Improved Recovery after Vestibular Schwannoma Excision with Intratympanic Gentamicin Prehabilitation.

OBJECTIVE: Translabyrinthine excision of a vestibular schwannoma is associated with acute vestibular failure. Preoperative intratympanic gentamicin (ITG) injections can improve objective balance function after surgery but its clinical benefits remain to be established. METHODS: Adult patients undergoing translabyrinthine removal of a vestibular schwannoma between January 2014 and February 2018 underwent preoperative vestibular function testing. Patients were divided in to 3 groups, those with vestibular function (VF) who received ITG injections, those with VF but did not receive ITG and those with no VF. Groups were compared according to degree of vertigo, length of stay, time to unassisted mobilization, and postoperative anti-emetic consumption. RESULTS: Forty six patients had ITG injections (Group 1), 7 had residual VF but refused treatment (Group 2), 21 had no VF (Group 3). Group 1 had a significant improvement in vertigo over time whereas groups 2 and 3 did not. There was a statistically significant 70% decrease in time to independent mobilization between Group 1 and other groups and a 19% decrease in length of stay in Group 1 compared to other groups although this did not reach statistical significance. Two patients had injection-related complications. Group 1 used less anti-emetics than other groups but this was not statistically significant. CONCLUSION: Preoperative intratympanic gentamicin injection with vestibular rehabilitation exercises is associated with less postoperative vertigo and earlier postoperative mobilization. There was reduced duration of hospitalization and decreased consumption of anti-emetic but not significantly so possibly because of low numbers of patients in the no treatment group. LEVEL OF EVIDENCE: 2 Laryngoscope, 134:3316-3322, 2024.

Hearing Rehabilitation in Patients With Neurofibromatosis Type 2: The Quebec's Experience With Auditory Implants.

OBJECTIVE: To review a single-center experience with hearing rehabilitation in patients with neurofibromatosis type 2 (NF2) and to describe the auditory outcomes of cochlear implants (CIs) and auditory brainstem implants (ABI) in this population. STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral center. PATIENTS: Twelve adult patients with NF2 who received a CI (n = 10) and/or an ABI (n = 5) between 2000 and 2021. INTERVENTIONS: Insertion of a CI and/or an ABI in NF2 patients with bilateral vestibular schwannomas (VSs). MAIN OUTCOME MEASURES: Patients' demographic data, treatment history, hearing evolution, hearing rehabilitation methods, implant details, and auditory outcomes after implantation. RESULTS: Among those who received a CI, five patients had a stable untreated VS, one patient underwent a cochlear nerve preserving surgery, and four patients received radiotherapy treatments. Six patients became regular users of their device. The median open-set sentence recognition scores were as follows: 0.5% preoperatively, 60% at 1 year postoperatively, and 80% on the most recent audiological evaluation. All patients with an ABI were implanted concomitantly with VS surgical excision, and three of them also received radiotherapy treatments. The median open-set sentence recognition scores were as follows: 4% preoperatively, 26% at 1 year postoperatively, and 0% on the most recent evaluation. Three patients became regular ABI users. CONCLUSION: Despite major technological advances, auditory outcomes with ABIs remain deceiving. Considering the overall improvement in postoperative auditory performances provided by CIs compared with ABIs, cochlear implantation standouts as a primary mean of hearing rehabilitation in NF2 patients.

Transtympanic Visualization of Cochlear Implant Placement With Optical Coherence Tomography: A Pilot Study.

OBJECTIVE: This study aimed to evaluate the ability of transtympanic middle ear optical coherence tomography (ME-OCT) to assess placement of cochlear implants (CIs) in situ. PATIENT: A 72-year-old man with bilateral progressive heredodegenerative sensorineural hearing loss due to work-related noise exposure received a CI with a slim modiolar electrode for his right ear 3 months before his scheduled checkup. INTERVENTION: A custom-built swept source ME-OCT system (λo = 1550 nm, ∆λ = 40 nm) designed for transtympanic middle ear imaging was used to capture a series of two- and three-dimensional images of the patient's CI in situ. Separately, transtympanic OCT two-dimensional video imaging and three-dimensional imaging were used to visualize insertion and removal of a CI with a slim modiolar electrode in a human cadaveric temporal bone through a posterior tympanotomy. MAIN OUTCOME MEASURE: Images and video were analyzed qualitatively to determine the visibility of implant features under ME-OCT imaging and quantitatively to determine insertion depth of the CI. RESULTS: After implantation, the CI electrode could be readily visualized in the round window niche under transtympanic ME-OCT in both the patient and the temporal bone. In both cases, characteristic design features of the slim modiolar electrode allowed us to quantify the insertion depth from our images. CONCLUSIONS: ME-OCT could potentially be used in a clinic as a noninvasive, nonionizing means to confirm implant placement. This study shows that features of the CI electrode visible under ME-OCT can be used to quantify insertion depth in the postoperative ear.

