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Introduction: The aim of the study was to evaluate the vestibulo-ocular reflex (VOR) using the video head impulse test (vHIT) as a vestibular function assessment method in patients with unilateral chronic otitis media (COM). Methods: Twenty-eight patients with unilateral COM scheduled for middle ear surgery, including 12 males and 16 females with a mean age of 54.5 years, successfully underwent preoperative vHIT. vHIT variables, including VOR gain, VOR gain asymmetry, and the incidence of corrective saccades, were compared between the ipsilesional and contralesional ears. Results: Ten (35.7%) patients reported varying degrees of disequilibrium or lightheadedness. The VOR gain in the ipsilesional and contralesional ears was 1.01 ± 0.10 and 1.04 ± 0.11, 0.97 ± 0.12 and 0.94 ± 0.13, and 0.97 ± 0.13 and 1.04 ± 0.15 in the horizontal canal (HC), anterior canal, and posterior canal (PC), respectively. The average VOR gains in the ipsilesional ears were within the predefined range of normal values; however, the gains in HC and PC significantly decreased compared with those in contralesional ears (p = 0.038 in HC and p = 0.015 in PC). The prevalence of corrective saccades, including overt and/or covert saccades, did not differ significantly between the two ears. Conclusion: This study assessed the pathophysiology of the inner ear affected by chronic middle ear infection by quantitatively comparing the VOR using vHIT. It demonstrated that vHIT can be a practical assessment of vestibular function in patients with COM. [ABSTRACT FROM AUTHOR]

We report a rare case of isolated malleus dislocation into the external auditory canal with lateralized intact tympanic membrane following a head trauma. The patient was a 63-year-old woman who presented at the outpatient department of our institute with hearing loss of 10 years' duration after a bicycle accident. During physical examination of the patient, total dislocation of the malleus-like bony structure into the external auditory canal on the right side was observed. In the computed tomography scan, an isolated malleus dislocation with intact incus-stapes articulation was identified. The patient was successfully treated with endoscopic exploratory tympanotomy and partial ossicular replacement prosthesis. The isolated malleus dislocation can rarely occur after trauma. A careful diagnostic step through history, physical examination, and temporal bone computed tomography scan are needed to confirm this rare condition. [ABSTRACT FROM AUTHOR]

Objective: Tinnitus is a common symptom of idiopathic sudden sensorineural hearing loss (ISSHL). This study aimed to investigate the characteristics and outcomes of acute tinnitus in patients with ISSHL. Methods: A total of 59 patients with ISSHL and acute tinnitus were enrolled. All patients underwent audiological assessment, tinnitus matching for loudness and frequency, and steroid treatment for sudden hearing loss. Tinnitus‐related distress was assessed using the tinnitus handicap inventory (THI) questionnaire. The outcomes of hearing recovery and tinnitus remission were investigated retrospectively. Results: The loudness and pitch of acute tinnitus were 63.2 ± 22.4 dB HL and 2010.63 ± 2368.99 Hz, respectively. Complete and partial recovery rates of acute tinnitus in ISSHL patients were 32.2% and 39% according to the THI scores. The group with complete recovery of hearing showed significantly greater improvement in tinnitus distress than the group with no improvement in hearing. The loudness and pitch of tinnitus did not correlate with tinnitus remission. Conclusion: Hearing recovery is more important for tinnitus remission than the psychoacoustic characteristics of acute tinnitus. Timely identification and proper treatment of hearing loss are important to improve tinnitus remission in patients with ISSHL. Level of Evidence: Level 4. [ABSTRACT FROM AUTHOR]

