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The present study aimed to investigate the clinical features of patients with direction-changing spontaneous nystagmus (DCSN) and gain insight into its underlying mechanisms.Medical records and vestibular function test results collected in our dizziness clinic between February 2013 and February 2020 were retrospectively reviewed. Spontaneous nystagmus was recorded while sitting upright using videonystagmography for 2 min to confirm the spontaneous changes in nystagmus direction. Causative disease diagnoses were based on the patients' clinical history, audiometry results, vestibular function tests, and imaging studies.Of 4786 patients, DCSN was observed in 41 (0.86%). Causative disease diagnoses included vestibular neuritis (n = 9), lateral semicircular canal cupulopathy (n = 9), cerebellopontine angle tumor (n = 8), vestibular paroxysmia (n = 2), vestibular migraine (n = 2), vestibular nucleus infarction (n = 1), sudden sensorineural hearing loss with vertigo (n = 2), Meniere's disease (n = 2), Ramsay Hunt syndrome (n = 1), labyrinthine fistula due to middle ear cholesteatoma (n = 1), lateral semicircular canal dysplasia (n = 1), post tympanomastoidectomy dizziness (n = 1), and head trauma (n = 2).Although the periodicity of DCSN could not be determined because of insufficiently long observation times, it was observed in various central and peripheral vestibulopathies. Careful examination of spontaneous nystagmus over a sufficient period may ensure the detection of DCSN when evaluating dizziness.

Objective To examine the treatment effects of repositioning maneuvers with the head turned 45° downwards (Gufoni maneuver) and 45° upwards (Appiani maneuver) in 25 patients with ageotropic horizontal semicircular canal (HSCC) benign paroxysmal positional vertigo (BPPV). Methods Patients were randomly assigned to the Gufoni or Appiani maneuvers, and their immediate and short‐term efficacy was investigated. Results The immediate treatment response was successful in four of 16 patients who were treated with the Appiani maneuver and were not successful in any of the nine patients treated with the Gufoni maneuver. The patients who showed spontaneous resolution during follow‐up outnumbered the patients who showed resolution of ageotropic HSCC BPPV immediately after the repositioning maneuvers. Conclusion This study showed a very low immediate resolution rate of ageotropic HSCC BPPV after the Gufoni maneuver; this may be attributed to the less effectiveness of the Gufoni maneuver against debris that often exists in the canal side of the cupula. Another interesting finding was that the rate of spontaneous resolution during the follow‐up period was higher than that of immediate resolution after the repositioning maneuvers, which may support the recently proposed hypothesis that ageotropic positional nystagmus can also be evoked by causes other than the otolithic attachment on the cupula, such as a slight change in the biochemical composition of the inner ear fluids. Level of Evidence 3.

There have been no reports regarding nystagmus observed immediately after the end of an acute vertiginous attack in patients with Meniere’s disease. The aim of this study was to demonstrate positional direction-changing nystagmus in patients with Meniere’s disease, and to discuss the mechanism that underlies this nystagmus. Video-nystagmography was recorded in two patients with definite Meniere’s disease, who showed positional direction-changing nystagmus during the period immediately after a vertigo attack. In one patient, video-nystagmographic recording was conducted 5 h after an episode of vertigo attack, and it showed very weak, persistent positional geotropic direction-changing nystagmus. In the other patient, video-nystagmographic recording was conducted 23 h after an episode of vertigo attack, and it showed very weak, persistent positional apogeotropic direction-changing nystagmus. Our patients exhibited very weak, persistent positional direction-changing nystagmus, which was geotropic in one and apogeotropic in the other. This type of positional nystagmus has been reported in other inner ear disorders and it cannot be clearly explained by typical benign paroxysmal positional vertigo. The change in chemical composition and/or electrolyte concentration of the inner ear fluid, although still unclear, may underlie the production of this characteristic nystagmus in these patients.

