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Objective: To study the surgical results, intracochlear position of the electrode array (EA) and auditory performance of the LISTENT LCI-20PI cochlear implant device, and daily use status at 3 years., Study Design: A retrospective study., Setting: A single-tertiary referral center., Patients: Between January and December 2016, 20 patients underwent cochlear implantation using the LISTENT LCI-20PI (lateral wall EA)., Intervention: Cochlear implantation., Main Outcome Measures: Measurement of cochlear size, extent of posterior tympanotomy, and insertion depth. Scalar position of the EA evaluated by 3D reconstruction. Auditory outcomes 1 year after implantation and daily use status at 3 years., Results: EAs were completely inserted in all cases with an insertion depth of 288 ± 36.8 degrees. One year later, the average sentence recognition score (SRS) was 90 ± 21.7%. EA scalar location was analyzed in 18 patients. Thirteen EAs (72.2%) were fully inserted into the scala tympani (ST) and 5 (27.8%) had shifted from the ST to the scala vestibuli (SV). There was no statistically significant difference in cochlear size, extent of posterior tympanotomy, or insertion depth between these two groups. EAs inserted by cochleostomy had a higher chance of scalar shift than those inserted via the round window (60% vs 15.4%, p = 0.099). SRS at 1 year with full ST insertion was significantly better than in those with scalar shift (99 ± 1.3% vs 83 ± 16.5%, p = 0.002). Three years after implantation, 92% of patients were daily users and 46% were telephone users., Conclusions: The LISTENT LCI-20PI provided accredited hearing rehabilitation with a short insertion depth. Full insertion into the ST was associated with better cochlear implantation outcomes., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2020, Otology & Neurotology, Inc.)

Objective: To describe the first cochlear array insertions using a robot-assisted technique, with different types of straight or precurved electrode arrays, compared with arrays manually inserted into the cochlea., Study Design: Retrospective review., Setting: Tertiary otologic center., Patients: Twenty cochlear implantations in the robot-assisted group and 40 in the manually inserted group., Interventions: Cochlear implantations using a robot-assisted technique (RobOtol) with straight (eight Cochlear CI522/622, and eight Advanced Bionics Hifocus Slim J) or precurved (four Advanced Bionics Hifocus Mid-Scala) matched to manual cochlear implantations. Three-dimensional reconstruction images of the basilar membrane and the electrode array were obtained from pre- and postimplantation computed tomography., Main Outcome Measures: Rate and localization of scalar translocations., Results: For straight electrode arrays, scalar translocations occurred in 19% (3/16) of the robot-assisted group and 31% (10/32) of the manually inserted group. Considering the number of translocated electrodes, this was lower in the robot-assisted group (7%) than in the manually inserted group (16%) (p < 0.0001, χ2 test). For precurved electrode arrays, scalar translocations occurred in 50% (2/4) of the robot-assisted group and 38% (3/8) of the manually inserted group., Conclusion: This study showed a safe and reliable insertion of different electrode array types with a robot-assisted technique, with a less traumatic robotic insertion of straight electrode arrays when compared with manual insertion., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2020, Otology & Neurotology, Inc.)

Objective: Intravestibular schwannomas (IVS) are uncommon tumors in Neurofibromatosis type 2 (NF2) and are mainly associated with multiple internal auditory meatus (IAM) and cerebellopontine angle (CPA) tumors. They usually induce profound hearing loss which can be rehabilitated by cochlear implantation (CI). The aim of this study was to analyze the long-term outcomes of CI during the unpredictable evolution of NF2 disease., Study Design: Retrospective case review., Setting: Tertiary referral center., Patients: Three adults with neurofibromatosis type 2 and intravestibular schwannomas, and who were cochlear implant recipients., Interventions: Periodic radiologic follow-up, tumor resection, and hearing rehabilitation., Main Outcome Measures: Audiological evolution, tumor evolution, surgical outcome, cochlear implant outcome., Results: Three NF2 patients (mean age at diagnosis, 26.3 ± 3.2 yr) were identified with IVS in the period between 2000 and 2017. IVS were first observed by serial MRI and profound hearing loss occurred in this ear after 4 ± 1.5 years of follow-up. IVS were removed via a translabyrinthine approach, and ipsilateral cochlear implantations were simultaneously performed. In two patients, large contralateral CPA tumors had previously been removed without hearing preservation, whereas in the third patient, a small, growing contralateral VS was excised via a retrosigmoid approach 6 months after IVS removal/cochlear implantation with serviceable hearing preservation. In all cases, CI provided good hearing outcomes. In two cases, hearing outcomes were even better for more than 5 years when ipsilateral intracanalicular vestibular schwannomas were removed in either the same or subsequent procedures., Conclusions: Rehabilitation of hearing with CI provides a favorable long-term outcome in patients with NF2-related IVS which could be altered by the occurrence of other intracanalicular and/or CPA NF2-related tumors.

Hypothesis: An inappropriate insertion axis leads to intracochlear trauma during cochlear implantation (CI)., Background: Few studies assessed the relationship between the insertion axis and the electrode scalar location., Methods: Preimplantation cone-beam CT (CBCT) was performed on 12 human temporal bones. In five temporal bones, an optimal insertion axis was planned, due to the impossibility to attain the ST centerline from the posterior tympanotomy, because of facial canal position. In the seven other temporal bones, an inaccurate insertion axis was intentionally planned (optimal axis+15 degrees). Automated CI array insertion according to the planned axis was performed with a motorized insertion tool driven by a navigated robot-based arm. The cochlea and basilar membrane were segmented from the preimplantation CBCT and the array segmented from the postimplantation CBCT to construct a merged final three-dimensional (3D) model. Microscopical and 3D analysis were performed to determine the intracochlear trauma at the level of each electrode., Results: A good agreement was observed in determining electrode position between microscopic analysis and the 3D model (Cohen's kappa k = 0.67). The angle of approach to the ST centerline was associated with the number of electrodes inserted into the ST (r = -0.65, p = 0.02, [95% CI -0.90 to -0.11] Spearman's rank correlation)., Conclusion: A 3D reconstruction model was effective in determining the array position in the cochlea scalae. Our data indicate that the angle of approach to the ST centerline is a critical factor in intracochlear trauma. Additional studies should be conducted to assess the importance of the insertion axis with other array designs.

