Audiology and Neuro-Otology最新文献

Introduction: Current clinical practice considers the vestibulo-ocular reflex (VOR) gain as registered by the video head impulse test (vHIT) as the primary measure for semicircular canal function, while the role of the re-fixation saccades (RSs) is still under evaluation. The goal of the study was to appraise the added benefit of RS towards the improvement of vHIT diagnostic accuracy in cases of suspected left horizontal semicircular canal dysfunction.

Methods: The vHIT recordings of 40 patients with left-sided horizontal VOR gains <0.8 were retrospectively evaluated for the presence of RS. The study groups included 20 patients with a final diagnosis of left horizontal semicircular canal dysfunction and 20 patients for whom vestibular dysfunction was ruled out.

Results: Gain values >0.72 were found in all patients with no vestibular disease and in 4 (20%) patients having vestibulopathy. Significantly higher average left-sided RS velocity and frequency were found among the vestibular patients. VOR gain <0.72 was found to be highly specific for the diagnosis of vestibular dysfunction. However, for gain values in the range of 0.72-0.79, the presence of RS with frequency >80% largely improved vHIT diagnostic accuracy.

Conclusions: Although VOR gain <0.8 is considered to reflect dysfunction, a significant false-positive rate for left-sided horizontal vHIT was found for gains in the range of 0.72-0.79. The presence of RS with frequency >80% could improve vHIT diagnostic ability in these patients.

Introduction: Tinnitus is one of the most common complaints, distressing about 15-24% of the adult population. Because of its pathophysiology heterogeneity, no curable treatment has been attained yet. Even though a neuromodulation management technique based on the tinnitus network model is currently being developed, it has not yet worked because the most involved brain areas still remain unpredictable from the patient's individual clinical and functional profile. A remarkable correlation between tinnitus network activity and the subjective measures of tinnitus like perceived loudness and annoyance and functional handicap is well established. Therefore, this study aimed to develop software for predicting the involved brain areas in the tinnitus network based on the subjective characteristics and clinical profile of patients using a supervised machine-learning method.

Methods: The involved brain areas of 30 tinnitus patients ranging from 6 to 80 months in duration were recognized by using QEEG and sLORETA software. There was a correlation between subjective information and those areas of activities in all rhythms by which we wrote our software.

Results: For verification and validation of the software, we compared and analyzed the results with SPSS data and the receiver operating characteristic (ROC) curves.

Conclusions: The findings of this study confirmed the effectiveness of the software in predicting the brain activity in tinnitus subjects; however, some other important parameters can be added to the model to strengthen its reliability and feasibility in clinical use.

Background: Mutations in TMPRSS3 are an important cause of autosomal recessive non-syndromic hearing loss. The hearing loss associated with mutations in TMPRSS3 is characterized by phenotypic heterogeneity, ranging from mild to profound hearing loss, and is generally progressive. Clinical presentation and natural history of TMPRSS3 mutations vary significantly based on the location and type of mutation in the gene. Understanding these genotype-phenotype relationships and associated natural disease histories is necessary for the successful development and application of gene-based therapies and precision medicine approaches to DFNB8/10. The heterogeneous presentation of TMPRSS3-associated disease makes it difficult to identify patients clinically. As the body of literature on TMPRSS3-associated deafness grows, there is need for better categorization of the hearing phenotypes associated with specific mutations in the gene.

Summary: In this review, we summarize TMPRSS3 genotype-phenotype relationships including a thorough description of the natural history of patients with TMPRSS3-associated hearing loss to lay the groundwork for the future of TMPRSS3 treatment using molecular therapy.

Key messages: TMPRSS3 mutation is a significant cause of genetic hearing loss. All patients with TMPRSS3 mutation display severe-to-profound prelingual (DFNB10) or a postlingual (DFNB8) progressive sensorineural hearing loss. Importantly, TMPRSS3 mutations have not been associated with middle ear or vestibular deficits. The c.916G>A (p.Ala306Thr) missense mutation is the most frequently reported mutation across populations and should be further explored as a target for molecular therapy.