Objective: To expand our understanding of the pathophysiological mechanisms underlying vestibular neuritis and labyrinthitis by identifying any difference in the vestibulo‐ocular reflex for each semicircular canal. Study Design: Retrospective analysis. Setting: The Department of Otorhinolaryngology – Head and Neck Surgery, Chosun University Hospital, from January 2015 to December 2021. Methods: We included 23 vestibular neuritis and 27 labyrinthitis patients who had been hospitalized. Pure‐tone audiometry, a bithermal caloric test, and a video head‐impulse test were performed within 5 days of symptom onset. Results: In the vestibular neuritis group, mean vestibulo‐ocular reflex gains were decreased to 0.51 in the ipsilesional horizontal canal and 0.55 in anterior canal, leading to marked asymmetry, whereas the gain of the ipsilesional posterior canal was relatively preserved at 0.85. In the labyrinthitis group, the mean vestibulo‐ocular reflex gain was 0.72 in the ipsilesional horizontal canal, 0.73 in the ipsilesional anterior canal, and 0.55 in the ipsilesional posterior canal. We observed statistical differences in the vestibulo‐ocular reflex gain and incidence of corrective saccades on the ipsilesional side in three semicircular canals between the groups (p =.002 for horizontal canal, p =.003 for anterior canal, and p <.001 for posterior canal). The receiver operating characteristic curve showed that pure‐tone audiometry, ipsilesional posterior canal gain, and gain asymmetry of posterior canal were excellent parameters for distinguishing labyrinthitis from vestibular neuritis. Conclusion: Vestibular neuritis and labyrinthitis patients have different degrees and patterns of video head‐impulse test involvement in the three semicircular canals, suggesting that the two distinct disorders may have different etiologies. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate the efficacy of galvanic vestibular stimulation (GVS) for recovering from the locomotor and spatial memory deficits of a murine bilateral vestibular deafferentation (BVD) model. Methods: Male C57BL/6 mice (n = 36) were assigned to three groups: bilateral labyrinthectomy with (BVD_GVS group) and without (BVD_non-GVS group) the GVS intervention, and a control group with the sham operation. We used the open field and Y maze, and Morris water maze (MWM) tests to assess locomotor and visuospatial cognitive performance before (baseline) and 3, 7, and 14 days after surgical bilateral labyrinthectomy. For the GVS group, a sinusoidal current at the frequency at 1 Hz and amplitude 0.1 mA was delivered for 30 min daily from the postoperative day (POD) 0 to 4 via electrodes inserted subcutaneously close to both the bony labyrinths. Results: Short-term spatial memory was significantly impaired in bilaterally labyrinthectomized mice (BVD_non-GVS group), as reflected by decreased spontaneous alternation performance in the place recognition test and time spent in the novel arm and increased same arm return in the Y-maze test, compared with the control. Long-term spatial memory was also impaired, as indicated by a longer escape latency in the hidden platform trial and a lower percentage of time spent in the target quadrant in the probe trial of the MWM. GVS application significantly accelerated the recovery of locomotion and short-term and long-term spatial memory deficits in the BVD mice. Conclusions: Our data demonstrate that locomotion, short-term, and long-term (at least 2 weeks) spatial memory were impaired in BVD mice. The early administration of sinusoidal GVS accelerated the recovery of those locomotion and spatial memory deficiencies. GVS could be applied to patients with BVD to improve their locomotion and vestibular cognitive functioning. [ABSTRACT FROM AUTHOR]

This study aimed to investigate the disparity in locomotor and spatial memory deficits caused by left- or right-sided unilateral vestibular deafferentation (UVD) using a mouse model of unilateral labyrinthectomy (UL) and to examine the effects of galvanic vestibular stimulation (GVS) on the deficits over 14 days. Five experimental groups were established: the left-sided and right-sided UL (Lt.-UL and Rt.-UL) groups, left-sided and right-sided UL with bipolar GVS with the cathode on the lesion side (Lt.-GVS and Rt.-GVS) groups, and a control group with sham surgery. We assessed the locomotor and cognitive-behavioral functions using the open field (OF), Y maze, and Morris water maze (MWM) tests before (baseline) and 3, 7, and 14 days after surgical UL in each group. On postoperative day (POD) 3, locomotion and spatial working memory were more impaired in the Lt.-UL group compared with the Rt.-UL group (p < 0.01, Tamhane test). On POD 7, there was a substantial difference between the groups; the locomotion and spatial navigation of the Lt.-UL group recovered significantly more slowly compared with those of the Rt.-UL group. Although the differences in the short-term spatial cognition and motor coordination were resolved by POD 14, the long-term spatial navigation deficits assessed by the MWM were significantly worse in the Lt.-UL group compared with the Rt.-UL group. GVS intervention accelerated the vestibular compensation in both the Lt.-GVS and Rt.-GVS groups in terms of improvement of locomotion and spatial cognition. The current data imply that right- and left-sided UVD impair spatial cognition and locomotion differently and result in different compensatory patterns. Sequential bipolar GVS when the cathode (stimulating) was assigned to the lesion side accelerated recovery for UVD-induced spatial cognition, which may have implications for managing the patients with spatial cognitive impairment, especially that induced by unilateral peripheral vestibular damage on the dominant side. [ABSTRACT FROM AUTHOR]

