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Downbeat nystagmus (DBN) observed in head-hanging positions, may be of central or peripheral origin. Central DBN in head-hanging positions is mostly due to a disorder of the vestibulo-cerebellum, whereas peripheral DBN is usually attributed to canalolithiasis of an anterior semicircular canal. Here, we describe an atypical case of a patient who, after head trauma, experienced severe and stereotypic vertigo attacks after being placed in various head-hanging positions. Vertigo lasted 10–15 s and was always associated with a robust DBN. The provocation of transient vertigo and DBN, which both showed no decrease upon repetition of maneuvers, depended on the yaw orientation relative to the trunk and the angle of backward pitch. On a motorized, multi-axis turntable, we identified the two-dimensional Helmholtz coordinates of head positions at which vertigo and DBN occurred (y-axis: horizontal, space-fixed; z-axis: vertical, and head-fixed; x-axis: torsional, head-fixed, and unchanged). This two-dimensional area of DBN-associated head positions did not change when whole-body rotations took different paths (e.g., by forwarding pitch) or were executed with different velocities. Moreover, the intensity of DBN was also independent of whole-body rotation paths and velocities. So far, therapeutic approaches with repeated liberation maneuvers and cranial vibrations were not successful. We speculate that vertigo and DBN in this patient are due to macular damage, possibly an unstable otolithic membrane that, in specific orientations relative to gravity, slips into a position causing paroxysmal stimulation or inhibition of macular hair cells.

To investigate transcranial transmission (TT) and the dampening effect of the skin in patients and cadaver heads. In patients a pure tone bone conduction audiogram for ipsilateral and contralateral stimulation was performed. The TT was defined as the difference between ipsilateral and contralateral hearing thresholds. In cadaver heads ipsilateral and contralateral promontory motion was measured using a three-dimensional Laser Doppler Vibrometer system. Seven single-sided deaf patients fitted with a Baha® Connect, fifteen single-sided deaf patients without a bone conduction hearing aid and five Thiel-embalmed cadaver heads were included. The TT decreased with increasing frequency in patients and cadaver heads. No significant difference was seen between patients and cadaver heads. Measurements on patients and cadaver heads showed increasing skin attenuation with increasing frequency. However, the dampening effect was 3–12 dB higher in patients than in cadavers at all frequencies. The TT was not significantly different for patients compared to cadaver heads. The value of promontory motion to estimate TT in patients need to be further evaluated. The skin attenuates a BC stimulus by 10–20 dB in patients and by a smaller amount in cadaver heads, probably due to changes in the properties of the Thiel-conserved skin.

Current surgical microscopes suffer from inherent constraints in achievable viewing angles and require manual interaction to control settings. Overcoming these limitations may provide benefits for otologic surgical procedures.We present a first feasibility and usability assessment of a novel commercial surgical microscope featuring a virtual-reality interface and hands-free control of a robot-mounted 3D-camera.Overall practicability of using the device for otologic procedures could be confirmed in theThe novel virtual reality 3D-microscope bears potential to provide benefits for various otologic procedures, which will be evaluated more quantitatively in clinical follow-up studies.

Objectives: (1) To correlate simultaneously recorded intra- and extracochlear electrocochleography (ECochG) signals during electrode insertion into the cochlea, (2) to track changes in the ECochG signal during insertion and removal of an electrode, and (3) to correlate the findings with the preoperative residual hearing. We hypothesized that intracochlear ECochG recordings show signal changes not reflected in simultaneous extracochlear ECochG recordings. Design: During cochlear implantation in human cochlear implant recipients, a short, slim, custom-made electrode was inserted and removed in a stepwise manner. At each step, ECochG recordings were simultaneously recorded by an extracochlear electrode near the round window and via the inserted electrode. The acoustic stimulus was a 500 Hz tone burst at 110 to 130 dB SPL. Results: The mean amplitude difference between intra- and extracochlear ongoing ECochG responses was 14 dB (range 9 to 24 dB; n = 10) at the beginning of insertion. Intracochlear ECochG responses were larger in all cases. Extracochlear ECochG responses remained stable while intracochlear recordings showed large variations regarding amplitude and phase during the electrode array insertion. Intracochlear signal changes during insertion were reversible with retraction of the electrode. There were only weak to moderate (rs = 0.006 to 0.4), nonsignificant correlations of residual preoperative hearing with maximum amplitudes and amplitude changes during electrode insertion and removal in intracochlear recordings. Conclusions: Signals in intracochlear ECochG recordings are reliably larger than ECochG signals recorded simultaneously from an extracochlear location. Intracochlear ECochG recordings show reversible amplitude and phase changes during insertion, not reflected in simultaneous extracochlear ECochG recordings. Such changes are most likely due to the movement of the recording electrode in relation to the signal generators. Residual high-frequency hearing is associated with larger ECochG signal amplitudes. Modeling of expected intracochlear ECochG changes during electrode insertions may allow detection of cochlear trauma in the future.

Purpose To establish a standardized reporting system of cholesteatoma, the ChOLE classification has recently been introduced. We here aimed to systematically investigate the association between the ChOLE classification and (i) hearing, (ii) recidivism rate, and (iii) postoperative complications. These data may increase the utility of the ChOLE classification in clinical practice and research by stratifying patients according to expected outcomes or risks for complications. Methods In this prospective multicentric study, we included adult patients undergoing tympanomastoid surgery due to cholesteatoma. Main outcome measures included the association of the ChOLE classification system with (i) audiometric data including air conduction (AC) and bone conduction (BC) pure-tone average (PTA), and the air–bone gap (ABG), (ii) recidivism and complication. Results A total of 160 patients suffering from cholesteatoma were included. ChOLE stage distribution was stage I in 23 (14%), stage II in 128 (80%), and stage III in 9 (6%) patients. The ChOLE stage was associated with the postoperative AC PTA (p = 0.05) and the postoperative BC PTA (p = 0.02). Further, the status of the ossicular chain after surgery (ChOLE subdivision “O”) was associated with both the postoperative ABG (p = 0.0001) and the postoperative AC PTA (p = 0.003). Moreover, we found an association between complications (ChOLE subdivision “L) and both the postoperative BC PTA (p = 0.04) and the postoperative ABG (p = 0.04). No association between the ChOLE stage was found to both cholesteatoma recidivism and surgical complications. Conclusion The ChOLE classification is a new system to classify cholesteatomas. We provide evidence that hearing outcomes vary among different ChOLE stages. In particular, hearing outcomes are associated with the ChOLE subdivision “O” and “L”. Thus, the ChOLE classification system has a predictive value regarding hearing outcomes.

ObjectiveTo investigate the prevalence of bony dehiscence in the tympanic facial canal in patients with acute otitis media with facial paresis compared to those without facial paresis.MethodA retrospective case–control study was conducted on acute otitis media patients with facial paresis undergoing high-resolution temporal bone computed tomography.ResultsForty-eight patients were included (24 per group). Definitive determination of the presence of a bony dehiscence was possible in 44 out of 48 patients (91.7 per cent). Prevalence of bony dehiscence in acute otitis media patients with facial paresis was not different from that in acute otitis media patients without facial paresis (p = 0.21). Presence of a bony dehiscence was associated with a positive predictive value of 66.7 per cent in regard to development of facial paresis. However, an intact bony tympanic facial canal did not prevent facial paresis in 44.8 per cent of cases (95 per cent confidence interval = 34.6–55.6).ConclusionPrevalence of bony dehiscence in acute otitis media patients with facial paresis did not differ from that in acute otitis media patients without facial paresis. An intact tympanic bony facial canal does not protect from facial paresis development.