Objective: The relationship between hearing loss and cognitive function has already been established. The objective of our study was to determine whether the two short cognitive tests, COgnitive Disorders EXamination (CODEX) and Montreal Cognitive Assessment (MoCA), could be used in daily clinical practice to detect cognitive impairment, and its changes after cochlear implantation., Patients: Eighteen patients with severe to profound postlingual progressive hearing loss (mean age ± SEM: 64 ± 3.5 yr; range, 23-83 yr) were tested before, and 12 months after cochlear implantation, with adapted visual presentation of CODEX and MoCA tests. Auditory performance was tested under best-aided conditions in quiet and noise., Results: Twelve months after cochlear implantation, hearing performance had clearly improved (paired t tests, p < 0.05). In addition, among the eight patients who had an abnormal cognitive score before implantation, four improved their cognitive performance, and were thus considered to be normal. No major alteration of cognitive performance was observed in the 10 patients who had normal cognitive performance before implantation., Conclusion: CODEX and MoCA are rapid tests that could be considered to be relevant cognitive performance screening tests. They could be used in daily clinical practice to improve the multidisciplinary sensory-cognitive monitoring of the elderly population.

Aim of the Study: To review indications, anatomical results, and complications of cochlear implant (CI) surgery in adults for which middle ear and mastoid obliterations were performed., Patients and Methods: Thirty cases (26 patients, 4 bilaterally implanted) of 837 CI surgeries (3.5%) performed between January 2009 and December 2013 have been included in this retrospective study. The mean follow-up was 21 ± 18 months (mean ± SD, range 3-58). There were 11 males and 15 females. The mean age was 59 ± 19 years (range 35-82). All surgeries were performed with a single-stage technique including a canal wall down mastoidectomy with external auditory canal closure and mastoid obliteration with fat. A postoperative CT scan was performed in all cases., Results: Etiologies of hearing loss were mainly chronic otitis with or without cholesteatoma in 24 cases. Other etiologies were meningitis with cochlear ossification in one case, progressive hearing loss in two cases, enlarged vestibular aqueduct in one case, temporal bone fracture with CSF leak in one case, and congenital aural atresia in one case. Four of those 30 cases were revision CI surgery for electrode array misplacement (one case with cochlear ossification) or extrusion from an open cavity (one case) and recurrent cholesteatomas (two cases). All surgeries were uneventful and performed in a single stage. The electrode array was inserted in the basal turn (29 cases) or in the middle turn (one case) of the cochlea. No complications were observed. Two cases of postoperative abdominal hematoma were drained under local anesthesia. A major failure of the CI device occurred 5 months after surgery., Conclusion: CI with mastoid and middle ear obliteration is a safe and effective technique for selected cases of cochlear implantation. Mastoid obliteration prevents from recurrent disease and lowering the facial ridge allows more space to manage extensive cochlear ossification or malformation.

Hypothesis: Loading otoprotective drug into cochlear implant might change its mechanical properties, thus compromising atraumatic insertion. This study evaluated the effect of incorporation of dexamethasone (DXM) in the silicone of cochlear implant arrays on insertion forces., Background: Local administration of DXM with embedded array can potentially reduce inflammation and fibrosis after cochlear implantation procedure to improve hearing preservation and reduce long-term impedances., Methods: Four models of arrays have been tested: 0.5-mm distal diameter array (n = 5) used as a control, drug-free 0.4-mm distal diameter array (n = 5), 0.4-mm distal diameter array with 1% eluded DXM silicone (n = 5), and 0.4-mm distal diameter array with 10% eluded DXM silicone (n = 5). Via a motorized insertion bench, each array has been inserted into an artificial scala tympani model. The forces were recorded by a 6-axis force sensor. Each array was tested seven times for a total number of 140 insertions., Results: During the first 10-mm insertion, no difference between the four models was observed. From 10- to 24-mm insertion, the 0.5-mm distal diameter array presented higher insertion forces than the drug-free 0.4-mm distal diameter arrays, with or without DXM. Friction forces for drug-free 0.4-mm distal diameter array and 0.4-mm distal diameter DXM eluded arrays were similar on all insertion lengths., Conclusion: Incorporation of DXM in silicone for cochlear implant design does not change electrode array insertion forces. It does not raise the risk of trauma during array insertion, making it suitable for long-term in situ administration to the cochlea.

