Auditory outcomes after scala vestibuli array insertion are similar to those after scala tympani insertion 1 year after cochlear implantation.

PURPOSE: In cochlear implantation, a scala vestibuli (SV) insertion of an electrode array is a rare occurrence and is reported to be linked to poor hearing outcomes. Using the same electrode array, the auditory performance of patients with a complete SV location was compared with that of patients having a complete scala tympani (ST) location 1 year after implantation. METHODS: Thirty-three patients were included in this retrospective case-control study (SV, n = 12; ST, n = 21). The matching criteria were electrode array type, age at implantation, and duration of severe or profound deafness. The array location was analyzed using 3D reconstruction of postoperative CT scans. Postoperative audiological evaluation of the implanted ear was performed using pure-tone audiometry, speech recognition of monosyllabic words in quiet, and words and sentences in noise. RESULTS: On the preoperative CT scan, six patients in the SV group presented with both round window (RW) and ST ossification, three with RW ossification alone, and three with no RW ossification. Auditory performance did not differ between SV and ST groups 1 year after cochlear implantation. Speech recognition of words was 49 ± 7.6% and 56 ± 5.0% in quiet and 75 ± 9.5% and 66 ± 6.0% in noise in SV and ST groups, respectively. CONCLUSION: ST insertion is the gold standard that allows the three cochlear scalae to preserve scalar cochlear integrity. However, 1 year after implantation, a planned or unexpected SV insertion is not detrimental to hearing outcomes, providing similar auditory performance in quiet and noise to ST insertion.

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.

Cochlear implantation and facial nerve anastomosis to rehabilitate a burnt middle ear.

A 48-year-old man presented to the ENT department of a general hospital after a traumatic event that occurred at work. During arc welding, an incandescent metallic projectile entered the left external auditory canal immediately causing earache, tinnitus, hearing loss, vertigo, and completed peripheral facial palsy on the left side. A burnt middle ear is a rare situation for which very few cases are described in the literature. We describe and discuss our therapeutic strategy in an emergency setting. This can be of interest to any ENT surgeon who may face such a case of ear burn. Then we report the first repair of the facial nerve by interposition of an anastomosed intermediate graft associated with cochlear implantation during a single surgical intervention. This case illustrates the multimodal rehabilitation of the damage that can be a consequence of petrous trauma. The patient recovered hearing and facial motor skills.

Dual AAV-mediated gene therapy restores hearing in a DFNB9 mouse model.

Autosomal recessive genetic forms (DFNB) account for most cases of profound congenital deafness. Adeno-associated virus (AAV)-based gene therapy is a promising therapeutic option, but is limited by a potentially short therapeutic window and the constrained packaging capacity of the vector. We focus here on the otoferlin gene underlying DFNB9, one of the most frequent genetic forms of congenital deafness. We adopted a dual AAV approach using two different recombinant vectors, one containing the 5' and the other the 3' portions of otoferlin cDNA, which exceed the packaging capacity of the AAV when combined. A single delivery of the vector pair into the mature cochlea of Otof-/- mutant mice reconstituted the otoferlin cDNA coding sequence through recombination of the 5' and 3' cDNAs, leading to the durable restoration of otoferlin expression in transduced cells and a reversal of the deafness phenotype, raising hopes for future gene therapy trials in DFNB9 patients.

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.

Cochlear Implant Outcomes in Patients with Neurosarcoidosis.

INTRODUCTION: Audiovestibular symptoms are rare in sarcoidosis, but they may also be the first manifestation of the disease. Sudden or progressive bilateral hearing loss is usually associated with vestibular impairment. The mechanism of hearing loss remains unclear, but clinical presentation and magnetic resonance imaging suggest a retrocochlear site for the lesion in most patients. Several cases of hearing recovery after corticosteroid treatment have been reported. In patients with severe or profound hearing loss, the benefit of cochlear implantation is challenging to predict in the case of auditory neuropathy and is rarely described. We present a case series of cochlear implantation in patients with documented neurosarcoidosis. RESULTS: Seven cases of cochlear implantation in 4 patients with neurosarcoidosis are reported. All of the patients showed a great improvement very quickly in both quiet and noise. Speech performance remained stable over time with a follow-up ranging from 4 to 11 years, even in patients who had disease exacerbation. CONCLUSION: Cochlear implantation is possible in deaf patients with neurosarcoidosis. The excellent benefit obtained in our patients suggests a particular type of neuropathy, but endocochlear involvement cannot be entirely ruled out.

