MRI-confirmed cochlear artery infarct clinically diagnosed in a patient with sickle cell disease: a case report.

PURPOSE: To corroborate the vascular etiology of sudden sensorineural hearing loss (SNHL) utilizing magnetic resonance imaging (MRI). PATIENT: A 24-year-old male with a history of sickle cell disease experienced sudden SNHL and right horizontal nystagmus, without accompanying vertigo. INTERVENTION: Audiometric evaluation revealed left-sided SNHL, predominantly affecting high frequencies. Video head impulse testing demonstrated isolated dysfunction of the left posterior semicircular canal. An urgent brain MRI identified a recent punctiform ischemic stroke in the frontal region. A subsequent MRI, conducted with a 4-hour delay and post-contrast enhancement, highlighted a hyperintense signal within the left cochlear region and the left posterior semicircular canal. CONCLUSION: The investigative results substantiate an infarction in the territory of the cochlear artery, precipitated by a vaso-occlusive event, thereby reinforcing the vascular hypothesis of cochleovestibular artery syndrome. This case underscores the congruence between clinical observations and delayed post-contrast MRI findings.

Development of a Semi-Automated Approach for the Quantification of Neuronal Cells in the Spiral Ganglion of the Whole Implanted Gerbil Cochlea, Acquired by Light-Sheet Microscopy.

INTRODUCTION: Assessing cochlear implantation's impact on cell loss and preventing post-implant cochlear damage are key areas of focus for hearing preservation research. The preservation of auditory neuronal and sensory neural hearing cells has a positive impact on auditory perception after implantation. This study aimed to provide details on a semi-automated spiral ganglion neuronal cell counting method, developed using whole implanted gerbil cochlea acquisitions with light-sheet microscopy. METHODS: Mongolian gerbils underwent right cochlear implantation with an electrode array whose silicone was loaded with dexamethasone or not and were euthanized 10 weeks after implantation. The cochleae were prepared according to a 29-day protocol, with the electrode array in place. Light-sheet microscopy was used for acquisition, and Imaris software was employed for three-dimensional analysis of the cochleas and semi-automatic quantification of spiral ganglion cells. The imaJ software was used for the manual quantification of these cells. RESULTS: Six cochleae were acquired by light-sheet microscopy, allowing good identification of cells. There was no significant difference between the mean number of spiral ganglion cells obtained by manual and semi-automatic counting (p = 0.25). CONCLUSION: Light-sheet microscopy provided complete visualization of the spiral ganglion and cell identification. The semi-automated counting method developed using Imaris software tools proved reliable and efficient and could be applied to a larger sample to assess post-cochlear implant cell damage and the efficacy of protective drugs delivered to the inner ear.

Reference data for a quick speech-in-noise hearing test in the French language.

INTRODUCTION: Difficulties in understanding speech in noise is the most common complaint of people with hearing impairment. Thus, there is a need for tests of speech-in-noise ability in clinical settings, which have to be evaluated for each language. Here, a reference dataset is presented for a quick speech-in-noise test in the French language (Vocale Rapide dans le Bruit, VRB; Leclercq, Renard & Vincent, 2018). METHODS: A large cohort (N=641) was tested in a nationwide multicentric study. The cohort comprised normal-hearing individuals and individuals with a broad range of symmetrical hearing losses. Short everyday sentences embedded in babble noise were presented over a spatial array of loudspeakers. Speech level was kept constant while noise level was progressively increased over a range of signal-to-noise ratios. The signal-to-noise ratio for which 50% of keywords could be correctly reported (Speech Reception Threshold, SRT) was derived from psychometric functions. Other audiometric measures were collected for the cohort, such as audiograms and speech-in-quiet performance. RESULTS: The VRB test was both sensitive and reliable, as shown by the steep slope of the psychometric functions and by the high test-retest consistency across sentence lists. Correlation analyses showed that pure tone averages derived from the audiograms explained 74% of the SRT variance over the whole cohort, but only 29% for individuals with clinically normal audiograms. SRTs were then compared to recent guidelines from the French Society of Audiology (Joly et al., 2021). Among individuals who would not have qualified for hearing aid prescription based on their audiogram or speech intelligibility in quiet, 18.4% were now eligible as they displayed SRTs in noise impaired by 3 dB or more. For individuals with borderline audiograms, between 20 dB HL and 30 dB HL, the prevalence of impaired SRTs increased to 71.4%. Finally, even though five lists are recommended for clinical use, a minute-long screening using only one VRB list detected 98.6% of impaired SRTs. CONCLUSION: The reference data suggest that VRB testing can be used to identify individuals with speech-in-noise impairment.

