Validation of the Tinnitus Acceptance Questionnaire: Japanese Version

This study aimed to develop and validate a Japanese version of the Tinnitus Acceptance Questionnaire (TAQ), an instrument that measures the process of intentional acceptance of adverse experiences associated with tinnitus. A total of 125 patients with chronic tinnitus from multiple institutions participated in this study. Participants completed the Japanese versions of the TAQ, Tinnitus Handicap Inventory, Valuing Questionnaire, Acceptance and Action Questionnaire-II, and Hospital Anxiety and Depression Scale. A second TAQ was administered 1–2 weeks later. Because the model fitted poorly in confirmatory factor analysis, exploratory factor analysis was conducted, which yielded a two-factor structure that was divided into forward and reversed item groups. Hypotheses regarding criterion and construct validity were clearly supported. A high Cronbach’s α coefficient value was obtained for the TAQ total score (0.88). The interclass correlation coefficient for test–retest reliability was within the acceptable range (0.95). The results of the exploratory factor analysis were considered to be due to artifacts caused by the characteristics of the Japanese language. The present study confirmed the validity and reliability of the Japanese version of the TAQ in measuring tinnitus-specific receptivity.

Usher Syndrome

Usher syndrome (USH) is the most common genetic condition responsible for combined loss of hearing and vision. Balance disorders and bilateral vestibular areflexia are also observed in some cases. The syndrome was first described by Albrecht von Graefe in 1858, but later named by Charles Usher, who presented a large number of cases with hearing loss and retinopathy in 1914. USH has been grouped into three main clinical types: 1, 2, and 3, which are caused by mutations in different genes and are further divided into different subtypes. To date, nine causative genes have been identified and confirmed as responsible for the syndrome when mutated: MYO7A, USH1C, CDH23, PCDH15, and USH1G (SANS) for Usher type 1; USH2A, ADGRV1, and WHRN for Usher type 2; CLRN1 for Usher type 3. USH is inherited in an autosomal recessive pattern. Digenic, bi-allelic, and polygenic forms have also been reported, in addition to dominant or nonsyndromic forms of genetic mutations. This narrative review reports the causative forms, diagnosis, prognosis, epidemiology, rehabilitation, research, and new treatments of USH.

Cochlear Implant in Patients with Intralabyrinthine Schwannoma without Tumor Removal

(1) Background: Schwannomas of the vestibulocochlear nerve are benign, slow-growing tumors, arising from the Schwann cells. When they originate from neural elements within the vestibule or cochlea, they are defined as intralabyrinthine schwannomas (ILSs). Cochlear implant (CI) has been reported as a feasible solution for hearing restoration in these patients. (2) Methods: Two patients with single-sided deafness (SSD) due to sudden sensorineural hearing loss and ipsilateral tinnitus were the cases. MRI detected an ILS. CI was positioned using a standard round window approach without tumor removal. (3) Results: The hearing threshold was 35 dB in one case and 30 dB in the other 6 mo after activation. Speech audiometry with bisillables in quiet was 21% and 27% at 65 dB, and the tinnitus was completely resolved or reduced. In the localization test, a 25.9° error azimuth was obtained with CI on, compared to 43.2° without CI. The data log reported a daily use of 11 h and 14 h. In order to not decrease the CI’s performance, we decided not to perform tumor exeresis, but only CI surgery to restore functional binaural hearing. (4) Conclusions: These are the sixth and seventh cases in the literature of CI in patients with ILS without any tumor treatment and the first with SSD. Cochlear implant without tumor removal can be a feasible option for restoring binaural hearing without worsening the CI’s performance.

Characterization of Balance Problems and Rehabilitation Needs of Patients with Ménière’s Disease

Background: To explore and characterize balance problems in subjects with Ménière’s disease (MD). Methods: A total of 539 people with MD with a mean age of 61.9 years, mean disease history of 15.6 years, and 79.5% females were recruited. The online questionnaire, consisting of 39 questions, including both structured and open-ended questions, focused on symptoms of MD, balance problems, impacts of the complaints, and quality of life (QoL). Results: After hearing loss (58%) and tinnitus (50%), balance problems (44%) were among the most commonly reported MD complaints, even higher than the impact of vertigo (40%). However, only 22% reported that those balance problems made obvious impacts in their daily lives. The most common balance problem that significantly reduced QoL was tripping (34%). Swaying (25%) had a limited impact on QoL, whereas rocking (10%) was less common but caused a significant impact on QoL. Non-defined balance problems were reported at 18%; these were occasional and correlated with vertigo attacks. Older participants had more frequent tripping problems. Younger participants more frequently reported swaying and rocking. Conclusions: Risk factors predicting poor postural control were mostly related to complaints reflecting otolith pathology. Different types of postural problems require different strategies to manage balance control and cope with the disease.

Fifty Years of Development of the Skull Vibration-Induced Nystagmus Test

This review enumerates most of the studies on the Skull Vibration-Induced Nystagmus Test (SVINT) in the past 50 years from different research groups around the world. It is an attempt to demonstrate the evolution of this test and its increased interest around the globe. It explores clinical studies and animal studies, both permitting a better understanding of the importance of SVINT and its pathophysiology.

