Decompression sickness of the inner ear and relationship with a patent oval foramen: a study of 61 cases.

OBJECTIVE: To discuss the link between inner ear decompression sickness and patent foramen ovale. MATERIALS AND METHODS: Monocentric and retrospective study on decompression sickness of the inner ear requiring hyperbaric chamber treatment, from 2014 to 2021. RESULTS: Sixty-one patients of inner ear decompression sickness were included in this study. Twenty-four patients had vestibular injuries, 28 cochlear injuries and 9 cochleo-vestibular injuries. Compression chamber treatment was given, using an oxygen-helium mixture with oxygen partial pressure (PIO2) limited to 2.8 atmosphere absolute (ATA). All vestibular accidents completely recovered without clinical sequelae. For cochlear accident only 10 out of 37 patients (27%) recovered completely. A right-left shunt (patent foramen oval or intra-pulmonary shunt) was found in 31.1% of patients with inner ear decompression sickness (p > 0.05). CONCLUSION: The presence of patent foramen oval in patients with inner ear decompression was not statistically significant in our study. Understanding of the pathophysiology of decompression illness and the physiology and anatomy of the labyrinth would suggest a mechanism of supersaturation with degassing in intra-labyrinthine liquids.

Improving rehabilitation of deaf patients by advanced imaging before cochlear implantation.

INTRODUCTION: Cochlear implants have advanced the management of severe to profound deafness. However, there is a strong disparity in hearing performance after implantation from one patient to another. Moreover, there are several advanced kinds of imaging assessment before cochlear implantation. Microstructural white fiber degeneration can be studied with Diffusion weighted MRI (DWI) or tractography of the central auditory pathways. Functional MRI (fMRI) allows us to evaluate brain function, and CT or MRI segmentation to better detect inner ear anomalies. OBJECTIVE: This literature review aims to evaluate how helpful pre-implantation anatomic imaging can be to predict hearing rehabilitation outcomes in deaf patients. These techniques include DWI and fMRI of the central auditory pathways, and automated labyrinth segmentation by CT scan, cone beam CT and MRI. DESIGN: This systematic review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Studies were selected by searching in PubMed and by checking the reference lists of relevant articles. Inclusion criteria were adults over 18, with unilateral or bilateral hearing loss, who had DWI acquisition or fMRI or CT/ Cone Beam CT/ MRI image segmentation. RESULTS: After reviewing 172 articles, we finally included 51. Studies on DWI showed changes in the central auditory pathways affecting the white matter, extending to the primary and non-primary auditory cortices, even in sudden and mild hearing impairment. Hearing loss patients show a reorganization of brain activity in various areas, such as the auditory and visual cortices, as well as regions involved in language and emotions, according to fMRI studies. Deep Learning's automatic segmentation produces the best CT segmentation in just a few seconds. MRI segmentation is mainly used to evaluate fluid space of the inner ear and determine the presence of an endolymphatic hydrops. CONCLUSION: Before cochlear implantation, a DWI with tractography can evaluate the central auditory pathways up to the primary and non-primary auditory cortices. This data is then used to generate predictions on the auditory rehabilitation of patients. A CT segmentation with systematic 3D reconstruction allow a better evaluation of cochlear malformations and predictable difficulties during surgery.

Binaural squelch effect in unilateral otosclerosis surgery: comparison of speech intelligibility in noise before-after surgery.

PURPOSE: We aimed to evaluate the speech intelligibility benefit in noise provided by stapedotomy in the treatment of unilateral otosclerosis. METHODS: We enrolled adults suffering from unilateral conductive hearing loss and followed them up until 9 months after surgery. The patients underwent a free field speech hearing evaluation using the French Matrix test before and after stapedotomy. Speech material was sent to the front of the patients (S0) and noise was presented either at the front (N0), or at the operated ear (N-90) or at the non-operated ear (N + 90). The speech intelligibility benefit in noise was assessed by comparing Squelch effect (SE), Head shadow effect (HS) and Binaural redundancy (BR) before and after surgery. SE was measured as the difference in speech reception thresholds (SRT) between S0N + 90 situations before and after surgery, HS as the difference in SRT between S0N + 90 and S0N-90 situations, and BR as the difference in SRT between S0N0 situations before and after surgery. In addition, two quality of life's questionnaires were completed by patients to evaluate their discomfort. RESULTS: Among 25 patients, 19 were followed up during 9 months, 4 were excluded and 2 were lost for the following-up. Stapedotomy provided a restoration of SE of 3.7 dB SNR (p < 0.001) and a BR gain of 1.8 dB SNR (p < 0.001). HS did not show any statistical variation after surgery (p = 0.077). Finally, the questionnaires showed a residual hearing discomfort. CONCLUSION: Stapedotomy provided a binaural benefit with the restoration of the SE and BR but which remained lower than in the normal-hearing population. TRIAL REGISTRATION: The 07/02/2018 on Clinical.Trial.Gouv: NCT03587792.

