Audiology and Neuro-Otology最新文献

Introduction: Cochlear implantation is the standard treatment for severe to profound hearing loss. While cochlear implant (CI) users can communicate effectively in quiet environments, speech understanding in noise remains challenging. Bimodal hearing, combining a CI in one ear and a hearing aid (HA) in the other, has shown advantages over unilateral electrical hearing, especially for speech understanding in noisy conditions. Beamforming is a technique used to improve speech understanding in noise by detecting sound direction and enhancing frontal (speech) sounds while attenuating background noise. One specific beamformer, Stereozoom, combines signals from microphones in both ears to create a focused beam toward the front resulting in a binaural beamformer (BB), in order to improve speech intelligibility in noise for bilateral and bimodal CI users.

Methods: A prospective crossover study involving 17 bimodal CI users was conducted, and participants were tested with various device configurations (CI, HA, CI + HA) with and without BB. Speech recognition testing with the Dutch/Flemish matrix test was performed in a sound-attenuated booth with diffuse noise to simulate realistic listening conditions.

Results: The results showed a statistically significant benefit of bimodal hearing over the CI configuration and showed a statistical significant benefit of BB for the CI and CI + HA configuration. The benefit of BB in the HA configuration was not statistically significant probably due to the higher variance. The benefit of BB in the three configurations did not differ statistically significant.

Conclusion: In conclusion, bimodal hearing offers advantages for speech understanding in noise for CI users. BB provides a benefit in various device configurations, leading to improved speech intelligibility when speech comes from the front in challenging listening environments.

Introduction: The main objective of the study was to validate the Norwegian translation of the Speech, Spatial and Qualities of Hearing Scale (SSQ) and investigate the SSQ disability profiles in a cochlear implant (CI) user population.

Methods: The study involved 152 adult CI users. The mean age at implantation was 55 (standard deviation [SD] = 16), and the mean CI experience was 5 years (SD = 4.8). The cohort was split into three groups depending on the hearing modality: bilateral CIs (BCIs), a unilateral CI (UCI), and bimodal (CI plus contralateral hearing aid; HCI). The SSQ disability profiles of each group were compared with those observed in similar studies using the English version and other translations of the SSQ. Standard values, internal consistency, sensitivity, and floor and ceiling effects were investigated, and the missing-response rates to specific questions were calculated. Relationships to speech perception were measured using monosyllabic word scores and the Norwegian Hearing in Noise Test scores.

Results: In the BCI group, the average scores were around 5.0 for the speech and spatial sections and 7.0 for the qualities section (SD ∼2). The average scores of the UCI and HCI groups were about one point lower than those of the BCI group. The SSQ disability profiles were comparable to the profiles in similar studies. The slopes of the linear regression lines measuring the relationships between the SSQ speech and monosyllabic word scores were 0.8 per 10% increase in the monosyllabic word score for the BCI group (explaining 35% of the variation) and 0.4 for the UCI and HCI groups (explaining 22-23% of the variation).

Conclusion: The Norwegian version of the SSQ measures hearing disability similar to the original English version, and the internal consistency is good. Differences in the recipients' pre-implantation variables could explain some variations we observed in the SSQ responses, and such predictors should be investigated. Data aggregation will be possible using the SSQ as a routine clinical assessment in global CI populations. Moreover, pre-implantation variables should be systematically registered so that they can be used in mixed-effects models.

Introduction: The aim of the study was to evaluate the vestibulo-ocular reflex (VOR) using the video head impulse test (vHIT) as a vestibular function assessment method in patients with unilateral chronic otitis media (COM).

Methods: Twenty-eight patients with unilateral COM scheduled for middle ear surgery, including 12 males and 16 females with a mean age of 54.5 years, successfully underwent preoperative vHIT. vHIT variables, including VOR gain, VOR gain asymmetry, and the incidence of corrective saccades, were compared between the ipsilesional and contralesional ears.

Results: Ten (35.7%) patients reported varying degrees of disequilibrium or lightheadedness. The VOR gain in the ipsilesional and contralesional ears was 1.01 ± 0.10 and 1.04 ± 0.11, 0.97 ± 0.12 and 0.94 ± 0.13, and 0.97 ± 0.13 and 1.04 ± 0.15 in the horizontal canal (HC), anterior canal, and posterior canal (PC), respectively. The average VOR gains in the ipsilesional ears were within the predefined range of normal values; however, the gains in HC and PC significantly decreased compared with those in contralesional ears (p = 0.038 in HC and p = 0.015 in PC). The prevalence of corrective saccades, including overt and/or covert saccades, did not differ significantly between the two ears.

Conclusion: This study assessed the pathophysiology of the inner ear affected by chronic middle ear infection by quantitatively comparing the VOR using vHIT. It demonstrated that vHIT can be a practical assessment of vestibular function in patients with COM.