Objectives: To investigate the ameliorating effects of sinusoidal galvanic vestibular stimulation (GVS) on vestibular compensation from unilateral vestibular deafferentation (UVD) using a mouse model of unilateral labyrinthectomy (UL). Methods: Sixteen male C57BL/6 mice were allocated into two groups that comprise UL groups with GVS (GVS group, n = 9) and without GVS intervention (non-GVS group, n = 7). In the experimental groups, we assessed vestibulo-ocular reflex (VOR) recovery before (baseline) and at 3, 7, and 14 days after surgical unilateral labyrinthectomy. In the GVS group, stimulation was applied for 30 min daily from postoperative days (PODs) 0–4 via electrodes inserted subcutaneously next to both bony labyrinths. Results: Locomotion and VOR were significantly impaired in the non-GVS group compared to baseline. The mean VOR gain of the non-GVS group was attenuated to 0.23 at POD 3 and recovered continuously to the value of 0.54 at POD 14, but did not reach the baseline values at any frequency. GVS intervention significantly accelerated recovery of locomotion, as assessed by the amount of circling and total path length in the open field tasks compared to the non-GVS groups on PODs 3 (p < 0.001 in both amount of circling and total path length) and 7 (p < 0.01 in amount of circling and p < 0.001 in total path length, Mann–Whitney U -test). GVS also significantly improved VOR gain compared to the non-GVS groups at PODs 3 (p < 0.001), 7 (p < 0.001), and 14 (p < 0.001, independent t -tests) during sinusoidal rotations. In addition, the recovery of the phase responses and asymmetry of the VOR was significantly better in the GVS group than in the non-GVS group until 2 weeks after UVD (phase, p = 0.001; symmetry, p < 0.001 at POD 14). Conclusion: Recoveries for UVD-induced locomotion and VOR deficits were accelerated by an early intervention with GVS, which implies that GVS has the potential to improve vestibular compensation in patients with acute unilateral vestibular failure. [ABSTRACT FROM AUTHOR]

Objective: In the present study, we characterized the vestibulo-ocular reflex (VOR) gain and properties of corrective saccades (CS) in patients with posterior inferior cerebellar artery (PICA) stroke and determined the best parameter to differentiate PICA stroke from benign peripheral vestibular neuritis (VN). In particular, we studied CS amplitude and asymmetry in video head impulse tests (vHITs) to discriminate these two less-studied disease conditions. Methods: The vHITs were performed within 1 week from symptom onset in patients with PICA stroke (n = 17), patients with VN (n = 17), and healthy subjects (HS, n = 17). Results: PICA stroke patients had bilaterally reduced VOR gains in the horizontal semicircular canal (HC) and the posterior semicircular canal (PC) compared with HSs. When compared with VN patients, PICA stroke patients showed preserved gains in the HC and anterior semicircular canal (AC) bilaterally (i.e., symmetric VOR gain). Similar to VOR gain, smaller but bilaterally symmetric CS in the HC and AC were observed in PICA stroke patients compared with VN patients; the mean amplitude of CS for the ipsilesional HC was reduced (p < 0.001, Mann–Whitney U -test), but the mean amplitude of CS for the contralesional HC was increased (p < 0.03, Mann–Whitney U -test) in PICA stroke compared with VN. The receiver operating characteristic (ROC) curve showed that CS amplitude asymmetry (CSs) and VOR gain asymmetry (Gs) of HC are excellent parameters to distinguish PICA stroke from VN. Conclusion: In the current study, we quantitatively investigated the VOR gain and CS using vHITs for three semicircular canals in PICA stroke and VN patients. In addition to VOR gain, quantitative assessments of CS using vHITs can provide sensitive and objective parameters to distinguish between peripheral and central vestibulopathies. [ABSTRACT FROM AUTHOR]