The remaining obstacles for a totally implantable cochlear implant.

PURPOSE OF THE REVIEW: For years, the development of a totally implantable cochlear implant (TICI) has faced several technical challenges hindering any prototypes from reaching full commercialization. This article aims to review the necessary specifications for a viable TICI. An overview of the remaining challenges when designing TICIs will be provided, focusing on energy supply and implantable microphones. RECENT FINDINGS: The literature review highlights how research efforts to generate sufficient power to supply a fully implantable CI could take advantage of microelectromechanical systems (MEMS)-based energy harvesters incorporating piezoelectric materials. Using one of the various energy sources in the vicinity of the temporal bone would allow the development of a self-sufficient implant, overcoming the limitations of electrochemical batteries. Middle ear implantable microphones could also use similar fabrication techniques and transduction mechanisms to meet the sensor requirements for a TICI. SUMMARY: Recent breakthroughs in power supply using MEMS-based energy harvesting technologies and piezoelectric implantable microphones may make TICIs become a more practical reality in the foreseeable future. Once available, TICIs will have major impact on our patients' quality of life and may help to make hearing rehabilitation a more appealing option to a greater proportion of those who fulfill our candidacy criteria.

Cochlear Implantation in Neurofibromatosis Type 2: Experience From the UK Neurofibromatosis Type 2 Service.

OBJECTIVE: To review the outcomes of cochlear implants (CI) in patients with neurofibromatosis type 2 (NF2) in a large cohort, and identify factors associated with poor hearing benefit. STUDY DESIGN: Fifteen-year retrospective national observational case series. SETTING: United Kingdom regional NF2 multidisciplinary teams. PATIENTS: Consecutive patients with NF2 receiving a CI. INTERVENTIONS: CI for hearing rehabilitation. MAIN OUTCOME MEASURES: 1) Audiometric performance at 9 to 12 months after implantation using City University of New York (CUNY) sentence recognition score, and Bamford- Kowal-Bench (BKB) word recognition score in quiet (BKBq), and in noise (BKBn). 2) CI use at most recent review. RESULTS: Sixty four consecutive patients, median age 43 years, were included. Nine to 12 months mean audiometric scores were: CUNY 60.9%, BKBq 45.8%, BKBn 41.6%. There was no difference in audiometric outcomes between VS treatment modalities. At most recent review (median 3.6 years from implantation), 84.9% with device in situ/available data were full or part-time users. Between 9 and 12 months and most recent review there was an interval reduction in mean audiometric scores: CUNY -12.9%, BKBq -3.3%, BKBn -4.9%. Larger tumor size and shorter duration of profound hearing loss were the only variables associated with poorer audiometric scores. Tumor growth at the time of surgery was the only variable associated with CI non-use. Individual patient response was highly variable. CONCLUSIONS: CI can provide significant and sustained auditory benefits to patients with NF2 independent of tumor treatment modality, with the majority of those implanted becoming at least part-time users. Larger datasets are required to reliably assess the role of independent variables.

Low-Grade Surface Osteosarcoma of the Temporal Bone in Paediatric Patients: A Case Report and Literature Review.

PURPOSE OF THE STUDY: Primary osteosarcoma of the temporal bone is an exceedingly rare pathology in the paediatric population. As of now, only 3 cases have been reported in the English literature. We describe the additional case of a 16-year-old girl with an osteosarcoma of the mastoid bone. This study aims to report a rare paediatric case of low-grade surface osteosarcoma of the temporal bone. MATERIALS AND METHODS USED: A literature review was performed to better understand paediatric osteosarcomas of the head and neck region, to optimize their investigation, to describe their histopathological and radiological characteristics, and to establish the optimal modalities of medical and surgical treatments. The research of previous published data was done using PubMed and Embase library with the keywords mentioned below. RESULTS: The patient presented with a rapidly progressive left retroauricular lesion over a 3-week period. Radiological studies demonstrated aggressive and invasive features. An open biopsy followed and confirmed the diagnosis of a low-grade surface osteosarcoma. In accordance with the multidisciplinary team, we decided to perform a complete surgical resection with wide surgical margins. We did not administer any adjuvant therapies. A control computed tomography (CT) scan obtained 26 months postoperatively still showed no signs of recurrence. CONCLUSION: Osteosarcomas are aggressive malignant neoplasms found in the head and neck region in only 6% to 10% of cases. They represent approximately 1% of head and neck cancers, and these are generally high-grade lesions. Temporal bone involvement is rare, particularly for low-grade lesions in paediatric patients. In addition to reporting the fourth paediatric case of primary temporal bone osteosarcoma, this study describes its specific clinical, histopathological, and radiological findings, to improve the management and the prognostic of patients affected with this particular clinical entity.