Introduction: The aims of the present study were to evaluate postural balance performance of the subjects on the time-restricted feeding (TRF) and reveal the effect of TRF on the vestibular system by comparing the results to those of traditional daily dietary (DD) condition.

Methods: Sixteen adults (3 males, 13 females; mean age: 25.4 ± 4 years) who had experienced at least 1 month of TRF were included in the study. The Sensory Organization Test (SOT) and Head-Shake SOT (HS-SOT) - which evaluate proprioceptive, visual, and vestibular systems - were performed on TRF and DD conditions via the Computerized Dynamic Posturography system.

Results: Significant differences were obtained between TRF and DD situations in SOT-5 (p = 0.008), SOT-6 (p = 0.01), and HS-SOT5 (p = 0.007) conditions in which the vestibular system dominated.

Conclusion: We revealed that TRF has an effect on postural balance in the absence of proprioceptive and visual systems. This feeding model is a negative stressor that has a substantial effect on the vestibular system, but this impact is minimal once the proprioceptive and visual systems are intact. To the best of our knowledge, it is the first study to evaluate postural balance utilizing vestibular parameters in TRF.

Introduction: Despite the high genetic heterogeneity of hearing loss, mutations in the GJB2 gene are a major cause of autosomal recessive nonsyndromic hearing loss (NSHL) worldwide. However, the mutation profile of GJB2 in NSHL is under-investigated in Morocco, especially among simplex cases. This study aimed to identify the spectrum and frequency of GJB2 mutations in the Moroccan population among simplex and multiplex families with NSHL.

Methods: Moroccan families with NSHL were selected according to well-defined criteria. Selected families were screened for GJB2 gene variants using direct sequencing of the entire coding region of GJB2.

Results: A total of 145 affected individuals from 115 families with NSHL were included in this study (49 simplex, 66 multiplex). Mutations in the GJB2 gene were noted in 28.69% of the families (33/115), of which 75.75% were multiplex families and 24.24% were simplex. In total, seven different mutations were detected: c.35delG(p.G12fs), c.551G>A(p.R184Q), c.139G>T(p.E47X), c.109G>A(p.V37I), c.167delT(p.L56fs), c.617A>G(p.N206S), c.94C>T(p.R32C). The last three mutations have not previously been reported in Morocco. The most common GJB2 mutation was c.35delG (21.73%), followed by p.V37I (2.60%) and p.E47X (1.73%).

Conclusions: Our study confirms a high prevalence of GJB2 variants in the Moroccan population, particularly the c.35delG mutation. Additionally, we have identified previously unreported or rarely reported mutations, revealing a greater diversity of GJB2 mutations. These findings emphasize the importance of comprehensive screening beyond the 35delG mutation for patients with NSHL, regardless of their family history. Integrating this approach into clinical care will enhance diagnosis and management of hearing loss in the Moroccan population.

Introduction: Spatial hearing is most accurate using both ears, but accuracy decreases in persons with asymmetrical hearing between ears. In participants with deafness in one ear but normal hearing in the other ear (single-sided deafness [SSD]), this difference can be compensated by a unilateral cochlear implant (CI). It has been shown that a CI can restore sound localization performance, but it is still unclear to what extent auditory spatial discrimination can be improved.

Methods: The present study investigated auditory spatial discrimination using minimum audible angles (MAAs) in 18 CI-SSD participants. Results were compared to 120 age-matched normal-hearing (NH) listeners. Low-frequency (LF) and high-frequency (HF) noise bursts were presented from 4°, 30°, and 60° azimuth on the CI side and on the NH side. MAA thresholds were tested for correlation with localization performance in the same participants.

Results: There were eight good performers and ten poor performers. There were more poor performers for LF signals than for HF signals. Performance on the CI side was comparable to performance on the NH side. Most difficulties occurred at 4° and at 30°. Eight of the good performers in the localization task were also good performers in the MAA task. Only the localization ability at 4° on the CI side was positively correlated with the MAA at that location.

Conclusion: Our data suggest that a CI can restore localization ability but not necessarily auditory spatial discrimination at the same time. The ability to discriminate between adjacent locations may be trainable during rehabilitation to enhance important auditory skills.

Background: The current pandemic of COVID-19, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has resulted in significant morbidity and mortality primarily associated with respiratory failure. However, it has also been reported that COVID-19 can evolve into a nervous system infection. The direct and indirect mechanisms of damage associated with SARS-CoV-2 neuropathogenesis could affect our sensory functionality, including hearing and balance.

Summary: In order to investigate a possible association between SARS-CoV-2 viral infection and possible damage to the vestibular system, this review describes the main findings related to diagnosing and evaluating otoneurological pathologies.