Objective: To analyze the difference between the endolymphatic sac tumors (ELSTs) in sporadic cases and in von Hippel-Lindau (VHL) disease., Study Design: Retrospective case review in a tertiary referral center., Patients and Methods: Fourteen cases of ELST, occurring since 1998, were reviewed. We analyzed the initial symptoms, characteristics of the tumor, treatment, sequelae, and follow-up for each group., Results: The ELSTs were sporadic in 6 cases and associated with VHL disease in 8 cases. The mean age at the time of the first surgery was 26 years (range, 12-41). All except two of the patients presented with a unilateral tumor. The initial symptoms were hearing loss (n = 9), tinnitus (n = 7), and/or vertigo (n = 5). Hearing loss was more prevalent in the sporadic cases. Preoperative arteriography was performed for 4 patients, with embolization performed for 1 patient. The size of the tumor was significantly larger in the sporadic cases (31.7 mm) than in the cases of VHL disease (19.3 mm). The surgical approach was more extensive in the sporadic cases. The surgeons found 2 types of tumors. Cystic tumors with massive bleeding invading the surrounding structures (the dura mater or jugular bulb) were more common in the sporadic cases. Fibrous tumors that infiltrate the bone and have moderate bleeding were more common in the cases associated with VHL disease.Two patients with small lesions were not operated on but were followed for 6 years without tumor growth. They died of metastasis from gastric and kidney cancer. Four recurrences occurred during the 14 years of follow-up. Four facial palsies and 8 cases of profound deafness were encountered postoperatively., Conclusion: Sporadic tumors are more aggressive than those associated with VHL disease. Complete surgical resection should be the goal of treatment. Preoperative angiography with embolization is recommended. In some cases, embolization may be impossible, and preoperative or postoperative radiotherapy should be discussed.

Hypothesis: The aim of the study was to evaluate force profiles during array insertion in human cochlea specimens and to evaluate a mechatronic inserter using a 1-axis force sensor., Background: Today, the surgical challenge in cochlear implantation is the preservation of the anatomic structures and the residual hearing. In routine practice, the electrode array is inserted manually with a limited sensitive feedback., Materials and Methods: Hifocus 1J electrode arrays were studied. The bench test comprised a mechatronic inserter combined to a 1-axis force sensor between the inserter and the base of the array and a 6-axis force sensor beneath the cochlea model. Influence of insertion tube material, speed (0.15, 0.5, and 1.5 mm/s) and lubricant on frictions forces were studied (no-load). Different models were subsequently evaluated: epoxy scala tympani model and temporal bones., Results: Frictions forces were lower in the plastic tube compared with those in the metal tube (0.09 ± 0.028 versus 0.14 ± 0.034 at 0.5 mm/s, p < 0.001) and with the use of hyaluronic acid gel. Speed did not influence frictions forces in our study. Insertion force profiles provided by the 1- and 6-axis force sensors were similar when friction forces inside the insertion tool (no-load measurements) were subtracted from the 1-axis sensor data in the epoxy and temporal bone models (mean error, 0.01 ± 0.001 N)., Conclusion: Using a sensor included in the inserter, we were able to measure array insertion forces. This tool can be potentially used to provide real-time information to the surgeon during the procedure.

Objective: This study aimed to evaluate the interaction of electric and acoustic cues in diotic condition in cochlear implantees., Materials and Methods: Five adult cochlear implantees with residual contralateral hearing were prospectively evaluated in hearing aid only (HA), cochlear implant only (CI), and HA + CI modes by audiometry (pure tone, dissyllabic words, and sentences), and sound quality questionnaires. CI electrodes corresponding to preserved frequencies in the contralateral ear (free-field aided thresholds, <50 dB) were then deactivated, and patients were retested after 20 to 30 days., Results: Sentences in silence showed a benefit of CI and the additive effect of HA + CI. As expected, performances with CI alone decreased after apical electrode deactivation. In contrast, speech performances (Marginal Benefit from Acoustic Amplification sentences) in HA + CI mode were not altered by electrode deactivation in silence (90 ± 5.9% before versus 81 ± 10.1% after deactivation, not significant, 2-way analysis of variance) or in noise (78 ± 4.8% before versus 66 ± 11.9% after deactivation, not significant, 2-way analysis of variance). Performances for dissyllabic words confirmed these results. Questionnaires showed a significant compensation of partial electrode deactivation by the contralateral hearing. Moreover, the human voice was reported to be significantly less metallic., Conclusion: These results suggested a significant complementarity of acoustic and electric diotic cues but also some redundancy affecting the sound quality.

Objective: To evaluate a continuous facial nerve (FN) stimulating burr (the StimBurGard) during otologic/neurotologic procedures in terms of safety and reliability when drilling in contact with the Fallopian canal (FC) of the mastoid segment of the FN., Study Design: Prospective clinical trial., Setting: Tertiary referral center., Patients: Thirty-five patients operated through translabyrinthine (TL) approach for vestibular schwannoma removal were divided into 3 groups. Group 1 (5 patients): the stimulation current was set at 3 and then at 2 mA visualizing the localization of the burr when the first response at 100-μV threshold was obtained in the mastoid cavity. Group 2 (15 patients): exposure of the FC in the mastoid segment during TL approach was stopped when the first response was obtained at 1-mA stimulation; FC thickness in the second genu and mastoid segment of the FC was evaluated on a postoperative computed tomographic (CT) scan, and FC dehiscence observed on CT scan was compared with surgical observation. Group 3 (15 patients), exposure of the FC was performed as routinely done during a TL approach and surgical observation of FC dehiscence; stimulation values in mA at the 100 μV threshold and FC thickness on postoperative CT scan were evaluated. In all cases, the stimulation value at the cerebellopontine angle root of the FN with a 100-μV response threshold was measured before tumor resection., Results: Group 1: stimulation at 3 mA occurred in aditus ad antrum and at 2 mA near the FC. Group 2: mean thickness of 1.09 ± 0.69 mm with 2 cases of radiologic dehiscence of the FN. Group 3: the stimulation threshold was 0.6 ± 0.37 mA, and the thickness was 0.41 ± 0.56 mm with 9 cases of uncovered FN (p = 0.0082). In all patients, FN at brainstem was stimulated at 0.03 mA before VS dissection., Conclusion: Continuous FN stimulating burr by means of the StimBurGard system is a safe and effective tool for FN stimulation and identification. The integrity of FC is preserved in most cases when the stimulation intensity is 1 mA.

