To assess the safety and efficacy of navigation-guided balloon Eustachian tuboplasty (BET) compared to medical management (MM) alone in patients with chronic Eustachian tube dilatory dysfunction ...(ETD). This is a prospective, multicenter, 1:1 parallel-group, randomized controlled trial (RCT). It aims to assess the efficacy of navigation-guided BET compared to MM alone in patients with chronic ETD. The primary outcome measure was an improvement in the Eustachian tube dysfunction questionnaire (ETDQ)-7 score at the 6-week follow-up compared with baseline. Secondary outcome measures included changes in the signs and symptoms during the follow-up, changes in the score for each subcategory of ETDQ-7, type of tympanometry, pure tone audiometry, and the availability of a positive modified Valsalva maneuver. Navigation-guided BET was safely performed in all patients. A total of 38 ears of 31 patients (19 ears of 16 patients in the BET group and 19 ears of 15 patients in the control group) completed the planned treatment and 6 weeks of follow-up. More patients in the BET group (1.99 ± 0.85) had less symptomatic dysfunction than in the control group (3.40 ± 1.29) at 6 weeks post-procedure (P = 0.001). More patients experienced tympanogram improvement in the BET group at 6 weeks compared to the control group (36.5% vs. 15.8%) with a positive modified Valsalva maneuver (36.6% vs. 15.8%, P = 0.014). Additionally, air-bone gap change was significantly decreased in the BET group compared to the control group at the 6-week follow-up visit (P = 0.037). This prospective, multicenter, RCT study suggests that navigation-guided BET is a safe and superior treatment option compared to MM alone in patients with chronic ETD.
Although balloon dilation has shown promising results in the treatment of dilatory Eustachian tube (ET) dysfunction, the histological effects of ET balloon dilation (ETBD) is unknown because ...histological examination of the whole human cartilaginous ET is impossible. Animal studies are needed to elucidate the effect of ETBD so we evaluated the histological changes after ETBD in a rat model. The left ET of 20 Wistar rats was dilated with a balloon catheter and the right ET was used as a control. Five rats were sacrificed immediately after ETBD, at 1, 4 and 12 weeks after the procedure for histological examination. The epithelial cells, presence of epithelial hyperplasia, and the proportion of the goblet cells in the epithelium; the vascular structures and dimensions of the submucosa; and presence of cartilage fracture and the area of the ET lumen were evaluated and compared between the groups. Desquamation of nearly all epithelial cells and the fracture of tubal cartilages were observed immediately after ETBD. At 1-week post-ETBD, the ciliated epithelial cells started to recover with epithelial hyperplasia. The goblet cells recovered by 4 weeks post-ETBD and epithelial hyperplasia decreased but was still present at 12 weeks post-ETBD. The depth of the submucosa increased and neovascularization in this region was observed at 1-week post-ETBD and persisted up to 12 weeks post-ETBD. The lumen of the cartilaginous ET increased immediately after ETBD but decreased at 1-week post-ETBD. The cartilaginous ET lumen recovered to the normal value at 4 weeks post-ETBD. This study is the first to describe the serial histological changes to the cartilaginous ET after ETBD and helps our understanding of the histological changes that occur after an ETBD intervention for intractable ET dysfunction.
In the early days of cochlear implantation (CI) surgery, when the types of electrodes were limited and the etiology of sensorineural hearing loss (SNHL) was not well understood, the one-size-fits-all ...approach to CI held true, as in all other fields. However, in the era of personalized medicine, there have been attempts to associate CI performance with the etiology of SNHL and to establish customized surgical techniques that can maximize performance according to individual cochlear dimensions. Personalized genomic-driven assessments of CI candidates and a better understanding of genotype-phenotype correlations could provide clinically applicable diagnostic and prognostic information about questions such as who, how, and when to implant. Rigorous and strategic imaging assessments also provide better insights into the anatomic etiology of SNHL and cochlear dimensions, leading to individualized surgical techniques to augment CI outcomes. Furthermore, the precision medicine approach to CI is not necessarily limited to preoperative planning, but can be extended to either intraoperative electrode positioning or even the timing of the initial switch-on. In this review, we discuss the implications of personalized diagnoses (both genetic and nongenetic) on the planning and performance of CI in patients with prelingual and postlingual SNHL.
Oxidative stress is an important mechanism underlying cellular damage of the inner ear, resulting in hearing loss. In order to prevent hearing loss, several types of antioxidants have been ...investigated; several experiments have shown their ability to effectively prevent noise-induced hearing loss, age-related hearing loss, and ototoxicity in animal models. Exogenous antioxidants has been used as single therapeutic agents or in combination. Antioxidant therapy is generally administered before the production of reactive oxygen species. However, post-exposure treatment could also be effective. Preconditioning refers to the phenomenon of pre-inducing a preventative pathway by subtle stimuli that do not cause permanent damage in the inner ear. This renders the inner ear more resistant to actual stimuli that cause permanent hearing damage. The preconditioning mechanism is also related to the induction of antioxidant enzymes. In this review, we discuss the mechanisms underlying antioxidant-associated therapeutic effects and preconditioning in the inner ear.