Benign recurrent vestibulopathy: MRI and vestibular tests results in a series of 128 cases.

PURPOSE: Benign recurrent vestibulopathy is a recent entity, close to probable Meniere's disease and vestibular-migraine. So far, no study has systematically investigated the presence of endolymphatic hydrops of the lateral semicircular canal in benign recurrent vestibulopathy using magnetic resonance imaging. The aim of this study was to determine magnetic resonance imaging data and vestibular test results in patients with benign recurrent vestibulopathy. METHODS: 128 patients with benign recurrent vestibulopathy included since 2010 were retrospectively analyzed. Patients had magnetic resonance imaging with a delayed acquisition, audiogram, head shaking test, caloric-test, skull-vibration-induced-nystagmus-test, video-head- impulse-test, and vestibular evoked myogenic potential. Endolymphatic hydrops presence was classified into four categories: cochlear, saccular, lateral semicircular canal and association with saccule. RESULTS: In benign reccurent vestibulopathy, 23% of cases showed an endolymphatic hydrops on magnetic resonance imaging, more frequently located in the lateral semicircular canal (59%) and related to the disease duration. The most often impaired tests were caloric-test (49%) with fluctuations of hypofunction in 67% and skull-vibration-induced-nystagmus-test (61%). No correlation between the caloric-test and the presence and location of the endolymphatic hydrops was observed. CONCLUSION: In our series of benign reccurent vestibulopathy, a rare endolymphatic hydrops was most often observed for the lateral semicircular canal and correlated with the seniority of the pathology. Hydrops identified at the magnetic resonance imaging was not correlated with the caloric-test results. Skull-vibration-induced-nystagmus-test and caloric-test were the most often modified vestibular tests.

Cochlear Implantation and Other Treatments in Single-Sided Deafness and Asymmetric Hearing Loss: Results of a National Multicenter Study Including a Randomized Controlled Trial.

INTRODUCTION: Cochlear implantation is a recent approach proposed to treat single-sided deafness (SSD) and asymmetric hearing loss (AHL). Several cohort studies showed its effectiveness on tinnitus and variable results on binaural hearing. The main objective of this study is to assess the outcomes of cochlear implantation and other treatment options in SSD/AHL on quality of life. METHODS: This prospective multicenter study was conducted in 7 tertiary university hospitals and included an observational cohort study of SSD/AHL adult patients treated using contralateral routing of the signal (CROS) hearing aids or bone-anchored hearing systems (BAHSs) or who declined all treatments, and a randomized controlled trial in subjects treated by cochlear implantation, after failure of CROS and BAHS trials. In total, 155 subjects with SSD or AHL, with or without associated tinnitus, were enrolled. After 2 consecutive trials with CROS hearing aids and BAHSs on headband, all subjects chose any of the 4 treatment options (abstention, CROS, BAHS, or cochlear implant [CI]). The subjects who opted for a CI were randomized between 2 arms (CI vs. initial observation). Six months after the treatment choice, quality of life was assessed using both generic (EuroQoL-5D, EQ-5D) and auditory-specific quality-of-life indices (Nijmegen Cochlear implant Questionnaire [NCIQ] and Visual Analogue Scale [VAS] for tinnitus severity). Performances for speech-in-noise recognition and localization were measured as secondary outcomes. RESULTS: CROS was chosen by 75 subjects, while 51 opted for cochlear implantation, 18 for BAHSs, and 11 for abstention. Six months after treatment, both EQ-5D VAS and auditory-specific quality-of-life indices were significantly better in the "CI" arm versus "observation" arm. The mean effect of the CI was particularly significant in subjects with associated severe tinnitus (mean improvement of 20.7 points ± 19.7 on EQ-5D VAS, 20.4 ± 12.4 on NCIQ, and 51.4 ± 35.4 on tinnitus). No significant effect of the CI was found on binaural hearing results. Before/after comparisons showed that the CROS and BAHS also improved significantly NCIQ scores (for CROS: +7.7, 95% confidence interval [95% CI] = [4.5; 10.8]; for the BAHS: +14.3, 95% CI = [7.9; 20.7]). CONCLUSION: Cochlear implantation leads to significant improvements in quality of life in SSD and AHL patients, particularly in subjects with associated severe tinnitus, who are thereby the best candidates to an extension of CI indications.

