Long-term clinical and radiological results for fat graft obliteration in subtotal petrosectomy and cochlear implant surgery: a retrospective clinical study.

PURPOSE: The study aimed to evaluate the long-term clinical, radiological, and functional results for subtotal petrosectomy and cochlear implant surgery with closure of the external auditory canal and fat obliteration. METHODS: We retrospectively included all consecutive cases of simultaneous subtotal petrosectomy and cochlear implant surgery performed at a tertiary referral center between 2009 and 2016 using the same surgical technique. All patients underwent postoperative high-resolution computed tomography (HRCT) and annual audiological assessments. A 5-year minimum clinical, radiological, and audiological follow-up was performed. The early and late postoperative results were compared. The main outcome measures were complications, postauricular retraction, fat graft reabsorption, and audiological outcomes. RESULTS: Twenty-nine procedures performed in 23 patients (six bilateral) met the inclusion criteria. The mean age of the patients was 67 ± 13.4 years and mean follow-up duration was 7.5 ± 2 years. At follow-up, postauricular retraction was detected in 24 cases (82.8%), including five cases (17.1%) with subcutaneous protrusion of implant and array. Fat graft volume was significantly reduced at late-HRCT in terms of maximum diameter (2.24 ± 1.0 cm vs 3.69 ± 0.7 cm; p < 0.0005) and surface area (1.88 ± 1.2 vs 4.24 ± 1.6 cm2, p < 0.0005). Six patients had extracochlear electrodes at late-HRCT (3/6 had an increased number of extracochlear electrodes), with a lowering of this group's performance of - 15% (p < 0.005) in the follow-up speech comprehension test. CONCLUSIONS: Subtotal petrosectomy with cochlear implantation is an effective long-term technique in selected cases. Fat grafts showed significant reabsorption at long-term follow-up with reaeration of the middle ear spaces. Prolonged clinical and radiological follow-up is recommended for monitoring implant performances and late complications.

3D 4K Exo-Endoscopic Temporal Bone Dissection: A Novel Approach for Sharing the Anatomy.

BACKGROUND: Temporal bone dissection is overwide recognized as an ideal training method for otologic surgeons. The knowledge of temporal bone anatomy and especially of the course of infratemporal facial nerve is pivotal in practice. The 3D exoscope is an innovative and promising tool, that was recently introduced in ear surgery. METHODS: A high-definition 3D exoscope (3D VITOM®) mounted on the VERSACRANETM holding system (Karl Storz) was used to perform two temporal bone dissection, with the aim to study the anatomy of infratemporal facial nerve. The 3D endoscope (TIPCAM®1 S 3D ORL, Karl Storz) was used in combination to provide a close-up high-quality view and to provide a different angle of view on fine anatomical relationships. RESULTS: The high-definition 3D exoscope allowed to conduct the dissection with high quality visualization and to share the same surgical field with trainees. Moreover, it showed a high interchangeability with the 3D endoscope. CONCLUSIONS: 3D 4 K Exo-endoscopic temporal bone dissection seems to have benefits in terms of educational purpose, especially concerning anatomy understanding. The superiority in teaching value of this tool should be further investigated in cohort studies.

French adaptation and validation of the Chronic Otitis Media Questionnaire-12 (COMQ-12).

OBJECTIVE: Chronic otitis media (COM) can seriously impact quality of life (QOL). Disease-specific questionnaires are essential for an accurate assessment of this impact. There is no questionnaire available for French-speaking patients with COM. This study aims to adapt and validate the French version of the COMQ-12 questionnaire. DESIGN: This is a controlled, prospective study, conducted between May 2020 and December 2021. Translation was performed using a forward-backward technique, and statistical validation was performed with a COM patients and a controls adult cohorts. STUDY SAMPLE: 100 patients (mean age 48 ± 16), and 50 controls (mean age 41 ± 16; p = 0.02) completed the test. RESULTS: Internal consistency, assessed by Cronbach's alpha, was 0.870 for the cases group. The mean COMQ-12 total score was 23.92 ± 11.3 for cases versus 3.70 ± 6.15 for controls (p < 0.0001). Individual items all had high discriminatory performances. The three items concerning ear discharge (Q1, Q2, Q9) had weaker correlation with the other items and the global score, but did not have a negative impact on internal consistency. CONCLUSION: The French version of the COMQ-12 is a short and easy-to-use test, with robust statistical properties, for assessing QOL in patients with COM.