Hearing Outcomes from Gamma Knife Treatment for Intracanalicular Vestibular Schwannomas with Good Initial Hearing.

Background: The objective of this study was to describe the long-term hearing outcomes of gamma knife treatment for unilateral progressing vestibular schwannomas (VS) presenting with good initial hearing using audiologic data. Methods: A retrospective review was performed between 2010 and 2020 to select patients with progressing unilateral VS and good hearing (AAO-HNS class A) treated with stereotactic gamma knife surgery (GKS). Their audiograms were analyzed along with treatment metrics and patient data. Results: Hearing outcomes with a median follow-up of 5 years post-treatment showed statistically significant loss of serviceable hearing: 34.1% of patients maintained good hearing (AAO-HNS class A), and 56.1% maintained serviceable hearing (AAO-HNS class A and B). Non-hearing outcomes are favorable with excellent tumor control and low facial nerve morbidity. Conclusions: Hearing declines over time in intracanalicular VS treated with GKS, with a significant loss of serviceable hearing after 5 years. The mean cochlear dose and the presence of cochlear aperture obliteration by the tumor are the main statistically significant factors involved in the hearing outcomes.

Early management of presbycusis: recommendations from the French Society of Otorhinolaryngology and Head and Neck Surgery, the French Society of Audiology, and the French Society of Geriatrics and Gerontology.

INTRODUCTION: Presbycusis is the physiological decrease in hearing due to advancing age and begins well before the sixth decade. These recommendations recall the principles of early diagnosis of presbycusis and the means of optimal rehabilitation as soon as the first symptoms appear. MATERIAL AND METHODS: The recommendations are based on a systematic analysis of the literature carried out by a multidisciplinary group of ENT physicians, audiologists, geriatricians and hearing specialists from all over France. They are classified as grade A, B, C or professional agreement according to a decreasing level of scientific evidence. RESULTS: The diagnosis of presbycusis is more difficult at the beginning of its evolution but a certain number of tools are available for its early diagnosis and its face-to-face or remote management. CONCLUSION: In the case of a clinical profile suggestive of presbycusis in a young subject, especially if there are several family cases, it is recommended to propose a genetic investigation. Free-field speech audiometry in noise is recommended to measure intelligibility in a realistic environment. Questionnaires in addition to audiometric tests would allow the best assessment of the patient's disability. Hearing rehabilitation with a hearing aid or cochlear implant may slow or prevent cognitive decline. Combined auditory and cognitive rehabilitation should be offered regardless of the time since the hearing was fitting. It is recommended to integrate programs accessible via smartphones, tablets or the Internet, that include different training domains to complement face-to-face sessions.

[Early management of presbycusis: recommendations from the French Society of Otorhinolaryngology and Head and Neck Surgery, the French Society of Audiology, and the French Society of Geriatrics and Gerontology]. / Recommandations de la Société française d'otorhinolaryngologie et de chirurgie de la face et du cou, la Société française d'audiologie et la Société française de gériatrie et gérontologie pour la prise en charge précoce de la presbyacousie.

INTRODUCTION: Presbycusis is the physiological decrease in hearing due to advancing age and begins well before the sixth decade. These recommendations recall the principles of early diagnosis of presbycusis and the means of optimal rehabilitation as soon as the first symptoms appear. MATERIAL AND METHODS: The recommendations are based on a systematic analysis of the literature carried out by a multidisciplinary group of doctors and audioprosthetists from all over France. They are graded A, B, C or expert opinion according to decreasing level of scientific evidence. RESULTS: The diagnosis of presbycusis is more difficult at the beginning of its evolution but a certain number of tools are available for its early diagnosis and its management in face-to-face or even distance learning. CONCLUSION: In case of a clinical profile suggestive of presbycusis in a young subject, especially if there are several family cases, it is recommended to propose a genetic investigation. It is recommended to perform free-field speech audiometry in noise to measure intelligibility in an environment as close as possible to reality. Questionnaires can be used in addition to audiometry to best assess the patient's disability. It is recommended that hearing rehabilitation with a hearing aid or cochlear implant may slow or prevent cognitive decline. Combined auditory and cognitive rehabilitation should be offered regardless of the time elapsed since the fitting. It is recommended to integrate programs accessible via smartphones, tablets or the Internet, integrating different training domains in addition to face-to-face sessions.