In terms of diagnosis, a painless isolated mass lesion around the mastoid area is rarely encountered in general practice. In the present study, we report two cases of painless benign mastoid tumors located in the postauricular region. The first patient visited our department with a painless progressing mass lesion behind the right ear, which was later revealed as an intramuscular lipoma in the mastoid origin site of the sternocleidomastoid muscle. The second patient similarly presented to our department with a chief complaint of a painless, palpable mass in the mastoid region. Biopsy results confirmed the diagnosis of an osteoma. In both cases, the tumor was surgically removed by a postauricular approach. Although osteoma and lipoma are benign tumors rarely involved in the mastoid area, presenting without symptoms, it is recommended of complete excision, especially in cases with symptoms or cosmetic deformity.

Objectives Considering that otolith particles pass through the canal until attaching to the cupula in the canal-side horizontal semicircular canal (HSCC) cupulolithiasis, comorbidity of HSCC canalolithiasis and cupulolithiasis may occur. We aimed to investigate the incidence of comorbidity of cupulolithiasis in patients with HSCC canalolithiasis and to improve treatment efficacy. Study design Retrospective study. Setting Tertiary referral academic center. Patients Ninety-seven consecutive patients with HSCC canalolithiasis between March 2017 and March 2019 were included. Main outcome measures Coexistence of HSCC cupulolithiasis was hierarchically investigated. 1) Spontaneous reversal of initial nystagmus is observed bilaterally in a head-roll test (HRT), 2) nystagmus is in the same direction at each of the three times the supine position was tested, and 3) both bowing and leaning nystagmus with opposite direction are observed. Results Of 97 patients with HSCC canalolithiasis, 2 patients (2%) had comorbid HSCC cupulolithiasis. Conclusions Although coexistence of HSCC canalolithiasis and cupulolithiasis should be considered when spontaneous reversal of nystagmus direction is observed without position change during a HRT, the incidence of coexistence is very low. However, canalith repositioning maneuvers for both canalolithiasis and cupulolithiasis should be performed in cases with comorbidity.

Ceruminous glands are located in the skin of the cartilaginous portion of the external auditory canal, and ceruminous gland adenoma originating from the middle ear mucosa is extremely rare. We report a case of middle ear ceruminous gland adenoma which caused long-standing otomastoiditis and mixed hearing loss with a large air-bone gap by obstructing the bony Eustachian tube. We discuss the clinical characteristics and histologic features of the present case.

The present study aimed to investigate the incidence and patterns of nystagmus in adult patients with acute otitis media (AOM) or otitis media with effusion (OME) without dizziness or vertigo, and discuss possible mechanisms. From February 2018 to November 2018, 34 consecutive patients with AOM or OME without dizziness were included. Nystagmus was examined with video Frenzel glasses. Of 34 adult AOM or OME patients without dizziness, nystagmus was observed in 28 patients (82%). In unilateral AOM or OME (n = 30), the most commonly observed nystagmus pattern was irritative-type direction-fixed nystagmus (n = 13), followed by paretic-type direction-fixed nystagmus (n = 8), and direction-changing positional nystagmus (n = 4). In bilateral AOM or OME (n = 4), direction-fixed nystagmus and direction-changing positional nystagmus were observed in two and one patients, respectively. Nystagmus was observed in as many as 82% of adult AOM or OME patients even though they did not complain of dizziness, and the pattern of nystagmus was either direction-fixed or direction-changing. Direct effect of inflammatory mediators penetrated from the middle ear and biochemical alteration in the inner ear fluids due to blood-perilymph barrier dysfunction may result in the presence of nystagmus in AOM or OME patients without dizziness.

Dizziness is not a common symptom in the lateral semicircular canal (LSCC) dysplasia, and nystagmus findings has been rarely described in LSCC dysplasia.To investigate the characteristics of spontaneous and positional nystagmus in patients with LSCC dysplasia.Patterns of spontaneous and positional nystagmus of twelve patients with LSCC dysplasia, who visited our clinic with a chief complaint of dizziness, were investigated.LSCC dysplasia was observed unilaterally in 4, and bilaterally in 8 patients. Non-rotatory dizziness with various onset times was the most common description of dizziness, and unilateral caloric weakness was observed in 82% of patients. Direction-fixed nystagmus, in which the direction of spontaneous nystagmus was same with that of positional nystagmus, was observed in 9 patients, and direction-changing positional nystagmus, in which the direction of nystagmus was changed from that of spontaneous nystagmus by positioning maneuvers, was observed in 3 patients, of whom down-beating or direction-changing spontaneous nystagmus was observed in one patient each.While direction-fixed horizontal nystagmus is the most commonly observed type of nystagmus in LSCC dysplasia, atypical spontaneous nystagmus, such as down-beating nystagmus or direction-changing spontaneous nystagmus, may be observed in patients with bilateral LSCC dysplasia.