Outcomes and prognostic factors of facial nerve repair: a retrospective comparative study of 31 patients and literature review.

PURPOSE: To evaluate functional results of facial nerve repair by direct nerve suture or grafting, compare results between a traumatic and a tumoral group and identify prognostic factors. METHODS: A retrospective monocentric cohort study was studied in a university ENT department. Thirty-one patients who benefited from facial nerve suture or grafting, with at least 12 months postoperative follow-up were included. Patients were divided into two groups according to the lesion type: traumatic (accident of the public road or iatrogenic) and tumoral. Preoperative data studied were sex, side, etiology documented by CT and/or MRI, facial palsy duration and grade according to House Brackmann grading system. Intraoperative data included: surgeon, age of patient, surgical technique, graft type, use of biological glue, facial nerve derivation, lesioned site. Postoperative data included: histological diagnosis, radiotherapy history, time to onset of the first signs of reinnervation, follow-up duration, and final facial function. RESULTS: Success rate, including grade III and IV, was 68% in the whole cohort, 79% in the traumatic group and 59% in the tumoral group. Patients presenting with facial palsy evolving less than 6 months had better recovery results than those evolving longer than 6 months (p = 0.02). No other prognostic factors were identified. CONCLUSIONS: The best outcome that can be achieved by suture or grafting of the facial nerve in traumatic or tumoral lesions is a grade III. Preoperative facial palsy duration is a prognostic factor and must be considered when establishing an operative indication.

Hearing recovery after surgical resection of non-vestibular schwannoma cerebellopontine angle tumors.

PURPOSE: Post-operative outcomes for hearing after resection surgery to remove cerebellopontine angle (CPA) tumors other than vestibular schwannomas (VS) are not well understood. This study presents a series of patients with significant post-operative hearing recovery, trying to define the incidence among all patients operated on for removal of non-VS CPA tumors. METHODS: This is a retrospective observational case series of 8 patients among 69 operated on for removal of non-VS CPA tumors between 2012 and 2020. All patients had pre- and post-operative hearing measurement with pure-tone average (PTA) and speech discrimination score (SDS), according to the American Academy of Otolaryngology-Head and Neck Surgery recommendations, auditory brainstem response (ABR) measurements and imaging. RESULTS: Six meningiomas and two lower cranial nerve schwannomas operated on with a retrosigmoid approach were included for analysis. The mean pre-operative PTA and SDS were 58 ± 20.7 dB and 13 ± 17.5%, respectively. All patients had pre-operative class D hearing and asynchronous ABRs. They all showed significant hearing recovery, with an improvement of 36 ± 22.2 dB (p = 0.0025) and 85 ± 16.9% (p = 0.0001) in PTA and SDS, respectively, with mean follow-up of 21 ± 23.5 months. Seven patients recovered to a class A hearing level and one patient to class B. The ABRs became synchronous for three patients. The incidence of auditory recovery was 13% for patients operated on with a conservative approach (n = 60). CONCLUSION: A significant post-operative improvement in hearing could be a reasonable expectation in non-VS tumors extending into the CPA and a retrosigmoid approach should always be considered regardless of pre-operative hearing status.

Presentation of dizziness in individuals with chronic otitis media: data from the multinational collaborative COMQ-12 study.

