Audiological and Vestibular Functions in Patients With Lateral Semicircular Canal Dysplasia and Aplasia

Objectives: The aim of the present study was to evaluate audiologic and vestibular functions in patients with lateral semicircular canal (LSCC) dysplasia/aplasia. Methods: We conducted a retrospective study of a patients with LSCC dysplasia and aplasia at tertiary referral center. The subjects included 15 patients with LSCC dysplasia or aplasia, with or without combined inner ear anomalies. Medical history, temporal bone computed tomography scans, pure-tone audiograms, and vestibular function test results were analyzed. Results: LSCC anomaly was identified in 15 patients (20 ears). Nine patients had unilateral LSCC dysplasia only and showed a mean pure-tone average of 45.5±28.7 dB, while three patients (33.3%) among them had normal hearing. Six patients had bilateral LSCC dysplasia/aplasia combined with other inner ear anomalies and profound bilateral hearing loss. Notably, only four out of 15 patients (26.7%) had dizziness symptoms. On caloric test, patients with isolated LSCC dysplasia showed a 51.8%±29.3% level of canal paresis (eight out of nine patients showed anomalies), whereas patients with bilateral LSCC dysplasia/aplasia presented bilateral vestibular loss. One patient with isolated LSCC underwent video-head impulse test; horizontal canal gain decreased to 0.62 (17% asymmetry) and anterior canal gain was 0.45 (52.6% asymmetry), whereas posterior canal gain was normal. Conclusion: Bilateral LSCC dysplasia/aplasia is comorbid with other inner ear anomalies and presents as profound bilateral hearing loss and vestibulopathy. In contrast, isolated unilateral LSCC dysplasia presents as ipsilateral horizontal canal paresis. Hearing function in isolated LSCC dysplasia is usually, but not always, impaired with varying severity.ope

Supplementary Effect of Choline Alfoscerate on Speech Recognition in Patients With Age-Related Hearing Loss: A Prospective Study in 34 Patients (57 Ears)

To investigate the effect of choline alfoscerate (CA) on hearing amplification in patients with age related hearing loss, we performed a prospective case-control observational study from March 2016 to September 2020. We assessed patients with bilateral word recognition score (WRS) <50% using monosyllabic words. The patients were 65-85 years old, without any history of dementia, Alzheimer's disease, parkinsonism, or depression. After enrollment, all patients started using hearing aids (HA). The CA group received a daily dose of 800 mg CA for 11 months. We performed between-group comparisons of audiological data, including pure tone audiometry, WRS, HA fitting data obtained using real-ear measurement (REM), and the Abbreviated Profile of Hearing Aid benefit scores after treatment. After CA administration, the WRS improved significantly in the CA group (4.2 ± 8.3%), but deteriorated in the control group (-0.6 ± 8.1%, p = 0.035). However, there was no significant between-group difference in the change in pure tone thresholds and aided speech intelligibility index calculated from REM. These findings suggest that the difference in WRS was relevant to central speech understanding rather than peripheral audibility. Therefore, administering oral CA could effectively enrich listening comprehension in older HA users.ope

The Era of Precision Medicine: Reshaping Usher Syndrome

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Diagnosis and Intervention of Genetic Hearing Loss

Sensorineural hearing loss (SNHL) is the most common sensory disorder in humans. A wide range of genetic and environmental factors that can cause congenital or late-onset, stable or progressive, age-related, drug-related, noise-causing, post-infection, or traumatic hearing loss leads to the SNHL. More and more comprehensive genetic studies have recently been published in patients with hearing loss in the era of target genome and massively parallel sequencing. Although rehabilitation options, to date, typically have focused on amplification with wearables or implantable devices, intriguing new gene therapy-based strategies to restore and prevent SNHL are actively being investigated using an animal model. Correcting or preventing an underlying genetic cause of hearing loss is ready to become a reality. In this article, several methods for diagnosis and treatment strategies for hereditary hearing loss in progress to date are introduced.ope

Which Patients With a Unilateral Hearing Aid for Symmetric Sensorineural Hearing Loss Have Auditory Deprivation?

Objectives: The aim of study is to find conditions that aggravate auditory deprivation in patients with symmetric hearing loss after unilateral digital, non-linear hearing aid (HA). Methods: In the retrospective case-comparison study, we assessed 47 patients with symmetric sensorineural hearing loss (SNHL), wearing unilateral conventional HAs. Audiological outcomes were assessed >1 year after HA fitting (mean duration, 31.0 months). Pure-tone audiometry in HA-aided and HA-unaided conditions was performed over time. Word recognition score (WRS) was evaluated at the most comfortable listening level. Results: The initial pure tone average of four frequency thresholds at 500, 1,000, 2,000, and 4,000 Hz (PTA4) did not show a difference of >5 dB HL between HA-aided and HA-unaided ears. WRS progressively decreased for both HA-aided and HA-unaided ears although the extent of decrease was significantly greater for HA-unaided (7.6%) than for HA-aided ears (5.1%, P<0.05). Notably, auditory deprivation in HA-unaided ears was significantly greater in patients with an initial PTA4 ≥53 dB HL (P<0.001). Conclusion: Bilateral HAs are strongly recommended, particularly for patients with moderate to severe SNHL to prevent auditory deprivation in the contralateral ear.ope

Does calmodulin regulate the bicarbonate permeability of ANO1/TMEM16A or not?