The brain can compensate for the vestibular imbalance. When the unilateral labyrinthine function is lost, the asymmetry between the peripheral vestibular inputs is compensated centrally by ...readjusting the signal difference from both ears and regaining vestibular balance. If the other healthy labyrinth is destroyed, the vestibular nuclei become imbalanced again, creating spontaneous nystagmus even though there is no input to the vestibular nuclei from either labyrinth. This is called Bechterew's phenomenon; a rare and not widely recognized phenomenon that occurs in cases of bilateral sequential vestibular neuritis. This is of clinical importance because spontaneous nystagmus with bilaterally absent or diminished caloric responses may give a misleading impression of a central lesion rather than a second peripheral lesion superimposed upon the effects of central compensation for the first. Although well-documented in experimental animals, this phenomenon rarely occurs in human beings. The objective of this study is to highlight the characteristics and the progression of test results from two patients from our own experience. Along with careful history taking and physical examination, a complex interpretation of various vestibular function tests, including induced nystagmus, head impulse test, caloric test, and fundus photography, is needed to make an accurate diagnosis of bilateral sequential vestibular neuritis (BSVN).
There are still debates about timing and effectiveness of cochlear implants (CI) in pediatric subjects with significant residual hearing who do not belong to traditional indication of CI. In this ...study, we aimed to investigate the outcomes of CI, specifically on improvement of pronunciation, among hearing-impaired children already with a substantial degree of language skills as evaluated by Categories of Auditory Perception (CAP) scores or sentence score. Our cohort comprised pediatric CI recipients from July 2018 through October 2020. Among them, cases with CAP scores of 5 or 6 preoperatively were defined as "borderline cases". We investigated prevalence and etiologies, and compared speech evaluation data preoperatively and postoperatively at three time points (3, 6 and 9-12 months after implantation). Among 86 pediatric CI recipients, 13 subjects (15.12%) had language development that reached CAP scores of 5 or 6 before implantation. Postoperative speech evaluation data 6 months after implantation revealed significant improvement of pronunciation (Urimal Test of Articulation and Phonation scores: UTAP), Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) and word perception scores, but not of CAP and sentence perception scores. Notably, the significant improvement of pronunciation based on UTAP scores outstripped that of other speech parameters and this continued steadily up to one-year postoperatively. The result of the study serves as evidence for what to expect from cochlear implantation in hearing-impaired children who have already achieved a substantial degree of language development in terms of CAP scores or sentence perception scores, preoperatively.
Novel hearing loss (HL) genes are constantly being discovered, and evidence from independent studies is essential to strengthen their position as causes of hereditary HL. To address this issue, we ...searched our genetic data of families with autosomal dominant HL (ADHL) who had been tested with high-throughput DNA sequencing methods. For
CD164
, only one pathogenic variant in one family has so far been reported. For
LMX1A
, just two previous studies have revealed its involvement in ADHL. In this study we found two families with the same pathogenic variant in
CD164
and one family with a novel variant in
LMX1A
(c.686C>A; p.(Ala229Asp)) that impairs its transcriptional activity. Our data show recurrence of the same
CD164
variant in two HL families of different geographic origin, which strongly suggests it is a mutational hotspot. We also provide further evidence for haploinsufficiency as the pathogenic mechanism underlying
LMX1A
-related ADHL.
Semicircular canal and otolith functions came to be evaluated recently, but comprehensive and comparative analysis of canal and otolith dysfunction in common vestibular disorders is lacking. We aimed ...to analyze the abnormal rates of canal and otolith function in common vestibular disorders. We enrolled 302 patients who were managed for 2 months in a dizziness clinic. Results of caloric, video head impulse test (vHIT), and cervical and ocular vestibular evoked myogenic potential (cVEMP and oVEMP) tests were analyzed and compared among various diagnoses. Vestibular disorders diagnosed included recurrent vestibulopathy (RV, 27%), vestibular migraine (VM, 21%), benign paroxysmal positional vertigo (BPPV, 17%), Meniere's disease (MD, 11%), vestibular neuritis (VN, 10%), orthostatic dizziness (7%), and central lesions (3%). Lateral canal dysfunction was found most in VN (100%) and less commonly in definite MD (75%), RV (46%) and definite VM (29%). Abnormal caloric results were more common than abnormal vHIT in all disorders. Otolith dysfunction was found more frequently than lateral canal dysfunction in most vestibular disorders except VN. An abnormal cVEMP was more frequent in definite MD than the other disorders. Isolated otolith dysfunction without lateral canal dysfunction was the most found in BPPV, followed by definite VM, RV, and definite MD in decreasing order. Various patterns of involvement in canal and otoliths were revealed in vestibular disorders, suggesting different pathogenesis.
Abstract
Determining the etiology of severe-to-profound sensorineural hearing loss (SP-SNHL) in pediatric subjects is particularly important in aiding the decision for auditory rehabilitation. We ...aimed to update the etiologic spectrum of pediatric SP-SNHL by combining internal auditory canal (IAC)-MRI with comprehensive and state-of-the-art genetic testings. From May 2013 to September 2020, 119 cochlear implantees under the age of 15 years with SP-SNHL were all prospectively recruited. They were subjected to genetic tests, including exome sequencing, and IAC-MRI for etiologic diagnosis. Strict interpretation of results were made based on ACMG/AMP guidelines and by an experienced neuroradiologist. The etiology was determined in of 65.5% (78/119) of our cohort. If only one of the two tests was done, the etiologic diagnostic rate would be reduced by at least 21.8%. Notably, cochlear nerve deficiency (n = 20) detected by IAC-MRI topped the etiology list of our cohort, followed by DFNB4 (n = 18), DFNB1 (n = 10), DFNB9 (n = 10) and periventricular leukomalacia associated with congenital CMV infection (n = 8). Simultaneous application of state-of-the-art genetic tests and IAC-MRI is essential for etiologic diagnosis, and if lesions of the auditory nerve or central nerve system are carefully examined on an MRI, we can identify the cause of deafness in more than 65% of pediatric SP-SNHL cases.
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