Interdisciplinary Approaches to the Study of Listening Effort in Young Children with Cochlear Implants

Very early bilateral implantation is thought to significantly reduce the attentional effort required to acquire spoken language, and consequently offer a profound improvement in quality of life. Despite the early intervention, however, auditory and communicative outcomes in children with cochlear implants remain poorer than in hearing children. The distorted auditory input via the cochlear implants requires more auditory attention resulting in increased listening effort and fatigue. Listening effort and fatigue may critically affect attention to speech, and in turn language processing, which may help to explain the variation in language and communication abilities. However, measuring attention to speech and listening effort is demanding in infants and very young children. Three objective techniques for measuring listening effort are presented in this paper that may address the challenges of testing very young and/or uncooperative children with cochlear implants: pupillometry, electroencephalography, and functional near-infrared spectroscopy. We review the studies of listening effort that used these techniques in paediatric populations with hearing loss, and discuss potential benefits of the systematic evaluation of listening effort in these populations.

Executive Functions and Deafness: Results in a Group of Cochlear Implanted Children

Objects: This study aimed to evaluate the Executive Function (EF) domains in a group of profoundly deaf children treated with cochlear implant (CI) in comparison to normal hearing (NH) children. The secondary aim was to evaluate the influence exerted by the age at cochlear implant activation on EFs. Materials and Methods: 32 children were enrolled into two groups: group A of 17 CI users with a mean age of 8.78 years and group B of 15 NH subjects with a mean age of 7.99 years (SD + 2.3). All subjects were tested using the following tests: the subtests for working memory of the neuropsychological evaluation battery for the developmental age (Batteria di valutazione neuropsicologica per l’età evolutive), inhibition and control of the impulsive response—CAF, and the tower of London test. Results: No children with CIs scored within the normal range in the tests administered for the evaluation of EF domains. The same scores were significantly lower when compared with scores obtained by NH children. Children with younger age at CI activation showed better executive performances in planning, working memory (backward digit span), and cognitive flexibility (categorical fluency). Conclusion: The results of this study highlight that cochlear implantation plays a role in improving hearing and consequently influences the development of EFs in deaf children.

Verification of a Mobile Psychoacoustic Test System

Many hearing difficulties can be explained as a loss of audibility, a problem easily detected and treated using standard audiological procedures. Yet, hearing can be much poorer (or more impaired) than audibility predicts because of deficits in the suprathreshold mechanisms that encode the rapidly changing, spectral, temporal, and binaural aspects of the sound. The ability to evaluate these mechanisms requires well-defined stimuli and strict adherence to rigorous psychometric principles. This project reports on the comparison between a laboratory-based and a mobile system’s results for psychoacoustic assessment in adult listeners with normal hearing. A description of both systems employed is provided. Psychoacoustic tests include frequency discrimination, amplitude modulation detection, binaural encoding, and temporal gap detection. Results reported by the mobile system were not significantly different from those collected with the laboratory-based system for most of the tests and were consistent with those reported in the literature. The mobile system has the potential to be a feasible option for the assessment of suprathreshold auditory encoding abilities.

Intracorporeal Cortical Telemetry as a Step to Automatic Closed-Loop EEG-Based CI Fitting: A Proof of Concept

Electrically evoked auditory potentials have been used to predict auditory thresholds in patients with a cochlear implant (CI). However, with exception of electrically evoked compound action potentials (eCAP), conventional extracorporeal EEG recording devices are still needed. Until now, built-in (intracorporeal) back-telemetry options are limited to eCAPs. Intracorporeal recording of auditory responses beyond the cochlea is still lacking. This study describes the feasibility of obtaining longer latency cortical responses by concatenating interleaved short recording time windows used for eCAP recordings. Extracochlear reference electrodes were dedicated to record cortical responses, while intracochlear electrodes were used for stimulation, enabling intracorporeal telemetry (i.e., without an EEG device) to assess higher cortical processing in CI recipients. Simultaneous extra- and intra-corporeal recordings showed that it is feasible to obtain intracorporeal slow vertex potentials with a CI similar to those obtained by conventional extracorporeal EEG recordings. Our data demonstrate a proof of concept of closed-loop intracorporeal auditory cortical response telemetry (ICT) with a cochlear implant device. This research breaks new ground for next generation CI devices to assess higher cortical neural processing based on acute or continuous EEG telemetry to enable individualized automatic and/or adaptive CI fitting with only a CI.

Benefits of Parent Training in the Rehabilitation of Deaf or Hard of Hearing Children of Hearing Parents: A Systematic Review

The present study is a systematic review on the effectiveness of Parent Training (PT) and coaching in deaf and hard of hearing (DHH) rehabilitation programs which reviews and synthesizes the existing body of evidence to assess the benefits of these programs in enhancing parents’ sensitivity, responsivity and promoting language development in DHH children during the first years after HA fitting or CI activation. Five published studies met the Population, Intervention, Comparison and Outcomes (PICO) inclusion criteria and were eligible to be included, but heterogeneity in terms of the study design, interventions and outcomes did not allow for performing a meta-analysis. All included studies shared the view that a parent’s learning is a circular (rather than frontal) process, and the results appear promising in terms of enhancing parents’ responsiveness and promoting DHH child language development. Nevertheless, the available evidence was judged to not be robust enough due to limitations in the studies’ designs. Further high-quality evidence is needed to evaluate the true degree of clinical value and the cost effectiveness of PT programs aimed at increasing parents’ responsiveness to their DHH children.