Objective: To evaluate the predictive value of electromyographic recordings for the immediate facial nerve outcome after solitary vestibular schwannoma (VS) removal., Study Design: Prospective cohort study., Setting: Tertiary referral center., Patients and Methods: From November 2005 to July 2007, 120 operated solitary VSs monitored by NIM Response 2.0 (Medtronic, Xomed, Jacksonville, FL, USA) were included. Intraoperative direct facial nerve stimulation at the brainstem (proximal pre site) before resection and at the brainstem (proximal post and distal sites, respectively) and internal acoustic meatus after resection were performed. Intraoperative recordings were performed on 4 muscles: Frontalis, Orbicularis oculi, Orbicularis oris, and chin muscles (CM). Postoperative facial function (House-Brackmann classification) was evaluated at postoperative Days (D) 2, 8, and 30. Grades 1 or 2 were considered as a good result., Results: A good postoperative facial function was obtained in 71, 63, and 77% at postoperative D2, D8, and D30, respectively. Maximal conduction block was found at Day 8. Poor facial nerve outcomes were observed for large tumor with a facial nerve superior to the tumor with a strong adhesion to the nerve. With a stimulation threshold at proximal site after proximal site tumor resection at less than or equal to 0.04 mA, a good facial function was observed in 77% at Day 8. Using these criteria together with CM responses greater than 800 muV to supramaximal stimulation at (proximal post site) and a proximal/distal post ratio at CM response greater than 0.6, 93% of patients presented with a good facial function at Day 8., Conclusion: Using CM recording stimulation and supramaximal stimulation of the facial nerve in combination with thresholds seem to increase the predictive value of the monitoring for the immediate facial nerve function for VS surgery.

Objective: The aim of this study was to investigate the clinical aspects of balance disorders and to assess the audiovestibular functions of patients with otosclerosis., Materials and Methods: Among 13,800 patients examined for balance disorders between 2002 and 2006, 98 (0.7%) presented with otosclerosis. Seventy-three patients (28 treated nonoperatively, 27 operated on unilaterally, and 18 operated on bilaterally for otosclerosis) with an audiovestibular assessment were included in this retrospective study., Results: Complaints were dizziness in 42 patients (56%), benign paroxysmal positional vertigo in 37 patients (51%), and rotatory vertigo in 21 patients (29%), with no difference between patients operated on and those treated nonoperatively. In patients treated nonoperatively, despite the absence of correlation for hearing loss between the right and left ears, a strong correlation was observed for the bithermic caloric response (BCR; Y = 2.54 + 0.73X, Y = left, X = right, R2 = 0.71, n = 26, p < 0.0001, analysis of variance). In contrast, the correlation between the ipsilateral and contralateral ears in BCR was weak in patients operated on 1 side (Y = 15.1 + 0.66X, Y = contralateral, X = ipsilateral, n = 27, R2 = 0.20, p < 0.05), and this was associated to a caloric paresis suggesting a surgery-induced deficit. There was no correlation between hearing thresholds and the BCR., Conclusion: The proportion of patients with otosclerosis among those with balance disorders was similar to the estimated prevalence of otosclerosis in the general population. Despite the asymmetric hearing loss, BCR was symmetrical in patients treated nonoperatively. In contrast, the loss of vestibular function symmetry in patients suggested a surgery-induced vestibular trauma.

Objective: To assess the nerve facial displacement by a vestibular schwannoma and nerve adhesion to tumor as predictive factors of facial function outcome., Study Design: A prospective cohort study., Setting: Tertiary referral center., Patients: Ninety-six patients undergoing a solitary vestibular schwannoma surgery during 2005 were included. Data concerning tumor size (Stage 1, intracanalicular; Stage 2, < or =15 mm in the cerebellopontine angle [CPA]; Stage 3, 15-30 mm in the CPA; and Stage 4, >30 mm in the CPA), intraoperative facial nerve displacement (Type 1, anterior to the tumor; Type 2, anterior and superior to the tumor and separated from the cochlear nerve; Type 3, superior to the tumor; and Type 4, posterior to the tumor), degree of tumor adhesion (weak, intermediate, and strong) and postoperative facial function according to the House and Brackmann classification at days 10, 30, 90 and 180 were collected., Results: A good facial function (Grade 1 or 2) was reported in 73% at postoperative Day 180. Univariate analysis showed that facial outcome was better in small tumors, in displacement Types 1 and 2 (Type 1, 46%; Type 2, 34%; and Type 3, 20%), and in tumors with weak and intermediate adhesion (weak, 10%; intermediate, 38%; and strong, 52%). Facial nerve displacement and adhesion were related to tumor stage. The combination of tumor stage, adhesion, and nerve displacement in a logistic regression model was highly predictive of postoperative facial function., Conclusion: Facial nerve displacement and nerve adhesion to tumor are significant predictive factors of facial function outcome after vestibular schwannoma surgery in addition to tumor size.