Objective: To determine the correlation between intraoperative changes of electrocochleography (ECochG) responses and traumatic cochlear implant insertions as well as postoperative hearing loss. Methods: ECochG, radiological, and audiological data were collected prospectively in a cochlear implant recipient with otosclerosis and assumed cochlear trauma during electrode insertion. A systematic review was conducted within PubMed-NCBI, EMBASE, and the Cochrane Library using the terms “Cochlear implant” and “Electrocochleography.” Original studies that evaluated intraoperative ECochG responses and postoperative hearing loss were selected and analyzed. Results: The case report revealed a drop of intra- and extracochlear ECochG signals during electrode insertion. The postoperative computed tomography scan suggested a scalar dislocation. There was no measurable hearing 4 weeks after surgery. Within the database search, nine articles met the inclusion criteria. All were case series reports (range from 2 to 36 subjects) with a total of 173 subjects. Due to the heterogeneous data, a meta-analysis was unfeasible. Conclusions: In concordance with some findings in the literature, the presented case report suggests that a drop of intra- and extracochlear ECochG signals during the insertion of the electrode array is associated with cochlear trauma and postoperative hearing loss in some cases. However, the literature is inconclusive regarding the correlation between intraoperative changes of the ECochG signals and postoperative hearing preservation. More studies investigating the correlation are needed to provide sufficient data.

Objective To assess the prevalence and implications of phase changes in extracochlear electrocochleography (ECochG) recordings during cochlear implantation. Materials and methods Extracochlear ECochG recordings were performed before and after insertion of the cochlear implant (CI) electrode by a recording electrode placed on the promontory. Acoustic stimuli were tone bursts at 250, 500, 750, and 1,000 Hz. The pure tone average (PTA) was determined before and approximately 4 weeks after surgery. Results Extracochlear ECochG recordings in 69 ears of 68 subjects were included. At 250 Hz, the mean phase change was 43° (n = 50, standard deviation (SD) 44°), at 500 Hz 36° (n = 64, SD 36°), at 750 Hz 33° (n = 42, SD 39°), and at 1,000 Hz 22° (n = 54, SD 27°). Overall, in 48 out of 210 ECochG recordings a phase change of ≥45° (23%) was detectable. Ears with an amplitude drop >3 dB and a phase change ≥45° (n = 3) had a complete or near complete loss of residual cochlear function in all cases. A phase change of ≥90° in one recording was not associated with a larger amplitude change of the ECochG signal (1.9 dB vs. -0.9 dB, p = 0.1052, n = 69), but with a significantly larger postoperative hearing loss (17 dB vs. 26 dB, p = 0.0156, n = 69). Conclusions Phase changes occur regularly in extracochlear ECochG recordings during cochlear implantation. Phase changes of ≥90° with or without amplitude changes in the ECochG signal are associated with a larger postoperative hearing loss and could therefore represent an independent marker for cochlear trauma or changes of inner ear mechanics relevant for the postoperative hearing outcome.

Contains fulltext : 251696.pdf (Publisher’s version ) (Closed access) In this study, we investigated the association of ACAN variants with otosclerosis, a frequent cause of hearing loss among young adults. We sequenced the coding, 5'-UTR and 3'-UTR regions of ACAN in 1497 unrelated otosclerosis cases and 1437 matched controls from six different subpopulations. The association between variants in ACAN and the disease risk was tested through single variant and gene-based association tests. After correction for multiple testing, 14 variants were significantly associated with otosclerosis, ten of which represented independent association signals. Eight variants showed a consistent association across all subpopulations. Allelic odds ratios of the variants identified four predisposing and ten protective variants. Gene-based tests showed an association of very rare variants in the 3'-UTR with the phenotype. The associated exonic variants are all located in the CS domain of ACAN and include both protective and predisposing variants with a broad spectrum of effect sizes and population frequencies. This includes variants with strong effect size and low frequency, typical for monogenic diseases, to low effect size variants with high frequency, characteristic for common complex traits. This single-gene allelic spectrum with both protective and predisposing alleles is unique in the field of complex diseases. In conclusion, these findings are a significant advancement to the understanding of the etiology of otosclerosis.

BACKGROUND: Surgical treatment of vestibular schwannoma (VS) leads to acute ipsilateral vestibular loss if there is residual vestibular function before surgery. To overcome the sequelae of acute ipsilateral vestibular loss and to decrease postoperative recovery time, the concept of preemptive vestibular ablation with gentamicin and vestibular prehabilitation before surgery has been developed (“vestibular prehab”). OBJECTIVE: Studying postural stability during walking and handicap of dizziness over a 1-year follow-up period in VS patients undergoing vestibular prehab before surgical treatment of VS. METHODS: A retrospective review of consecutive patients with a diagnosis of a VS undergoing surgical therapy from June 2012 to March 2018 was performed. All patients were included with documentation of the length of hospital duration and the Dizziness Handicap Inventory (DHI) and the Functional Gait Assessment (FGA) assessed preoperatively as well as 6 weeks and 1 year postoperatively. RESULTS: A total 68 VS patients were included, of which 29 patients received preoperative vestibular ablation by intratympanic injection of gentamicin. Mean VS diameter was 20.2 mm (SD 9.4 mm) and mean age at surgery was 49.6 years (SD 11.5 years). Vestibular prehab had no effect on DHI and FGA at any time point studied. CONCLUSIONS: We found no effect of vestibular prehab on postural stability during walking and on the handicap of dizziness. These findings add to the body of knowledge consisting of conflicting results of vestibular prehab. Therefore, vestibular prehab should be applied only in selected cases in an experimental setting.

Zusammenfassung Hintergrund Es sind unterschiedliche Knochenleitungshörgeräte (KLHG) verfügbar, die auf unterschiedliche Art mit dem Stimulationsort in Kontakt stehen. Ziel dieser Arbeit ist eine experimentelle Evaluation eines neuen transkutanen KLHG, das ohne Anpressdruck an die Haut angekoppelt wird. Material und Methoden Die Messungen wurden an einem Thiel-konservierten Ganzkopfkadaver durchgeführt. Zur Stimulation zwischen 0,1 und 10 kHz wurde ein Adhear verwendet, das über einen Signalgenerator und Verstärker direkt mit 1Vrms aktiviert wurde. Die Kopplung des KLHG erfolgte am Mastoid über ein Hautpflaster. Die Vibrationen des Schädels wurden am ipsi- und kontralateralen Promontorium und an der ipsi-, top- und kontralateralen Schädeloberfläche mittels 3D-Laser-Doppler-Vibrometers registriert. Insgesamt wurden ~200 Punkte auf der Schädeloberfläche (~15–20 mm Abstand) gemessen. Die Daten wurden mit entsprechenden Messungen verglichen, bei denen ein Baha Power als KLHG verwendet wurde. Dieses wurde am Mastoid über einen 5-Newton-Stahlbügel an die Haut gekoppelt. Ergebnisse Die ipsi- und kontralateralen promontorialen Vibrationen bei Stimulation mit dem Adhear sind in ihrer frequenzspezifischen Amplitude und Phase wie auch in der Zusammensetzung der Bewegungskomponenten mit der Stimulation mittels Baha Power am Stahlbügel vergleichbar. Ebenso erfährt die Schädeloberfläche unter beiden Ankopplungsarten eine vergleichbare komplexe räumliche Bewegung. Schlussfolgerungen Obwohl das Adhear ohne Anpressdruck und das Baha Power am Stahlband mit 5N an die Haut über dem Mastoid gekoppelt werden, unterscheiden sich die untersuchten Parameter kaum.

Vestibular neurectomy for Meniere's disease is considered as a treatment option when conservative methods fail to control debilitating vestibular symptoms. Most common surgical approaches today consist of transtemporal supralabyrinthine (middle fossa), retrolabyrinthine and retrosigmoid craniotomies. The aim of this article is to describe the procedure of vestibular neurectomy via a retrosigmoid approach. This approach can be performed alone or in combination with a retrolabyrinthine approach and allows good exposure of the cranial nerves VII and VIII in the cerebellopontine angle.