Treatment choice in single-sided deafness and asymmetric hearing loss. A prospective, multicentre cohort study on 155 patients.

OBJECTIVES: To describe the treatment choice in a cohort of subjects with single-sided deafness (SSD) and asymmetric hearing loss (AHL). To assess the reliability of the treatment trials. DESIGN: In this national, multicentre, prospective study, the choice of subjects was made after two consecutive trials of Contralateral Routing Of the Signal (CROS) hearing aids and a Bone Conduction Device (BCD) on a headband. Subjects could proceed with one of these two options, opt for cochlear implantation or decline all treatments. SETTING: Seven tertiary university hospitals. PARTICIPANTS: One hundred fifty-five subjects with SSD or AHL fulfilling the candidacy criteria for cochlear implantation, with or without associated tinnitus. MAIN OUTCOME MEASURES: After the two trials, the number of subjects choosing each option was described. Repeated assessments of both generic and auditory-specific quality of life were conducted, as well as hearing assessments (speech recognition in noise and horizontal localization). RESULTS: CROS was chosen by 75 subjects, followed by cochlear implantation (n = 51), BCD (n = 18) and abstention (n = 11). Patients who opted for cochlear implantation had a poorer quality of life (P = .03). The improvement of quality of life indices after each trial was significantly associated with the final treatment choice (P = .008 for generic indices, P = .002 for auditory-specific indices). The follow-up showed that this improvement had been overestimated in the CROS group, with a long-term retention rate of 52.5%. CONCLUSIONS: More than one third of SSD/AHL subjects are unsatisfied after CROS and BCD trials. Repeated quality of life assessments help counselling the patient for his/her treatment choice.

Intratemporal facial nerve schwannomas: multicenter experience of 80 cases.

PURPOSE: To provide more data on the clinical presentation and natural evolution of facial nerve schwannomas and to provide guidance for therapeutic decision making. METHODS: A retrospective case review of eighty patients diagnosed with a facial nerve schwannoma between 1990 and 2018 in ten tertiary referral centers in Europe was performed. Patients' demographics, symptomatology, audiometry, anatomical site (segments involved), size and whenever possible volume measurement were registered. RESULTS: At presentation, transient or persistent facial palsy was the most common symptom, followed by hearing loss. The schwannoma involved more than one segment in the majority of the patients with the geniculate ganglion being most commonly involved. Initial treatment consisted of a wait and scan approach in 67.5%, surgery in 30% and radiation therapy in 2.5% of the patients. Tympanic segment schwannomas caused mainly conductive hearing loss and were more prone to develop facial palsy at follow-up. Internal auditory canal or cerebellopontine angle schwannomas presented with significantly more sensorineural hearing loss. CONCLUSIONS: Although modern imaging has improved diagnosis of this tumor, choosing the best treatment modality remains a real challenge. Based on the literature review and current findings, more insights into the clinical course and the management of facial nerve schwannomas are provided.

The vestibular aqueduct sign: Magnetic resonance imaging can detect abnormalities in both ears of patients with unilateral Meniere's disease.

