Treatment of patients with glomus jugulare tumours (GJT) and its subjective effect on quality of life (QoL) measures.

Cerebellopontine angle (CPA) tumours account for 6-10% of intracranial tumours. The most common CPA tumours are vestibular schwannomas (VS), also known as acoustic neuromas, benign tumours of the vestibulocochlear nerve. Less common but symptomatic skull base lesions are glomus jugulare tumours (GJT), of which approximately 40% are identified as CPA tumours. Initial symptoms for GJT may include hearing loss and tinnitus and progress to various cranial nerve dysfunctions. Three well-accepted treatment modalities for such tumours include surgical resection, radiotherapy and/or conservative management employing serial MR or CT imaging. Patients' quality of life may be impacted by different treatment methods, so treatment decisions should be client centered.

Quality of life related to symptomatic outcomes in patients with vestibular schwannomas: A Canadian Centre perspective.

Patients with vestibular schwannomas (VS) typically present with hearing loss and tinnitus as well as variable cranial nerve dysfunctions. Surgical resection, stereotactic radiotherapy and/or conservative management employing serial magnetic resonance or computed tomography imaging serve as the main treatment options. Quality of life (QoL) may be impacted by the extent of tumour burden and exacerbated or relieved by treatment. Subjective assessment and quality of life inventories provide valuable information in client centered approaches with important implications for treatment. The intention of QoL measurements affecting VS patients within a clinical setting is to facilitate discussions regarding treatment options and objectively evaluate patient- centered clinical outcomes in a naturalistic setting.

Hearing preservation in vestibular schwannoma management.

OBJECTIVE: To compare hearing preservation between stereotactic radiotherapy (SRT) and conservative treatment of patients with unilateral vestibular schwannoma. DATA SOURCES: Retrospective case series comparing hearing outcomes of patients with a unilateral vestibular schwannoma managed conservatively or with stereotactic radiotherapy in a tertiary care academic centre. PATIENTS: Tumor database patients with American Academy of Otolaryngology Head and Neck Surgery Class A or B hearing at the onset of study. Stereotactic radiotherapy patients were predominantly those who failed conservative management. INTERVENTIONS: Audiometric pure tone averages and speech discrimination scores as well as patient demographics, tumor location, size and growth were extracted. MAIN OUTCOME MEASURES: Hearing outcome measures were: 1) Hearing Preservation, i.e. no drop from Class A/B to Class C/D hearing, 2) Hearing Survival of Class A/B hearing in months, 3) Audiometric Pure Tone Averages, Difference between post-treatment and pre-treatment, and 4) Speech Discrimination Score Difference (pre-treatment-post treatment). Survival analysis and non-parametric tests were used for hearing outcome measures, with multiple covariates tested. RESULTS: Overall, serviceable hearing preservation among the 123 patients was 51%. The median hearing survival time was 46 months (mean 59 months). The Pure Tone averages and Speech Discrimination score differences were 16 dB and 82% respectively over a median follow-up time of 43 months. No significant difference was found between the conservative and SRT groups in any hearing outcome. Class A patients showed far better hearing survival than Class B patients. CONCLUSIONS: No significant difference was demonstrated as to measures of hearing outcomes between stereotactic radiotherapy and conservative management. Excellent existing hearing appears to be the best predictor of long term hearing survival in the cohort studied.

Presentation of atrial fibrillation following oral dexamethasone treatment in a NF2 patient.

Atrial fibrillation (A-fib) is the most common cardiac arrhythmia which is associated with an increased risk of mortality secondary to stroke and coronary artery disease. Intravenous glucocorticoid therapy (such as dexamethasone and hydrocortisone) is frequently used peri-operatively in patients undergoing cardiac surgery to prevent A-fib. Dexamethasone is also frequently used in patients with single or bilateral vestibular schwannomas (VS), to reduce tumor swelling both before and after radiation treatment. We describe a case of A-fib in a 50 year-old female patient with neurofibromatosis type 2 (NF-2), who was prescribed dexamethasone for post-radiation tumor edema.

Geometrically accurate real-time volumetric visualization of the middle ear using optical coherence tomography.