Objectives/Hypothesis: To investigate the efficacy of direct implantation of a Vibrant Soundbridge (VSB) implant in the oval window (OW) without the use of an OW coupler in patients with severe mixed hearing loss. Study Design Retrospective chart review Methods: A total of 62 patients underwent VSB implantation between July 2016 and December 2018 at Severance Hospital in Seoul, South Korea. Among them, eight patients (nine ears) with moderate‐to‐severe mixed hearing loss were implanted with a VSB directly in the OW. A floating mass transducer (FMT) was attached to the stapes footplate and covered with tragal cartilage. The outcomes were evaluated using pure‐tone audiogram and speech audiogram preoperatively and postoperatively. Word recognition score (WRS; % correct) were measured at the most comfortable loudness (MCL) level to evaluate speech perception. Results: All cases posed difficulty with round window vibroplasty during surgery, and eventually, an FMT was appropriately placed in the OW without a coupler. Preoperative and postoperative bone conduction thresholds were not different. VSB‐aided threshold improved in terms of functional and effective gains. Interestingly, four cases showed improved air conduction thresholds without the use of a VSB. In addition, MCL level with a VSB was significantly lower than that with a hearing aid, and VSB‐aided WRS improved over time. Conclusions: Direct implantation of a VSB in the OW without the use of a coupler showed favorable hearing outcomes, and the OW vibroplasty was safe. Direct OW vibroplasty without a coupler is a reliable procedure and can be a good option for hearing rehabilitation in patients with severe mixed hearing loss. Level of Evidence: 4 Laryngoscope, 2020 [ABSTRACT FROM AUTHOR]

Vestibular schwannoma (VS) originates from Schwann cells in the superior or inferior vestibular nerve. Identifying the precise origin will help in determining the optimal surgical approach. We retrospectively analyzed the preoperative vestibular function test according to VS origin to determine whether the test is a valuable indicator of tumor origin. Forty-seven patients with VS (male:female = 18:29, mean age: 54.06 ± 13.50 years) underwent the cochleovestibular function test (pure-tone audiometry, caloric test, video head impulse test (vHIT), cervical and ocular vestibular-evoked myogenic potential, and posturography). All patients then underwent surgical removal of VS, and the schwannoma origin was confirmed. The tumor originated from the superior vestibular nerve (SVN group) in 21 patients, the inferior vestibular nerve (IVN group) in 26 patients, and an undetermined site in eight patients. The only value that differed significantly among the groups was the gain of the vestibular-ocular reflex (VOR) in the ipsilesional posterior canal (iPC) during the vHIT. Our results indicate that VOR gain in the iPC may be used to predict the nerve origin in patients with VS. Other cochleovestibular function tests have limited value to discriminate nerve origins, especially in cases of medium to large VS. [ABSTRACT FROM AUTHOR]

We report an extremely rare case with bilateral horizontal semicircular canal (HSC) dysplasia presenting as hearing loss with recurrent vertigo mimicking Meniere's disease in a previously healthy 49-year-old male patient. HSC malformation is one of the common isolated developmental anomalies of the bony labyrinth usually associated with varying degree of hearing loss. He suffered from recurrent episodes of vertigo lasting more than 20 minutes accompanied by left hearing loss and tinnitus for 3 years. Pure-tone audiometry revealed a mild to moderate hearing loss at low frequencies in the left ear. Bithermal caloric testing revealed significant left-sided canal paralysis, but other vestibular testing was normal. Computerized tomography (CT) of the temporal bone showed the left HSC hypoplasia lacking a central bony island fused together with an enlarged vestibule, and right dysplasia, finally diagnosed with bilateral HSC dysplasia. Meniere's disease-like recurrent vertigo attack and hearing loss were suspected to be caused by subsequent endolymphatic hydrops which could be due to HSC dysplasia., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Introduction: The international outcome inventory for hearing aids (IOI-HA) is a questionnaire widely used to assess the subjective benefits of hearing aids. This study aimed to evaluate the relationship between IOI-HA outcomes and target mismatch in real-ear measurement (REM)., Methods: Thirty-four ears of 25 patients who had worn hearing aids were evaluated using the Korean version of the IOI-HA and REM after fitting for 2 months. Real-ear insertion gain (REIG) was measured at three different levels of input intensity - 50, 65, and 80 dB sound pressure level (SPL) - and a frequency range of 0.25-6 kHz. Factors 1 and 2 and total IOI-HA scores were compared with the mismatches of REIGs and target gains of REM., Results: Factor 1, factor 2, and total IOI-HA scores were 14.6 ± 3.5, 11.4 ± 2.2, and 25.9 ± 5.1, respectively. The averages of the difference of REIGs and target gains in REM at 50, 65, and 80 dB SPL input levels were -3.1 ± 6.7, -2.3 ± 7.2, and -3.0 ± 8.2, respectively. Factors 1 and 2 scores of the IOI-HA showed significant correlations with target mismatch in REM at 1 kHz and 0.75 kHz frequencies, respectively. Total IOI-HA scores had significant correlations with target mismatches in REM at 0.75 and 1 kHz frequencies., Conclusion: IOI-HA scores correlated with target mismatch in REM at mid frequencies. The IOI-HA can be a useful screening measure for evaluating the necessity of further adjustments in hearing aids through REM at mid frequencies., (© 2024 S. Karger AG, Basel.)