BACKGROUND AND PURPOSE: In patients with Meniere's disease (MD), saccular hydrops can only be studied by magnetic resonance imaging (MRI) at a late stage when the disease is already responsible for moderate to severe hearing loss. However, these patients may also present vestibular aqueduct (VA) abnormalities. MATERIALS AND METHODS: In this prospective study (38RC14.428 for healthy subjects/38RC15.173 for patients), imaging was carried out on a 3T MRI scanner. Twenty healthy subjects (13 women, median age 53.5 [52.2-66.7]) and twenty MD patients (9 women, median age 54.5 [52-66.7]) had MRI scans with 3D-FLAIR sequences without injection, then 4 hours after a single intra-venous dose of contrast agent. Two radiologists independently ranked the morphology of the VA in the healthy subjects and in MD patients, using a three-level score (completely visible, discontinuous and not visible). Each subject was then graded, based on both the VA's appearance and on saccular hydrops presence. Inter-reader agreement tests were performed. RESULTS: In controls and patients, VA modifications were symmetrical without significant difference between the symptomatic and asymptomatic ears. The presence of at least one ear with discontinuous VA showed a correlation with clinical MD (P < 0.001) with a sensitivity of 90%. Ten patients had saccular hydrops, but only in the symptomatic ears. The evaluation of VA did not differ between MRI, both within MRI series or between the two radiologists (kappa without and with contrast agent = 0.9 and 0.92 respectively). CONCLUSION: Analysis of the vestibular aqueduct by MRI detects abnormalities in both ears of patients with unilateral MD.

Relationship Between Audio-Vestibular Functional Tests and Inner Ear MRI in Meniere's Disease.

OBJECTIVES: Meniere's disease is an inner ear disorder generally attributed to an endolymphatic hydrops. Different electrophysiological tests and imaging techniques have been developed to improve endolymphatic hydrops diagnosis. The goal of our study was to compare the sensitivity and the specificity of delayed inner ear magnetic resonance imaging (MRI) after intravenous injection of gadolinium with extratympanic clicks electrocochleography (EcochG), phase shift of distortion product otoacoustic emissions (shift-DPOAEs), and cervical vestibular-evoked myogenic potentials (cVEMP) for the diagnosis of Meniere's disease. DESIGN: Forty-one patients, with a total of 50 affected ears, were included prospectively from April 2015 to April 2016 in our institution. Patients included had definite or possible Meniere's disease based on the latest American Academy of Otolaryngology-Head and Neck Surgery guidelines revised in 2015. All patients went through delayed inner ear MRI after intravenous injection of gadolinium (three dimension-fluid attenuated inversion recovery sequences), pure-tone audiometry, extratympanic clicks EcochG, shift-DPOAEs, and cVEMP on the same day. Endolymphatic hydrops was graded on MRI using the saccule to utricle ratio inversion defined as when the saccule appeared equal or larger than the utricle. RESULTS: Abnormal EcochG and shift-DPOAEs in patients with definite Meniere's disease (DMD) were found in 68 and 64.5%, respectively. The two methods were significantly associated in DMD group. In DMD group, 25.7% had a positive MRI. The correlation between MRI versus EcochG and MRI versus shift-DPOAEs was not significant. MRI hydrops detection was correlated with hearing loss. Finally, 22.9% of DMD group had positive cVEMP. CONCLUSIONS: EcochG and shift-DPOAEs were both well correlated with clinical criteria of Meniere's disease. Inner ear MRI showed hydrops when hearing loss was higher than 35 dB. The shift-DPOAEs presented the advantage of a rapid and easy measurement if DPOAEs could be recorded (i.e., hearing threshold <60dB). In contrast, EcochG can be performed regardless of hearing loss. In combination with shift-DPOAEs, it enhances the chances to confirm the diagnosis with a better confidence.

In vivo imaging of saccular hydrops in humans reflects sensorineural hearing loss rather than Meniere's disease symptoms.

OBJECTIVES: A case-controlled imaging study demonstrated that saccular hydrops was specific to Meniere's disease (MD), but only present in a subset of patients. Here, we compared patients with definite MD, vertigo and sensorineural hearing loss (SNHL) to elucidate the relationship between saccular hydrops and extent of SNHL. METHODS: In this prospective study, we performed 3D-FLAIR sequences between 4.5 and 5.5 h after contrast media injection in patients with MD (n=20), SNHL (n=20), vertigo (n=20) and 30 healthy subjects. Two radiologists independently graded saccular hydrops. ROC analysis was performed to determine the hearing loss threshold to differentiate patients with saccular hydrops. RESULTS: Saccular hydrops was found in 11 of 20 MD patients, 10 of 20 SNHL patients and in none of the vertigo patients and healthy subjects. In SNHL patients, 45 dB was the threshold above which there was a significant association with saccular hydrops, with sensitivity of 100 % and specificity of 90 %. In MD patients, 40 dB was the threshold above which there was a significant association with saccular hydrops, with sensitivity of 100 % and specificity of 44 %. CONCLUSIONS: Our results indicate saccular hydrops as a feature of worse than moderate SNHL rather than MD itself. KEY POINTS: • MRI helps clinicians to assess patients with isolated low-tone sensorineural hearing loss. • Saccular hydrops correlates with sensorineural hearing loss at levels above 40 dB. • Vertigo patients without sensorineural hearing loss do not have saccular hydrops. • Saccular hydrops is described in patients without clinical diagnosis of Meniere's disease.