Intraoperative facial nerve electromyography parameters to optimize postoperative facial nerve outcome in patients with large unilateral vestibular schwannoma.

BACKGROUND: Decision-making for large sporadic vestibular schwannomas (VS) resection guided by the intraoperative change in supramaximal facial nerve (FN) amplitude and latency response to optimize post-operative FN outcome. METHODS: Prospectively study of 43 patients, from January to December 2018, of large sporadic VS with preoperative normal FN function at our center. Tumors were removed through retrosigmoid (81%) or translabyrinthine (19%) approaches with FN monitoring. Intraoperative pre- and post-VS resection supramaximal (2 mA) amplitude and latency responses at the proximal FN root were recorded. RESULTS: Total, near-/subtotal VS resections (TR, NTR, STR) were achieved in 51%, 38%, and 11% of tumors, respectively, guided by no more than 40% decrease in supramaximal amplitude. Pre- and post-resection supramaximal amplitude and latency responses were lower and longer, respectively, in NTR+STR than in TR. At day 8, FN function was grade I-II in 77% of patients and grade III-V in 23%, and after 6 months, it was in grade I-II in 95% and grade III in 5%, and there was no significant difference between TR and NTR+STR. Facial palsy occurred in older patients and in the case of severe FN adhesion. At day 8, pre- and post-resection supramaximal amplitude but not latency responses were different between FN grade III-V and grade I-II. Serviceable hearing was preserved in 28% of large VS. CONCLUSIONS: Intraoperative FN monitoring guided VS resection in large VS so that 49% retained some residual tumor. Accordingly, 95% good postoperative FN function and significant hearing preservation were achieved after 6 months.

Benefits in noise from sound processor upgrade in thirty-three cochlear implant users for more than 20 years.

PURPOSE: Some oldest patients rehabilitated with a cochlear implant more than 20 years ago could still be upgraded with new generations of speech processor (SP). The aim of this study was to show the benefit of a recent generation of SP in this population. METHODS: A monocentric prospective study was designed to evaluate the performance of 33 ancient CI22M users implanted between 1989 and 1997 and upgraded with the late compatible sound processor CP900. Performance was evaluated in quiet and noise with Framatix, an automated adaptative test. RESULTS: Performance using Framatix significantly improved with the CP900, with a decrease of the median speech perception threshold of 6 dB in quiet (p < 0.05) and 5,3 dB in noise (p < 0.0005). No subjective benefit using the APHAB questionnaire was observed. CONCLUSION: Upgrading of cochlear implant recipients who were implanted more than 20 years ago with recent compatible and new technological SP provide benefit in speech recognition in noise.

Robot-based assistance in middle ear surgery and cochlear implantation: first clinical report.

PURPOSE: Middle ear surgery may benefit from robot-based assistance to hold micro-instruments or an endoscope. However, the surgical gesture performed by one hand may perturb surgeons accustomed to two-handed surgery. A robot-based holder may combine the benefits from endoscopic exposure and a two-handed technique. Furthermore, tremor suppression and accurate tool control might help the surgeon during critical surgical steps. The goal of this work was to study the safety of an otological robot-based assistant under clinical conditions in a limited series of patients. METHODS: The RobOtol system has been used as an endoscope or a micro instrument holder for this series. Eleven cases were operated on with the robot as an endoscope holder for chronic otitis. Twenty-one cases were operated on with the robot as a micro-instrument holder for otosclerosis (9 cases), transtympanic tube placement (2 cases), or cochlear implantation (10 cases). RESULTS: No complications related to the robot manipulation occurred during surgery nor in postoperative. In the chronic otitis group, all perforations were sealed and 3-month postoperative pure-tone average air-bone gap (PTA ABG) was 15 ± 2.6 dB. In the otosclerosis group, 1-month post-op PTA ABG was 10 ± 1 dB. For cochlear implantation cases, a scala tympani insertion, a vestibular scala translocation occurred and a full scala vestibuli insertion was observed in 7, 2 and 1 case, respectively. CONCLUSION: The RobOtol system has reached the clinical stage. It could be used safely and with accurate control as an endoscope holder or a micro instrument holder in 32 cases.

Local gene therapy durably restores vestibular function in a mouse model of Usher syndrome type 1G.