There is a lack of comparative studies examining changes in vestibulo-ocular reflex (VOR) gain with head velocity in the video head impulse test (vHIT) of patients with vestibular neuritis (VN). Thus, the purpose of present study was to identify the effect of head impulse velocity on the gain of the VOR during the vHIT in patients with VN. Head impulse velocities ranging from 100%-200°/s [158.08 ± 23.00°/s in the horizontal canal (HC), 124.88 ± 14.80°/s in the anterior canal (AC), and 122.92 ± 14.26°/s in the posterior canal (PC) were used during vHIT trials of 32 patients with VN. Differences in VOR gain on the ipsilesional and contralesional sides according to head velocity were analyzed. The mean VOR gains in ipsilesional side were decreased to 0.47 in the HC and 0.56 in the AC, leading to marked asymmetry compared to the contralesional side; PC gain was relatively preserved at 0.82 in the ipsilesional side. The mean head impulse velocity applied during vHIT trials in each semicircular canal plane did not differ bilaterally. On the contralesional side, VOR gain was negatively correlated with head impulse velocity (R2=0.25, P=.004 in HC; R2=0.17, P=.021 in AC; R2=0.24, P=.005 in PC), while VOR gain on the ipsilesional sides of the HC and AC was not. Head impulse velocity may have a differential impact on VOR gain, depending on the degree of deficit. Increasing head velocity in vHIT may be considered to identify subtle deficits on the contralesional side of patients with VN.

Objectives: To investigate the deficits of spatial memory and navigation from unilateral vestibular deafferentation (UVD) and to determine the efficacy of galvanic vestibular stimulation (GVS) for recovery from these deficits using a mouse model of unilateral labyrinthectomy (UL). Methods: Thirty-six male C57BL/6 mice were allocated into three groups that comprise a control group and two experimental groups, UVD with (GVS group) and without GVS intervention (non-GVS group). In the experimental groups, we assessed the locomotor and cognitive behavioral function before (baseline) and 3, 7, and 14 days after surgical UL, using the open field (OF), Y maze, and Morris water maze (MWM) tests. In the GVS group, the stimulations were applied for 30 min daily from postoperative day (POD) 0-4 via the electrodes inserted subcutaneously close to both bony labyrinths. Results: Locomotion and spatial cognition were significantly impaired in the mice with UVD non-GVS group compared to the control group. GVS significantly accelerated recovery of locomotion compared to the control and non-GVS groups on PODs 3 ( p < 0.001) and 7 ( p < 0.05, Kruskal-Wallis and Mann-Whitney U tests) in the OF and Y maze tests. The mice in the GVS group were better in spatial working memory assessed with spontaneous alternation performance and spatial reference memory assessed with place recognition during the Y maze test than those in the non-GVS group on POD 3 ( p < 0.001). In addition, the recovery of long-term spatial navigation deficits during the MWM, as indicated by the escape latency and the probe trial, was significantly better in the GVS group than in the non-GVS group 2 weeks after UVD ( p < 0.01). Conclusions: UVD impairs spatial memory, navigation, and motor coordination. GVS accelerated recoveries in short- and long-term spatial memory and navigation, as well as locomotor function in mice with UVD, and may be applied to the patients with acute unilateral vestibular failure., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Nguyen, Nam, Kang, Han, Kim, Dieterich and Oh.)

