliteration after canal wall-down technique for cholesteatoma.

CONCLUSION: The TricOs™/MBCP(®) and fibrin sealant composite was a convenient, effective, and well-tolerated material for mastoid cavity filling and immediate reconstruction of the external auditory meatus after cholesteatoma surgery with canal wall down (CWD). OBJECTIVE: To assess the tolerance and osteointegration of a bone graft substitute, TricOs™/MBCP(®), in association with fibrin sealant for filling the mastoid cavity after cholesteatoma surgery using the CWD technique. METHODS: In this prospective observational study 57 patients with cholesteatoma suitable for CWD were recruited from April 2006 to April 2008 and followed up for 1 year. The mastoid cavity was filled with TricOs™/MBCP(®) followed by immediate reconstruction of the external auditory meatus covered with fascia temporalis and/or cartilage. The main outcome was skin tolerance assessed by a novel weighted score emphasizing long-term results. The typical weighted reference score was 1.67; skin tolerance was considered acceptable if 75% of patients had a score ≤ 1.67. Secondary outcomes were otorrhea and/or otalgia, hearing, and osteointegration assessed through computed tomography scanning at 12 months. RESULTS: Forty-one patients had a complete follow-up; 34 (82.3%) patients achieved the main end point with scores ≤ 1.67. Otorrhea decreased postoperatively. No otalgia interfering with daily tasks was reported. Ossicular reconstruction was carried out in 29 patients. Absence of cochlear toxicity was confirmed by unimpaired bone conduction. Preoperative and postoperative speech audiometry results were similar. No serious adverse events were observed. Osteointegration was satisfactory with hyperdensity or intermediate density in 95% of patients at 12 months.

Inflammatory myofibroblastic tumour of the skull base.

Inflammatory myofibroblastic tumors (IMTs) are rare benign clinical and pathological entities. IMTs have been described in the lungs, abdomen, retroperitoneum, and extremities but rarely in the head and neck region. A 38-year-old man presented with headache, right exophthalmia, and right 6th nerve palsy. A CT scan revealed enlargement of the right cavernous sinus and osteolytic lesions of the right sphenoid and clivus. MR imaging showed a large tumor of the skull base which was invading the sella turcica, right cavernous sinus, and sphenoidal sinus. A biopsy was performed and revealed an IMT. Corticosteroids were given for 3 months but were inefficient. In the framework of our pluridisciplinary consultation, fractionated conformal radiotherapy (FRT) was indicated at a low dose; 20 Gy in 10 fractions of 2 Gy over 12 days were delivered. Clinical response was complete 3 months after FRT. Radiological response was subtotal 6 months after FRT. Two years later, the patient is well.

Comparison between bilateral cochlear implants and Neurelec Digisonic(®) SP Binaural cochlear implant: speech perception, sound localization and patient self-assessment.

An alternative to bilateral cochlear implantation is offered by the Neurelec Digisonic(®) SP Binaural cochlear implant, which allows stimulation of both cochleae within a single device. The purpose of this prospective study was to compare a group of Neurelec Digisonic(®) SP Binaural implant users (denoted BINAURAL group, n = 7) with a group of bilateral adult cochlear implant users (denoted BILATERAL group, n = 6) in terms of speech perception, sound localization, and self-assessment of health status and hearing disability. Speech perception was assessed using word recognition at 60 dB SPL in quiet and in a 'cocktail party' noise delivered through five loudspeakers in the hemi-sound field facing the patient (signal-to-noise ratio = +10 dB). The sound localization task was to determine the source of a sound stimulus among five speakers positioned between -90° and +90° from midline. Change in health status was assessed using the Glasgow Benefit Inventory and hearing disability was evaluated with the Abbreviated Profile of Hearing Aid Benefit. Speech perception was not statistically different between the two groups, even though there was a trend in favor of the BINAURAL group (mean percent word recognition in the BINAURAL and BILATERAL groups: 70 vs. 56.7% in quiet, 55.7 vs. 43.3% in noise). There was also no significant difference with regard to performance in sound localization and self-assessment of health status and hearing disability. On the basis of the BINAURAL group's performance in hearing tasks involving the detection of interaural differences, implantation with the Neurelec Digisonic(®) SP Binaural implant may be considered to restore effective binaural hearing. Based on these first comparative results, this device seems to provide benefits similar to those of traditional bilateral cochlear implantation, with a new approach to stimulate both auditory nerves.