PURPOSE: In chronic otitis media (COM), disease chronicity and severity of middle ear inflammation may influence the development of inner ear deficits, increasing the risk of vestibular impairment. This secondary analysis of the multinational collaborative Chronic Otitis Media Questionnaire-12 (COMQ-12) dataset sought to determine the prevalence of vestibular symptoms in patients with COM and identify associated disease-related characteristics. METHODS: Adult patients with a diagnosis of COM in outpatient settings at nine otology referral centers across eight countries were included. We investigated the presence of vestibular symptoms (dizziness and/or disequilibrium) using participant responses to item 6 of a native version of the COMQ-12. Audiometric data and otoscopic assessment were also recorded. RESULTS: This analysis included 477 participants suffering from COM, with 56.2% (n = 268) reporting at least mild inconvenience related to dizziness or disequilibrium. There was a significant association between air conduction thresholds in the worse hearing ear and presence of dizziness [adjusted odds ratio (AOR), 1.01; 95% CI 1.00-1.02; p = 0.0177]. Study participants in European countries (AOR 1.53; 95% CI 1.03-2.28; p = 0.0344) and Colombia (AOR 2.48; 95% CI 1.25-4.92; p = 0.0096) were more likely to report dizziness than participants in Asian countries. However, ear discharge and cholesteatoma showed no association with dizziness in the adjusted analyses. CONCLUSION: Vestibular symptoms contribute to burden of disease in patients with COM and associates with hearing disability in the worse hearing ear. Geographical variation in presentation of dizziness may reflect financial barriers to treatment or cultural differences in how patients reflect on their health state.

Cochlear Implantation and Other Treatments in Single-Sided Deafness and Asymmetric Hearing Loss: Results of a National Multicenter Study Including a Randomized Controlled Trial.

INTRODUCTION: Cochlear implantation is a recent approach proposed to treat single-sided deafness (SSD) and asymmetric hearing loss (AHL). Several cohort studies showed its effectiveness on tinnitus and variable results on binaural hearing. The main objective of this study is to assess the outcomes of cochlear implantation and other treatment options in SSD/AHL on quality of life. METHODS: This prospective multicenter study was conducted in 7 tertiary university hospitals and included an observational cohort study of SSD/AHL adult patients treated using contralateral routing of the signal (CROS) hearing aids or bone-anchored hearing systems (BAHSs) or who declined all treatments, and a randomized controlled trial in subjects treated by cochlear implantation, after failure of CROS and BAHS trials. In total, 155 subjects with SSD or AHL, with or without associated tinnitus, were enrolled. After 2 consecutive trials with CROS hearing aids and BAHSs on headband, all subjects chose any of the 4 treatment options (abstention, CROS, BAHS, or cochlear implant [CI]). The subjects who opted for a CI were randomized between 2 arms (CI vs. initial observation). Six months after the treatment choice, quality of life was assessed using both generic (EuroQoL-5D, EQ-5D) and auditory-specific quality-of-life indices (Nijmegen Cochlear implant Questionnaire [NCIQ] and Visual Analogue Scale [VAS] for tinnitus severity). Performances for speech-in-noise recognition and localization were measured as secondary outcomes. RESULTS: CROS was chosen by 75 subjects, while 51 opted for cochlear implantation, 18 for BAHSs, and 11 for abstention. Six months after treatment, both EQ-5D VAS and auditory-specific quality-of-life indices were significantly better in the "CI" arm versus "observation" arm. The mean effect of the CI was particularly significant in subjects with associated severe tinnitus (mean improvement of 20.7 points ± 19.7 on EQ-5D VAS, 20.4 ± 12.4 on NCIQ, and 51.4 ± 35.4 on tinnitus). No significant effect of the CI was found on binaural hearing results. Before/after comparisons showed that the CROS and BAHS also improved significantly NCIQ scores (for CROS: +7.7, 95% confidence interval [95% CI] = [4.5; 10.8]; for the BAHS: +14.3, 95% CI = [7.9; 20.7]). CONCLUSION: Cochlear implantation leads to significant improvements in quality of life in SSD and AHL patients, particularly in subjects with associated severe tinnitus, who are thereby the best candidates to an extension of CI indications.