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Effect of Specific Retinoic Acid Receptor Agonists on Noise-Induced Hearing Loss

Noise is one of the most common causes of hearing loss in industrial countries. There are many studies about chemical agents to prevent noise-induced hearing loss (NIHL). However, there is no commercially available drug yet. Retinoic acid is an active metabolite of Vitamin A; it has an anti-apoptic role in NIHL. This study aims to verify the differences among selective agonists of retinoic acid receptors (RARs) in NIHL. All-trans retinoic acid (ATRA), AM80 (selective retinoic acid receptor α agonist), AC261066 (Selective retinoic acid receptor β1 agonist), and CD1530 (Selective retinoic acid λ agonist) were injected to 6-7 weeks old CJ5BL/6 mice before noise (110 dB for 3 h) exposure. In the auditory brainstem response test pre-, post 1, 3, and 7 days after noise exposure, not only ATRA but all kinds of selective RAR agonists showed protective effects in hearing threshold and wave I amplitude. Though there was no significant difference in the level of protective effects between agonists, α agonist showed the most prominent effect in preserving hearing function as well as outer hair cells after noise exposure. In conclusion, selective agonists of RAR demonstrate comparable protective effects against NIHL to retinoic acid. Given that these selective RAR agonists have less side effects than retinoic acid, they may be promising potential drugs against NIHL.ope

Feasibility of Revision Cochlear Implant Surgery for Better Speech Comprehension

BACKGROUND AND OBJECTIVES: The purpose of this study was to evaluate the efficacy of revision cochlear implant (CI) surgery for better speech comprehension targeting patients with low satisfaction after first CI surgery. SUBJECT AND METHODS: Eight patients who could not upgrade speech processors because of an too early CI model and who wanted to change the whole system were included. After revision CI surgery, we compared speech comprehension before and after revision CI surgery. Categoies of Auditory Performance (CAP) score, vowel and consonant confusion test, Ling 6 sounds, word and sentence identification test were done. RESULTS: The interval between surgeries ranged from eight years to 19 years. Same manufacturer's latest product was used for revision surgery in six cases of eight cases. Full insertion of electrode was possible in most of cases (seven of eight). CAP score (p-value=0.01), vowel confusion test (p-value=0.041), one syllable word identification test (p-value=0.026), two syllable identification test (p-value=0.028), sentence identification test (pvalue=0.028) had significant improvement. Consonant confusion test (p-value=0.063), Ling 6 sound test (p-value=0.066) had improvement but it is not significant. CONCLUSIONS: Although there are some limitations of our study design, we could identify the effect of revision (upgrade) CI surgery indirectly. So we concluded that if patient complain low functional gain or low satisfaction after first CI surgery, revision (device upgrade) CI surgery is meaningful even if there is no device failure.ope

전정신경염 환자에서 비디오두부충동검사 및 칼로릭검사의 장기추적결과의 분석

Background and Objectives : Video head impulse tests (vHITs) and caloric tests are widely used to assess the loss of vestibular function in acute vestibular neuritis. Although previous studies have reported on the results of each test, longitudinal comparison of these tests is rare. In the present study, vHITs and caloric tests were performed in patients with unilateral vestibular neuritis during the acute phase and after a long follow-up period (＞6 months). The goal of this study was to evaluate the changes in vHIT and caloric test results and to analyze the relationships between them. Subjects and Method : Between September 2013 and December 2015, charts from 13 patients with unilateral vestibular neuritis were retrospectively reviewed. Among the 13 patients, caloric tests and vHITs were performed in 9 and 10 patients, respectively. Results of the vHITs and caloric tests were analyzed and the changes were compared. Results : During the acute phase of vestibular neuritis, the results of the caloric test showed an increase in canal paresis (CP), and the results of the vHIT showed a decrease in horizontal gain. Although subjective symptoms improved in all patients after a long follow-up period (mean: 13.9 months), the occurrence of CP determined from the caloric test was not significantly changed (p;=0.889). On the other hand, the mean horizontal gain of the vHIT had improved significantly (p;＜0.05). Conclusion : While CP determined from the caloric test did not change after a long follow-up period, the decreased horizontal gain in the vHIT was significantly recovered in patients with unilateral vestibular neuritis.ope

Synergistic effect of smoking on age-related hearing loss in patients with diabetes

This study investigated the synergistic effects of risk factors on age-related hearing loss (ARHL) using nationwide cross-sectional data of 33,552 individuals from the 2010‒2013 Korea National Health and Nutrition Examination Survey. Patients with ARHL were selected based on their pure-tone audiometry results. Previously reported risk factors for ARHL were analyzed using logistic regression and propensity score-matching, and synergistic effects between risk factors were analyzed using propensity score-matching. Of the 12,570 individuals aged 40-79 years, 2002 (15.9%) met the criteria for ARHL. Male sex, exposure to occupational noise, and diabetes showed a significant relationship with ARHL (p < 0.05) in both the logistic regression and propensity score-matching analyses. Smoking and diabetes showed the strongest significant synergistic effect on ARHL (odds ratio [OR] 1.963, 95% confidence interval [CI] 1.285‒2.998; p = 0.002). In the subgroup analysis based on smoking status, current smokers with diabetes had a significant relationship with ARHL (OR 1.883, CI 1.191‒2.975; p = 0.009), whereas ex-smokers with diabetes did not (OR 1.250; CI 0.880‒1.775; p = 0.246). This implies that current smokers with diabetes may benefit from the cessation of smoking. In conclusion, patients with diabetes should strictly avoid or cease smoking to prevent the progression of ARHL.ope