We introduce a novel system for geometrically accurate, continuous, live, volumetric middle ear optical coherence tomography imaging over a 10.9mm×30∘×30∘ field of view (FOV) from a handheld imaging probe. The system employs a discretized spiral scanning (DC-SC) pattern to rapidly collect volumetric data and applies real-time scan conversion and lateral angular distortion correction to reduce geometric inaccuracies to below the system's lateral resolution over 92% of the FOV. We validate the geometric accuracy of the resulting images through comparison with co-registered micro-computed tomography (micro-CT) volumes of a phantom target and a cadaveric middle ear. The system's real-time volumetric imaging capabilities are assessed by imaging the ear of a healthy subject while performing dynamic pressurization of the middle ear in a Valsalva maneuver.

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.

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.

Optical Clearing Agents for Optical Imaging Through Cartilage Tympanoplasties: A Preclinical Feasibility Study.

HYPOTHESIS: Optical clearing agents (OCAs) can render cartilage tympanoplasty grafts sufficiently transparent to permit visualization of middle ear structures in an operated ear using optical coherence tomography (OCT) imaging. METHODS: Pieces of human tragal cartilage were treated with glycerol, a commonly used OCA. A reference reflector was imaged with OCT through the tympanoplasty as it cleared and the optical attenuation of the graft was measured. The reversibility of clearing and the dimensional changes associated with glycerol absorption were also measured. In a separate experiment, a human cadaveric temporal bone was prepared to simulate an ossiculoplasty surgery with cartilage replacement of the tympanic membrane. A partial ossicular replacement prosthesis (PORP) inserted in the ear was imaged with OCT through a 0.4mm cartilage graft optically cleared with glycerol. MAIN OUTCOME MEASURE: The optical attenuation of 0.4mm cartilage grafts decreased at 2.3+/-1.1 dB/min following treatment with glycerol, reaching a total decrease in attenuation of 13.6+/-5.9 dB after 7 minutes. The optical and dimensional effects of glycerol absorption were reversable following saline washout. In the temporal bone preparation, treatment of a cartilage graft with glycerol resulted in a 13 dB increase in signal-to-noise ratio and a 13 dB increase in contrast for visualizing the PORP through the graft with OCT. CONCLUSIONS: Optical clearing agents offer a potential pathway towards optical coherence tomography imaging of the middle ear in post-surgical ears with cartilage grafts.

Clinical efficiency and safety of the oticon medical neuro cochlear implant system: a multicenter prospective longitudinal study.

OBJECTIVE: This prospective longitudinal cohort study at six tertiary referral centers in Canada and Denmark describes the clinical efficiency and surgical safety of cochlear implantation with the Oticon Medical Neuro cochlear implant system, including the Neuro Zti implant, the EVO electrode array, and the Neuro One sound processor. METHODS: Patients were adult cochlear implant candidates with bilateral sensorineural hearing loss. RESULTS: The mean HINT scores in quiet pre-operatively and at 3, 6, and 12 months post-activation were 13%, 58%, 67%, and 72%, respectively, and in noise (+10 dB SNR) 13%, 46%, 53%, and 59%, respectively. The mean improvement from baseline to 6 months post-activation was 54% in quiet and 40% in noise. The surgical major complication incidence rate was 0% and the post-surgical major complication incidence rate (until 12 months post-activation) was 4%. There was no adverse event that was fatal, that required explantation, or that resulted in sound processor nonuse, and no implant failure. CONCLUSION: Cochlear implantation with the Oticon Medical Neuro system enables speech identification both in quiet and in noise and audiologic outcomes continue to improve in the year following activation. No substantial adverse events occurred during the surgical implantation procedure and during the 12 months post-activation.

Optical Coherence Tomography Doppler Vibrometry Measurement of Stapes Vibration in Patients With Stapes Fixation and Normal Controls.