Safety, tolerability, pharmacokinetics and pharmacokinetic-pharmacodynamic modelling of the novel H4 receptor inhibitor SENS-111 using a modified caloric test in healthy subjects.

AIM: A Phase 1 study was performed to evaluate safety, pharmacokinetics (PK) and pharmacodynamics (PD) of the selective histamine H4 receptor antagonist SENS-111, an oral small molecule. METHODS: One hundred healthy subjects were randomized in a placebo-controlled, double-blind study evaluating single-ascending doses (SAD; 100-500 mg) and multiple-ascending doses (MAD; 50-150 mg day-1 , 4 days; 200-250 mg day-1 , 7 days). Effects of SENS-111 on nystagmus and vertigo induced by modified caloric tests were measured in the MAD studies. Population PK and PK/PD models were developed using a nonlinear mixed-effects approach. RESULTS: SENS-111 was well tolerated with mild to moderate events. No sedation was reported. A maximal tolerated dose was not reached. Dose-proportional increases in concentrations were seen up to 200 mg and more than dose-proportional thereafter, with mean half-life between 24 and 56 h. The caloric test induced mild but measurable vertigo and nystagmus with large intra/inter-individual variation for all parameters. SENS-111 did not significantly impact nystagmus but significantly improved latency of vertigo appearance/disappearance, duration and European Evaluation of Vertigo questionnaire parameters vs. baseline. A two-compartment model with first-order absorption, distribution and elimination best fit the data. PK/PD indirect modelling applied to vertigo duration and latency of appearance indicated maximum activity between 100 and 500 ng ml-1 plasma concentrations, corresponding to 100 and 200 mg day-1 , which are appropriate for clinical efficacy evaluations in vestibular diseases. CONCLUSIONS: SENS-111 is a well-tolerated first-in-class H4 receptor antagonist with acceptable PK for oral daily dosing. PK/PD modelling determined plasma concentrations and doses for efficacy studies in patients with vertigo symptoms.

MRI of endolymphatic hydrops in patients with Meniere's disease: a case-controlled study with a simplified classification based on saccular morphology.

OBJECTIVES: Endolymphatic hydrops (EH) can be studied in patients by MRI. With the semi-quantitative grading system, previous imaging studies showed discrepancies in the occurrence and grading of EH in patients with Meniere's disease (MD). Here, we compared the inversion of the saccule to utricle area ratio (SURI) with the semi-quantitative method of grading conventionally used to diagnose MD. METHODS: Imaging was carried out on a 3-T MRI scanner. We performed 3D-FLAIR sequences 4 h after a single intravenous dose of contrast agent. Two radiologists independently studied the morphology of the inner ear structures in the healthy subjects and MD patients. Each subject was then graded on the basis of the EH semi-quantitative analysis and on saccular morphology using axial and sagittal reference slices in the vestibule plane. RESULTS: Thirty healthy subjects and 30 MD patients had MRI scans. Using the semi-quantitative method, we found no significant difference in the number of subjects with EH between the two groups. SURI was found in 15 out of 30 MD patients and in none of the 30 healthy subjects. In three MD patients the saccule was not visible. CONCLUSION: SURI is currently the most specific criterion for imaging diagnosis of MD. KEY POINTS: • Half of MD patients presented with inversion of the saccule to utricle ratio. • Saccular analysis is crucial when assessing patients with Meniere's disease. • In some patients, the saccule is not visible, suggestive of intra-labyrinthine fistulae.

Safety and effectiveness of the Bonebridge transcutaneous active direct-drive bone-conduction hearing implant at 1-year device use.

