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International audience; 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 postoperative 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.

Treatment of neurofibromatosis type 2 (NF2) associated vestibular schwannomas (VS) can prove challenging because of the various choices, from observation to surgery and radiosurgery with medical treatment opportunities, sometimes within clinical trials. Some factors direct the treatment decision tree such as size of VS and hearing level. Others factors have to be considered such as patient clinical status, inherited situation, and contralateral VS characteristics. All these factors are detailed, discussed, and weighted in this review. Another essential part of the clinical management of patients with NF2 includes the hearing rehabilitation process using either cochlear or auditory brainstem implants. The management of VS in patients with NF2 has become difficult, owing to a multitude of solution options, and should be performed in reference NF2 centers.

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. 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). 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. 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

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). 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. 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. 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.

To evaluate the appearance of mastoid and epitympanic obliteration using S53P4 bioactive glass (BG) granules in high-resolution computed tomography (HRCT) and MRI. 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. 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. 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. • 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.

International audience; 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.

International audience; 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 %.

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.

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.

International audience; Most cochlear implantations are unilateral. To explore the benefits of a binaural cochlear implant, we used water-labelled oxygen-15 positron emission tomography. Relative cerebral blood flow was measured in a binaural implant group (n = 11), while the subjects were passively listening to human voice sounds, environmental sounds non-voice or silence. Binaural auditory stimulation in the cochlear implant group bilaterally activated the temporal voice areas, whereas monaural cochlear implant stimulation only activated the left temporal voice area. Direct comparison of the binaural and the monaural cochlear implant stimulation condition revealed an additional right temporal activation during voice processing in the binaural condition and the activation of a right fronto-parietal cortical network during sound processing that has been implicated in attention. These findings provide evidence that a bilateral cochlear implant stimulation enhanced the spectral cues associated with sound perception and improved brain processing of voice stimuli in the right temporal region when compared to a monaural cochlear implant stimulation. Moreover, the recruitment of sensory attention resources in a right fronto-parietal network allowed patients with bilateral cochlear implant stimulation to enhance their sound discrimination, whereas the same patients with only one cochlear implant stimulation had more auditory perception difficulties.

The objective of this study was to measure performance benefits obtained by upgrading recipients of the Cochlear Nucleus CI24 cochlear implant to the new CP810 sound processor. Speech recognition in quiet and in spatially separated noise was measured in established users of the Cochlear ESPrit 3G (n = 22) and Freedom (n = 13) sound processors, using the "Everyday" listening program. Subjects were then upgraded to the CP810 processor and were re-assessed after a 3-month period, using both the "Everyday" program and the new "Noise" program, which incorporates several pre-processing features including a new directional microphone algorithm ("Zoom"). Subjective perceptions were also recorded using the abbreviated profile of hearing aid benefit (APHAB) questionnaire. Mean scores for monosyllables in quiet, presented at 50 and 60 dB SPL, increased by 11% (p < 0.0001) and 8% (p < 0.001), respectively, after upgrade, for all subjects combined. Significant increases were also recorded for both processor groups. In noise, the mean scores were 60.0 and 67.4% for the original and CP810 Everyday programs, respectively (difference not significant). With the CP810 Noise programs the mean score increased to 82.5% (p < 0.01), with significant increases in both processor groups. There was evidence of slightly greater upgrade benefit in users of the ESPrit 3G processor and in relatively poor performers. The APHAB questionnaire also indicated significant reduction in perceived difficulty in the background noise and reverberation sub-scales after upgrade. The findings of the study appear to support the expectation of increased benefit from the new CP810 sound processor.