Silicone matrices for controlled dexamethasone release: toward a better understanding of the underlying mass transport mechanisms.

Dexamethasone-loaded silicone matrices offer an interesting potential as innovative drug delivery systems, e.g. for the treatment of inner ear diseases or for pacemakers. Generally, very long drug release periods are targeted: several years/decades. This renders the development and optimization of novel drug products cumbersome: experimental feedback on the impact of the device design is obtained very slowly. A better understanding of the underlying mass transport mechanisms can help facilitating research in this field. A variety of silicone films were prepared in this study, loaded with amorphous or crystalline dexamethasone. Different polymorphic drug forms were investigated, the film thickness was altered and the drug optionally partially/completely exchanged by much more water-soluble dexamethasone 'phosphate'. Drug release studies in artificial perilymph, scanning electron microscopy, optical microscopy, differential scanning calorimetry, X-ray diffraction and Raman imaging were used to elucidate the physical states of the drugs and polymer, and of the systems' structure as well as dynamic changes thereof upon exposure to the release medium. Dexamethasone particles were initially homogeneously distributed throughout the systems. The hydrophobicity of the matrix former very much limits the amounts of water penetrating into the system, resulting in only partial drug dissolution. The mobile drug molecules diffuse out into the surrounding environment, due to concentration gradients. Interestingly, Raman imaging revealed that even very thin silicone layers (<20 µm) can effectively trap the drug for prolonged periods of time. The physical state of the drug (amorphous, crystalline) did not affect the resulting drug release kinetics to a noteworthy extent.

One Year Assessment of the Hearing Preservation Potential of the EVO Electrode Array.

BACKGROUND: A prospective longitudinal multicentre study was conducted to assess the one-year postsurgical hearing preservation profile of the EVOTM electrode array. METHODS: Fifteen adults presenting indications of electro-acoustic stimulation (pure-tone audiometry (PTA) thresholds ≤70 dB below 750 Hz) were implanted with the EVO™ electrode array. Hearing thresholds were collected at five time-points from CI activation to twelve months (12M) after activation. Hearing thresholds and hearing preservation profiles (HEARRING group classification) were assessed. RESULTS: All subjects had measurable hearing thresholds at follow-up. No case of complete loss of hearing or minimal hearing preservation was reported at any time point. At activation (Nact = 15), five participants had complete hearing preservation, and ten participants had partial hearing preservation. At the 12M time point (N12m = 6), three participants had complete hearing preservation, and three participants had partial hearing preservation. Mean hearing loss at activation was 11 dB for full range PTA and 25 dB for PTAs low-frequency (125-500 Hz). CONCLUSIONS: This study provides the first longitudinal follow-up on associated hearing profiles to the EVO™ electrode array, which are comparable to the literature. However, other studies on larger populations should be performed.

Evaluation of the Efficacy of Dexamethasone-Eluting Electrode Array on the Post-Implant Cochlear Fibrotic Reaction by Three-Dimensional Immunofluorescence Analysis in Mongolian Gerbil Cochlea.

Cochlear implant is the method of choice for the rehabilitation of severe to profound sensorineural hearing loss. The study of the tissue response to cochlear implantation and the prevention of post-cochlear-implant damages are areas of interest in hearing protection research. The objective was to assess the efficacy of dexamethasone-eluting electrode array on endo canal fibrosis formation by three-dimensional immunofluorescence analysis in implanted Mongolian gerbil cochlea. Two trials were conducted after surgery using Mongolian gerbil implanted with dexamethasone-eluting or non-eluting intracochlear electrode arrays. The animals were then euthanised 10 weeks after implantation. The cochleae were prepared (electrode array in place) according to a 29-day protocol with immunofluorescent labelling and tissue clearing. The acquisition was carried out using light-sheet microscopy. Imaris software was then used for three-dimensional analysis of the cochleae and quantification of the fibrotic volume. The analysis of 12 cochleae showed a significantly different mean volume of fibrosis (2.16 × 108 µm3 ± 0.15 in the dexamethasone eluting group versus 3.17 × 108 µm3 ± 0.54 in the non-eluting group) (p = 0.004). The cochlear implant used as a corticosteroid delivery system appears to be an encouraging device for the protection of the inner ear against fibrosis induced by implantation. Three-dimensional analysis of the cochlea by light-sheet microscopy was suitable for studying post-implantation tissue damage.