Clinicians sometimes see patients with relatively persistent geotropic direction-changing positional nystagmus (DCPN) as a variant of lateral semicircular canal-benign paroxysmal positional vertigo (LSCC-BPPV). Recently, the concept of a “light cupula” in the lateral semicircular canal, exhibiting persistent geotropic DCPN, has been introduced. However, the underlying pathogenesis of light cupula is not known. We investigated the efficacy of a modified cupulopathy repositioning maneuver (mCuRM), designed to reduce light debris attached to the cupula in patients with persistent geotropic DCPN. Retrospective cohort study. Participants included 65 patients with a persistent geotropic DCPN: 35 underwent treatment (mCuRM group), and 30 were followed-up but received no treatment (No CuRM group). We compared the therapeutic and survival rate of persistent geotropic DCPN between two groups. On Day 1, the persistent geotropic DCPN did not resolve in either group. On the first and second follow-up days, persistent geotropic DCPN was observed in 28 (80%) and 21 (60%) of patients, respectively, in the mCuRM group, and in 28 (93.3%) and 24 (80%) patients, respectively, in the no mCuRM group. The differences between groups were not statistically significant. Furthermore, no between-group differences were found in the time from diagnosis to resolution of nystagmus, or the time from symptom onset to resolution of nystagmus. Kaplan–Meier analysis of the time course of persistent geotropic DCPN resolution from the day of diagnosis and day of symptom onset revealed no significant differences between the groups. Our findings indicate that mCuRM had no therapeutic benefit for a persistent geotropic DCPN and suggest that the pathophysiology of persistent geotropic DCPN is less likely to be a light debris attached to the cupula.

Objective The purpose of this study was to evaluate and compare the results of cervical vestibular evoked myogenic potential (cVEMP) and video head impulse test (p-vHIT) of posterior semicircular canal considered tools of inferior vestibular nerve function in vestibular neuritis. Study design Prospective cohort study. Setting Tertiary otology clinic. Patients and interventions Seventy-nine patients with vestibular neuritis participated in this study. We analyzed the interaural amplitude difference in cVEMP with a positive rate of p-vHIT according to gain and corrective saccade in the study population. Main outcome measure To evaluate the concordance rate of both tests, we analyzed Fleiss' Kappa value inter-test agreement of cVEMP with p-vHIT. Finally, we performed detailed analysis of the bilaterally absent response on cVEMP according to the p-vHIT results. Results The inter-test agreement between cVEMP and p-vHIT was 69.8% as we also considered the lesion side. This result indicated a statistically fair to good agreement in both tests. In mostly elderly patients with a bilaterally absent response (11 patients) on cVEMP, as a result of vHIT, nine patients with a bilaterally negative response on p-vHIT showed only canal paresis. Two patients showed canal paresis and a unilaterally positive response on p-vHIT. Conclusions Inter-test agreement between cVEMP and p-vHIT assessed in vestibular neuritis was relatively lower than we had predicted. Probably, p-vHIT can provide additional information on the differential diagnosis of dysfunction of the inferior vestibular nerve which is composed of the saccular nerve and the posterior ampullary nerve.

To investigate the incidence of positional nystagmus (PN) using a head-roll test in patients with Ramsay Hunt syndrome with vertigo (RHS_V) and discuss possible mechanisms.Retrospective study.Tertiary referral academic medical center.Twenty-eight patients with RHS_V were enrolled.Eye movements were recorded at positions of head roll to the right or left, and PN was classified as direction-fixed or direction-changing. Vestibular function tests including caloric test were performed.Direction-fixed nystagmus beating away from the affected side was the most common type of PN (61%), followed by direction-changing geotropic type (18%), direction-fixed nystagmus beating toward the affected side (14%), and direction-changing apogeotropic type (7%). The duration of nystagmus was longer than 60 seconds in all patients exhibiting direction-changing PN. Postcontrast T1-weighted internal auditory canal (IAC) magnetic resonance imaging showed enhancement of not only the facial and vestibulocochlear nerves, but also the inner ear structures or dura along the IAC, suggesting inflammatory changes within the labyrinthine membrane or IAC dura.Although direction-fixed PN was more commonly observed (75%), direction-changing PN was also observed in some RHS_V patients (25%). The mechanism of direction-changing PN may be, at least in part, explained by the alteration of specific gravity of the lateral semicircular canal cupula or endolymph due to inflammation in the inner ear membrane.