HYPOTHESIS: Ears with otosclerotic stapes fixation will exhibit lower-than-normal levels of ossicular mobility as measured by Optical Coherence Tomography Doppler Vibrometry (OCT-DV). BACKGROUND: OCT-DV measures the vibration of middle ear structures in response to sound non-invasively through the intact tympanic membrane. This allows, in particular, direct measurement of the vibration at the lenticular process of the incus which is expected to be lower in patients with otosclerotic stapes fixation. METHODS: OCT-DV was performed on ears presumptively diagnosed with otosclerosis (n = 13) and a group of normal control ears (n = 42). Displacement was measured at the umbo and the lenticular process of the incus in response to 500 and 1000 Hz stimulus tones at 100 dBSPL. MAIN OUTCOME MEASURE: The ability to discriminate between groups was assessed using receiver operator characteristic analysis, with the main outcome measures being the area-under-curve (AUC) and the sensitivity and specificity. RESULTS: For the best condition tested (500 Hz at the incus), the AUC was 0.998 and discriminated the otosclerotic from normal ears with a sensitivity/specificity of 1.00/0.98. One anomalous patient with surgically confirmed stapes fixation exhibited hypermobility at the umbo, possibly due to a partial ossicular discontinuity. Despite the high umbo mobility, this patient's stapes fixation was correctly discriminated based on the measured incus vibration levels. CONCLUSIONS: OCT-DV is a promising tool for preoperatively assessing ossicular mobility non-invasively in the clinic. Our results suggest OCT-DV may also be useful in discriminating other ossicular pathologies that result in conductive hearing loss.

Long term follow-up demonstrating stability and patient satisfaction of minimally invasive punch technique for percutaneous bone anchored hearing devices.

OBJECTIVE: Minimally Invasive Ponto Surgery (MIPS) was recently described to facilitate the placement of percutaneous bone anchored hearing devices. As early adopters of this new procedure, we sought to perform a quality assurance project using our own small prospective cohort to justify this change in practice. We chose to examine device stability and to gauge our patients' perspective of the surgery and their overall satisfaction with the process. METHODS: A total of 12 adult patients who underwent MIPS between 2016 and 2017 with a minimum post-operative follow-up of 12 months were included in this study. A prospective MIPS research clinic was used to follow patients, assess the implant site soft tissue status and gather qualitative information through patient interviews and surveys. RESULTS: The mean (SD) soft tissue status score averages using the IPS Scale were low for inflammation 0.1 (0.1), pain 0.1 (0.1), skin height 0.2 (0.1) and total IPS score 0.4 (0.3) indicating minimal soft tissue changes. Patient experiences with MIPS were overwhelmingly positive in reports through the MIPS modified SSQ-8. All patients reported speedy recoveries and no long-term complications. There were zero device losses. CONCLUSION: The series presented in this paper represents the first MIPS cohort with long term follow-up to be published to date in North America. Our findings conclude both device stability and patient satisfaction with no loss of fixtures. Consequently, we have adopted MIPS as our procedure of choice for the placement of all percutaneous BAHDs.

Shared decision making and decisional conflict in the Management of Vestibular Schwannoma: a prospective cohort study.

BACKGROUND: Patients with vestibular schwannomas (VS) are faced with complex management decisions. Watchful waiting, surgical resection, and radiation are all viable options with associated risks and benefits. We sought to determine if patients with VS experience decisional conflict when deciding between surgery or non-surgical management, and factors influencing the degree of decisional conflict. METHODS: A prospective cohort study in two tertiary ambulatory skull-base clinics was performed. Patients with newly diagnosed or newly growing vestibular schwannomas were recruited. Patients were given a demographic form and the decisional conflict scale (DCS), a validated measure to assess the degree of uncertainty when making medical decisions. The degree of shared decision making (SDM) experienced by the patient and physician were assessed via the SDM-Q-10 and SDM-Q-Doc questionnaires, respectively. Non-parametric statistics were used. Questionnaires and demographic information were correlated with DCS using Spearman correlation coefficient and Mann-Whitney U. Logistic regression was performed to determine factors independently associated with DCS scores. RESULTS: Seventy-seven patients participated (55% female, aged 37-81 years); VS ranged in size from 2 mm-50 mm. Significant decisional conflict (DCS score 25 or greater) was experienced by 17 (22%) patients. Patients reported an average SDM-Q-10 score of 86, indicating highly perceived level of SDM. Physician and patient SDM scores were weakly correlated (p = 0.045, Spearman correlation coefficient 0.234). DCS scores were significantly negatively correlated with a decision to pursue surgery, presence of a trainee, and higher SDM-Q-10 score. DCS was higher with female gender. Using logistic regression, the SDM-Q-10 score was the only variable associated with significantly reduced DCS. CONCLUSIONS: About one fifth of patients deciding how to manage their vestibular schwannoma experienced a significant degree of decisional conflict. Involving the patients in the process through shared decision-making significantly reduced the degree of uncertainty patients experienced.

Cochlear implantation in Cockayne syndrome: our experience of two cases with different outcomes.