Objective: To evaluate the auditory brainstem implant (ABI) performances in neurofibromatosis type 2 (NF2) and non-NF2 patients., Study Design: Retrospective cohort study., Setting: Tertiary referral center., Patients: Between 1996 and 2006, 31 adult patients (mean age, 41 yr; range, 17-65 yr) were implanted with a 21-electrode Nucleus device (Cochlear Inc., Lane Cove, Australia). The population comprised 23 NF2, 3 postmeningitis bilateral ossified cochleas, 3 solitary vestibular schwannomas on the only hearing ear, 1 inner ear malformation, and 1 bilateral cochlear destruction by otosclerosis., Intervention: Auditory brainstem implant was placed through a translabyrinthine or a retrosigmoid approach., Main Outcome Measures: Auditory brainstem implant was evaluated by open-set words and sentences in sound, vision, and sound-plus-vision modes., Results: In NF2 patients, 16 (70%) were daily users of their implants. In these patients, the open-set dissyllabic word recognition was 36 +/- 6.0 % for vision-only mode, 33 +/- 6.5 % for sound-only mode, and 65 +/- 8.0 % in vision-plus-sound mode with a high interindividual variation. Negative prognostic factors were long duration of total hearing loss (>10 yr), low number of active electrodes (<10), and local complications (meningitis, hematoma). Six non-NF2 patients (75%) were daily ABI users. The performances of patients with ossified cochleas were similar to best NF2 cases., Conclusion: A clear benefit of ABI could be evidenced in NF2 patients, especially in case of small tumor and short duration of hearing loss. Auditory brainstem implant may also be indicated in patients with bilateral profound hearing loss and a predictable failure of cochlear implantation.

Objective: To evaluate the functional results of otosclerosis surgery using diode laser., Study Design: Retrospective cohort analysis., Patients: One hundred seven patients operated on for otosclerosis with a diode laser (119 ears, all primary cases) and 141 patients operated on with a conventional technique (141 ears, all primary cases). Revision cases using the diode laser were also described., Methods: Preoperative tomographic computed scan findings and intraoperative observations were collected. Pure-tone and vocal audiometry was performed preoperatively and postoperatively (at 3 mo and 1 yr)., Results: In the laser group, the air-bone gap was 29 +/- 0.8 dB (n= 112) preoperatively and 9 +/- 0.6 dB (n = 58) at 1 year. Air conduction was improved by 22 +/- 1.7 dB at 1 year (n = 58). In the conventional group, the air-bone gap was 32 +/- 0.9 dB (n=127) preoperatively and 10 +/- 0.6 dB (n = 127) at 1 year. Air conduction was improved by 25 +/- 1.1 dB (n = 127) at 1 year. No difference of hearing gain was observed between the 2 groups at 1 year. A decreased rate of footplate fracture was observed with the diode laser (3.6%) compared with the conventional technique (21.3%)., Conclusion: Diode laser is a reliable and safe device for otosclerosis surgery. The functional results were similar to those reported in other series.

Objective/hypothesis: Angiotensin II (Ang II) may be implicated in the regulation of bone remodeling, and its activity is related to several gene polymorphisms including AGT M235T for plasmatic and tissular concentrations of angiotensinogen (AGT), ACE I/D for the angiotensin-converting enzyme activity, and AT(1)R A/C(1166) for the Ang II receptor function. The objective of this study was to investigate the implication of this hormone in otosclerosis., Study Design: Prospective case-control study., Methods: The above-mentionedpolymorphisms were investigated in 186 patients with otosclerosis and 526 healthy controls, both groups originated from the French Caucasian population. Primary cell cultures of stapedial bone from patients with otosclerosis (n = 6) and control subjects (n = 5) were investigated for the messenger ribonucleic acid expressions of Ang II receptors (Types 1 and 2) and cellular AGT and the effect of Ang II (10(-7) mol/L, 24 h) on the alkaline phosphatase activity and the interleukin-6 secretion in the culture media., Results: A significant association was found between otosclerosis and the AGT M235T and the ACE I/D polymorphisms. Higher proportions of TT (29% versus 16%; p < 0.01) and DD (50% versus 38%; p < 0.05) genotypes were observed in cases versus controls. No association was found between the AT(1)R A/C(1166) polymorphism and otosclerosis. Ang II receptor Types 1 and 2 and AGT were detected in the cultures. Ang II increased the in vitro secretion of interleukin-6 and decreased the alkaline phosphatase activity only in otosclerotic cells., Conclusion: These observations suggest a relation between the local renin angiotensin system activity and otosclerosis, opening new therapeutic insights.

Objective: To describe and analyze a series of intracochlear schwannomas., Methods: From 1987 to 2005, 19 patients with schwannomas involving the cochlea were included in this series. Clinical, audiovestibular, and imaging data concerning initial and follow-up visits were collected., Results: The mean age was 54 years (range, 25-71 yr). The series comprised 10 women and 9 men, 18 solitary tumors and 1 neurofibromatosis Type 2 patient. The mean diagnosis delay was 11 years (range, 1-31 yr). At diagnosis, a total or profound hearing loss (Class D of American Academy of Otolaryngology-Head and Neck Surgery classification) was noted in 17 patients (89%), and a severe loss (Class C) was reported in 2 patients (11%). Facial paresis was reported in 2 patients (11%; Grades 2 and 4 of House and Brackmann classification). Magnetic resonance imaging showed an involvement of the posterior labyrinth in 8 patients (37%), an extension of the schwannoma to the internal auditory meatus in 8 patients (37%), and a cerebellopontine angle extension in 7 patients (32%). In 11 patients (58%), the schwannoma was removed through a transotic route. In the 8 remaining patients, a watch-and-rescan policy was decided. The postoperative course was uneventful. Postoperative follow-up period was 27 months (range, 6-88 mo). Postoperative facial function was assessed as Grade 1 in 7 patients, as Grade 2 in 4, and as Grade 4 in 1 patient., Conclusion: The diagnosis of intracochlear schwannomas can be difficult on magnetic resonance imaging. The diagnosis should be considered in all unilateral hearing losses.