The objective of this study is to evaluate the safety and efficacy of a new transcutaneous bone-conduction implant (BCI BB) in patients with conductive and mixed hearing loss or with single-sided deafness (SSD), 1 year after surgical implantation. The study design is multicentric prospective, intra-subject measurements. Each subject is his/her own control. The setting is nine university hospitals: 7 French and 2 Belgian. Sixteen subjects with conductive or mixed hearing loss with bone-conduction hearing thresholds under the upper limit of 45 dB HL for each frequency from 500 to 4000 Hz, and 12 subjects with SSD (contralateral hearing within normal range) were enrolled in the study. All subjects were older than 18 years. The intervention is rehabilitative. The main outcome measure is the evaluation of skin safety, audiological measurements, benefit, and satisfaction questionnaires with a 1-year follow up. Skin safety was rated as good or very good. For the mixed or conductive hearing loss groups, the average functional gain (at 500 Hz, 1, 2, 4 kHz) was 26.1 dB HL (SD 13.7), and mean percentage of speech recognition in quiet at 65 dB was 95 % (vs 74 % unaided). In 5/6 SSD subjects, values of SRT in noise were lower with BB. Questionnaires revealed patient benefit and satisfaction. The transcutaneous BCI is very well tolerated at 1-year follow up, improves audiometric thresholds and intelligibility for speech in quiet and noise, and gives satisfaction to both patients with mixed and conductive hearing loss and patients with SSD.

The relationship between post-traumatic ossicular injuries and conductive hearing loss: A 3D-CT study.

PURPOSE: After a trauma, the conductive ossicular chain may be disrupted by ossicular luxation or fracture. Recent developments in 3D-CT allow a better understanding of ossicular injuries. In this retrospective study, we compared patients with post-traumatic conductive hearing loss (CHL) with those referred without CHL to evaluate the relationship between ossicular injuries and CHL. We also assessed the added value of 3D reconstructions on 2D-CT scan to detect ossicular lesions in patients surgically managed. METHODS: The CT scans were performed using a 40-section spiral CT scanner in 49 patients with post-traumatic CHL (n=29) and without CHL (n=20). Three radiologists performed independent blind evaluations of 2D-CT and 3D reconstructions to detect ossicular chain injury. We used the t-test to explore differences regarding the number of subjects with ossicular injury in the two groups. We also estimated the diagnostic accuracy and the inter-rater agreement of the 3D-CT reconstructions associated to 2D-CT scan. RESULTS: We identified ossicular abnormality in 14 patients out of 29 and in one patient out of 20 in the CHL and non-CHL groups respectively. There was a significant difference regarding the number of subjects with ossicular lesions between the two groups (P≤0.01). The diagnostic sensitivity of 3D-CT reconstructions associated with 2D-CT ranged from 66% to 100% and the inter-reader agreement ranged from 0.85 to 1, depending of the type of lesion. CONCLUSION: The relationship between ossicular lesion and the presence of CHL tightly correlated. 3D-CT reconstructions of the temporal bone are useful to assess patients in a post-traumatic context.

Realisation of voicing by French-speaking CI children after long-term implant use: An acoustic study.

Studies of speech production in French-speaking cochlear-implanted (CI) children are very scarce. Yet, difficulties in speech production have been shown to impact the intelligibility of these children. The goal of this study is to understand the effect of long-term use of cochlear implant on speech production, and more precisely on the coordination of laryngeal-oral gestures in stop production. The participants were all monolingual French children: 13 6;6- to 10;7-year-old CI children and 20 age-matched normally hearing (NH) children. We compared /p/, /t/, /k/, /b/, /d/ and /g/ in word-initial consonant-vowel sequences, produced in isolation in two different tasks, and we studied the effects of CI use, vowel context, task and age factors (i.e. chronological age, age at implantation and duration of implant use). Statistical analyses show a difference in voicing production between groups for voiceless consonants (shorter Voice Onset Times for CI children), with significance reached only for /k/, but no difference for voiced consonants. Our study indicates that in the long run, use of CI seems to have limited effects on the acquisition of oro-laryngeal coordination needed to produce voicing, except for specific difficulties located on velars. In a follow-up study, further acoustic analyses on vowel and fricative production by the same children reveal more difficulties, which suggest that cochlear implantation impacts frequency-based features (second formant of vowels and spectral moments of fricatives) more than durational cues (voicing).