Cockayne syndrome is a rare autosomal recessive defect in DNA repair resulting in a classic facies with potential visual and auditory impairment. The hearing loss begins peripherally and may become central as the condition progresses. Coexisting sensory deprivation from visual impairment and the possibility of progressive deterioration in mental function conspire with a lack of published experience to produce many challenges for the cochlear implant team. To the best of our knowledge, we present the first case reports with documented follow-up of cochlear implantation in two patients with different manifestations of Cockayne syndrome.

How does prosthesis head size affect vibration transmission in ossiculoplasty?

OBJECTIVES: The transmission of vibrations from the tympanic membrane to the stapes footplate by an ossicular reconstruction prosthesis is affected by the size of the prosthesis head. We sought to determine if augmenting or reducing the head size of prosthesis had a systematic effect on transmission of vibrations to the stapes. STUDY DESIGN: We conducted a fresh cadaveric temporal bone middle ear study. METHODS: The incus was replaced with a prosthesis using a tympanic membrane to stapes head (TASH)-type hydroxyapatite prosthesis in nine fresh cadaveric temporal bones. Three prosthesis head sizes were created: unaltered, reduced, and augmented. Stapes vibrations were measured with a laser Doppler vibrometer in response to acoustic frequency chirps at 90 dB SPL. RESULTS: All three head size prostheses resulted in smaller stapes vibrations than the intact ear. There was no difference in the vibration transmission between the three different head sizes. All prostheses showed a vibration loss of 10 to 15 dB compared to the intact ear. CONCLUSIONS AND SIGNIFICANCE: Within the range of sizes tested, prosthesis head size had little impact on vibration transmission to the stapes footplate.

Direct cost comparison of minimally invasive punch technique versus traditional approaches for percutaneous bone anchored hearing devices.

BACKGROUND: Minimally Invasive Ponto Surgery (MIPS) was recently described as a new technique to facilitate the placement of percutaneous bone anchored hearing devices. The procedure has resulted in a simplification of the surgical steps and a dramatic reduction in surgical time while maintaining excellent patient outcomes. Given these developments, our group sought to move the procedure from the main operating suite where they have traditionally been performed. This study aims to test the null hypothesis that MIPS and open approaches have the same direct costs for the implantation of percutaneous bone anchored hearing devices in a Canadian public hospital setting. METHODS: A retrospective direct cost comparison of MIPS and open approaches for the implantation of bone conduction implants was conducted. Indirect and future costs were not included in the fiscal analysis. A simple cost comparison of the two approaches was made considering time, staff and equipment needs. All 12 operations were performed on adult patients from 2013 to 2016 by the same surgeon at a single hospital site. RESULTS: MIPS has a total mean reduction in cost of CAD$456.83 per operation from the hospital perspective when compared to open approaches. The average duration of the MIPS operation was 7 min, which is on average 61 min shorter compared with open approaches. CONCLUSION: The MIPS technique was more cost effective than traditional open approaches. This primarily reflects a direct consequence of a reduction in surgical time, with further contributions from reduced staffing and equipment costs. This simple, quick intervention proved to be feasible when performed outside the main operating room. A blister pack of required equipment could prove convenient and further reduce costs.

Preoperative headband assessment for semi-implantable bone conduction hearing devices in conductive hearing loss: is it useful or misleading?

OBJECTIVE: To establish whether preoperative assessment using a conventional, percutaneous bone conducting implant (pBCI) processor on a headband accurately represents postoperative performance of a semi-implantable BCI (siBCI). STUDY DESIGN: Retrospective case series. SETTING: Tertiary otology unit. PATIENTS: Five patients with chronic otitis media (implanted unilaterally) and one with bilateral congenital ossicular fixation (implanted bilaterally). INTERVENTION(S): Semi-implantable bone conduction hearing implant. MAIN OUTCOME MEASURE(S): Functional hearing gain; preoperative (headband) versus postoperative (aided) speech discrimination; unaided bone conduction (BC) versus postoperative (aided) soundfield threshold. RESULTS: Significant functional gain was seen at all frequencies (one-tailed t test p G 0.01; n = 7). There was a 50 dB improvement in median speech reception threshold (SRT) from 70 dB unaided to 20 dB aided. Compared to the preoperative BC, aided siBCI thresholds were worse at 0.5 kHz, but at frequencies from 1 to 6 kHz, the siBCI closely matched the bone curve ( p G 0.01). The siBCI performed better than both pBCI processors on a headband at 3 to 4 kHz, except 1 kHz ( p G 0.01). CONCLUSIONS: BC thresholds may be a better indicator of implant performance than headband assessment. Candidacy assessment for siBCI implantation that relies on headband testing with pBCI processors should be interpreted with caution because the headband may under-represent the implanted device. This seems to be especially true at 3 kHz and above and may make it difficult for surgeons to conduct accurate informed consent discussions with patients about the realistic anticipated outcomes and benefits of the procedure.