Objective: To assess the conservation of residual hearing in recipients of the Nucleus 24 Contour Advance cochlear implant (CI) and the benefits of combined electrical and acoustic stimulation., Study Design: Prospective multicenter study., Setting: CI clinics in Western Europe., Patients: Adult candidates for conventional cochlear implantation with a minimum preoperative word recognition score of 10% in the ear to be implanted., Intervention: "Soft-surgery" protocol, including a 1- to 1.2-mm anterior and inferior cochleostomy hole with the electrode array, inserted 17 mm using the "advance-off-stylet" technique. Patients with postoperative pure-tone hearing threshold levels (HTLs) of 80 dB hearing loss or less at 125 and 250 Hz and 90 dB hearing loss or less at 500 Hz were refitted with an in-the-ear hearing aid for combined ipsilateral electrical and acoustic (El-Ac) stimulation., Main Outcome Measures: A questionnaire to collect information regarding surgery. Pure-tone HTLs measured at intervals. Word recognition tested in quiet and sentence recognition tested in noise at 10 and 5 dB signal-to-noise ratio (SNR). HEARING CONSERVATION RESULTS: HTL data were available for 27 patients. HTLs were conserved within 20 dB of preoperative levels for 33, 26, and 19% of patients for 125, 250, and 500 Hz, respectively. However, the recommended soft-surgery protocol was strictly followed in only 12 of 27 patients. For these 12 patients, hearing thresholds were conserved within 20 dB for 50, 50, and 33% of patients. Median threshold increases were 40 dB (range, 250-500 Hz) for the whole group and 23 dB for the strict surgery group. Ten patients retained sufficient HTLs to enter the El-Ac user group. SPEECH RECOGNITION RESULTS: Group mean recognition scores for nine El-Ac users for words presented at 65 dB sound pressure level were 45% for CI alone and 55% for CI + ipsilateral hearing aid (p < 0.05, paired t). For sentences presented in noise at 5 dB SNR, mean word scores were 46% CI alone and 56% CI + ipsilateral hearing aid (p < 0.01, paired t)., Conclusion: Hearing was conserved for conventional candidates for cochlear implantation where the recommended soft-surgery protocol was strictly adhered. Combined ipsilateral electrical and acoustic stimulation provided considerable benefits for speech recognition in noise, equivalent to between 3 and 5 dB SNR, compared with CI alone.

Objective: To report the first case of spontaneous intratumoral and brainstem hemorrhage in a patient with a vestibular schwannoma., Study Design: Case report and review of the literature., Setting: University-based, tertiary referral center., Patient: A 73-year-old woman with a vestibular schwannoma, anti-vitamin K overdose, and arterial hypertension experienced a rapid onset of headache, facial palsy, diplopia, and hoarseness., Interventions: The patient was admitted to the intensive care unit and treated with vitamin K and corticoids., Main Outcome Measures: Clinical and radiologic findings., Results: At admission, the magnetic resonance imaging and computed tomographic scans showed an intratumoral hemorrhage. The patient entered a coma 24 hours after the onset of the symptoms. At that time, the computed tomographic scan demonstrated a massive intratumoral and brainstem hemorrhage. The patient died 3 days later., Conclusion: Intratumoral, subarachnoid, and brainstem hemorrhages can occur in patients with vestibular schwannoma. Oral anticoagulant therapy is a risk factor for tumor-related hemorrhage.

Objective: To evaluate the short-term facial prognostic value of a four-channel facial electromyographic device in vestibular schwannoma surgery., Study Design: Eighty-nine vestibular schwannomas operated on and intraoperatively monitored by a four-channel facial electromyographic device between October 2002 and September 2003 were included in this prospective study. Detection was performed in frontal, orbicularis oculi, orbicularis oris, and platysma muscles., Main Outcome Measure: Facial function grading at postoperative Days 1, 8, 30, and 180 (House-Brackmann classification)., Setting: Tertiary referral center., Results: Postoperative facial function at Day 180 was assessed as Grade 1 or 2 in 80%, as Grade 3 or 4 in 16%, and as Grade 5 or 6 in 4% (n=80). The postoperative facial function was related to the intraoperative nerve stimuli thresholds (range, 0.01-3 mA for a response >100 microV) near the brainstem and the proximal-to-distal ratio of the stimulation threshold. A proximal threshold between 0.01 and 0.04 mA had a positive predictive value of 94% for good facial function (Grade 1 or 2). The proximal threshold was lower in patients with improving or stable facial function in comparison with those with a delayed deterioration between Days 8 and 30. The stimulation threshold at the adhesion zone was related to the immediate facial function outcome. The maximal electromyographic response was detected in the frontal muscle or the platysma in 27% of cases and in orbicularis oris and oculi in 73% of cases., Conclusion: A four-channel device may enhance electromyographic sensitivity. Determination of stimulation threshold below 0.05 mA yields facial prognostic information.

Objective: To assess the value of cerebrospinal fluid pressure as a decisional factor for immediate surgical revision in cerebrospinal fluid leakage after acoustic neuroma removal., Study Design: Prospective study., Setting: Tertiary referral center., Patients: Between 1998 and 2001, 220 patients were operated on for acoustic neuroma by different transpetrosal approaches. Among 24 patients (12%) presenting postoperative cerebrospinal fluid leakage, those with meningitis or with hydrocephalus were excluded. Fifteen patients were included in this study., Methods: Each patient had initial conservative treatment with serial depletive lumbar punctures and cerebrospinal fluid pressure measurements associated with oral acetazolamide. Surgical revision was decided on in case of persistent cerebrospinal fluid leakage., Results: In eight patients with high cerebrospinal fluid pressure (18+/-1.4 cm H2O; range, 14-28 cm H2O), cerebrospinal fluid leak disappeared in 3 days after conservative treatment. Seven other patients required surgical revision for persistent cerebrospinal fluid leakage. Revision surgery was efficient in six patients with low cerebrospinal fluid pressure (8+/-1.3 cm H2O, range, 3-12 cm H2O). In the remaining patient with high cerebrospinal fluid pressure (18 cm H2O), cerebrospinal fluid leakage continued despite surgical revision, requiring lumboperitoneal shunting., Conclusion: The cerebrospinal fluid pressure value may be used as a decisional indicator for cerebrospinal fluid leakage treatment after acoustic neuroma surgery. Low cerebrospinal fluid pressure leakage would imply a revision surgery procedure without delay, whereas high cerebrospinal fluid pressure leakage would imply conservative treatment.