Introduction: This study aimed to evaluate the incidence of balance disorders and the efficacy of dexamethasone in protecting patients undergoing cisplatin-based cancer treatment against vestibulototoxicity.

Methods: This study was a randomized controlled phase IIIB clinical trial. The subjects participating in the clinical trial were patients with a neoplastic disease whose treatment protocol included cisplatin. The average dose of cisplatin was 444.87 mg (SD 235.2 mg). Treatment consisted of intratympanically administering dexamethasone via a passive diffusion device called Microwick (8 mg/24 h dose) from the start of treatment with cisplatin to 3 weeks after the last cycle. Patients were administered the medication to one ear, and the contralateral ear was used as the control. The treated ears were randomly chosen using a computer system (randomization). Vestibular system was evaluated by video head impulse test before each cisplatin cycle.

Results: Thirty-four patients were recruited over a 2-year period at a reference tertiary hospital, of whom 11 were excluded. Forty-six ears were analyzed (23 treated and 23 control ears). Vestibular analysis presented no changes in the mean increase in the vestibulo-ocular response in all patients evaluated, both in treated and control ears. Both 8.69% infection complications during treatment and 34.8% permanent perforation at 6 months were detected after device removal.

Conclusion: Ototoxicity related to cisplatin-based treatment does not affect the vestibular system. Long-term high-dose intratympanic dexamethasone treatment is safe for the vestibular system.

Background: Speech perception in noise is especially challenging for cochlear implant (CI) recipients; thus, speech in noise tests are used to clinically evaluate functional hearing with CIs. The coordinate response measure (CRM) corpus can be utilized in an adaptive speech perception test with competing speakers as the masker. Determining the critical difference for CRM thresholds can enable it to be used to evaluate changes in CI outcomes for clinical and research purposes. If a change in CRM exceeds the critical difference, then this would indicate significant improvement or decrement in speech perception. Additionally, this information provides figures for power calculations that could be used for planning studies and clinical trials [Bland JM: An Introduction to Medical Statistics, 2000].

Objectives: This study determined the test-retest reliability of the CRM for adults with normal hearing (NH) and adults with CIs. The replicability, variability, and repeatability of the CRM were evaluated for the two groups separately.

Method: Thirty-three NH adults and thirteen adult CI recipients were recruited and tested with the CRM twice, 1 month apart. The CI group was tested with two talkers only, while the NH group was tested with seven talkers as well as two talkers.

Results: CRM had better replicability, repeatability and lower variability for the CI adults compared to NH adults. The critical difference (at p < 0.05) in the two-talker CRM speech reception thresholds (SRTs) among CI users was >5.2 dB, and it was >6.2 dB for the NH if an individual were to be tested under two different conditions. The critical difference (at p < 0.05) in the seven-talker CRM SRT was >6.49. The Mann-Whitney U test showed that CI recipients' CRM scores' variance (Mdn = -0.94) was significantly less than the NH group's (Mdn = 2.2) (U = 54, p < 0.0001). Although the NH had significantly better SRTs in the two-talker condition than in the seven-talker condition (t = -20.29, df = 65, p < 0.0001), the Wilcoxon signed ranks test showed no significant difference between the CRM scores' variance in the two conditions (Z = -1, N = 33, p = 0.08).

Conclusions: NH adults had significantly lower CRM SRTs than the CI recipients; t (31.16) = -23.91, p < 0.001. CRM had better replicability, stability and lower variability for the CI adults compared to NH adults.

Introduction: In individuals with single-sided deafness (SSD), who are characterised by profound hearing loss in one ear and normal hearing in the contralateral ear, binaural input is no longer present. A cochlear implant (CI) can restore functional hearing in the profoundly deaf ear, with previous literature demonstrating improvements in speech-in-noise intelligibility with the CI. However, we currently have limited understanding of the neural processes involved (e.g., how the brain integrates the electrical signal produced by the CI with the acoustic signal produced by the normal hearing ear) and how modulation of these processes with a CI contributes to improved speech-in-noise intelligibility. Using a semantic oddball paradigm presented in the presence of background noise, this study aims to investigate how the provision of CI impacts speech-in-noise perception of SSD-CI users.