Purpose: The concept of hidden hearing loss can explain the discrepancy between a listener's perception of hearing ability and hearing evaluation using pure tone audiograms. This study investigated the utility of the suprathreshold auditory brainstem response (ABR) for the evaluation of hidden hearing loss in noise-exposed ear with normal audiograms., Materials and Methods: A total of 15 patients (24 ears) with normal auditory thresholds and normal distortion product otoacoustic emissions were included in a retrospective analysis of medical records of 80 patients presenting with histories of acute noise exposure. The control group included 12 subjects (24 ears) with normal audiograms and no history of noise exposure. Pure tone audiometry and suprathreshold ABR testing at 90 dB peSPL were performed. The amplitudes and latencies of ABR waves I and V were compared between the noise-exposed and control groups., Results: We found no significant difference in the wave I or V amplitude, or the wave I/V ratio, between the two groups. The latencies of ABR wave I, V, and I-V interpeak interval were compared, and no significant intergroup difference was observed., Conclusion: The results suggest that either hidden hearing loss may not be significant in this cohort of patients with acute noise exposure history, or the possible damage by noise exposure is not reflected in the ABRs. Further studies are needed to inquire about the role of ABR in identification of hidden hearing loss., Competing Interests: The authors have no potential conflicts of interest to disclose., (© Copyright: Yonsei University College of Medicine 2021.)

Objectives: The aim of this study was to investigate whether preserved vestibular function in the high-frequency range influences the prognosis of patients with bilateral vestibulopathy (BVP) after vestibular rehabilitation., Methods: Twenty-four patients followed up with vestibular rehabilitation were recruited. The enrolled patients were divided into two groups according to the preservation of the high-frequency vestibulo-ocular reflex (VOR) based on the video head impulse test (vHIT). The results of computerized dynamic posturography and the Dizziness Handicap Inventory (DHI) survey collected at baseline and at the 6-month follow-up after vestibular rehabilitation therapy were analyzed., Results: Both groups showed significantly increased composite and DHI scores after follow-up with vestibular rehabilitation. The group with preserved high-frequency VOR showed a better composite score (p=0.064) and vestibular score (p= 0.008) than the group with lost high-frequency VOR at the 6-month follow up. The DHI score significantly decreased only in the group with lost high-frequency VOR (p=0.047). Among the three vestibular function tests (caloric test, rotary chair test, and vHIT) used to diagnose BVP, only vHIT showed a significant correlation (p=0.015) with a favorable prognosis (composite score ≥70)., Conclusion: Better treatment outcomes are likely in patients with BVP with preserved vestibular function in response to high-frequency stimulation, as measured by the vHIT.

Background and Purpose: The associations between hearing loss (HL) and the mechanisms underlying cognitive impairment (CI) remain unclear. We evaluated the effects of clinical factors, vascular magnetic resonance imaging (MRI) markers, and CI mechanisms on HL., Methods: In total, 112 patients with CI (59% demented) and subjective HL prospectively underwent MRI, amyloid positron-emission tomography (PET), hearing evaluations, and neuropsychological tests including a language comprehension test. Patients were categorized into pure-Alzheimer's disease-related CI (ADCI), pure-Lewy-body disease-related CI (LBCI), mixed-ADCI/LBCI, and non-ADCI/LBCI groups based on clinical features and PET biomarkers., Results: The risk of peripheral HL [defined as a pure-tone average (PTA) threshold >40 dB] was higher in the pure-LBCI group than in the pure-ADCI and mixed-ADCI/LBCI groups, and lower in the presence of ADCI. The non-ADCI/LBCI group had the most-severe vascular MRI markers and showed a higher risk of peripheral HL than did the pure-ADCI and mixed-ADCI/LBCI groups. While the pure-LBCI group had a higher risk of comprehension dysfunction than the pure-ADCI group regardless of the PTA and the score on the Korean version of the Mini Mental State Examination (K-MMSE), those in the pure-LBCI group even with a better K-MMSE score had a risk of comprehension dysfunction comparable to that in the mixed-ADCI/LBCI group due to a worse PTA., Conclusions: Peripheral HL could be associated with the absence of significant β-amyloid deposition in patients with CI and characteristic of the pure-LBCI and non-ADCI/LBCI groups., Competing Interests: The authors have no potential conflicts of interest to disclose., (Copyright © 2021 Korean Neurological Association.)