Comparative Impacts of Scala Vestibuli Versus Scala Tympani Cochlear Implantation on Auditory Performances and Programming Parameters in Partially Ossified Cochleae.

OBJECTIVE: To compare scala vestibuli versus scala tympani cochlear implantation in terms of postoperative auditory performances and programming parameters in patients with severe scala tympani ossification. STUDY DESIGN: Retrospective case-control study. SETTING: Tertiary referral center. PATIENTS: One hundred three pediatric and adult patients who underwent cochlear implant surgery between 2000 and 2016. Three groups were formed: a scala vestibuli group, a scala tympani with ossification group, and a scala tympani without ossification group. Patients were matched based on their age, sex, duration of deafness, and side of implantation (ratio of 1:2:2). INTERVENTIONS: Postoperative evaluation of auditory performances and programming parameters following intensive functional rehabilitation program completion. MAIN OUTCOME MEASURES: Multimedia adaptive test (MAT), hearing in noise test (HINT SNR +10 dB, HINT SNR +5 dB, and HINT SNR +0 dB), impedances, neural response telemetry thresholds (NRT), neural response imaging thresholds (NRI), comfortable levels (C-levels), and threshold levels (T-levels) were compared between groups. RESULTS: Twenty-one patients underwent scala vestibuli cochlear implantation: 19 adults and two children. Auditory performances were similar between groups, although sentence recognition in a noisy environment was slightly higher in the scala vestibuli group. Impedance values were also higher in the scala vestibuli group, but all other programming parameters were similar between groups. CONCLUSIONS: We present the largest series of patients with scala vestibuli cochlear implantation. This approach provides at least comparable auditory performances without having any deleterious effects on programming parameters. This viable and useful insertion route might be the primary surgical alternative when facing partial cochlear ossification.

Musculo-mucous web velum and velopharyngeal dysfunction associated with 8q22.1-22.2 microduplication.

This report presents a rare case of isolated non-cleft velopharyngeal dysfunction (VPD). An eight-year-old child presented 1. a phenotypically unique band-gap pattern of the velar musculature with anteroposterior insertion; 2. a mosaic partial trisomy on chromosome 19 as well as microduplications on chromosomes 8 and 22. Following cytogenetic analysis, microduplication on chromosome 8 was found in another member of her family. A family history of VPI with hypernasality and nasal regurgitation was reported over three different generations on the patient's maternal side. Since only one case of velum malformation is found in this family, we cannot conclude to a link between the palatal anomaly or VPD and the DNA rearrangements.

Improving Quality of Life With Nabilone During Radiotherapy Treatments for Head and Neck Cancers: A Randomized Double-Blind Placebo-Controlled Trial.

OBJECTIVES: Patients treated for head and neck carcinomas experience a significant deterioration of their quality of life during treatments because of severe side effects. Nabilone has many properties that could alleviate symptoms caused by radiotherapy and improve patients' quality of life. The aim of the present study was to compare the effects of nabilone versus placebo on the quality of life and side effects during radiotherapy for head and neck carcinomas. METHODS: Fifty-six patients were randomized to nabilone or placebo. Patients filled the European Organisation for Research and Treatment of Cancer (EORTC) QLQ-C30 and the EORTC QLQ-H&N35; three independent questionnaires assessing appetite, nausea, and toxicity; and a visual analog scale for pain. These data were collected before radiotherapy, each week during radiotherapy, and 4 weeks after radiotherapy. Patients were weighed every week. RESULTS: Nabilone did not lengthen the time necessary for a 15% deterioration of quality of life (P = .4279), and it was not better than placebo for relieving symptoms like pain (P = .6048), nausea (P = .7105), loss of appetite (P = .3295), weight (P = .1454), mood (P = .3214), and sleep (P = .4438). CONCLUSION: At the dosage used, nabilone was not potent enough to improve the patients' quality of life over placebo.

Improving audiologic performance with partial insertion of a compressed array despite intracochlear retention of four electrodes during revision cochlear implant surgery: A case report.