Meningiomas of the posterior fossa represent a heterogeneous group of tumors regarding difficulty of resection and functional outcome. The aim of this review was to focus on tumors located mainly on the posterior surface of the petrous bone and threatening hearing and facial functions. An anatomical classification was used to evaluate surgical outcome depending on the relationship of the tumor to the internal auditory meatus. The authors performed a retrospective chart review of 53 consecutive patients operated on for a posterior petrous bone meningioma in a tertiary referral center and a literature review. Tumors were classified in four groups according to the modified Desgeorges and Sterkers classification: posterior petrous (P; 17 tumors), meatus and internal auditory canal (M; 12 tumors), petrous apex without invasion of the internal auditory canal (A; 9 tumors), and cerebellopontine angle with invasion of the internal auditory canal (AMP; 15 tumors). The facial function was preserved in 100% of P and A tumors, 75% of group M tumors, and 53% of AMP tumors. Hearing preservation was attempted only in P, A, and M groups where rates of serviceable hearing preservation were 82%, 80%, and 66%, respectively. The cumulative rate of hearing preservation for AMP tumors was 45% in our literature review. The increased facial morbidity associated with AMP tumors compared with other groups was also confirmed by the review of the literature. In conclusion, this study shows that among posterior petrous bone meningiomas, tumors invading the internal auditory canal present with increased postoperative functional morbidity in spite of a tailored approach regarding dural insertion.

Our objective is to prospectively report very early complications and outcomes of cerebellopontine angle (CPA) surgery. Between January and August 2007, 72 patients were operated on by different transpetrosal approaches in a tertiary referral center. During preoperative assessment, facial nerve function (House-Brackmann grading system), the presence of vertigo or tinnitus and caloric test results were recorded and correlated with complications and symptoms occurring daily from day (D) 1 to D 8. The overall number of complications did not differ from those of former retrospective studies; nevertheless, the prospective feature of this study prompts several comments. Even slight (grade II) preoperative facial impairments increased the risk of severe postoperative facial dysfunction. Keratitis was frequent (42%) even in patients with normal facial function. Thrombo-embolic complications only occurred after long air-travel (≥5 h). Preoperative caloric test status was predictive of postoperative vestibular disturbance occurrence. With respect to the activity recovery; younger patients (40 years old) displayed faster central compensations than the older (60 years old) patients. This study highlights several features that may be used for preoperative patient counseling and complication management. In particular, the practitioner has to pay attention to even minor preoperative clinical signs of facial dysfunction to properly inform the patient of facial outcome. Routine ophthalmologic evaluation should be practiced, even when facial function is normal or subnormal.1b.

Recent advances in the field of the physiology of inner ear fluids permitted the characterization of the molecular mechanisms involved in critical processes such as the absorption of K(+) through cochlear sensory hair cells (mechanoelectrical transduction) or the secretion of K(+) by marginal cells of the stria vascularis. In addition, new pathways for ion circulations were evidenced. Mutations of transporters involved in some of these pathways, especially in K(+) recycling through gap junction systems, and in local pH regulation, are among the most frequent etiologies of genetic deafness in humans.

The aim of this study was to evaluate the functional outcome of facial nerve repair with fibrin glue in end-to-end anastomosis and intermediate nerve graft. Thirty-six patients undergoing facial nerve repair by end-to-end anastomosis or facial nerve grafting using exclusively fibrin glue between 1986 and 1999 were included in this retrospective study. The population comprised ten vestibular schwannomas (28%), nine temporal bone fractures (25%), seven facial nerve schwannomas (19%), four facial nerve hemangiomas (11%), two iatrogenic facial nerve interruptions (6%) and four miscellaneous facial nerve lesions (11%). Data were reviewed concerning etiology, location of the nerve interruption, type of repair and postoperative facial function according to the repaired facial nerve recovery scale (A: normal; B: independent movements of eyelid and mouth; C: strong closure of eyelids and mouth; D: incomplete eyelid closure; E: minimal movement; F: no movement). Eleven patients (31%) underwent end-to-end nerve anastomosis and 25 (69%) underwent intermediate facial nerve grafting. The mean follow-up period was 50 months (range: 3-95). Among patients followed-up more than 18 months (n = 20), a score of B or C was obtained in 16 patients (80%), a score D in 2 cases (10%) and a score E in 2 cases (10%). The type of repair and the site of interruption did not influence the results. Fibrin glue is a simple, rapid and efficient means of facial nerve repair. In case of intraoperative facial nerve interruption, this type of repair can be attempted in any location at the time of the tumor removal.