Vestibular results after intratympanic gentamicin therapy in disabling Menière's disease.

Intratympanic injection of gentamicin is increasingly used in the treatment of unilateral disabling Menière's disease (MD). Several objective functional and subjective tests have been developed to assess the control of vertigo after gentamicin treatment. The aim of this study was to show that subjective results require a vestibular deafferentation as profound as possible, evidenced with multifrequency vestibular assessment. Sixty four patients with intractable MD in situation of medical treatment failure longer than 6 months were included between 1998 and 2013 in this case control study. A 2-year follow-up was performed after the last intratympanic gentamicin performed with the titration technique. A vestibular assessment was applied before and after 2 years of treatment with a functional level score using the AAOHNS vertigo scale and multifrequency vestibular assessment: skull vibration-induced nystagmus test (SVINT), head-shaking test (HST) and caloric test (CaTe). The correlation between the results of the questionnaire and the level of the deafferentation as evaluated by the tests was analyzed with the Spearman test. Among the 64 included patients, 56 (87.5 %) described vertigo control. There was a correlation (=-0.33 [-0.53; -0.09], p = 0.008) between subjective improvement (AAO -HNS 1 or 2) and the degree of vestibular deafferentation as evidenced by a destructive nystagmus (beating toward the safe side) with the HST and the SVINT, as well as a caloric hypofunction >90 % with the CaTe. The present study demonstrates that a profound vestibular deafferentation confirmed with multifrequency test evaluation is needed to have a subjective improvement in the treatment of unilateral disabling MD with intratympanic gentamicin.

Skull Vibration-Induced Nystagmus in Superior Semicircular Canal Dehiscence: A New Insight into Vestibular Exploration-A Review.

The third window syndrome, often associated with the Tullio phenomenon, is currently most often observed in patients with a superior semicircular-canal dehiscence (SCD) but is not specific to this pathology. Clinical and vestibular tests suggestive of this pathology are not always concomitantly observed and have been recently complemented by the skull-vibration-induced nystagmus test, which constitutes a bone-conducted Tullio phenomenon (BCTP). The aim of this work was to collect from the literature the insights given by this bedside test performed with bone-conducted stimulations in SCD. The PRISMA guidelines were used, and 10 publications were included and analyzed. Skull vibration-induced nystagmus (SVIN), as observed in 55 to 100% of SCD patients, usually signals SCD with greater sensitivity than the air-conducted Tullio phenomenon (ACTP) or the Hennebert sign. The SVIN direction when the test is performed on the vertex location at 100 Hz is most often ipsilaterally beating in 82% of cases for the horizontal and torsional components and down-beating for the vertical component. Vertex stimulations are more efficient than mastoid stimulations at 100 Hz but are equivalent at higher frequencies. SVIN efficiency may depend on stimulus location, order, and duration. In SCD, SVIN frequency sensitivity is extended toward high frequencies, with around 400 Hz being optimal. SVIN direction may depend in 25% on stimulus frequency and in 50% on stimulus location. Mastoid stimulations show frequently diverging results following the side of stimulation. An after-nystagmus observed in 25% of cases can be interpreted in light of recent physiological data showing two modes of activation: (1) cycle-by-cycle phase-locked activation of action potentials in SCC afferents with irregular resting discharge; (2) cupula deflection by fluid streaming caused by the travelling waves of fluid displacement initiated by sound or vibration at the point of the dehiscence. The SVIN direction and intensity may result from these two mechanisms' competition. This instability explains the SVIN variability following stimulus location and frequency observed in some patients but also discrepancies between investigators. SVIN is a recent useful insight among other bedside examination tests for the diagnosis of SCD in clinical practice.

Posterior Semicircular Canal Dehiscence with Vestibulo-Ocular Reflex Reduction for the Affected Canal at the Video-Head Impulse Test: Considerations to Pathomechanisms.