Objectives Investigation of bone conduction sound propagation by osseous and non-osseous pathways and their interactions based upon the stimulation site and coupling method of the actuator from a bone conduction hearing aid (BCHA). Methods Experiments were conducted on five Thiel embalmed whole head cadaver specimens. The electromagnetic actuator from a commercial bone conduction hearing aid (BCHA) (Baha® Cordelle II) was used to provide a stepped sine stimulus in the range of 0.1-10 kHz. Osseous pathways (direct bone stimulation or transcutaneous stimulation) were sequentially activated by stimulation at the mastoid or the BAHA side using several methods including a percutaneously implanted screw, Baha® Attract transcutaneous magnet and a 5-N (5-N) steel headband. Non-osseous pathways (only soft tissue or intra-cranial contents) were activated by actuator stimulation on the eye or neck via attachment to a 5-N steel headband, and were compared with stimulation via equivalent attachment on the mastoid and forehead. The response of the skull was measured as motions of the ipsi- and contralateral promontory and intracranial pressure (ICP) in the central, anterior, posterior, ipsilateral and contralateral temporal regions of the cranial space. Promontory motion was monitored using a 3-dimensional Laser Doppler vibrometer (3D LDV) system. Results The promontory undergoes spatially complex motion with similar contributions from all motion components, regardless of stimulation mode. Combined 3D promontory motion provided lower inter-sample variability than did any individual component. Transcranial transmission showed gain for the low frequencies and attenuation above 1 kHz, independent of stimulation mode This effect was not only for the magnitude but also its spatial composition such that contralateral promontory motion did not follow the direction of ipsilateral stimulation above 0.5 kHz. Non-osseous stimulation on the neck and eye induced comparable ICP relative to percutaneous (via screw) mastoid stimulation. Corresponding phase data indicated lower phase delays for ICP when stimulation was via non-osseous means (i.e., to the eye) versus osseous means (i.e., to the mastoid or forehead). Sound propagation due to skull stimulation passes through the thicker bony sections first before activating the CSF. Conclusion Utilization of 3D promontory motion measurements provides more precise (lower inter-sample variability) information about bone vibrations than does any individual component. It also provides a more detailed description of transcranial attenuation. A comprehensive combination of motion and pressures measurements across the head, combined with a variation of the stimulation condition, could reveal details about sound transmission within the skull.

Otosclerosis is a common form of hearing loss (HL) due to abnormal remodeling of the otic capsule. The genetic causes of otosclerosis remain largely unidentified. Only mutations in a single gene, SERPINF1, were previously published in patients with familial otosclerosis. To unravel the contribution of genetic variation in this gene to otosclerosis, this gene was re-sequenced in a large population of otosclerosis patients and controls. Resequencing of the 5' and 3' UTRs, coding regions, and exon-intron boundaries of SERPINF1 was performed in 1604 unrelated otosclerosis patients and 1538 unscreened controls, and in 62 large otosclerosis families. Our study showed no enrichment of rare variants, stratified by type, in SERPINF1 in patients versus controls. Furthermore, the c.392C>A (p.Ala131Asp) variant, previously reported as pathogenic, was identified in three patients and four controls, not replicating its pathogenic nature. We could also not find evidence for a pathogenic role in otosclerosis for 5' UTR variants in the SERPINF1-012 transcript (ENST00000573763), described as the major transcript in human stapes. Furthermore, no rare variants were identified in the otosclerosis families. This study does not support a pathogenic role for variants in SERPINF1 as a cause of otosclerosis. Therefore, the etiology of the disease remains largely unknown and will undoubtedly be the focus of future studies.

There are no instruments available to comprehensively assess health-related quality of life (HRQoL) in chronic otitis media (COM) in Italian-speaking countries. The Zurich chronic middle ear inventory (ZCMEI-21) is a well-established instrument for the assessment of HRQoL in COM. The objective of this study was to translate and cross-culturally adapt the ZCMEI-21 into Italian and validate this questionnaire for measuring HRQoL in patients with COM.Prospective multicenter study.Three University hospitals (northern Italy, central Italy, southern Italy).Adult patients suffering from COM (n = 128).Following international guidelines, the ZCMEI-21 was translated into Italian (ZCMEI-21-It). Validation was performed by psychometric test statistics. Moreover, ZCMEI-21-It total and subscale scores were compared and correlated with 1) the scores of the original validation study, 2) to a question that directly addresses HRQoL, and 3) to the scores of the EQ-5D-5L, a generic questionnaire assessing HRQoL.From three study centers, a total of 128 patients with COM were included. Cronbach's α was 0.86 indicating a high reliability. Between the ZCMEI-21-It total score and the question that directly addresses HRQoL, we found a strong correlation (r = 0.62, p 0.0001). Between the ZCMEI-21-It total score and the EQ-5D-5L scores, we expectedly found moderate correlations (descriptive system score: r = 0.39, p 0.0001; visual analog scale: r = 0.30, p = 0.008).We translated the ZCMEI-21 questionnaire into Italian and validated the ZCMEI-21-It in a prospective multicenter study. The ZCMEI-21-It is the first instrument that comprehensively assesses relevant dimensions of HRQoL in Italian-speaking patients affected by COM.

Objectives Predicting the course of cranial nerves (CNs) VII and VIII in the cerebellopontine angle on preoperative imaging for vestibular schwannoma (VS) may help guide surgical resection and reduce complications. Diffusion magnetic resonance imaging dMRI is commonly used for this purpose, but is limited by its resolution. We investigate the use of super-resolution reconstruction (SRR), where several different dMRIs are combined into one dataset. We hypothesize that SRR improves the visualization of the CN VII and VIII. Design Retrospective case review. Setting Tertiary referral center. SRR was performed on the basis of axial and parasagittal single-shot epiplanar diffusion tensor imaging on a 3.0-tesla MRI scanner. Participants Seventeen adult patients with suspected neoplasms of the lateral skull base. Main Outcome Measures We assessed separability of the two distinct nerves on fractional anisotropy (FA) maps, the tractography of the nerves through the cerebrospinal fluid (CSF), and FA in the CSF as a measure of noise. Results SRR increases separability of the CN VII and VIII (16/17 vs. 0/17, p = 0.008). Mean FA of CSF surrounding the nerves is significantly lower in SRRs (0.07 ± 0.02 vs. 0.13 ± 0.03 [axial images]/0.14 ± 0.05 [parasagittal images], p = 0.00003/p = 0.00005). Combined scanning times (parasagittal and axial) used for SRR were shorter (8 minute 25 seconds) than a comparable high-resolution scan (15 minute 17 seconds). Conclusion SRR improves the resolution of CN VII and VIII. The technique can be readily applied in the clinical setting, improving surgical counseling and planning in patients with VS.

The aim of this study was to investigate the contralateral hearing of patients with sporadic vestibular schwannoma (VS).Retrospective cohort study.Pure-tone audiograms of the contralateral ear from patients with a wait-and-scan strategy were compared to the ones who received therapy. Due to a possible bias caused by the therapy, hearing thresholds before and after radiotherapy or surgery were compared separately with the wait-and-scan group.From 1979 to 2017, 508 patients with sporadic VS could be included in the study. Of these, 240 received regular controls in the sense of wait-and-scan, 72 underwent radiotherapy (63 audiograms before and 43 after radiotherapy), and 196 had a surgery (186 audiograms before and 146 after surgery). Age-normalized hearing thresholds of the contralateral ear from patients with a wait-and-scan strategy were compared to ones who received therapy. In addition, hearing thresholds were compared to norm values.There was no evidence for a difference in the contralateral hearing of patients with sporadic VS between the wait-and-scan and therapy groups. The mean difference of hearing thresholds in our sample to norm values was found to be larger for the high frequencies and more pronounced in male patients.There was no evidence for a difference in the contralateral hearing loss of patients with sporadic VS between the wait-and-scan and therapy groups. However, there was some indirect indication of poorer contralateral hearing in all patients with sporadic VS compared to normative values.