Objective improvement in adults with cerebellopontine angle arachnoid cysts after surgical treatment.

BACKGROUND: Intracranial arachnoid cysts are extra-axial benign lesions mainly found in the middle cerebral fossa. Rare case series report various cranial nerve dysfunctions associated with cerebellopontine angle (CPA) cysts and there is no consensus with regard to their surgical management; some reports claiming that subjective improvement in adults with intracranial arachnoid cysts cannot justify surgical treatment. METHODS: This retrospective study included all 12 consecutive adult patients treated by microsurgical fenestration for symptomatic CPA arachnoid cysts between 2010 and 2019 and using a retrosigmoid approach. Demographic, clinical, surgical, and radiological data were collected from medical files. RESULTS: The main symptoms were audiovestibular in 9 patients (75%) complaining of dizziness and 6 patients (50%) with hearing loss. In addition, 3 patients (25%) reported tinnitus, 3 patients (25%) presented vasovagal syncope, and 1 patient (8.3%) reported facial pain. Surgery improved 5 patients (83%) with pre-operative hearing loss, 7 patients (78%) reporting dizziness, and all patients with vasovagal syncope. All of the patients recovered from at least one symptom. No recurrence was observed with a mean follow-up of 5.5 years. CONCLUSION: Although most arachnoid cysts are asymptomatic, the CPA location may lead to cranial nerve impairments. Microsurgical fenestration seems to be a simple, safe, and effective technique.

Treatment choice in single-sided deafness and asymmetric hearing loss. A prospective, multicentre cohort study on 155 patients.

OBJECTIVES: To describe the treatment choice in a cohort of subjects with single-sided deafness (SSD) and asymmetric hearing loss (AHL). To assess the reliability of the treatment trials. DESIGN: In this national, multicentre, prospective study, the choice of subjects was made after two consecutive trials of Contralateral Routing Of the Signal (CROS) hearing aids and a Bone Conduction Device (BCD) on a headband. Subjects could proceed with one of these two options, opt for cochlear implantation or decline all treatments. SETTING: Seven tertiary university hospitals. PARTICIPANTS: One hundred fifty-five subjects with SSD or AHL fulfilling the candidacy criteria for cochlear implantation, with or without associated tinnitus. MAIN OUTCOME MEASURES: After the two trials, the number of subjects choosing each option was described. Repeated assessments of both generic and auditory-specific quality of life were conducted, as well as hearing assessments (speech recognition in noise and horizontal localization). RESULTS: CROS was chosen by 75 subjects, followed by cochlear implantation (n = 51), BCD (n = 18) and abstention (n = 11). Patients who opted for cochlear implantation had a poorer quality of life (P = .03). The improvement of quality of life indices after each trial was significantly associated with the final treatment choice (P = .008 for generic indices, P = .002 for auditory-specific indices). The follow-up showed that this improvement had been overestimated in the CROS group, with a long-term retention rate of 52.5%. CONCLUSIONS: More than one third of SSD/AHL subjects are unsatisfied after CROS and BCD trials. Repeated quality of life assessments help counselling the patient for his/her treatment choice.

Bioactive glass granules for mastoid and epitympanic surgical obliteration: CT and MRI appearance.

