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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

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.

To analyze the difference between the endolymphatic sac tumors (ELSTs) in sporadic cases and in von Hippel-Lindau (VHL) disease.Retrospective case review in a tertiary referral center.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.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.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.

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 μV 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 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 (

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: 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.

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.Prospective multicenter study.CI clinics in Western Europe.Adult candidates for conventional cochlear implantation with a minimum preoperative word recognition score of 10% in the ear to be implanted."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.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 (p0.05, paired t). For sentences presented in noise at 5 dB SNR, mean word scores were 46% CI alone and 56% CI + ipsilateral hearing aid (p0.01, paired t).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 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.

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.

To evaluate the results of partial and total ossicular reconstruction prostheses entirely made of hydroxylapatite.A retrospective review of cases followed-up between 1997 and 2000.Tertiary referral center.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%).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.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.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, p0.05). 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.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 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.

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.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.Five tertiary referral and teaching hospitals.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.Rehabilitative.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.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.