Objective To analyze the cost effectiveness of cochlear implantation (CI) for the treatment of single-sided deafness (SSD). Study design Cost-utility analysis in an adapted Markov model. Setting Adults with single-sided deafness in a high-income country. Intervention Unilateral CI was compared with no intervention. Main outcome measure Incremental cost-effectiveness ratios were compared with different cost-effectiveness thresholds ($10,000 to $150,000) for different age, sex, and cost combinations. The calculations were based on the quality-adjusted life year (QALY), national life expectancy tables, and different cost settings. The health utility values for the QALY were either directly collected from published data, or, derived from published data using a regression model of multiple utility indices (regression estimate). Results The regression estimate showed an increase of the health utility value from 0.62 to 0.74 for SSD patients who underwent CI. CI for SSD was cost effective for women up to 64 years ($50,000 per-QALY threshold), 80 years ($100,000 per-QALY threshold), and 86 years ($150,000 per-QALY threshold). For men, these values were 58, 77, and 84, respectively. Changing the discount rate by up to 5% further increased the cutoff ages up to 5 years. A detailed cost and age sensitivity analysis is presented and allows testing for cost effectiveness in local settings worldwide. Conclusions CI is a cost-effective option to treat patients with SSD.

Objectives: Predicting the course of cranial nerve (CN) VII in the cerebellopontine angle (CPA) on preoperative imaging for vestibular schwannoma (VS) may help guide surgical resection and reduce complications. Diffusion MRI based tractography has been used to identify cranial nerve trajectory, but intraoperative validation of this novel approach is challenging. Currently, validation is based on operative report descriptions of the course of cranial nerves, but yields a simplified picture of the three-dimensional (3D) course of CN VII. In this study, we investigate the accuracy of tractography with detailed patient-specific 3D-printed VS tumors. Design: Retrospective case review. Setting: Tertiary referral center. Participants: Twenty adult VS surgical candidates. Main Outcome Measures: We compared tractography with intraoperative 3D course of CN VII. The surgeons were blinded to tractography and drew the intraoperative course of the CN VII on a patient specific 3D-printed tumor model for detailed comparison with tractography. Results: Of 20 patients, one was excluded due to subtotal removal and inability to assess CN VII course. In the remaining 19 patients, 84% (16/19) tractography was successful. In 94% of tumors with tractography (15/16), the intraoperative description of CN VII course matched the tractography finding. The maximum distance, however, between tractography and intraoperative course of CN VII was 3.7 mm ± 4.2 mm. Conclusion: This study presents a novel approach to CN VII tractography validation in VS. Although descriptions of CN VII intraoperatively match tractography, caution is warranted as quantitative measures suggest a clinically significant distance between tractography and CN VII course.

The reliable prediction of cochlear implant (CI) speech perception outcomes is highly relevant and can facilitate the monitoring of postoperative hearing performance. To date, multiple audiometric, demographic, and surgical variables have shown some degree of correlation with CI speech perception outcomes. In the present study, postsurgical acoustic and electric hearing thresholds that are routinely assessed in clinical practice were compared to CI speech perception outcomes in order to reveal possible markers of postoperative cochlear health. A total of 237 CI recipients were included in this retrospective monocentric study. An analysis of the correlation of postoperative pure-tone averages (PTAs) and electric CI fitting thresholds (T-/C-levels) with speech perception scores for monosyllabic words in quiet was performed. Additionally, a correlation analysis was performed for postoperative acoustic thresholds in intracochlear electrocochleography (EcochG) and speech recognition scores in a smaller group (n = 14). The results show that neither postoperative acoustic hearing thresholds nor electric thresholds correlate with postoperative speech perception outcomes, and they do not serve as independent predictors of speech perception outcomes. By contrast, the postoperative intracochlear total EcochG response was significantly correlated with speech perception. Since the EcochG recordings were only performed in a small population, a large study is required to clarify the usefulness of this promising predictive parameter.

Introduction: Semicircular canal dehiscence (SCD) is defined as a defect of the bone overlying the semicircular canal. It has a relatively high prevalence of 3% in the general population, which makes it likely that a certain number of patients receiving a cochlear implant (CI) would have it. However, little is known about the influence of SCD on the CI outcome. Therefore, the aim of this study was to determine the influence of SCD on CI outcome with regard to short- and long-term word perception and hearing preservation. Methods: This study was a retrospective analysis of postoperative word perception ability in the electric-only condition after 6, 12, and ≥18 months and of hearing preservation 4 weeks after surgery in CI recipients with and without SCD. All patients received a preoperative 1.5- or 3-tesla magnetic resonance imaging. Results: Fifty-five patients were included. Forty-eight patients (87%) had no SCD, and 7 patients (13%) had SCD. Mean postoperative word perception scores were 66% in the non-SCD group versus 50% in the SCD group (p = 0.17) after 6 months, 74 versus 64% (p = 0.28) after 12 months, and 77 versus 73% (p = 0.62) after 18 or more months. The mean postoperative hearing loss in patients with functional residual hearing before surgery (n = 34) was 22 dB in the non-SCD group versus 31 dB in the SCD group (p = 0.15). Conclusions: CI outcome is comparable between recipients without and with SCD. Specifically, hearing preservation rate and word perception ability in the electric-only condition seem not affected by SCD. The rate of progress of word perception ability in the first 12 months after cochlear implantation is not influenced by SCD.

Introduction: The round window membrane (RWM) acts as a pressure-relieving membrane for incompressible cochlear fluid. The reinforcement of the RWM has been used as a surgical intervention for the treatment of superior semicircular canal dehiscence and hyperacusis. The aim of this study was to investigate how RWM reinforcement affects sound pressure variations in the cochlea. Methods: The intracochlear sound pressure (ICSP) was simultaneously measured in the scala tympani (ST) and scala vestibuli (SV) of cadaveric human temporal bones (HTBs) in response to acoustic stimulation for three RWM reinforcement materials (soft tissue, cartilage, and medical-grade silicone). Results: The ICSP in the ST was significantly increased after RWM reinforcement for frequencies below 2 kHz. Between 400 and 600 Hz, all three materials demonstrated the highest median pressure increase. The higher the RWM stiffness, the larger the pressure increase: silicone (7 dB) &lt, soft tissue (10 dB) &lt, cartilage (13 dB). The ICSP in the SV was less affected by reinforcement. The highest median pressure increase was 3 dB. The experimental findings can be explained with numerical models of cochlear mechanics. Discussion and conclusions: RWM reinforcement increases the sound pressure in ST at lower frequencies but only has a minor influence on the SV pressure.