Adult Users of the Oticon Medical Neuro Cochlear Implant System Benefit from Beamforming in the High Frequencies.

The Oticon Medical Neuro cochlear implant system includes the modes Opti Omni and Speech Omni, the latter providing beamforming (i.e., directional selectivity) in the high frequencies. Two studies compared sentence identification scores of adult cochlear implant users with Opti Omni and Speech Omni. In Study 1, a double-blind longitudinal crossover study, 12 new users trialed Opti Omni or Speech Omni (random allocation) for three months, and their sentence identification in quiet and noise (+10 dB signal-to-noise ratio) with the trialed mode were measured. The same procedure was repeated for the second mode. In Study 2, a single-blind study, 11 experienced users performed a speech identification task in quiet and at relative signal-to-noise ratios ranging from -3 to +18 dB with Opti Omni and Speech Omni. The Study 1 scores in quiet and in noise were significantly better with Speech Omni than with Opti Omni. Study 2 scores were significantly better with Speech Omni than with Opti Omni at +6 and +9 dB signal-to-noise ratios. Beamforming in the high frequencies, as implemented in Speech Omni, leads to improved speech identification in medium levels of background noise, where cochlear implant users spend most of their day.

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.

The Development of the "Telislife" Questionnaire for the Evaluation of Telephone Use in Cochlear Implant Users.

Purpose For cochlear implant users, the ability to use the telephone is often seen as an important landmark during rehabilitation and an indicator of cochlear implant benefit. The goal of this study was to develop a short questionnaire exploring the ability to use the telephone in cochlear implant users, named Telislife, and test it in a group of experienced users. Method This prospective multicenter study was based on the completion of self-administrated questionnaires. The Telislife includes 20 items using a 5-point Likert scale for answers. Speech recognition scores were obtained with monosyllabic word lists at 70 dB HL. Quality of life was evaluated with the Nijmegen Cochlear Implant Questionnaire. This study included 55 adult patients wearing a cochlear implant for over 1 year. Results The Telislife questionnaire showed excellent reliability (Cronbach's α = .91). A significant correlation was found between Telislife scores and Nijmegen Cochlear Implant Questionnaire scores (r = .69, p < .001) and speech recognition scores (r = .35, p = .007). Conclusion Given significant correlations between Telislife scores and both speech recognition and quality of life and given its short form, the Telislife questionnaire appears to be a reliable tool to evaluate cochlear implant outcomes in clinical practice. Supplemental Material https://doi.org/10.23641/asha.13322873.

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.

Ultrarare heterozygous pathogenic variants of genes causing dominant forms of early-onset deafness underlie severe presbycusis.

Presbycusis, or age-related hearing loss (ARHL), is a major public health issue. About half the phenotypic variance has been attributed to genetic factors. Here, we assessed the contribution to presbycusis of ultrarare pathogenic variants, considered indicative of Mendelian forms. We focused on severe presbycusis without environmental or comorbidity risk factors and studied multiplex family age-related hearing loss (mARHL) and simplex/sporadic age-related hearing loss (sARHL) cases and controls with normal hearing by whole-exome sequencing. Ultrarare variants (allele frequency [AF] < 0.0001) of 35 genes responsible for autosomal dominant early-onset forms of deafness, predicted to be pathogenic, were detected in 25.7% of mARHL and 22.7% of sARHL cases vs. 7.5% of controls (P = 0.001); half were previously unknown (AF < 0.000002). MYO6, MYO7A, PTPRQ, and TECTA variants were present in 8.9% of ARHL cases but less than 1% of controls. Evidence for a causal role of variants in presbycusis was provided by pathogenicity prediction programs, documented haploinsufficiency, three-dimensional structure/function analyses, cell biology experiments, and reported early effects. We also established Tmc1N321I/+ mice, carrying the TMC1:p.(Asn327Ile) variant detected in an mARHL case, as a mouse model for a monogenic form of presbycusis. Deafness gene variants can thus result in a continuum of auditory phenotypes. Our findings demonstrate that the genetics of presbycusis is shaped by not only well-studied polygenic risk factors of small effect size revealed by common variants but also, ultrarare variants likely resulting in monogenic forms, thereby paving the way for treatment with emerging inner ear gene therapy.