Background: Congenital cytomegalovirus (CMV) infection is the most common non-genetic cause of sensorineural hearing loss (SHNL) in children. Only about 10% to 15% of children with congenital CMV are symptomatic, and most are not diagnosed at birth. About 7% to 15% of clinically asymptomatic patients may develop later complications, including SNHL, which is the most common sequela in clinically asymptomatic patients. In this study, hearing status was investigated in children with confirmed CMV infection and neonatal hearing screening (NHS) histories were reviewed to explore hearing loss caused by CMV., Methods: The medical records of 58 children who were diagnosed with confirmed CMV infection were reviewed for clinical symptoms and signs of CMV infection. Hearing status was evaluated with age-appropriate audiological test batteries., Results: A total of 58 children (M:F = 32:26 patients; age at study: mean, 5.62 years, range, 1-10 years) were diagnosed serologically with CMV infection (14 patients, 21.1%), or diagnosed via PCR of serum (5, 7.9%) and/or PCR from urine (19, 26.8%). Hearing loss was confirmed in 11 children (19.0%), being bilateral in 6 (54.5%), and unilateral in 5 (45.5%). Note that 7 of 17 ears with hearing loss passed NHS and were diagnosed only after re-evaluation when CMV infection was identified., Conclusion: Hearing loss is a serious complication of CMV infection in children. Our results highlight the importance of timely audiological evaluation in children with clinically symptomatic CMV infection even if they pass NHS., Competing Interests: The authors have no potential conflicts of interest to disclose., (© 2020 The Korean Academy of Medical Sciences.)

Background and Objectives: This study was undertaken to investigate the educational status in bilateral prelingual deaf children with a cochlear implant (CI), also known as early cochlear implantees (CIs). Type of schooling and enrollment rate of tertiary education were analyzed as primary results., Subjects and Methods: Participants in this study comprised a highly homogeneous group of deaf patients who underwent cochlear implantation at a similar age. Sixty-four Korean patients were enrolled. Statistical data for disabled populations and the general population were obtained from the National Statistics Korea., Results: Among 64 patients, 46, 8, and 10 attended mainstream, integrated, and special schools, respectively. Notably, there was a significant difference in the type of school between hearing-impaired and CI groups (p=0.007). Ten of 13 patients enrolled in tertiary education., Conclusions: CI users were more likely than hearing impaired students to attend mainstream school. The enrollment rate of CI users in tertiary education was the same as that of the general population.

Background: The endolymphatic sac (ES) is a cystic structure situated on the posterior fossa dura and is connected to the luminal space of the vestibular organ through the endolymphatic duct, which branches into the utricular and saccular ducts. Unlike the cochlea and vestibule, the ES does not contain sensory epithelium in its luminal space, and a single layer of epithelial cells line the luminal surface area. The ES in the inner ear is thought to play a role in the regulation of inner ear homeostasis, fluid volume, and immune reaction. If these functions of the ES are disrupted, dysfunction of the inner ear may develop. The most well-known pathology arising from dysfunction of the ES is endolymphatic hydrops, characterized by an enlarged endolymphatic space due to the accumulation of excessive endolymphatic fluid. Although, molecular identities and functional evidence for the roles were identified in animal studies, basic studies of the human ES are relatively uncommon compared with those using animal tissues, because of limited opportunity to harvest the human ES., Methods: In this study, molecular and functional evidence for the role of the human ES in the development of endolymphatic hydrops are reviewed., Results and Conclusions: Although evidence is insufficient, studies using the human ES have mostly produced findings similar to those of animal studies. This review may provide a basis for planning further studies to investigate the pathophysiology of disorders with the finding of endolymphatic hydrops.