Method: Task performance (reaction time, reaction time variability, target accuracy, subjective listening effort) and high density electroencephalography from twelve SSD-CI participants were recorded, while they completed a semantic acoustic oddball task. Reaction time was defined as the time taken for a participant to press the response button after stimulus onset. All participants completed the oddball task in three different free-field conditions with the speech and noise coming from different speakers. The three tasks were: (1) CI-On in background noise, (2) CI-Off in background noise, and (3) CI-On without background noise (Control). Task performance and electroencephalography data (N2N4 and P3b) were recorded for each condition. Speech in noise and sound localisation ability were also measured.

Results: Reaction time was significantly different between all tasks with CI-On (M [SE] = 809 [39.9] ms) having faster RTs than CI-Off (M [SE] = 845 [39.9] ms) and Control (M [SE] = 785 [39.9] ms) being the fastest condition. The Control condition exhibited significantly shorter N2N4 and P3b area latency compared to the other two conditions. However, despite these differences noticed in RTs and area latency, we observed similar results between all three conditions for N2N4 and P3b difference area.

Conclusion: The inconsistency between the behavioural and neural results suggests that EEG may not be a reliable measure of cognitive effort. This rationale is further supported by different explanations used in past studies to explain N2N4 and P3b effects. Future studies should look to alternative measures of auditory processing (e.g., pupillometry) to gain a deeper understanding of the underlying auditory processes that facilitate speech-in-noise intelligibility.

Introduction: We sought to evaluate the correlation between whole cochlear T2 signal changes obtained with a novel automated segmentation method and hearing levels, both at diagnosis and over time, in patients with observed vestibular schwannoma.

Methods: This retrospective correlation study within an academic medical center neurotology practice evaluated 127 patients with vestibular schwannoma observed over time, each with ≥2 MRI scans (367 total) and ≥2 audiograms (472 total). 86 patients had T2-weighted sequences with sufficient resolution for cochlear signal analysis, yielding 348 unique timepoint intervals. The main outcome measure was correlation of the ipsilateral-to-contralateral ratio of whole cochlear T2 signal with hearing outcomes as measured by pure tone average (PTA) and word recognition score (WRS).

Results: Whole cochlear T2 signal ratios did not show a correlation with hearing levels at diagnosis. Change in signal ratio over time showed weak correlation with changes in PTA, but not WRS, over time. Cochlear signal ratio did not precede changes in hearing but did follow changes in both PTA and WRS.

Conclusion: Whole cochlear T2 signal ratios were weakly correlated with changes in hearing in patients with observed vestibular schwannoma. The technology of automated segmentation and signal processing holds promise for future evaluation of clinical entities causing cochlear signal changes.

Introduction: The aim of this study was to better understand the onset time and factors associated with cochlear obliteration following translabyrinthine approach (TLA) surgery for large cerebellopontine angle tumors.

Methods: This retrospective cohort study included 117 patients with large cerebellopontine angle tumor (tumor diameter >2 cm) treated by TLA surgery from June 2011 to March 2019 in a single tertiary referral center. The Kaplan-Meier method with log-rank test was used to estimate cochlear patency survival and the association between survival and covariates, and the Cox proportional hazards regression analysis was used to identify possible factors associated with cochlear obliteration.

Results: Of the 117 patients included in our analysis, the median follow-up was 24.8 months. There were 30 (25.6%) patients in the cochlear obliteration group, and 87 (74.4%) in the patent cochlear group. Various degrees of cochlear obliteration was found in 25.6% patients in final MRI scan, comprised of 50% grade I, 30% grade II, and 20% grade III. Cochlear patency survival curves showed 94.0% at 3 months, 73.0% at 18 months, which plateaued after 20 months with a survival rate of 71.6%. In the multivariate Cox proportional hazards model, patients presented with postoperative hyperintense T1W cochlear signal had poorer cochlear patency survival compared to isointense T1W (HR = 4.15). Similarly, postoperative deteriorated facial function (HR = 4.52) and full IAC involvement of tumor (HR = 2.33) demonstrated a higher risks of cochlear obliteration after TLA surgery.