Unilateral Cochlear Implants for Severe, Profound, or Moderate Sloping to Profound Bilateral Sensorineural Hearing Loss: A Systematic Review and Consensus Statements.

Importance: Cochlear implants are a treatment option for individuals with severe, profound, or moderate sloping to profound bilateral sensorineural hearing loss (SNHL) who receive little or no benefit from hearing aids; however, cochlear implantation in adults is still not routine. Objective: To develop consensus statements regarding the use of unilateral cochlear implants in adults with severe, profound, or moderate sloping to profound bilateral SNHL. Design, Setting, and Participants: This study was a modified Delphi consensus process that was informed by a systematic review of the literature and clinical expertise. Searches were conducted in the following databases: (1) MEDLINE In-Process & Other Non-Indexed Citations and Ovid MEDLINE, (2) Embase, and (3) the Cochrane Library. Consensus statements on cochlear implantation were developed using the evidence identified. This consensus process was relevant for the use of unilateral cochlear implantation in adults with severe, profound, or moderate sloping to profound bilateral SNHL. The literature searches were conducted on July 18, 2018, and the 3-step Delphi consensus method took place over the subsequent 9-month period up to March 30, 2019. Main Outcomes and Measures: A Delphi consensus panel of 30 international specialists voted on consensus statements about cochlear implantation, informed by an SR of the literature and clinical expertise. This vote resulted in 20 evidence-based consensus statements that are in line with clinical experience. A modified 3-step Delphi consensus method was used to vote on and refine the consensus statements. This method consisted of 2 rounds of email questionnaires and a face-to-face meeting of panel members at the final round. All consensus statements were reviewed, discussed, and finalized at the face-to-face meeting. Results: In total, 6492 articles were identified in the searches of the electronic databases. After removal of duplicate articles, 74 articles fulfilled all of the inclusion criteria and were used to create the 20 evidence-based consensus statements. These 20 consensus statements on the use of unilateral cochlear implantation in adults with SNHL were relevant to the following 7 key areas of interest: level of awareness of cochlear implantation (1 consensus statement); best practice clinical pathway from diagnosis to surgery (3 consensus statements); best practice guidelines for surgery (2 consensus statements); clinical effectiveness of cochlear implantation (4 consensus statements); factors associated with postimplantation outcomes (4 consensus statements); association between hearing loss and depression, cognition, and dementia (5 consensus statements); and cost implications of cochlear implantation (1 consensus statement). Conclusions and Relevance: These consensus statements represent the first step toward the development of international guidelines on best practices for cochlear implantation in adults with SNHL. Further research to develop consensus statements for unilateral cochlear implantation in children, bilateral cochlear implantation, combined electric-acoustic stimulation, unilateral cochlear implantation for single-sided deafness, and asymmetrical hearing loss in children and adults may be beneficial for optimizing hearing and quality of life for these patients.

The round window sign: a sensitive sign to detect perilymphatic fistulae on delayed postcontrast 3D-FLAIR sequence.

OBJECTIVES: The aim of this study is to assess the diagnostic performance of a new MR sign, named the round window sign (RWS), to diagnose perilymphatic fistula (PLF) in a population of patients with chronic cochleo-vestibular symptoms, classified as definite or probable Menière's disease (MD). METHODS: A total of 164 patients (mean age 52 ± 35 years) with chronic cochleo-vestibular symptoms underwent MRI, between 4 and 5 h after intravenous gadoteric acid injection (Dotarem®, 0.1 mmol/kg). MRI exploration was carried out on a 3-T Achieva® TX scanner. We analyzed the presence of the RWS, defined as a nodular FLAIR high signal in the round window (RW) and the presence of associated saccular hydrops. When this RWS was present, a temporal bone CT scan was performed and the RW was analyzed. RESULTS: Of the 164 patients with definite MD (85 patients) or probable MD (79 patients), we found the RWS in 18 (11%), and 17/18 were classified into the group of probable MD. All these 18 patients showed other MR sequences considered as normal, including heavily weighted T2 imaging. Among these 18 patients, the temporal bone CT examination presented a filling of the RW in 13 patients (72%) and no filling of the RW in 5 patients (28%). Seven patients were surgically managed confirming in vivo the PLF diagnosis. The RWS was associated with the presence of a saccular hydrops in 4 cases. CONCLUSION: Delayed postcontrast 3D-FLAIR may reveal perilymphatic fistulae in patients with probable Menière's disease using the round window sign. KEY POINTS: • MRI with delayed acquisition can detect perilymphatic fistulae with perfect sensitivity, based on the presence of the round window sign. • This visual sign is only visible on a 3D-FLAIR sequence. • 3D-FLAIR sequence with delayed acquisition is more sensitive than temporal bone CT scan examination in detecting PLF.