PURPOSE: To evaluate the appearance of mastoid and epitympanic obliteration using S53P4 bioactive glass (BG) granules in high-resolution computed tomography (HRCT) and MRI. MATERIALS AND METHODS: Patients undergoing mastoid and epitympanic obliteration between May 2013 and December 2015 were prospectively included in an uncontrolled clinical study. All patients underwent a temporal HRCT scan 1 year after surgery, aimed at evaluating the attenuation, homogeneity, and osseointegration of the BG granules, as well as the ventilation of the middle ear and the volume of the obliterated paratympanic spaces. If a cholesteatoma was found during surgery, additional MRI, including at least pre- and post-contrast T1-weighted, T2-weighted, and axial non-echo-planar diffusion-weighted (DW) sequences, was performed 1 year after surgery, to study the normal signal of the BG granules and the presence of residual cholesteatoma and/or other temporal bone pathologies. RESULTS: Seventy cases were included. On 1-year HRCT, the mean attenuation of the BG granules was 888.34 ± 166.10 HU. The obliteration was found to be mostly homogeneous with partial osseointegration. The appearance of the BG granules having a low-intensity signal in T2-weighted imaging and DW MRI was always different from the appearance of cholesteatoma. A longer follow-up has shown no attenuation or signal modification of the BG granules compared with the 1-year imaging. CONCLUSION: Radiological follow-up of patients operated on with mastoid and epitympanic obliteration using BG granules is effective using both HRCT and MRI. A cholesteatoma and/or other potential complications could easily be detected due to the specific radiological appearance of the BG granules. KEY POINTS: • The appearance of mastoid and epitympanic obliteration by S53P4 bioactive glass (BG) granules on high-resolution computed tomography (HRCT) scans was homogeneous with an attenuation significantly higher than the attenuation of cholesteatoma and lower than mastoid bone attenuation. • The granules have a low-intensity signal on non-echo-planar diffusion-weighted sequences and on T2-weighted images and present contrast enhancement allowing the differential diagnosis with cholesteatoma and effective for the detection of other underlying temporal bone pathologies. • The volume and radiological appearance of the obliteration appear to be stable with time.

Benefits of a contralateral routing of signal device for unilateral Naída CI cochlear implant recipients.

PURPOSE: Many bilaterally deaf adults are only able to receive one cochlear implant (CI), resulting in suboptimal listening performance, especially in challenging listening environments. Adding a contralateral routing of signal (CROS) device to a unilateral CI is one possibility to alleviate these challenges. This study examined the benefit of such a CROS device. METHODS: Thirteen adult subjects with at least 6 months of CI use, and no or limited benefit of a hearing instrument in the contralateral ear were included in the study. The perceived benefit of a CROS device in everyday listening environments was evaluated up to 1 year after initial fitting using several questionnaires. Speech intelligibility performance was determined using the French matrix sentence test in quiet and in two speech-in-noise setups and was followed for 3 months after CROS fitting. RESULTS: Subjects indicated high satisfaction with the practical usability of the CROS device and long-term device retention was high. Perceived benefits in everyday listening environments were reported. Formal speech intelligibility tests revealed statistically significant median improvements of 6.93 dB SPL (Wilcoxon Z = 2.380, p = 0.017) in quiet and up to 8.00 dB SNR (Wilcoxon Z = 2.366, p = 0.018) in noise. These benefits were accessible immediately without a need for prolonged acclimatization. CONCLUSIONS: Subjective satisfaction and device retention as well as speech intelligibility benefits in quiet and in noise prove the CROS device to be a valuable addition to a unilateral CI in cases of bilateral deafness where bilateral implantation is not an option.

3D endoscopic ear surgery: a clinical pilot study.

OBJECTIVE: To evaluate the feasibility and efficacy of a new high-resolution 3D endoscope in endoscopic ear and lateral skull base surgery. METHODS: Eight patients, five women and three men, were included in this clinical pilot prospective study. Their age was 38 ± 17.3 years (mean ± SD, range 17-54 years). The right side was involved in five cases, and the left side in three cases. There were three cases of chronic otitis, one case of cholesteatoma of the external auditory canal, one case of otosclerosis, one case of cochlear implant surgery, and two cases of stage 2 vestibular schwannoma operated on with a retrosigmoid approach to preserve hearing. RESULTS: There were no intraoperative or postoperative complications. The new system was used during all procedures without the necessity to switch to a 2D mode. The surgeons felt comfortable using the system and its major advantages were considered to be in measuring the distances for ossicular chain reconstruction and in the sense of depth provided in the middle ear and cerebellopontine angle. CONCLUSION: The sense of depth provided by the 3D system is effective in both middle ear and lateral skull base surgery. Further improvements (smaller and/or more angled endoscopes) should be considered for future developments in endoscopic ear surgery.