Conclusion: The 2-year estimated cochlear patency rate was 71.6% in patients that received TLA. Cochlear obliteration can develop as early as 3 months post-surgery, with no new obliteration 20 months after the surgery and half of these patients got severe obliteration. Three factors associated with cochlear obliteration were identified including full IAC involvement of tumor, postoperative facial function deterioration, and postoperative hyperintense T1W cochlear signal.

Introduction: Dizziness is a common complaint affecting up to 23% of the world population. Diagnosis is of utmost importance and routinely involves several tests to be performed in specialized centers. The advent of a new generation of technical devices would make envision their use for a valid objective vestibular assessment. Microsoft HoloLens 2 (HL2) mixed reality headset has the potential to be a valuable wearable technology that provides interactive digital stimuli and inertial measurement units (IMUs) to objectively quantify the movements of the user in response to various exercises. The aim of this study was to validate the integration of HoloLens with traditional methods used to analyze the vestibular function in order to obtain precise diagnostic values.

Methods: Twenty-six healthy adults completed the Dynamic Gait Index tests both with a traditional evaluation and while wearing HL2 headset, thus allowing to collect kinematic data of the patients' head and eyes. The subjects had to perform 8 different tasks, and the scores were independently assigned by two otolaryngology specialists.

Results: The maximum of the mean position of the walking axis of the subjects was found in the second task (-0.14 ± 0.23 m), while the maximum value of the standard deviation of the walking axis was found in the fifth task (-0.12 ± 0.27 m). Overall, positive results were obtained in regard to the validity of the HL2 use to analyze kinematic features.

Conclusion: The accurate quantification of gait, movement along the walking axis, and deviation from the normality using HL2 provide an initial evidence for its useful adoption as a valuable tool in gait and mobility assessment.

Introduction: A nonsurgical bone conduction hearing aid (BCHA) is a well-established treatment for children with congenital unilateral microtia and atresia (UMA). To date, limited studies have evaluated the audiological characteristics of the different wearing modes in the same nonsurgical BCHA.

Methods: Eighteen patients with UMA aged 5-24 years were included. Warble tones at frequencies of 0.5, 1, 2, and 4 kHz were presented to determine functional hearing gain (FHG) of hearing thresholds (in dB HL) in the sound field. The speech perception abilities were assessed by the speech discrimination score (SDS, in %) of monosyllables, disyllables, and sentences in quiet and noise using the Chinese Mandarin speech test materials. Hearing outcomes were evaluated with the ADHEAR™ worn on a softband and with an adhesive adapter. A correlational analysis was conducted to analyze the correlations between variables (e.g., age, height, weight, body mass index [BMI], bone conduction pure-tone threshold, and air conduction pure-tone threshold) and the differences in the two wearing modes.

Results: The mean FHG (standard deviation, SD) at 0.5-4 kHz was 20.63 (3.94) dB HL with the adhesive adapter and 26.39 (3.15) dB HL with the softband. When aided with the BCHA, significant improvements in SDS were revealed in all Mandarin speech test material lists either in quiet or noise for both wearing modes. Compared with the adapter mode, the softband provided higher aided SDS values. Correctional analyses revealed that higher BMI values were positively associated with larger delta outcomes between the two coupling methods of the softband and adhesive adapter in patients with UMA. Furthermore, a larger delta average FHG of 0.5-4 kHz was consistently associated with larger delta monosyllabic SDS in quiet, disyllabic SDS in quiet, and disyllabic SDS in noise.

Discussion: To the best of our knowledge, this is the first study to compare the hearing benefits of coupling methods using novel adhesive adapters and conventional softbands with the same audio processor (ADHEAR™). Under uniform internal settings, softband integration provided more hearing benefits than adhesive adapter integration, and the differences were more obvious in patients with higher BMI values. Besides, a brief measurement of FHG can be utilized to predict individualized speech perception levels.