OBJECTIVE AND IMPORTANCE: To report a case of partial extraction of the electrode array during revision cochlear implant surgery and to discuss the surgical management of this rare complication. Clinical presentation The safety of revision cochlear implant surgery has long been demonstrated. Only five cases of partial extraction of the electrode array with intracochlear retention have been reported in the literature. In this report, we describe the case of a 12-year-old boy with post-meningitis deafness who suffered this complication. INTERVENTION: Despite intracochlear retention of four electrodes, the surgical team was able to perform partial insertion of a Med-El compressed array: a total of 8 electrodes out of 12 were implanted in the same scala tympani. Five months after the surgery, the patient had access for the first time to open-set speech recognition. He could recognize 77% of open-set sentences in silence compared to 14% after initial implant activation. Seven months after the surgery, the patient was implanted in his contralateral ear with a Med-El Pulsar split array and now benefits from bilateral auditory stimulation. CONCLUSION: Partial insertion of a compressed array represents a viable option when facing incomplete extraction of a cochlear implant electrode array. Indeed, our patient's audiologic performance improved significantly and the results seem to surpass those obtained with partial insertion of a conventional electrode array.

A mobile phone based remote patient monitoring system for chronic disease management.

Rising concern over the poor state of chronic disease management led to the user-informed design and development of a home tele-monitoring system. Focus groups with patients and primary care providers guided the research team towards a design that would accommodate the workflow and concerns of the healthcare providers and the low use and comfort with technology found among the patient population. The system was trialed in a before-and-after pilot study of 34 patients with diabetes and hypertension. Findings demonstrate a significant improvement in systolic and diastolic blood pressure. An RCT beginning in 2007 is being conducted to confirm these findings. It is hypothesized that this user-centred approach, utilizing focus groups, iterative design and human factors methods of evaluation, will lead to the next-generation of home tele-monitoring applications that are more intuitive, less cumbersome, and ultimately bring about greater patient compliance and better physician management.

Mobile phone-based remote patient monitoring system for management of hypertension in diabetic patients.

BACKGROUND: Rising concern over the poor level of blood-pressure (BP) control among hypertensive patients has prompted searches for novel ways of managing hypertension. The objectives of this study were to develop and pilot-test a home BP tele-management system that actively engages patients in the process of care. METHODS: Phase 1 involved a series of focus-group meetings with patients and primary care providers to guide the system's development. In Phase 2, 33 diabetic patients with uncontrolled ambulatory hypertension were enrolled in a 4-month pilot study, using a before-and-after design to assess its effectiveness in lowering BP, its acceptability to users, and the reliability of home BP measurements. RESULTS: The system, developed using commodity hardware, comprised a Bluetooth-enabled home BP monitor, a mobile phone to receive and transmit data, a central server for data processing, a fax-back system to send physicians' reports, and a BP alerting system. In the pilot study, 24-h ambulatory BP fell by 11/5 (+/-13/7 SD) mm Hg (both P < .001), and BP control improved significantly. Substantially more home readings were received by the server than expected, based on the preset monitoring schedule. Of 42 BP alerts sent to patients, almost half (n = 20) were due to low BP. Physicians received no critical BP alerts. Patients perceived the system as acceptable and effective. CONCLUSIONS: The encouraging results of this study provide a strong rationale for a long-term, randomized, clinical trial to determine whether this home BP tele-management system improves BP control in the community among patients with uncontrolled hypertension.

Going, going, still there: using the WebCite service to permanently archive cited web pages.

Scholars are increasingly citing electronic "web references" which are not preserved in libraries or full text archives. WebCite is a new standard for citing web references. To "webcite" a document involves archiving the cited Web page through www.webcitation.org and citing the WebCite permalink instead of (or in addition to) the unstable live Web page. This journal has amended its "instructions for authors" accordingly, asking authors to archive cited Web pages before submitting a manuscript. Almost 200 other journals are already using the system. We discuss the rationale for WebCite, its technology, and how scholars, editors, and publishers can benefit from the service. Citing scholars initiate an archiving process of all cited Web references, ideally before they submit a manuscript. Authors of online documents and websites which are expected to be cited by others can ensure that their work is permanently available by creating an archived copy using WebCite and providing the citation information including the WebCite link on their Web document(s). Editors should ask their authors to cache all cited Web addresses (Uniform Resource Locators, or URLs) "prospectively" before submitting their manuscripts to their journal. Editors and publishers should also instruct their copyeditors to cache cited Web material if the author has not done so already. Finally, WebCite can process publisher submitted "citing articles" (submitted for example as eXtensible Markup Language [XML] documents) to automatically archive all cited Web pages shortly before or on publication. Finally, WebCite can act as a focussed crawler, caching retrospectively references of already published articles. Copyright issues are addressed by honouring respective Internet standards (robot exclusion files, no-cache and no-archive tags). Long-term preservation is ensured by agreements with libraries and digital preservation organizations. The resulting WebCite Index may also have applications for research assessment exercises, being able to measure the impact of Web services and published Web documents through access and Web citation metrics.