Face and content validity of a novel, web-based otoscopy simulator for medical education.

BACKGROUND: Despite the fact that otoscopy is a widely used and taught diagnostic tool during medical training, errors in diagnosis are common. Physical otoscopy simulators have high fidelity, but they can be expensive and only a limited number of students can use them at a given time. OBJECTIVES: 1) To develop a purely web-based otoscopy simulator that can easily be distributed to students over the internet. 2) To assess face and content validity of the simulator by surveying experts in otoscopy. METHODS: An otoscopy simulator, OtoTrain™, was developed at Western University using web-based programming and Unity 3D. Eleven experts from academic institutions in North America were recruited to test the simulator and respond to an online questionnaire. A 7-point Likert scale was used to answer questions related to face validity (realism of the simulator), content validity (expert evaluation of subject matter and test items), and applicability to medical training. RESULTS: The mean responses for the face validity, content validity, and applicability to medical training portions of the questionnaire were all ≤3, falling between the "Agree", "Mostly Agree", and "Strongly Agree" categories. The responses suggest good face and content validity of the simulator. Open-ended questions revealed that the primary drawbacks of the simulator were the lack of a haptic arm for force feedback, a need for increased focus on pneumatic otoscopy, and few rare disorders shown on otoscopy. CONCLUSION: OtoTrain™ is a novel, web-based otoscopy simulator that can be easily distributed and used by students on a variety of platforms. Initial face and content validity was encouraging, and a skills transference study is planned following further modifications and improvements to the simulator.

Optimum tension for partial ossicular replacement prosthesis reconstruction in the human middle ear.

OBJECTIVE: Hearing results from ossiculoplasty are unpredictable. There are many potentially modifiable parameters. One parameter that has not been adequately investigated in the past is the effect of tension on the mechanical functioning of the prosthesis. Our goal was to investigate this parameter further, with the hypothesis that the mechanical functioning of partial ossicular replacement prostheses (PORP) from the stapes head to the eardrum will be affected by the tension that they are placed under. METHODS: Fresh temporal bones were used to reconstruct a missing incus defect with a PORP-type prosthesis. Three different lengths of PORP were used, and the stapes vibrations were measured with a laser Doppler vibrometer using a calibrated standard sound in the ear canal. Eight temporal bones were used. RESULTS: Tension had a very significant effect on stapes vibration. In general, loose prostheses resulted in the best overall vibration transmission. The effects were most marked at the lower frequencies. There was a slight advantage to tight prostheses in the higher frequencies, but much less than the decrement in lower frequencies with tight prostheses. CONCLUSION: In ossicular reconstruction, best stapes vibration results in our model are achieved by shorter prostheses, which result in lower tension.

How do cartilage and other material overlay over a prosthesis affect its vibration transmission properties in ossiculoplasty?

OBJECTIVE: The prosthesis/eardrum interface is often deliberately modified by cartilage overlay, or by soft tissue in-growth. We examined the effects of vibration transmission to the footplate of inserting cartilage of varying sizes, and materials of varying rigidities. STUDY DESIGN AND SETTING: Using fresh human cadaveric temporal bones, stapes vibrations were measured. A partial ossicular reconstruction prosthesis was covered with differing materials and cartilage sizes. Materials used included glass (rigid), cartilage (intermediate), and Merocel (soft). Cartilage sizes varied in size relative to the prosthesis head. RESULTS: Rigidity of material had little impact. Cartilage size had an impact, with smaller cartilage covers performing best. CONCLUSION: Larger cartilage sizes performed worse. This could be a result of increased tension on the eardrum from larger cartilage size. There is little impact stiffness of interposed material stiffness. SIGNIFICANCE: Cartilage of the size used clinically has little impact on vibration transmission to the footplate. A wide range of materials could clinically be interposed over the prosthesis.