Different bone conduction hearing aids (BCHA) are commercially available. They are attached to the head in different ways. The aim of this work is an experimental evaluation of the performance of a new transcutaneous (surface mounted via adhesive pad) actuator of a BCHA.Experiments were conducted on a Thiel embalmed whole head cadaver specimen. The electromagnetic actuators from a commercial BCHA (Adhear) was used to provide stepped sine stimulus in the range of 0.1-10 kHz. The BCHA was coupled to a skin surface adhesion that was placed on the mastoid. The response was monitored as motions of the ipsi- and contralateral promontory, and as motions of the ipsi-, top- and contralateral skull surface. Promontory motion was quantified via a three-dimensional laser Doppler vibrometer (3D LDV) system. Analogously, surface motion was registered by sequentially measuring ~200 points on the skull surface (~ 15-20 mm pitch) via 3D LDV. The data were compared to corresponding measurements obtained with a Baha Power that was coupled to skin on the Mastoid via a 5 Newton steelband.Ipsilateral and contralateral promontory vibration for stimulation with the Adhear are comparable to stimulation with the Baha Power on the 5 Newton steelband with regard to frequency dependent amplitude and phase, as well as the contribution of the motion components. The surface motion of the skull experiences a similar complex motion for both stimulation modes.Although the Adhear is coupled without any pressure to the skin over the mastoid whereas the Baha power is attached with a 5 Newton steelband, the vibration parameters investigated are comparable.Es sind unterschiedliche Knochenleitungshörgeräte (KLHG) verfügbar, die auf unterschiedliche Art mit dem Stimulationsort in Kontakt stehen. Ziel dieser Arbeit ist eine experimentelle Evaluation eines neuen transkutanen KLHG, das ohne Anpressdruck an die Haut angekoppelt wird.Die Messungen wurden an einem Thiel-konservierten Ganzkopfkadaver durchgeführt. Zur Stimulation zwischen 0,1 und 10 kHz wurde ein Adhear verwendet, das über einen Signalgenerator und Verstärker direkt mit 1Vrms aktiviert wurde. Die Kopplung des KLHG erfolgte am Mastoid über ein Hautpflaster. Die Vibrationen des Schädels wurden am ipsi- und kontralateralen Promontorium und an der ipsi-, top- und kontralateralen Schädeloberfläche mittels 3D-Laser-Doppler-Vibrometers registriert. Insgesamt wurden ~200 Punkte auf der Schädeloberfläche (~15–20 mm Abstand) gemessen. Die Daten wurden mit entsprechenden Messungen verglichen, bei denen ein Baha Power als KLHG verwendet wurde. Dieses wurde am Mastoid über einen 5-Newton-Stahlbügel an die Haut gekoppelt.Die ipsi- und kontralateralen promontorialen Vibrationen bei Stimulation mit dem Adhear sind in ihrer frequenzspezifischen Amplitude und Phase wie auch in der Zusammensetzung der Bewegungskomponenten mit der Stimulation mittels Baha Power am Stahlbügel vergleichbar. Ebenso erfährt die Schädeloberfläche unter beiden Ankopplungsarten eine vergleichbare komplexe räumliche Bewegung.Obwohl das Adhear ohne Anpressdruck und das Baha Power am Stahlband mit 5N an die Haut über dem Mastoid gekoppelt werden, unterscheiden sich die untersuchten Parameter kaum.

Objectives To investigate the association between the “ChOLE” classification, hearing outcomes and disease-specific health-related quality of life (HRQoL). Methods In two tertiary referral centers, patients requiring primary or revision surgery for cholesteatoma were assessed for eligibility. Audiometric assessment was performed pre- and postoperatively. The ChOLE classification was determined intraoperatively and via the preoperative CT scan. HRQoL was assessed pre- and postoperatively using the Zurich Chronic Middle Ear Inventory (ZCMEI-21). Results A total of 87 patients (mean age 45.2 years, SD 16.2) were included in this study. ChOLE stage I cholesteatoma was found in 8 (9%), stage II cholesteatoma was found in 65 (75%), and stage III cholesteatoma was found in 14 (16%) patients. Postoperatively, the mean air–bone gap (0.5, 1, 2, 3 kHz) was significantly smaller than before surgery (14.3 dB vs. 23.0 dB; p = 0.0007). The mean ZCMEI-21 total score significantly decreased after surgery (26.8 vs. 20.7, p = 0.004). No correlation between the ZCMEI-21 total score and both the ChOLE stage and the extent of the cholesteatoma (ChOLE subdivision “Ch”) was found. A trend towards worse HRQoL associated with a poorer status of the ossicular chain (ChOLE subdivision “O”) was observed. The audiometric outcomes were not associated with the extent of the cholesteatoma. The ChOLE subdivision describing the ossicular status showed a strong association with the pre- and postoperative air conduction (AC) thresholds. Further, the ZCMEI-21 total score and its hearing subscore correlated with the AC thresholds. Conclusion The ChOLE classification does not show a clear association with HRQoL measured by the ZCMEI-21. The HRQoL neither seems to be associated with the extent of the disease nor with the ossicular chain status. Yet, surgical therapy significantly improved HRQoL by means of reduced ZCMEI-21 total scores, which were strongly associated with the AC thresholds. Intraoperative assessment of a cholesteatoma using the ChOLE classification and HRQoL complement each other and provide useful information.

Objective: The etiology of symptoms following primary repair of superior canal dehiscence (SCD) may be due to a persistent third window. However, the extent of surgery cannot be seen on postoperative computed tomography (CT) since most repair materials are not radiopaque. We hypothesize that the extent of superior semicircular canal (SSC) occlusion following primary repair can be quantified based on postoperative magnetic resonance imaging (MRI) data. Study design: Retrospective series. Setting: Tertiary care center. Patients: Adult patients with a history of SCD syndrome who 1) report persistent symptoms following primary SCD repair and 2) underwent heavily T2-weighted MRI postoperatively. Interventions: Analysis of SSC using 3D-reconstruction of CT co-registered with MRI data. Main outcome measures: Arc length of fluid void on MRI and quantification of persistent SCD based on CT/MRI co-registration. Results: We identified 9 revision cases from a cohort of 145 SCD repairs at our institution (2002-2017) with CT/MRI data. A fluid void on postoperative MRI (indicating occlusion of the SSC) was observed in all cases (anterior limb: 50.1 degrees [±21.8 SD] and posterior limb 48.1 degrees [±28.5 SD]). Co-registration of CT/MRI revealed a residual defect that was most commonly found along the posterior limb in most patients with persistent symptoms. Conclusions: The extent of SCD repair can be determined using reformatted or direct T2-weighted MRI sequences in the plane of Pöschl. Co-registration of CT/MRI may be useful to determine the location of a residual superior canal defect and when present was found most commonly along the posterior limb.

Aural fullness is a common symptom of middle ear diseases, most importantly Eustachian tube dysfunction (ETD). Yet, aural fullness may also be caused by inner ear disorders, such as hydropic ear diseases. Here, we report our experience with endolymphatic hydrops (EH) mimicking ETD. Furthermore, we review the literature related to (i) EH as a differential diagnosis of symptoms suggesting ETD and (ii) the pathophysiology and treatment of aural fullness due to inner ear disorders. We retrospectively included adult patients with aural fullness as chief complaint and radiographically diagnosed EH. Hearing and Eustachian tube function were assessed using audiometry, tympanometry, and tubomanometry. Primarily suspected ETD was treated by balloon dilatation of the Eustachian tube (BDET). The endolymphatic space of the inner ear was imaged using gadolinium-enhanced MRI (Gd-MRI) including a 3D-real inversion-recovery sequence after intravenous gadolinium administration. We report three affected ears of two patients (two females, age 42 and age 51) with aural fullness as chief complaint. Audiometry of main speech frequencies was normal in all affected ears. In one ear, there was a type A tympanogram and in two ears, there was a type B tympanogram. In both patients, medical treatment for ETD and BDET were unsuccessful. Gd-MRI of the inner ears revealed cochlear EH in 3/3 ears affected by aural fullness, but not in the unaffected ear. EH may underlay cases with aural fullness and could in these cases explain unsuccessful treatment for ETD. As ETD is often treated by invasive procedures, distinguishing ETD from EH as the underlying cause of aural fullness is important. Our findings raise the question whether Gd-MRI to rule out EH is indicated in patients with unexplained aural fullness, in particular after unsuccessful interventional treatment for ETD.