Objectives: Neonatal hyperbilirubinemia is considered one of the most common causative factors of hearing loss. Preterm infants are more vulnerable to neuronal damage caused by hyperbilirubinemia. This study aimed to evaluate the effect of hyperbilirubinemia on hearing threshold and auditory pathway in preterm infants by serial auditory brainstem response (ABR). In addition, we evaluate the usefulness of the unconjugated bilirubin (UCB) level compared with total serum bilirubin (TSB) on bilirubin-induced hearing loss., Methods: This study was conducted on 70 preterm infants with hyperbilirubinemia who failed universal newborn hearing screening by automated ABR. The diagnostic ABR was performed within 3 months after birth. Follow-up ABR was conducted in patients with abnormal results (30 cases). TSB and UCB concentration were compared according to hearing threshold by ABR., Results: The initial and maximal measured UCB concentration for the preterm infants of diagnostic ABR ≥40 dB nHL group (n=30) were statistically higher compared with ABR ≤35 dB nHL group (n=40) (P=0.031 and P=0.003, respectively). In follow-up ABR examination, 13 of the ABR ≥40 dB nHL group showed complete recovery, but 17 had no change or worsened. There was no difference in bilirubin level between the recovery group and non-recovery group., Conclusion: UCB is a better predictor of bilirubin-induced hearing loss than TSB in preterm infants as evaluated by serial ABR. Serial ABR testing can be a useful, noninvasive methods to evaluate early reversible bilirubin-induced hearing loss in preterm infants.

Objectives: Carcinomas of the external auditory canal (EAC) are rare, and management remains challenging. Previous studies seeking prognostic factors for EAC cancers included cancers other than carcinomas. In this study, we analyzed the treatment outcomes of, prognostic factors for, and survival rates associated with specifically squamous cell carcinoma (SCC) of the EAC., Methods: A retrospective review of 26 consecutive patients diagnosed with SCCs of the EAC in a 10-year period was performed in terms of clinical presentation, stage, choice of surgical procedure, and adjunct therapy. Overall survival (OS) and recurrence-free survival (RFS) were calculated and univariate analysis of prognostic factors was performed., Results: The median age of the 26 patients with SCCs of the EAC was 63 years (range, 40 to 72 years), and 16 males and 10 females were included. According to the modified University of Pittsburgh staging system, the T stages were T1 in 11, T2 in six, T3 in four, and T4 in five cases. The surgical procedures employed were wide excision in three cases, lateral temporal bone resection (LTBR) in 17, and extended LTBR in four, and subtotal temporal bone resection in two. Two patients underwent neoadjuvant chemotherapy, and two underwent adjuvant chemotherapy. One patient received preoperative radiation therapy, and eleven received postoperative radiation therapy. Of the possibly prognostic factors examined, advanced preoperative T stage and advanced overall stage were significant predictors of RFS, but not of OS., Conclusion: The advanced T stage and overall stage were associated with decreased survival after surgical treatment in patients with SCC of the EAC, highlighting the importance of clinical vigilance and early detection.

Objectives: Growth of vestibular schwannomas (VS) causes progressive vestibular symptoms and postural instability. Since the tumor grows slowly, compensation of decaying vestibular input may decrease subjective symptoms of dizziness. This study aims to estimate the relationship of subjective vestibular symptoms and objective postural instability in patients with VS., Methods: A retrospective review of 18 patients newly diagnosed with VS and with subjective vertigo symptoms was performed. The results of vestibular function tests including the sensory organization test (SOT) using computerized dynamic posturography, caloric test, and self-report measures of subjective dizziness handicap (Dizziness Handicap Inventory) and visual analogue scale were compared according to the onset of vertigo symptoms., Results: In VS patients, SOT showed decreased equilibrium score for all vestibular function related conditions, condition (C) 5 and 6, and composite (COMP) score. COMP scores were not correlated with visual analogue scale or Dizziness Handicap Inventory scores. Acute onset group included six patients and insidious onset group, 12 patients. Equilibrium scores for C5 and C6, and COMP scores were lower for insidious onset group, but the difference was not statistically significant., Conclusion: Our findings confirmed postural instability is prevalent in VS patients. SOT parameters did not differ significantly between acute onset and insidious onset groups, but increased tumor size and canal weakness were noted in the insidious onset group. Clinicians should consider that postural instability is likely present even in patients who do not complain of acute vertigo, and appropriate counseling should be discussed with the patients.