Modifications to a 3D-printed temporal bone model for augmented stapes fixation surgery teaching.

Functional outcomes and complications in otosclerosis surgery are governed by the surgeon's experience. Thus, teaching the procedure to residents to guide them through the learning process as quickly as possible is challenging. Artificial 3D-printed temporal bones are replacing cadaver specimens in many institutions to learn mastoidectomy, but these are not suitable for middle ear surgery training. The goal of this work was to adapt such an artificial temporal bone to aid the teaching of otosclerosis surgery and to evaluate this tool. We have modified a commercially available 3D-printed temporal bone by replacing the incus and stapes of the model with in-house 3D-printed ossicles. The incus could be attached to a 6-axis force sensor. The stapes footplate was fenestrated and attached to a 1-axis force sensor. Six junior surgeons (residents) and seven senior surgeons (fellows or consultants) were enrolled to perform piston prosthesis placement and crimping as performed during otosclerosis surgery. The time required to perform the tasks and the forces applied to the incus and stapes were collected and analyzed. No statistically significant differences were observed between the junior and senior groups for time taken to perform the tasks and the forces applied to the incus during crimping and placement of the prosthesis. However, significantly lower forces were applied to the stapes by the senior surgeons in comparison with the junior surgeons during prosthesis placement (junior vs senior group, 328 ± 202.9 vs 80 ± 99.6 mN, p = 0.008) and during prosthesis crimping (junior vs senior group, 565 ± 233 vs 66 ± 48.6 mN, p = 0.02). We have described a new teaching tool for otosclerosis surgery based on the modification of a 3D-printed temporal bone to implement force sensors on the incus and stapes. This tool could be used as a training tool to help the residents to self-evaluate their progress with recording of objective measurements.

Benefit of the UltraZoom beamforming technology in noise in cochlear implant users.

The objectives of the study were to demonstrate the audiological and subjective benefits of the adaptive UltraZoom beamforming technology available in the Naída CI Q70 sound processor, in cochlear-implanted adults upgraded from a previous generation sound processor. Thirty-four adults aged between 21 and 89 years (mean 53 ± 19) were prospectively included. Nine subjects were unilaterally implanted, 11 bilaterally and 14 were bimodal users. The mean duration of cochlear implant use was 7 years (range 5-15 years). Subjects were tested in quiet with monosyllabic words and in noise with the adaptive French Matrix test in the best-aided conditions. The test setup contained a signal source in front of the subject and three noise sources at +/-90° and 180°. The noise was presented at a fixed level of 65 dB SPL and the level of speech signal was varied to obtain the speech reception threshold (SRT). During the upgrade visit, subjects were tested with the Harmony and with the Naída CI sound processors in omnidirectional microphone configuration. After a take-home phase of 2 months, tests were repeated with the Naída CI processor with and without UltraZoom. Subjective assessment of the sound quality in daily environments was recorded using the APHAB questionnaire. No difference in performance was observed in quiet between the two processors. The Matrix test in noise was possible in the 21 subjects with the better performance. No difference was observed between the two processors for performance in noise when using the omnidirectional microphone. At the follow-up session, the median SRT with the Naída CI processor with UltraZoom was -4 dB compared to -0.45 dB without UltraZoom. The use of UltraZoom improved the median SRT by 3.6 dB (p < 0.0001, Wilcoxon paired test). When looking at the APHAB outcome, improvement was observed for speech understanding in noisy environments (p < 0.01) and in aversive situations (p < 0.05) in the group of 21 subjects who were able to perform the Matrix test in noise and for speech understanding in noise (p < 0.05) in the group of 13 subjects with the poorest performance, who were not able to perform the Matrix test in noise. The use of UltraZoom beamforming technology, available on the new sound processor Naída CI, improves speech performance in difficult and realistic noisy conditions when the cochlear implant user needs to focus on the person speaking at the front. Using the APHAB questionnaire, a subjective benefit for listening in background noise was also observed in subjects with good performance as well as in those with poor performance. This study highlighted the importance of upgrading CI recipients to new technology and to include assessment in noise and subjective feedback evaluation as part of the process.