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.

Prospective Multicentric Follow-up Study of Cochlear Implantation in Adults With Single-Sided Deafness: Tinnitus and Audiological Outcomes.

OBJECTIVE: This study investigated the audiological and tinnitus outcomes of cochlear implantation (CI) in adults with single-sided deafness (SSD) and tinnitus. STUDY DESIGN: Multicentered prospective, non-randomized intervention study. SETTING: Six French CI centers. PATIENTS: Twenty-six patients with SSD and incapacitating tinnitus (Tinnitus Handicap Inventory [THI] >58) underwent cochlear implantation. INTERVENTIONS: First, CIs delivered only masking white noise stimulation for 1 month and then standard CI stimulation. MAIN OUTCOME MEASURES: Before and after CI surgery, patients completed the THI, Tinnitus Reaction Questionnaire (TRQ), Subjective Tinnitus Severity Scale (STSS), and two visual analogue scales quantifying tinnitus loudness and annoyance. Speech perception in spatialized noise was tested at 13 months. RESULTS: The first month of white noise stimulation triggered a significant improvement in THI scores (72 ±â€Š9 to 55 ±â€Š20, p < 0.05). No change was observed for the other measures. After 1 year of standard CI stimulation, 23 patients (92%) reported a significant improvement in tinnitus. This improvement started 1 to 2 months after CI and exceeded 40% improvement for 14 patients (54%). Average speech-in-noise perception after 1 year significantly improved for the 23 patients who completed these measures. CONCLUSIONS: CI is efficacious to reduce the handicap of patient with SSD and incapacitating tinnitus, leading to a decrease in reported tinnitus and partial restoration of binaural hearing abilities.

The Oticon Medical Neuro Zti cochlear implant and the Neuro 2 sound processor: multicentric evaluation of outcomes in adults and children.

Objective: This study evaluated the outcomes of the Oticon Medical Neuro Zti cochlear implant and the Neuro 2 sound processor.Design: Neuro One users were upgraded to Neuro 2. Monosyllabic word identification was evaluated in adults with Neuro One after ≥5 months, with Neuro 2 at upgrade, and with Neuro 2 after 3 months. Self-reported listening ability, satisfaction, and usability were measured in adults and children.Study sample: Participants were 44 adults and 26 children.Results: Speech identification scores in quiet and noise were 58% and 45% with Neuro One and 67% and 55% with Neuro 2 after 3 months, respectively. Hearing impairment duration and number of active electrodes significantly predicted speech identification in noise with Neuro 2. Significantly higher questionnaire ratings were obtained for Neuro 2 than Neuro One regarding listening ability in complex listening situations, comfort and music, as well as nine aspects of satisfaction and usability.Conclusion: This study demonstrates the clinical superiority of the Neuro 2 sound processor over Neuro One in terms of speech identification in quiet and in noise and reported patient benefit and satisfaction. Given the study design, sources of improvement may include factors unrelated to the sound processor itself.

Development and validation of the FrBio, an international French adaptation of the AzBio sentence lists.

Objectives: The goal of this work was to develop and normalise an international French version of the AzBio sentence test. Design: A corpus of 1000 sentences was generated. These sentences were recorded with four talkers and processed through a four-channel cochlear implant simulation. The mean intelligibility for each sentence achieved by 16 normal-hearing listeners was computed. The consecutively ordered 165 sentences from each talker rendering an average score of 85% were sequentially assigned to 33 lists of 20 sentences. All lists were presented to 30 normal-hearing and 25 hearing-impaired listeners in order to verify their equivalency. Thirty normal-hearing adults were also recruited to assess the test's psychometrics and define norms. Results: The results of the list equivalency validation study showed no significant differences in percent correct scores for 30 sentence lists. A binomial distribution model was used to estimate the 95% critical differences for each potential percentage score. Normalization data showed an average performance between 96% and 99% with a very low standard deviation. Conclusions: With a set of 30 lists, researchers and clinicians can use the FrBio to evaluate a large number of experimental conditions; changes in performance over time or across conditions can then be tracked.