The purpose of this study was to prove the hypothesis that caloric response in Ménière's disease (MD) is reduced by hydropic expansion of the vestibular labyrinth, not by vestibular hypofunction, by evaluating the correlation morphologically using an intravenous Gadolinium (IV-Gd) inner ear MRI. In study I, the prevalence of abnormal video Head Impulse Test (vHIT) results among the patients with definite unilateral MD (n = 24) and vestibular neuritis (VN) (n = 22) were investigated. All patients showed abnormal canal paresis (CP) (> 26%) on caloric tests. The prevalence of abnormal vHIT in patients with abnormal CP was significantly lower in MD patients (12.5%) than that in VN patients (81.8%) (p < 0.001). In study II, morphological correlation between caloric tests and vestibular hydrops level was evaluated in unilateral MD patients (n = 16) who had normal vHIT results. Eleven patients (61%) had abnormal CP. After taking the images of IV-Gd inner ear MRI, the vestibular hydrops ratio (endolymph volume/total lymph volume = %VH) was measured. In addition, the relative vestibular hydrops ratio (%RVH = (%VHaffected ear—%VHunaffected ear) / (%VHaffected ear + %VHunaffected ear)) was calculated. Each ratio (%VH and %RVH) was compared with average peak slow phase velocity (PSPV) and CP, respectively. In the MD patients, %VH of the affected ear correlated significantly with mean PSPV on the same side (rs = -0.569, p = 0.024), while %RVH correlated significantly with CP (rs = 0.602, p = 0.014). In most MD patients (87.5%) compared to VN patients, vHIT results were normal even though the caloric function was reduced. In addition, the reduced caloric function with normal vHIT was related to the severity of the vestibular hydrops measured by the IV-Gd inner ear MRI. These findings concluded that the abnormal caloric tests with normal vHIT in MD indicated severe endolymphatic hydrops rather than vestibular hypofunction.

The aim of this study was to investigate hidden hearing loss in patients with Charcot-Marie-Tooth disease type 1 A (CMT1A), a common inherited demyelinating neuropathy. By using pure-tone audiometry, 43 patients with CMT1A and 60 healthy controls with normal sound detection abilities were enrolled. Speech perception in quiet and noisy backgrounds, spectral ripple discrimination (SRD), and temporal modulation detection (TMD) were measured. Although CMT1A patients and healthy controls had similar pure-tone thresholds and speech perception scores in a quiet background, CMT1A patients had significantly (p < 0.05) decreased speech perception ability in a noisy background compared to controls. CMT1A patients showed significantly decreased temporal and spectral resolution (both p < 0.05). Also, auditory temporal processing of CMT1A patients was correlated with speech perception in a noisy background (r = 0.447, p < 0.01) and median motor conduction velocity (r = 0.335, p < 0.05). Therefore, we assumed that demyelination of auditory nerve in CMT1A causes defective cochlear neurotransmission, which reduces temporal resolution and speech perception in a noisy background. Because the temporal resolution test was well correlated with the degree of demyelination in auditory and peripheral motor nerves, temporal resolution testing could be performed as an additional marker for CMT1A.

Background and Objectives: To assess the academic performance, communication skills, and psychosocial development of prelingual deaf children with cochlear implants (CIs) attending mainstream schools, and to evaluate the impact of auditory speech perception on their classroom performance.Subjects and Methods: As participant, 67 children with CI attending mainstream schools were included. A survey was conducted using a structured questionnaire on academic performance in the native language, second language, mathematics, social studies, science, art, communication skills, self-esteem, and social relations. Additionally, auditory and speech performances on the last follow-up were reviewed retrospectively.Results: Most implanted children attending mainstream school appeared to have positive self-esteem and confidence, and had little difficulty in conversing in a quiet classroom. Also, half of the implanted children (38/67) scored above average in general academic achievement. However, academic achievement in the second language (English), social studies, and science were usually poorer than general academic achievement. Furthermore, half of the implanted children had difficulty in understanding the class content (30/67) or conversing with peers in a noisy classroom (32/67). These difficulties were significantly associated with poor speech perception.Conclusions: Improving the listening environment for implanted children attending mainstream schools is necessary.