Audiological Results and Quality of Life of Sophono Alpha 2 Transcutaneous Bone-Anchored Implant Users in Single-Sided Deafness.

Single-sided deafness (SSD) represents one of the most difficult audiological conditions to rehabilitate. The aim of this prospective study was to evaluate the audiological benefits and quality of life of patients affected by SSD who had previously been users of the Alpha 1® when upgrading them to the Sophono Alpha 2® external processor (Boulder, Colo., USA). Nine patients were included in the study. They underwent physical examination, free-field speech audiometry at 40 and 60 dB, a hearing-in-noise test (Hirsch's test and the squelch test), the Glasgow Benefit Inventory (GBI) questionnaire, and a specific questionnaire on patient satisfaction with Alpha 1. Afterwards, the Alpha 2 external processor was delivered to all patients, and the above-mentioned protocol was repeated after 1 month with the Alpha 2. A statistically significant improvement was found in the speech discrimination score at 40 dB and in the squelch test when using the Alpha 2 external processor compared to the Alpha 1. Alpha 2 had a good clinical tolerance and gave similar results in the specific questionnaire and the GBI to Alpha 1. In conclusion, the new Alpha 2 external processor represents a safe and effective device for the rehabilitation of SSD, and there is an audiological benefit to upgrading to the Alpha 2 external processor for patients who had previously been users of the Alpha 1. The improvement in quality of life is similar to that with other bone-anchored hearing devices.

Intratemporal facial nerve schwannoma: clinical presentation and management.

Facial nerve schwannoma is the most common facial nerve tumor, but its therapeutic strategy remains debated. The aim of this study is to analyze the facial nerve function and the hearing outcomes after surgery or wait-and-scan policy in a facial nerve schwannoma series. A monocentric retrospective review of medical charts of patients followed for an intratemporal facial nerve schwannoma between 1988 and 2013 was performed. Twenty-two patients were included. Data were extracted pertaining to the following variables: patient demographics, tumor localization, clinical and imaging features, facial nerve function and hearing levels, and details of surgical intervention. The majority of tumors were located at the geniculate ganglion. Initial symptoms were mainly facial palsy and hearing loss. The average follow-up was 4.8 ± 4.5 years. Nineteen patients underwent surgery, and three patients were observed. After surgery, 11 patients had a stable or improved facial nerve function (57.9 %), and 8 patients had a worsened facial nerve function (42.1 %). Facial nerve function was in the majority of cases a HB grade III, depending on surgical strategy. No patient presented a postoperative HB grade V or VI. Regarding the hearing, it remained stable after surgery in 52.6 % of cases, and improved in 10.5 % of cases. Among monitored patients, facial nerve function and hearing remained stable. Surgery for facial nerve schwannoma is a safe and effective option in the treatment of these tumors.

Management of epi- and mesotympanic cholesteatomas by one-stage trans-canal atticotomy in adults.