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

Hypothesis: This study investigates the function of the diastrophic dysplasia sulfate transporter (DTDST) in otosclerotic bone and the effect on it of sodium fluoride (NaF)., Background: Otosclerosis is a localized bone dystrophy with increased bone turnover. DTDST is implicated in the regulation of the bone turnover., Materials and Methods: Primary cultures of cells were obtained from the stapes and external auditory canal (EAC) of 26 patients with otosclerosis and from nine control patients. Sulfate uptake was quantified under basal conditions and with NaF. The NaF signaling pathways were investigated using forskolin and verapamil., Results: The relative initial rates of sulfate uptake and the apparent Vmax values were: otosclerotic stapes > EAC > control stapes = control EAC. The sulfate uptake by the otosclerotic stapes was correlated with the loss of sensorineural hearing. The amounts of DTDST mRNA (RNase protection assay) in the four subgroups did not differ. NaF (10(-6)M, 1 hr) inhibited sulfate uptake by the otosclerotic stapes and EAC cells but not by control samples., Conclusion: The authors believe that whether the increased DTDST activity is a cause or an effect of otosclerosis, it appears to be a specific target for NaF treatment.

Objective: To evaluate the results of partial and total ossicular reconstruction prostheses entirely made of hydroxylapatite., Study Design: A retrospective review of cases followed-up between 1997 and 2000., Setting: Tertiary referral center., Patients: One hundred adult patients (60 men and 40 women) were studied. The mean age was 55 years (range, 25 to 65 years). Fifty patients had a previous tympanoplasty. The series included 45 cholesteatomas (45%), 37 chronic otitis media without cholesteatoma (37%), 13 retraction pockets (13%), and 5 posttraumatic ossicular displacements (5%)., Intervention: A one-stage ossiculoplasty with a partial ossicular reconstruction prosthesis (n = 65) or a total ossicular reconstruction prosthesis (n = 35) was performed. This was associated with a simple mastoidectomy in 70 cases and a radical mastoidectomy in 10 cases., Main Outcome Measures: All patients were followed-up at 1 year, and 50 were reexamined 2 years after surgery. Clinical and audiometric data were collected in a database at each visit., Results: A prosthetic extrusion was noted in two cases (2%). A prosthetic displacement occurred in 10 cases (10%). A revision surgery was carried out in 10 cases because of an ossiculoplasty failure or a disease recurrence. At 1 year, the residual air-bone gap was lower in cases with a partial ossicular reconstruction prosthesis than those with a total ossicular reconstruction prosthesis (16 +/- 9.3 versus 23 +/- 13.7 dB, p < 0.05). The gain in mean air conduction threshold was 21 +/- 12.8 dB in cases of partial ossicular reconstruction prosthesis and 19 +/- 11.3 dB in cases of total ossicular reconstruction prosthesis (not significant). Revision surgery, canal wall-down mastoidectomy, or cholesteatoma yielded poorer functional results., Conclusion: Partial ossicular reconstruction prosthesis and total ossicular reconstruction prosthesis entirely made of dense hydroxylapatite yielded high rates of air-bone gap reduction and high anatomic stability.

Objective: The Vibrant Soundbridge is an active semi-implantable middle ear implant for the rehabilitation of patients with a sensorineural hearing loss who are not able to derive adequate benefit from conventional hearing aids. A retrospective study was performed to assess the overall level of satisfaction of implanted patients and to investigate the potential determinants of postoperative success., Study Design: A retrospective survey of audiological data from repeated measures and subjective data from self-assessment scales administered postoperatively was conducted to determine the degree of benefit and satisfaction for Vibrant Soundbridge implantees., Setting: Twenty-one tertiary referral and teaching hospitals., Subjects: The first 125 VSB implantees implanted in France between August 1997 and May 2001 were included in the study., Results: No clinically significant change was observed for residual hearing postoperatively. Most patients (83%) reported they were either satisfied or very satisfied with the Vibrant Soundbridge. Analysis of correlation revealed a moderate correlation (Pearson coefficient r = 0.59) between the degree of benefit reported via the patient survey and the degree of benefit reported via the Glasgow Benefit Inventory. A moderate correlation (Pearson coefficient r = 0.66) was observed between speech comprehension scores in quiet for the preoperative unaided condition and the postoperative aided Vibrant Soundbridge condition. No correlation was observed between subjective reports of satisfaction postoperatively and performance on preoperative objective tests or patient characteristics., Conclusion: The results indicate a high level of satisfaction with the VSB as a treatment of sensorineural hearing impairment in patients with a wide range of characteristics. Preoperative scores for unaided speech comprehension tests in quiet may be a potential indicator of success on aided Vibrant Soundbridge speech comprehension tests postoperatively but do not reflect patient satisfaction with the device reported on self-assessment scales.