The aim of this retrospective study was to evaluate the functional results of surgical lowering of the high jugular bulb in the treatment of patients with Ménière's disease and pulsatile tinnitus. Fifteen patients with disabling Ménière's disease associated with pulsatile tinnitus and a high and medial jugular bulb were included in this study. As treatment a complete mastoidectomy was performed, after which the jugular bulb was freed by an infralabyrinthine and subfacial approach. The bulb was then displaced downwards with surgical wax. Functional results of surgery were assessed by a questionnaire according to the 1995 guidelines of the United States American Academy Committee on Hearing and Equilibrium, audiometric and vestibular tests, and by magnetic resonance and computed tomographic imaging with vascular sequences. Surgical treatment was contraindicated in two cases: one had hypoplasia of the contralateral sigmoid sinus and the other a small petrous hemangioma located around the jugular bulb that was discovered peroperatively. Among the 13 patients treated by definitive surgery, attacks of vertigo were reported as disabling in 12 cases preoperatively (92%) versus 1 (8%) after surgical treatment. No significant change in hearing was observed after surgery. Tinnitus had been reported in all patients preoperatively and decreased in intensity in four (31%) and disappeared in three (23%) after surgery.

Facial nerve schwannoma is an uncommon tumor and bilateral facial nerve tumors are extremely rare. A case is presented in which neuromas affecting the intra-canalicular and labyrinthine portions of both facial nerves occurred. Radiologic assessment demonstrated the origin of these tumors. Eventual tumor involvement of the sole functioning cochlea resulted in the development of total hearing loss. Management entailed symptomatic care and surgical resection. Auditory rehabilitation was attempted using cochlear implantation, but results have not been satisfactory. Genetic screening identified a mutation in the NF2 gene. It is proposed that this patient's condition should be considered a variant of neurofibromatosis 2 and that bilateral facial neuromas should be included in the clinical criteria for this condition.

Basal adenosine 3',5'-cyclic monophosphate (cAMP) content and the modulation of its production were studied in the frog's semicircular canal epithelium. This epithelium secretes endolymph, a K(+)-rich, positively polarized fluid. The basal cAMP content measured by microradioimmunoassay was 244 +/- 14.2 fmol/structure per 5 min (n = 30). This content was increased about 8 times by 10(-5) M forskolin. Vasotocin, the frog antidiuretic hormone, increased the cAMP production by factors of 1.3 and 3.3 at concentrations of 10(-8) M and 10(-7) M, respectively. This stimulatory effect of vasotocin was blunted by the addition of alpha 2-adrenergic agonists, such as 10(-8) M-10(-5) M norepinephrine, in the presence of 10(-5) M propranolol, or 10(-5) M clonidine. Prostaglandin E2 at a concentration of 10(-8) M, which did not affect the cAMP production, did not modify the response to vasotocin. Glucagon (10(-6) M), calcitonin (10(-6) M), and parathyroid hormone (10 units/ml) did not affect the cAMP content. Prostaglandin E2 (10(-7) M) and the beta-adrenergic agonist isoproterenol (10(-6) M) stimulated the cAMP production by a factor of 1.6. These results indicate that the frog semicircular canal is a target of both vasotocin and catecholamines and that catecholamines through alpha 2-receptors modulate vasotocin-induced cAMP generation. Further, this interaction might be of physiological relevance in the modulation of ion transport in this structure.

The purposes of this study were to test the pharmacokinetics and renal and otologic tolerances of a once-daily regimen of netilmicin and teicoplanin administered intramuscularly, alone or in combination (4.5 and 6 mg/kg, respectively), for 7 days in 30 healthy male volunteers. Teicoplanin induced only a mild increase in enzymuria. Nephrotoxicity was moderate and reversible with netilmicin; there was increased enzymuria and alteration in diluting ability, without significant changes in urinary β2-microglobulin levels, concentrating ability, and glomerular filtration rate. Ototoxicity was not detected in any of the subjects. Our results suggest that (1) teicoplanin and netilmicin given once daily induced only slight, reversible tubular damage, without any sign of ototoxicity; (2) their combination was not more toxic; and (3) clinical studies can be envisaged to evaluate the efficacy and tolerance of once-daily regimens in long-term treatment. Clinical Pharmacology and Therapeutics (1988) 44, 458–466; doi:10.1038/clpt.1988.180