This study aimed to investigate the usefulness of the intravenous gadolinium enhanced inner ear magnetic resonance imaging (IV-Gd inner ear MRI) in diagnosing Ménière’s disease(MD) and find a correlation between the degree of endolymphatic hydrops(EH) and the audiovestibular tests. Total 29 patients diagnosed with unilateral definite MD were enrolled. All patients underwent IV-Gd inner ear MRI and auditory and vestibular function tests such as pure tone audiometry (PTA), electrocochleography (ECoG), cervical vestibular evoked myogenic potential (cVEMP) and caloric test. The hydrops ratio in the cochlea and vestibule were significantly higher in the affected side than the unaffected side (p < 0.001). Average pure-tone thresholds for 0.5, 1 k, 2 k, and 4 k Hz correlated significantly with cochlear and vestibular hydrops (p < 0.01) in the affected side. When comparing the SP/AP ratio of ECoG with hydrops ratio in the vestibule, the affected and unaffected ears showed a significant difference (p < 0.05). Similarly, the results of the caloric test also showed a significant correlation (p < 0.05) with relative vestibular hydrops. However, the cVEMP response was not related to the hydrops ratio in the cochlea or vestibule. This study presents pertinent data with appropriate correlations with auditory vestibular functional testing which demonstrates the usefulness of IV-Gd inner ear MRI as a diagnostic method for visualizing the endolymphatic hydrops in MD.

The objective of this study was to examine the relationship between spectrotemporal modulation (STM) sensitivity and the ability to perceive music. Ten normal-hearing (NH) listeners, ten hearing aid (HA) users with moderate hearing loss, and ten cochlear Implant (CI) users participated in this study. Three different types of psychoacoustic tests including spectral modulation detection (SMD), temporal modulation detection (TMD), and STM were administered. Performances on these psychoacoustic tests were compared to music perception abilities. In addition, psychoacoustic mechanisms involved in the improvement of music perception through HA were evaluated. Music perception abilities in unaided and aided conditions were measured for HA users. After that, HA benefit for music perception was correlated with aided psychoacoustic performance. STM detection study showed that a combination of spectral and temporal modulation cues were more strongly correlated with music perception abilities than spectral or temporal modulation cues measured separately. No correlation was found between music perception performance and SMD threshold or TMD threshold in each group. Also, HA benefits for melody and timbre identification were significantly correlated with a combination of spectral and temporal envelope cues though HA.

This study aimed to compare functional hearing with the use of a personal sound amplification product (PSAP) or a basic hearing aid (HA) among sensorineural hearing impaired listeners. Subjects and. Nineteen participants with mild-to-moderate sensorineural hearing loss (SNHL) (26-55 dB HL; pure-tone average, 0.5-4 kHz) were prospectively included. No participants had prior experience with HAs or PSAPs. Audiograms, speech intelligibility in both quiet and noisy environments, speech quality, and preference were assessed in three different listening conditions: unaided, with the HA, and with the PSAP. The use of PSAP was associated with significant improvement in pure-tone thresholds at 1, 2, and 4 kHz compared to the unaided condition (all p <0.01). In the quiet environment, speech intelligibility was significantly improved after wearing a PSAP compared to the unaided condition (p <0.001), and this improvement was better than the result obtained with the HA. The PSAP also demonstrated similar improvement in the most comfortable levels compared to those obtained with the HA (p <0.05). However, there was no significant improvement of speech intelligibility in a noisy environment when wearing the PSAP (p =0.160). There was no significant difference in the reported speech quality produced by either device or in participant preference for the PSAP or HA. The current result suggests that PSAPs provide considerable benefits to speech intelligibility in a quiet environment and can be a good alternative to compensate for mild-to-moderate SNHL.