In order to better understand bone conduction sound propagation across the skull, three-dimensional (3D) wave propagation on the skull surface was studied, along with its dependence on stimulation direction and location of a bone conduction hearing aid (BCHA) actuator. Experiments were conducted on five Thiel embalmed whole head cadaver specimens. Stimulation, in the 0.1–10 kHz range, was sequentially applied at the forehead and mastoid via electromagnetic actuators from commercial BCHAs, supported by a 5-N steel band. The head response was quantified by sequentially measuring the 3D motion of ∼200 points (∼15–20 mm pitch) across the ipsilateral, top, and contralateral skull surface via a 3D laser Doppler vibrometer (LDV) system, guided by a robotic positioner. Low-frequency stimulation (

Objectives Evaluation of the transfer function efficiency of a newly-developed piezo-electric actuator for active subcutaneous bone conduction hearing aid. Methods The experiments were conducted on four Thiel embalmed whole head cadaver specimens. A novel actuator based on piezo-electric transduction (PZTA), part of a subcutaneous bone conduction hearing aid device, was sequentially implanted on three locations: 1) Immediately posterior to pinna; 2) 50–60 mm posterior to pinna, approximately the same distance as between the BAHA (bone anchored hearing aid) location and the ear canal, but the same horizontal level as location 1; 3) the traditional BAHA location. Using a single point 3-dimensional laser Doppler vibrometer (LDV) system, three types of motion measurements were performed at the cochlear promontory for each stimulation location: 1) ipsilateral side, 2) contralateral side, 3) measurements 1 and 2 were repeated after mastoidectomy on the ipsilateral side. Results On average, stimulation at locations 1 and 2 show a trend for higher promontory motion relative to location 3 (BAHA location) above 1 kHz. Stimulation at location 1 had an average improvement of 1–6 dB at 2–4 kHz, and 1–18 dB at 6–8 kHz. The spatial composition of the motion showed significant contributions from both in-plane and out-of-plane (along ear canal) motion components, with in-plane components being dominant at mid and high frequencies for locations 2 and 3. Stimulation at locations 1 and 3 produced similar transcranial attenuation at mid frequencies (0.6–4 kHz), with a potential trend of higher attenuation (seen in 3 or the 4 samples) for location 1 at higher frequencies (>4 kHz). The mastoidectomy affected negatively mostly the high frequencies (6–8 kHz) for stimulation at location 1, with no significant change for location 3. Conclusion The sound transfer function efficacy of a novel subcutaneous bone conduction device has been quantified, and the influence of stimulation location and mastoidectomy have been analyzed based on promontory motion in Thiel-preserved cadaver heads.

Intra-operative quantification of the ossicle mobility could provide valuable feedback for the current status of the patient's conductive hearing. However, current methods for evaluation of middle ear mobility are mostly limited to the surgeon's subjective impression through manual palpation of the ossicles. This study investigates how middle ear transfer function is affected by stapes quasi-static stiffness of the ossicular chain. The stiffness of the middle ear is induced by a) using a novel fiber-optic 3-axis force sensor to quantify the quasi-static stiffness of the middle ear, and b) by artificial reduction of stapes mobility due to drying of the middle ear.Middle ear transfer function, defined as the ratio of the stapes footplate velocity versus the ear canal sound pressure, was measured with a single point LDV in two conditions. First, a controlled palpation force was applied at the stapes head in two in-plane (superior-inferior or posterior-anterior) directions, and at the incus lenticular process near the incudostapedial joint in the piston (lateral-medial) direction with a novel 3-axis PalpEar force sensor (Sensoptic, Losone, Switzerland), while the corresponding quasi-static displacement of the contact point was measured via a 3-axis micrometer stage. The palpation force was applied sequentially, step-wise in the range of 0.1-20 gF (1-200 mN). Second, measurements were repeated with various stages of stapes fixation, simulated by pre-load on the stapes head or drying of the temporal bone, and with severe ossicle immobilization, simulated by gluing of the stapes footplate.Simulated stapes fixation (forced drying of 5-15 min) severely decreases (20-30 dB) the low frequency (1 kHz) response of the middle ear, while increasing (5-10 dB) the high frequency (4 kHz) response. Stapes immobilization (gluing of the footplate) severely reduces (20-40 dB) the low and mid frequency response (4 kHz) but has lesser effect (10 dB) at higher frequencies. Even moderate levels of palpation force (3gF,30 mN), regardless of direction, have negative effect (10-20 dB) on the low frequency (2 kHz) response, but with less significant (5-10 dB) effect at higher frequencies. Force-displacement measurements around the incudostapedial joint showed quasi-static stiffness in the range of 200-500 N/m for normal middle ears, and 1000-2500 N/m (5-8-fold increase) after artificially (through forced drying) reducing the middle ear transfer function with 10-25 dB at 1 kHz.Effects of the palpation force level and direction, as well as stapes fixation and immobilization have been analyzed based on the measurement of the stapes footplate motion, and controlled application of 3D force and displacement.

Objectives: (1) To assess dynamic postural stability before and after cochlear implantation using a functional gait assessment (FGA). (2) To evaluate the correlation between loss of residual hearing and changes in dynamic postural stability after cochlear implantation. Methods: Candidates for first-sided cochlear implantation were prospectively included. The FGAs and pure-tone audiograms were performed before and 4–6 weeks after cochlear implantation. Results: Twenty-three subjects were included. Forty-eight percent (n = 11) showed FGA performance below the age-referenced norm before surgery. One subject had a clinically relevant decrease of the FGA score after cochlear implantation. No significant difference between the mean pre- and postoperative FGA scores was detectable (p = 0.4). Postoperative hearing loss showed no correlation with a change in FGA score after surgery (r = 0.3, p = 0.3, n = 16). Conclusion: Single-sided cochlear implantation does not adversely affect dynamic postural stability 5 weeks after surgery. Loss of functional residual hearing is not correlated with a decrease in dynamic postural stability.

Animals are frequently used for the development and testing of new hearing devices. Dimensions of the middle ear and cochlea differ significantly between humans and commonly used animals, such as rodents or cats. The sheep cochlea is anatomically more like the human cochlea in size and number of turns. This study investigated the middle-ear ossicular velocities and intracochlear sound pressure (ICSP) in sheep temporal bones, with the aim of characterizing the sheep as an experimental model for implantable hearing devices. Measurements were made on fresh sheep temporal bones. Velocity responses of the middle ear ossicles at the umbo, long process of the incus and stapes footplate were measured in the frequency range of 0.25-8 kHz using a laser Doppler vibrometer system. Results were normalized by the corresponding sound pressure level in the external ear canal (PEC). Sequentially, ICSPs at the scala vestibuli and tympani were then recorded with custom MEMS-based hydrophones, while presenting identical acoustic stimuli. The sheep middle ear transmitted most effectively around 4.8 kHz, with a maximum stapes velocity of 0.2 mm/s/Pa. At the same frequency, the ICSP measurements in the scala vestibuli and tympani showed the maximum gain relative to the PEC (24 dB and 5 dB, respectively). The greatest pressure difference across the cochlear partition occurred between 4 and 6 kHz. A comparison between the results of this study and human reference data showed middle-ear resonance and best cochlear sensitivity at higher frequencies in sheep. In summary, sheep can be an appropriate large animal model for research and development of implantable hearing devices.

The aim of the study was to evaluate whether pretherapeutic metabolic tumor parameters from 18-fluorodeoxyglucose positron emission tomography (FDG-PET) imaging could predict larynx preservation in laryngeal and hypopharyngeal cancer patients prior to primary chemoradiation. Tumor metabolic parameters [maximum standardized uptake value (SUVmax), metabolic tumor volume (MTV), and total lesion glycolysis (TLG)] were retrospectively assessed in a consecutive cohort of laryngeal and hypopharyngeal cancer patients undergoing primary (chemo-)radiation. Main outcome measures were larynx preservation and survival. The study included 97 patients with a median follow-up of 32 months (IQR 20–54.5). For hypopharyngeal cancer, multivariable analysis showed that patients with a primary tumor’s SUVmax > 9.5 entailed a higher risk of undergoing salvage pharyngolaryngectomy after chemoradiation (HR = 8.64, 95% CI = 1.1–67.3, P = 0.040). In laryngeal cancer, SUVmax did not predict the need for salvage laryngectomy. The only predictor for larynx preservation in laryngeal cancer patients was T-classification at initial diagnosis (HR = 6.67, 95% CI = 0.82–53.9, P = 0.039). In conclusion, SUVmax of primary tumor could be used as a predictor of larynx preservation prior to primary chemoradiation in hypopharyngeal cancer patients. This information may be important for patient counseling, as high SUVmax was correlated with reduced probability of larynx preservation. However, in laryngeal cancer patients, SUVmax does not seem to be predictive of outcome.