Posterior semicircular canal dehiscence (PSCD) has been demonstrated to result in a third mobile window mechanism (TMWM) in the inner ear similar to superior semicircular canal dehiscence (SSCD). Typical clinical and instrumental features of TMWM, including low-frequency conductive hearing loss (CHL), autophony, pulsatile tinnitus, sound/pressure-induced vertigo and enhanced vestibular-evoked myogenic potentials, have been widely described in cases with PSCD. Nevertheless, video-head impulse test (vHIT) results have been poorly investigated. Here, we present six patients with PSCD presenting with a clinical scenario consistent with a TMWM and an impaired vestibulo-ocular reflex (VOR) for the affected canal on vHIT. In two cases, an additional dehiscence between the facial nerve and the horizontal semicircular canal (HSC) was detected, leading to a concurrent VOR impairment for the HSC. While in SSCD, a VOR gain reduction could be ascribed to a spontaneous "auto-plugging" process due to a dural prolapse into the canal, the same pathomechanism is difficult to conceive in PSCD due to a different anatomical position, making a dural herniation less likely. Alternative putative pathomechanisms are discussed, including an endolymphatic flow dissipation during head impulses as already hypothesized in SSCD. The association of symptoms/signs consistent with TMWM and a reduced VOR gain for the posterior canal might address the diagnosis toward PSCD.

A bone-conducted Tullio phenomenon-A bridge to understand skull vibration induced nystagmus in superior canal dehiscence.

Nystagmus produced in response to air-conducted sound (ACS) stimulation-the Tullio phenomenon-is well known in patients with a semicircular canal (SCC) dehiscence (SCD). Here we consider the evidence that bone-conducted vibration (BCV) is also an effective stimulus for generating the Tullio phenomenon. We relate the clinical evidence based on clinical data extracted from literature to the recent evidence about the physical mechanism by which BCV may cause this nystagmus and the neural evidence confirming the likely mechanism. The hypothetical physical mechanism by which BCV activates SCC afferent neurons in SCD patients is that traveling waves are generated in the endolymph, initiated at the site of the dehiscence. We contend that the nystagmus and symptoms observed after cranial BCV in SCD patients is a variant of Skull Vibration Induced Nystagmus (SVIN) used to identify unilateral vestibular loss (uVL) with the major difference being that in uVL the nystagmus beats away from the affected ear whereas in Tullio to BCV the nystagmus beats usually toward the affected ear with the SCD. We suggest that the cause of this difference is a cycle-by-cycle activation of SCC afferents from the remaining ear, which are not canceled centrally by simultaneous afferent input from the opposite ear, because of its reduced or absent function in uVL. In the Tullio phenomenon, this cycle-by-cycle neural activation is complemented by fluid streaming and thus cupula deflection caused by the repeated compression of each cycle of the stimuli. In this way, the Tullio phenomenon to BCV is a version of skull vibration-induced nystagmus.

Correlation Between Cochlear Length, Insertion Angle, and Tonotopic Mismatch for MED-EL FLEX28 Electrode Arrays.

OBJECTIVE: To investigate the relationship between cochlear length, insertion angle, and tonotopic mismatch and to compare the tonotopic mismatches with respect to the spiral ganglion and the organ of Corti. STUDY DESIGN: Retrospective. SETTING: Tertiary referral center with cochlear implant program. PATIENTS: Analyses of patients' computed tomography images after cochlear implant surgery. INTERVENTION: Cochlear implantation with 28-mm-long straight lateral wall electrode arrays. MAIN OUTCOME MEASURE: Cochlear length, insertion angle, and insertion depth were assessed using the OTOPLAN software. Tonotopic mismatch for each electrode contact was estimated using the Greenwood (organ of Corti) and the Stakhovskaya (spiral ganglion) maps and compared. RESULTS: 106 cochleae were analyzed. 99% of the electrode arrays were located in the tympanic ramp. The insertion was complete in 96% of cases. The mean cochlear length was 34.5 mm and the mean insertion angle of the apical electrode was 545°. Cochlear length was negatively correlated with the insertion angle of the contacts E1 to E9 (all p < 0.004). The tonotopic mismatch was greater at the organ of Corti than at the spiral ganglion. It was also greater at the organ of Corti in larger cochleae (correlation with mismatch for E1 r = 0.421, p < 0.0001) and in the apical than in the middle and basal regions of the cochlea. CONCLUSION: Small cochlea size corresponded to higher insertion angle and reduction of tonotopic mismatch on a 28-mm-long straight lateral wall electrode array. Tonotopic mismatch could be minimized preoperatively by choosing electrode arrays according to the individual cochlear morphology and postoperatively by appropriate frequency fitting.