Objective: To report and evaluate the results of auditory brainstem implantation in a case of postmeningitis hearing loss with totally ossified cochleae on both sides., Study Design: Case report., Setting: Tertiary referral center., Patient: A 49-year-old man was referred to the authors' department for bilateral total hearing loss subsequent to bacterial meningitis 2 years earlier. Pure tone audiometry, auditory brainstem response tests, and promontory tests did not reveal any cochlear activity. Computed tomography showed bilateral and totally ossified cochleae. Magnetic resonance imaging confirmed the diagnosis by showing no cochlear signal on T2-weighed images and ruled out brainstem and cerebellopontine angle abnormalities., Intervention: A left auditory brainstem implantation was performed through a translabyrinthine route, using a Nucleus 22-channel device., Main Outcome Measures: Word and sentence recognition tests in sound-only and sound plus vision modes., Results: No postoperative complication was observed. Twelve electrodes could be activated, and their tonotopy was defined. Nine other electrodes were inactivated because of an absence of auditory response (4 electrodes) or paresthesia (5 electrodes). At the last follow-up visit, 26 months after the implantation, 50% of disyllabic word scores and 60% of sentence scores were achieved using auditory brainstem implant sound only. These scores reached 80% and 93%, respectively, with lip-reading., Conclusion: Auditory brainstem implantation is an efficient means of auditory rehabilitation in cases of bilateral total hearing loss with totally ossified cochleae. It should be considered in cases of predictable failure in cochlear implantation.

Objective: The purpose of this study was to analyze the imaging characteristics of 12 cholesterol granulomas as a function of their clinical symptoms. The results of the different surgical approaches and the management of these lesions are discussed., Study Design: Retrospective case review., Setting: Five tertiary referral centers., Patients: Twelve patients managed for a cholesterol granuloma of the petrous apex., Interventions: All patients were evaluated via computed tomography and magnetic resonance imaging. Eight patients required surgical drainage: through a conservative approach in seven patients (infralabyrinthine, n = 5; infracochlear, n = 2) and a transotic approach in one patient. Clinical and radiologic follow-up without surgery was the mode of treatment for four patients. The mean follow-up period was 18 months for patients who underwent operations and ranged from 6 months to 10 years for patients without operations., Results: Four patterns of clinical symptoms were noted: retrocochlear signs by an involvement of the internal auditory meatus (n = 8), headaches by a traction of the dura (n = 4), serous otitis media by a compression of the eustachian tube (n = 2), and asymptomatic lesions with no involvement of the adjacent structures (n = 2). Hearing and facial functions were preserved in all the cases treated by a noninvasive procedure. No recurrence or complication was reported in the patients who underwent operations. None of the noninvasively treated patients with cholesterol granulomas showed significant enlargement on follow-up imaging., Conclusion: Clinical manifestations of cholesterol granulomas depend on their anatomic location and the involvement of the adjacent structures. Aggressive lesions in patients with residual hearing can be drained via an infralabyrinthine or an infracochlear approach with minimal morbidity. Follow-up must be preferred for patients with nonaggressive lesions. Although magnetic resonance imaging provides a specific diagnosis tool for cholesterol granulomas, computed tomography is essential for an accurate evaluation of the location of the cyst and choice of the surgical procedure.

Objective: To assess predictive factors of long-term facial nerve function in a series of patients undergoing vestibular schwannoma surgery and to evaluate the reproducibility of the relevant parameters., Study Design: Prospective., Setting: Three tertiary referral neurotology units in two separate countries., Patients: A total of 67 patients, with normal preoperative facial function and an anatomically intact facial nerve postoperatively, undergoing vestibular schwannoma surgery during a sequential 18-month period., Interventions: Recording of intraoperative stimulus amplitudes (minimum intensity medial to the tumor after excision) and postoperative facial nerve function up to 2 years after surgery., Main Outcome Measures: Long-term facial nerve function related to tumor size, early postoperative facial nerve function, and intraoperative electrophysiologic intensities., Results: Multivariate logistic regression model identified tumor size and the minimum intensity required to provoke a stimulus threshold event medial to the tumor after excision as independent predictors of a favorable initial outcome. Immediate facial nerve function was the only independent predictor of long-term normal function. The sensitivity of this predictor was 95% (95% confidence interval [CI], 89-100%); specificity, 83% (95% CI, 62-100%); positive predictive accuracy, 96% (95% CI, 91-100%); and negative predictive accuracy, 77% (95% CI, 54-100%)., Conclusion: The combination of electrophysiologic intensities and tumor size are reproducible and better predictors of initial facial nerve function than any individual parameter, but long-term facial nerve function is more likely to have a better outcome if the nerve is left intact and a per-operative graft repair is not performed. The study suggests that although the best available predictor of overall long-term facial nerve outcome is the level of early postoperative function, this factor is not useful in surgical rehabilitation decision making.

Objective: The Vibrant Soundbridge (VBS; Symphonix Devices, Inc., San Jose, CA, U.S.A.) is an active, semi-implantable, middle ear hearing device that directly drives the ossicular chain and is used in the treatment of patients with mild to severe sensorineural hearing loss. The benefits of the VBS and the effects of surgery were examined and compared with the preoperative aided condition in 25 patients with implants., Study Design: Single-subject repeated-measures evaluations were performed with each patient acting as his or her own control. Objective audiologic measures and subjective questionnaires also were used., Setting: Five tertiary referral and teaching hospitals., Subjects: Adult patients had bilateral sensorineural hearing loss (average hearing loss, 56 dB; range, 33-80 dB). Twenty-one patients had worn a conventional hearing aid before surgery (11 binaurally, 10 monaurally). Four patients had not used a conventional hearing aid before surgery., Intervention: Rehabilitative., Results: No significant change in residual hearing after surgery was observed. Functional gain was significantly superior with the VBS. No significant differences were observed for aided speech recognition in quiet. A significant improvement in communication in various listening conditions was reported with the VBS as compared with conventional hearing aids., Conclusion: The VBS surgical implantation procedure does not affect the residual hearing level in the implanted ear, nor does it present any unacceptable risk. Measurable benefit from the VBS in comparison with conventional amplification was demonstrated with regard to the provision of superior usable amplification and greater ease in communication in daily listening environments for the majority of patients.