Objective: To evaluate the quality of perceived sound after stapedotomy over a 1-year follow-up period focussing on incidence of dysacusis, particularly distorted sound perception (DSP). DSP was assessed by (i) determination of the frequencies and hearing level that such perceptions are elicited by pure tones (pure-tone-evoked distorted sound perception, PTE-DSP), a novel psychoacoustic measurement introduced in this paper, and (ii) assessment of patient-reported occurrence of DSP using the Amsterdam Post Operative Sound Evaluation (APOSE) questionnaire (APOSE-DSP). Design: Prospective study. Study sample: Patients (n = 23) with otosclerosis undergoing stapedotomy. Results: An air-bone gap of p = 0.03). Postoperatively, APOSE-DSP was associated with a smaller benefit (improvement in air conduction; p = 0.03), yet, a lower bone conduction pure-tone average was associated with PTE-DSP (p = 0.006). Conclusions: DSP after stapedotomy is associated with a smaller benefit 3 months after stapedotomy. DSP commonly occur after stapedotomy, but decrease over time. This is important information to be included in patient counselling before stapedotomy.

OBJECTIVES To translate and validate the Zurich Chronic Middle Ear Inventory (ZCMEI-21) for the English language in order to provide an English instrument to assess health-related quality of life in chronic otitis media (COM). DESIGN Pilot translation study including cognitive debriefings, prospective multicenter cross-sectional psychometric validation study. SETTING Four tertiary referral centers in three different English-speaking countries (United Kingdom, United States of America and Australia). PARTICIPANTS Adult patients suffering from COM. MAIN OUTCOME MEASURES The English translation of the ZCMEI-21 (ZCMEI-21-E) and the five-level version of the EQ-5D questionnaire. The EQ-5D, which constitutes a generic measure of health-related quality of life, consists of a descriptive system score and a visual analogue scale. Statistical outcomes included single item descriptive statistics, internal consistency (Cronbach's α) as an indicator of reliability, as well as construct validity. RESULTS A total of 124 patients suffering from COM were included. The mean age was 50.1 years (SD 16.9 years) and 72 (58.1%) were males. The Cronbach's α of the ZCMEI-21-E was 0.91, suggesting an excellent internal consistency. The Spearman's correlation coefficient of the ZCMEI-21-E total score was 0.55 (p < 0.0001) for convergent construct validity with EQ-5D descriptive system score and 0.57 (p < 0.0001) with the EQ-5D visual analogue scale. CONCLUSIONS The ZCMEI-21-E is a new validated questionnaire that provides clinicians with a short, comprehensive and reliable instrument to quantify health-related quality of life in patients suffering from COM. The ZCMEI-21-E may be of use in clinical routine as well as in outcome research and monitoring. This article is protected by copyright. All rights reserved.

OBJECTIVE: To assess by electrocochleography (ECoG) at which times during cochlear implantation changes of cochlear function occur. METHODS: Tone bursts with a frequency of 500 or 750 Hz were used as acoustic stimuli. The recording electrode was placed on the promontory and left in an unchanged position for all ECoG recordings. RESULTS: Eight subjects were included. After opening the cochlea, an increase of the amplitude of the ECoG signal was detectable in four subjects (mean change 3.9 dB, range from 0.2 to 10.8 dB). No decreases were detectable after opening the cochlea or during the first half of the insertion of the CI electrode array (mean change 0.5 dB, range from -2.2 to 1.6 dB). During the second half of the insertion, the amplitude of the ECoG signal decreased in four subjects (mean change -2.5 dB, range from -0.04 to -4.8 dB). If a decrease occurred during the second half of the insertion, then the decrease continued in the earliest phase after insertion of the CI electrode array (mean change -2.1 dB, range from -0.5 to -5.8 dB). CONCLUSION: Pressure changes inside the cochlea can lead to an increase of ECoG signals after opening the cochlea. If detectable, then a decrease of ECoG signals occurs during the second half of the insertion of the CI electrode array and continues in the earliest phase after completed insertion. These findings suggest that cochlear trauma occurs toward the end of the insertion and that trauma-dependent postoperative mechanisms contribute to postoperative hearing loss.

The incudo-malleal joint (IMJ) in the human middle ear is a true diarthrodial joint and it has been known that the flexibility of this joint does not contribute to better middle-ear sound transmission. Previous studies have proposed that a gliding motion between the malleus and the incus at this joint prevents the transmission of large displacements of the malleus to the incus and stapes and thus contributes to the protection of the inner ear as an immediate response against large static pressure changes. However, dynamic behavior of this joint under static pressure changes has not been fully revealed. In this study, effects of the flexibility of the IMJ on middle-ear sound transmission under static pressure difference between the middle-ear cavity and the environment were investigated. Experiments were performed in human cadaveric temporal bones with static pressures in the range of +/- 2 kPa being applied to the ear canal (relative to middle-ear cavity). Vibrational motions of the umbo and the stapes footplate center in response to acoustic stimulation (0.2-8 kHz) were measured using a 3D-Laser Doppler vibrometer for (1) the natural IMJ and (2) the IMJ with experimentally-reduced flexibility. With the natural condition of the IMJ, vibrations of the umbo and the stapes footplate center under static pressure loads were attenuated at low frequencies below the middle-ear resonance frequency as observed in previous studies. After the flexibility of the IMJ was reduced, additional attenuations of vibrational motion were observed for the umbo under positive static pressures in the ear canal (EC) and the stapes footplate center under both positive and negative static EC pressures. The additional attenuation of vibration reached 4~7 dB for the umbo under positive static EC pressures and the stapes footplate center under negative EC pressures, and 7~11 dB for the stapes footplate center under positive EC pressures. The results of this study indicate an adaptive mechanism of the flexible IMJ in the human middle ear to changes of static EC pressure by reducing the attenuation of the middle-ear sound transmission. Such results are expected to be used for diagnosis of the IMJ stiffening and to be applied to design of middle-ear prostheses.

Objective: To evaluate foreign language acquisition at school in cochlear implant patients. Study design: Cohort study. Setting: CI center. Patients: Forty three cochlear implants (CI) patients (10-18 yr) were evaluated. CI nonusers and patients with CI-explantation, incomplete datasets, mental retardation, or concomitant medical disorders were excluded. Intervention(s): Additional data (type of schooling, foreign language learning, and bilingualism) were obtained with questionnaires. German-speaking children with foreign tuition language (English and/or French) at school were enrolled for further testing. Main outcome measure(s): General patient data, auditory data, and foreign language data from both questionnaires and tests were collected and analyzed. Results: Thirty seven out of 43 questionnaires (86%) were completed. Sixteen (43%) were in mainstream education. Twenty-seven CI users (73%) have foreign language learning at school. Fifteen of these were in mainstream education (55%), others in special schooling. From 10 CI users without foreign language learning, one CI user was in mainstream education (10%) and nine patients (90%) were in special schooling. Eleven German-speaking CI users were further tested in English and six additionally in French. For reading skills, the school objectives for English were reached in 7 of 11 pupils (64%) and for French in 3 of 6 pupils (50%). For listening skills, 3 of 11 pupils (27%) reached the school norm in English and none in French. Conclusions: Almost 75% of our CI users learn foreign language(s) at school. A small majority of the tested CI users reached the current school norm for in English and French in reading skills, whereas for hearing skills most of them were not able to reach the norm.

