A clinical diagnosis of Ménière's disease (MD) is made based on medical history and audiometry findings. The 1995 American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) guidelines requires histopathological confirmation of endolymphatic hydrops (EH) for a diagnosis of “certain” MD. Symptoms such as dizziness and ear fullness are important diagnostic features; however, the descriptions provided by patients are frequently vague and non-specific. A recently developed magnetic resonance imaging (MRI) protocol to document EH is, therefore, useful for the evaluation of inner ear status in patients with MD. In this study, patients with MD were assessed using MRI and the HYDROPS (HYbriD of Reversed image Of Positive endolymph signal and native image of positive perilymph Signal) protocol to investigate the effectiveness of MRI for visualization of the endolymphatic space in the diagnosis of MD by correlating clinical laboratory parameters with the grade of EH. Of the 123 patients with MD recruited in this study, 80 had definite MD, 11 had probable MD, and 32 had possible MD based on the 1995 AAO-HNS guidelines. The EH grade based on HYDROPS MRI was determined independently by two otorhinolaryngologists and compared with several clinical parameters, including the diagnostic scale of MD (1995 AAO-HNS guidelines), pure tone average (PTA), low tone average (LTA), canal paresis (CP) on the caloric test, and disease duration. Cochlear hydrops and vestibular hydrops were detected in 58 and 80% of 80 definite MD ears, in 33 and 58% of 12 probable MD ears, and in 5 and 27% of 37 possible MD ears, respectively. The proportion of higher hydrops grades increased significantly with grade according to the MD diagnostic scale (p < 0.0001). Both PTA and LTA were significantly higher in patients with hydrops grade 2 than hydrops grade 0 in both the cochlea and the vestibule. CP was significantly higher in patients with grade 2 than grade 0 vestibular hydrops. Disease duration was not associated with hydrops grade. Radiological evaluation of MD using the HYDROPS protocol is useful for evaluation of the extent and severity of EH in the diagnosis of MD based on its pathophysiological mechanism.

Background and Objectives: Many otologists face a dilemma in the decision-making process of surgical management of patients with cochlear nerve (CN) aplasia. The goal of this study is to provide fresh evidence on cochlear implantation (CI) results in patients with CN aplasia. Materials and Methods: We scrutinized functional outcomes in 37 ears of 21 children with bilateral CN aplasia who underwent unilateral or bilateral CI based on cross-sectional and longitudinal assessments. Results: The Categories of Auditory Performance (CAP) scores gradually improved throughout the 3-year follow-up; however, variable outcomes existed between individuals. Specifically, 90% of recipients with a 1-year postoperative CAP score ≤1 could not achieve a CAP score over 1 even at 3-year postoperative evaluation, while the recipients with a 1-year postoperative CAP score >1 had improved auditory performance, and 72.7% of them were able to achieve a CAP score of 4 or higher. Meanwhile, intraoperative electrically evoked compound action potential was not correlated with postoperative CAP score. Conclusions: Our results further refine previous studies on the clinical feasibility of CI as the first treatment modality to elicit favorable auditory performance in children with CN aplasia. However, special attention should be paid to pediatric patients with an early postoperative CAP score ≤1 for identification of unsuccessful cochlear implants and switching to auditory brainstem implants.

Background and Objectives: We aim to explore the effects of residual auditory steady state response (ASSR) on cochlear implantation (CI) outcomes in children lacking auditory brainstem responses (ABRs). Subjects and Methods: We retrospectively reviewed the data of child CI recipients lacking ABRs. All ears were divided into two groups: with residual ASSR and without ASSR. For each frequency, the T- and C-levels and the electrical dynamic ranges of postoperative 3-month and 1-year mappings were compared between the groups. To evaluate speech perception, patients who received simultaneous bilateral CIs were divided into two groups: group 1 exhibited responses at all frequencies in both ears; in group 2, at least one ear evidenced no response. The Categories of Auditory Perception (CAP) and Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) scores were compared between the groups. Results: We enrolled 16 patients. At 2 kHz, the postoperative 3-month and 1-year T-levels of patients with residual hearing were lower than those of hearing loss group (p=0.001, p=0.035). In residual hearing group, the ASSR threshold correlated positively with the postoperative 1-year T-level (p=0.012, R-2=0.276) and C-level (p=0.002, R-2=0.374). Of 10 simultaneous bilateral CI recipients, 5 exhibited ASSRs at all frequencies and the other 5 showed no response at >= 1 frequency. The latter had higher CAP scores at the postoperative 1-year (p=0.018). Conclusions: In children exhibiting hearing loss in ABR testing, residual hearing at 2 kHz ASSR correlated positively with the post-CI T-level. Those with ASSRs at all frequencies had significantly lower CAP scores at the postoperative 1year. CI should not be delayed when marginal residual hearing is evident in ASSR.

Chang Y, et al. J Korean Med Sci. 2026 May;41:e89. https://doi.org/10.3346/jkms.2026.41.e89