Our understanding of the mechanisms underlying inherited forms of inner ear deficits has considerably improved during the past 20 y, but we are still far from curative treatments. We investigated gene replacement as a strategy for restoring inner ear functions in a mouse model of Usher syndrome type 1G, characterized by congenital profound deafness and balance disorders. These mice lack the scaffold protein sans, which is involved both in the morphogenesis of the stereociliary bundle, the sensory antenna of inner ear hair cells, and in the mechanoelectrical transduction process. We show that a single delivery of the sans cDNA by the adenoassociated virus 8 to the inner ear of newborn mutant mice reestablishes the expression and targeting of the protein to the tips of stereocilia. The therapeutic gene restores the architecture and mechanosensitivity of stereociliary bundles, improves hearing thresholds, and durably rescues these mice from the balance defects. Our results open up new perspectives for efficient gene therapy of cochlear and vestibular disorders by showing that even severe dysmorphogenesis of stereociliary bundles can be corrected.

Improving facial nerve outcome and hearing preservation by different degrees of vestibular schwannoma resection guided by intraoperative facial nerve electromyography.

BACKGROUND: Facial nerve (FN) outcome has been improved by deciding upon the degree of vestibular schwannoma (VS) resection guided by intraoperative facial nerve monitoring (IOFM). This study aimed to evaluate hearing and FN outcomes following IOFM-guided VS removal through a retrosigmoid approach (RS). METHODS: Between January 2015 and August 2017, 77 patients undergoing VS resection through RS, regardless of their preoperative hearing or tumor size, were included in this monocentric retrospective study. Total or near-/subtotal resection was decided intraoperatively according to IOFM. Partial resection was planned preoperatively. Pre- and postoperative FN function (House-Brackmann) and hearing level (AAO-HNS and Gardner-Robertson (GR) scales) were reported. Predictive factors for hearing preservation were analyzed according to a score developed to take into account pre-/postoperative hearing evolution. RESULTS: The mean extrameatal diameter was 17 ± 8.5 mm. VS resection was total, near-/subtotal, and partial in 71%, 21%, and 8% of cases, respectively. Postoperative grade I and II FN function was achieved in 91% of patients. Serviceable (A+B or GR I+II) hearing and hearing with intelligibility (A+B+C or GR I-III) were preserved in 30% and 43% of cases, respectively. In multivariate analysis, preoperative synchronized auditory brainstem responses (ABR) were the only predictive factor (p = 0.006) imparting a five times greater chance of hearing preservation. CONCLUSION: IOFM-guided VS excision through RS, whatever the VS size and hearing level, achieved a high rate of good postoperative FN function together with significant hearing preservation, and it should be used if hearing with some intelligibility is still present preoperatively, even in large VS.

Long-term residual hearing in cochlear implanted adult patients who were candidates for electro-acoustic stimulation.

PURPOSE: To evaluate the long-term hearing outcomes in cochlear implanted adults with residual hearing at low frequencies, and the proportion of patients using electro-acoustic stimulation (EAS). METHODS: A monocentric retrospective cohort study was performed in a tertiary referral center. Population demographics, surgical approach, pre- and postoperative hearing at low frequencies, in the implanted and contralateral ear, were recorded as well as duration of EAS use. The percentage hearing preservation was calculated according to the formula S (HEARRING group). RESULTS: In total, 63 adults (81 ears) with residual hearing underwent cochlear implantation with intent to use EAS processors. Six different types of electrode array were implanted. The mean pure tone audiometry (PTA) shift after cochlear implantation was 16 ± 15 dB HL (range 0-59 dB HL). Half of the implanted ears had minimal hearing preservation or total hearing loss (HL) at 5.5 years and the cumulative risk of total HL was 50% at 7 years. During the follow-up, total HL occurred in 22 ears. The decrease in hearing levels was similar in both implanted and contralateral ear during follow-up (ns, F = 2.46 ± 3, Linear Mixed Model (LMM)). Only 44 patients found a benefit from EAS at the first fitting. At the last visit, EAS processors were fitted in 30% of the cases. The pre- and postoperative mean PTA thresholds were not predictive of EAS use (Cox's proportional hazards analysis). CONCLUSIONS: Postoperative residual hearing was observed in 93% of implanted ears, but only half of them had an initial benefit from EAS. No predictive factors were found to influence the use of EAS processors.

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.

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.