Under large quasi-static loads, the incudo-malleolar joint (IMJ), connecting the malleus and the incus, is highly mobile. It can be classified as a mechanical filter decoupling large quasi-static motions while transferring small dynamic excitations. This is presumed to be due to the complex geometry of the joint inducing a spatial decoupling between the malleus and incus under large quasi-static loads. Spatial Laser Doppler Vibrometer (LDV) displacement measurements on isolated malleus-incus-complexes (MICs) were performed. With the malleus firmly attached to a probe holder, the incus was excited by applying quasi-static forces at different points. For each force application point the resulting displacement was measured subsequently at different points on the incus. The location of the force application point and the LDV measurement points were calculated in a post-processing step combining the position of the LDV points with geometric data of the MIC. The rigid body motion of the incus was then calculated from the multiple displacement measurements for each force application point. The contact regions of the articular surfaces for different load configurations were calculated by applying the reconstructed motion to the geometry model of the MIC and calculate the minimal distance of the articular surfaces. The reconstructed motion has a complex spatial characteristic and varies for different force application points. The motion changed with increasing load caused by the kinematic guidance of the articular surfaces of the joint. The IMJ permits a relative large rotation around the anterior-posterior axis through the joint when a force is applied at the lenticularis in lateral direction before impeding the motion. This is part of the decoupling of the malleus motion from the incus motion in case of large quasi-static loads.

OBJECTIVE To assess cochlear trauma during cochlear implantation by electrocochleography (ECoG) and cone beam computed tomography (CBCT) and to correlate intraoperative cochlear trauma with postoperative loss of residual hearing. METHODS ECoG recordings to tone bursts at 250, 500, 750, and 1000 Hz and click stimuli were recorded before and after insertion of the cochlear implant electrode array, using an extracochlear recording electrode. CBCTs were conducted within 6 weeks after surgery. Changes of intraoperative ECoG recordings and CBCT findings were correlated with postoperative threshold shifts in pure-tone audiograms. RESULTS Fourteen subjects were included. In three subjects a decrease of low-frequency ECoG responses at 250, 500, 750, and 1000 Hz occurred after insertion of the electrode array. This was associated with no or minimal residual hearing 4 weeks after surgery. ECoG responses to click stimuli were present in six subjects and showed a decrease after insertion of the electrode array in three. This was associated with a mean hearing loss of 21 dB in postoperative pure-tone audiograms. Scalar dislocation of the electrode array was assumed in one subject because of CBCT findings and correlated with a decrease of low-frequency ECoG responses and a complete loss of residual hearing. CONCLUSION Hearing loss of ≤11 dB is not associated with detectable decrease in ECoG recordings during cochlear implantation. However, in a majority of patients with threshold shifts of >11 dB or complete hearing loss, an intraoperative decrease of high- or low-frequency ECoG signals occurs, suggesting acute cochlear trauma.

This study explores the influence of stimulation position on bone conduction (BC) hearing sensitivity with a BC transducer attached using a headband.(1) The cochlear promontory motion was measured in cadaver heads using laser Doppler vibrometry while seven different positions around the pinna were stimulated using a bone anchored hearing aid transducer attached using a headband. (2) The BC hearing thresholds were measured in human subjects, with the bone vibrator Radioear B71 attached to the same seven stimulation positions.Three cadaver heads and twenty participants.Stimulation on a position superior-anterior to the pinna generated the largest promontory motion and the lowest BC thresholds. Stimulations on the positions superior to the pinna, the mastoid, and posterior-inferior to the pinna showed similar magnitudes of promontory motion and similar levels of BC thresholds.Stimulations on the regions superior to the pinna, the mastoid, and posterior-inferior to the pinna provide stable BC transmission, and are insensitive to small changes of the stimulation position. Therefore it is reliable to use the mastoid to determine BC thresholds in clinical audiometry. However, stimulation on a position superior-anterior to the pinna provides more efficient BC transmission than stimulation on the mastoid.

The use of electrocochleography (ECochG) for providing real-time feedback of cochlear function during cochlear implantation is receiving increased attention for preventing cochlear trauma and preserving residual hearing. Although various studies investigated the relationship between intra-operative ECochG measurements and surgical outcomes in recent years, the limited interpretability of ECochG response changes leads to conflicting study results and prevents the adoption of this method for clinical use. Specifically, the movement of the recording electrode with respect to the different signal generators in intracochlear recordings makes the interpretation of signal changes with respect to cochlear trauma difficult. Here, we demonstrate that comparison of ECochG signals recorded simultaneously from intracochlear locations and from a fixed extracochlear location can potentially allow a differentiation between traumatic and atraumatic signal changes in intracochlear recordings. We measured ECochG responses to 500 Hz tone bursts with alternating starting phases during cochlear implant insertions in six human cochlear implant recipients. Our results show that an amplitude decrease with associated near 180° phase shift and harmonic distortions in the intracochlear difference curve during the first half of insertion was not accompanied by a decrease in the extracochlear difference curve’s amplitude ( n = 1), while late amplitude decreases in intracochlear difference curves (near full insertion, n = 2) did correspond to extracochlear amplitude decreases. These findings suggest a role for phase shifts, harmonic distortions, and recording location in interpreting intracochlear ECochG responses.

This systematic review provides an overview of the available studies (published by January 29, 2018) with descriptive data analysis about the influence of cochlear implantation on tinnitus in patients with single-sided deafness (SSD).PubMed, EMBASE, Web of Science, Cochrane Library, and Google Scholar.Original studies about the influence of cochlear implantation on tinnitus, measured with different tinnitus questionnaires or visual analog scale, in patients with SSD were included. The pre- and postimplantation tinnitus scores of the included studies were extracted for the further systematic review.The systematic search yielded 1028 studies. After evaluating titles, abstracts, and full texts, 1011 of these were dismissed. From the remaining 17 studies, 4 showed a low directness of evidence or high risk of bias and were therefore excluded. Due to the nature of cochlear implantation in SSD, only cohort studies and no randomized trials exist, which limits the evaluation in a systematic review. Generally, the mean tinnitus questionnaire scores decreased after cochlear implantation in these 13 studies with a total of 153 patients. The most widely used tinnitus questionnaire was the Tinnitus Handicap Inventory. In these studies, 34.2% of patients demonstrated complete suppression, 53.7% an improvement, 7.3% a stable value, and 4.9% an increase of tinnitus, and none of the patients reported an induction of tinnitus.This review shows a clear improvement of tinnitus complaints after cochlear implantation in patients with SSD. Therefore, tinnitus might be considered as an additional indication for cochlear implantation in SSD.

Current cochlear implant (CI) systems provide substantial benefits for patients with severe hearing loss. However, they do not allow for 24/7 hearing, mainly due to the external parts that cannot be worn in all everyday situations. One of the key missing parts for a totally implantable CI (TICI) is the microphone, which thus far has not been implantable. The goal of the current project was to develop a concept for a packaging technology for state-of-the-art microelectromechanical systems (MEMS) microphones that record the liquid-borne sound inside the inner ear (cochlea) as a microphone signal input for a TICI. The packaging concept incorporates requirements, such as biocompatibility, long-term hermeticity, a high sensing performance and a form factor that allows sensing inside the human cochlea and full integration into the existing CI electrode array. The present paper (1) describes the sensor packaging concept and the corresponding numerical and experimental design verification process and (2) gives insight into new engineering solutions for sensor packaging. Overall, a packaging concept was developed that enables MEMS microphone technology to be used for a TICI system.