Key messages: The clinical evidence shows that SARS-CoV-2 causes acute damage to the vestibular system that would not leave significant sequelae. Recovery is similar to vestibular pathologies such as vestibular neuronitis and benign paroxysmal positional vertigo. Further basic science, clinical, and translational research is needed to verify and understand the short- and long-term effects of COVID-19 on vestibular function.

Introduction: We aimed to investigate the clinical significance of the loudness discomfort level (LDL) test in tinnitus patients and its relationship with pure-tone audiometry, tinnitogram, and questionnaires.

Methods: We retrospectively reviewed the medical records of 320 tinnitus patients who visited a tertiary university hospital's tinnitus clinic and completed LDL tests between March 2020 and December 2022. Epidemiological data and psychoacoustic test results were collected.

Results: The LDL showed no significant differences between frequencies for both ears. The mean LDL did not correlate with mean pure-tone average or hearing thresholds at each frequency. The hearing loss group had a higher LDL at 8 kHz compared to the normal hearing group (p < 0.01). Objective sound intolerance was found in a quarter, correlating with subjective hyperacusis, anxiety, and depression. Weak negative correlations were found between most of questionnaire's scores and LDL on the left side. Tinnitus loudness weak negatively correlated with LDL at most frequencies, except 8 kHz.

Discussion/conclusion: Our findings suggest a notable association between LDL levels and emotional factors in tinnitus patients, rather than with auditory thresholds. While lateralized differences in LDL responses were observed, specifically on the left side, these preliminary results do not confirm a causal link and thus do not warrant changes to current clinical testing protocols without further research.

Introduction: This work aimed to study the management of vestibular schwannoma (VS) patients with normal hearing (NH).

Methods: A retrospective study was undertaken in a Quaternary referral center for skull base pathologies. Among 4,000 VS patients 162 met our strict audiological criteria for NH. These patients were divided into 2 management groups, wait and scan (W&S) (45/162, 25%) and operated patients (123/162, 75%), and 6 patients were included in both groups.

Results: Our management strategy achieved the goals for treatment of VS. First goal, all tumors were completely removed except for 2 intentional residuals. Second goal, facial nerve (FN) function preservation (House Brackmann I, II, and III) was 95.9%. Third goal, possible hearing preservation (HP) attempts occurred in (50/122) (40.9%) with an HP rate in 44% of the patients. Additionally, there were only 2 cases of postoperative complications with no CSF leakage. The prospect of HP in NH patients did not differ with respect to tumor size. However, patients with normal preoperative ABR seemed to have better chances of HP and good FN function and vice versa. HP rate was superior for the MCFA as opposed to the RS + RLA. W&S group demonstrated hearing stability in 88.9% of the patients and FN function stability of HB I in 100% of the patients.

Conclusions: Surgical resection is a reasonable and definitive management option for VS with NH. Nevertheless, choosing to manage cases with observation remains an appropriate management option for NH patients. ABR might be considered as an adjuvant tool indicating better prognosis for HP.

Introduction: Usher syndrome (USH) is an autosomal recessive disorder that predominantly affects hearing, vision, and, in some cases, vestibular function. USH, according to the onset age, severity, and progression of symptoms, is categorized into four main types. In addition, there are a significant number of reports that patients' manifestations deviate from canonical phenotypic criteria of main types of USH, which are named atypical USH. CDH23 is the second most common USH gene in which its defects result in USH1D, non-syndromic autosomal recessive deafness-12 (DFNB12), and in a few cases, atypical USH1D. While some studies have suggested that missense and truncating damaging variants in the CDH23 gene cause DFNB12 and USH1D, respectively, no genotype-phenotype correlation for atypical USH1D has been established.

Methods: Using whole-exome sequencing, we studied an Iranian family with two affected siblings who manifested congenital bilateral hearing loss, late-onset nyctalopia, retinitis pigmentosa, and normal vestibular function, indicating that their clinical symptoms are consistent with USH2.

Results: Whole-exome data analysis revealed a novel bi-allelic nonsense variant (c.6562G>T; p.Glu2188Ter) in the CDH23 gene, which was confirmed by Sanger sequencing. Surprisingly, CDH23 is a member of the USH1 genes; therefore, our patients suffered from atypical USH1D. Also, by conducting a literature review, we provided a clinical and mutational profile of all reported patients with atypical manifestations or those who refuted the claimed genotype-phenotype correlation.

Conclusion: By reporting a novel damaging variant, we expand the mutational spectrum of the CDH23 gene that leads to atypical USH1D. Also, reviewing the literature shows that, contrary to previous claims, different genotypes occur in the CDH23 gene allelic disorders, and there is no clear-cut genotype-phenotype correlation.