Surgical management of cholesteatoma limited to the attic and/or mesotympanum remains controversial. The aim of this study is to evaluate the anatomical and the functional results of trans-canal atticotomy in this pathological condition. The records of 27 adult patients treated from 2008 to 2014 who underwent trans-canal atticotomy for primary cholesteatoma surgery were reviewed. Pre-operative physical examination, audiometry, and CT-scan have been analyzed. Intraoperative findings have been described as well as the surgical technique. Anatomical and functional results have been evaluated with a mean follow-up of 24 ± 12.2 months, and the results of a CT-scan performed 1 year after surgery were examined to assess the presence of residual disease. Surgeries were uneventful. During the follow-up, 1 patient (4 %) experienced a retraction of the attical reconstruction; all the other patients had a well-healed tympanic drum with stable attical reconstruction. The mean air-bone gap was 19 ± 12.2 and 10 ± 7.3 dB pre-operatively and post-operatively, respectively (mean ± SD, p = 0.001, paired t test). Twenty-two patients (81 %) had no opacity suggesting residual cholesteatoma in CT-scan. Four patients (15 %) presenting an opacity at CT-scan underwent MRI study that was negative for residual cholesteatoma. One patient (4 %) had displacement of the ossicular prosthesis. In conclusion, cholesteatomas restricted to the attic and/or mesotympanum can be removed in a one-stage technique with no visible residual at 1 year, and with closure of the air-bone gap by 50 %.

Variability of the mental representation of the cochlear anatomy during cochlear implantation.

The aim of this study was to assess the mental representation of the insertion axis of surgeons with different degrees of experience, and reproducibility of the insertion axis in repeated measures. A mastoidectomy and a posterior tympanotomy were prepared in five different artificial temporal bones. A cone-beam CT was performed for each temporal bone and the data were registered on a magnetic navigation system. In these five temporal bones, 16 surgeons (3 experts; >50 cochlear implant surgery/year; 7 fellows with few cochlear implant experience, and 6 residents) were asked to determine the optimal insertion axis according to their mental representation. Compared to a planned ideal axis, the insertion axis was better determined by the experts with higher accuracy (axial: 7° ± 1.5°, coronal: 6° ± 1.5°) than fellows (axial: 14° ± 1.7°, coronal: 13° ± 1.7°; p < 0.05), or residents (axial: 15° ± 1.5°; p < 0.001, coronal: 17° ± 1.9°; p < 0.001). This study suggests that mental representation of the cochlea is experience-dependent. A high variation of the insertion axis to the scala tympani can be observed due to the complexity of the temporal bone anatomy and lack of landmarks to determine scala tympani orientation. Navigation systems can be used to evaluate and improve mental representation of the insertion axis to the scala tympani for cochlear implant surgery.

Phase II study of mTORC1 inhibition by everolimus in neurofibromatosis type 2 patients with growing vestibular schwannomas.

Neurofibromatosis type 2 (NF2) is a genetic disorder with bilateral vestibular schwannomas (VS) as the most frequent manifestation. Merlin, the NF2 tumor suppressor, was identified as a negative regulator of mammalian target of rapamycin complex 1. Pre-clinical data in mice showed that mTORC1 inhibition delayed growth of NF2-schwannomas. We conducted a prospective single-institution open-label phase II study to evaluate the effects of everolimus in ten NF2 patients with progressive VS. Drug activity was monitored every 3 months. Everolimus was administered orally for 12 months and, if the decrease in tumor volume was >20 % from baseline, treatment was continued for 12 additional months. Other patients stopped when completed 12 months of everolimus but were allowed to resume treatment when VS volume was >20 % during 1 year follow-up. Nine patients were evaluable. Safety was evaluated using CTCAE 3.0 criteria. After 12 months of everolimus, no reduction in volume ≥20 % was observed. Four patients had progressive disease, and five patients had stable disease with a median annual growth rate decreasing from 67 %/year before treatment to 0.5 %/year during treatment. In these patients, tumor growth resumed within 3-6 months after treatment discontinuation. Everolimus was then reintroduced and VS decreased by a median 6.8 % at 24 months. Time to tumor progression increased threefold from 4.2 months before treatment to > 12 months. Hearing was stable under treatment. The safety of everolimus was manageable. Although the primary endpoint was not reached, further studies are required to confirm the potential for stabilization of everolimus.