Objectives/hypothesis To investigate the role of the bow and lean test (BLT) in the diagnosis of benign paroxysmal positional vertigo (BPPV). Study design Retrospective case-control study. Methods Between March 2015 and June 2017, we enrolled 113 patients with posterior semicircular canal (PSCC) BPPV, 74 patients with lateral semicircular canal (LSCC) canalolithiasis, 53 patients with LSCC cupulolithiasis, and 32 patients with light cupula. We retrospectively assessed bowing nystagmus (BN) and leaning nystagmus (LN). Results In PSCC BPPV, 75% of the patients showed at least one of BN and LN, and direction of nystagmus provoked by a Dix-Hallpike test on the affected side was consistent with that of LN and opposite to that of BN. In LSCC canalolithiasis, 65% (48 of 74) of the patients showed both BN and LN, which were in the same direction in 38 patients (of 48) and in the opposite direction in 10 patients (of 48). The affected side can be determined according to the results of THE BLT in 74% (55 of 74) of LSCC canalolithiasis patients, and among them, the side determined according to the results of head-roll test was discordant with that according to the BLT in 20 of 55 patients (36%). In LSCC cupulopathy (n = 85), both BN and LN were persistent and observed in all cases, but we could not distinguish LSCC cupulolithiasis from light cupula according to nystagmus direction in the BLT. Conclusions Although a BLT yields better lateralization in LSCC canalolithiasis, it may be more useful in predicting the diagnosis and lateralization of PSCC BPPV than LSCC canalolithiasis. Level of evidence 4 Laryngoscope, 2600-2604, 2018.

Objectives: To evaluate the therapeutic efficacy of the forced prolonged position (FPP) in patients with horizontal semicircular canal (HSCC) cupulolithiasis in whom the cupulolith repositioning maneuver (CuRM) failed. Methods: Fifty-four consecutive patients with HSCC cupulolithiasis were included, and immediate treatment efficacy of CuRM and short-term treatment efficacy of FPP were investigated. Results: We performed the CuRM in HSCC cupulolithiasis, and, if the CuRM did not show immediate success, instructed the patients to perform the FPP or the sham position (by random allocation) at home. The immediate therapeutic success of the CuRM was assessed by the absence of nystagmus and vertigo on positional testing after 30 minutes of the maneuver, which was 14.8% on the first visit day. And the resolution rate of HSCC cupulolithiasis was higher in the FPP group than in the sham position group at the second (78.3% vs 55.6%), third (75.0% vs 42.9%), and fourth visits (100% vs 25.0%). Conclusion: Although the CuRM has been considered to be a good therapeutic option for HSCC cupulolithiasis because it theoretically aims to detach otoconial particles attached both on the utricle and canal sides of the cupula, the immediate success rate was only 14.8% at the first visit. The FPP can be additionally recommended to improve the resolution rate in HSCC cupulolithiasis patients in whom the CuRM fails.

Dizziness and balance problems are common in the elderly, and benign paroxysmal positional vertigo (BPPV) is one of the most common causes of dizziness. The aim of this study is to investigate the subtype distribution of geriatric BPPV in a single tertiary referral center, and compare the treatment efficacy according to the subtype of BPPV.Retrospective study.Tertiary referral academic center.The consecutive 316 elderly patients diagnosed with BPPV between March 2013 and March 2019 were included.Using a head-roll and Dix-Hallpike tests, subtype of BPPV was determined. Once the diagnosis of BPPV was made, patients were treated by its corresponding canalith repositioning maneuver (CRM).Among 316 elderly patients with BPPV, 143 patients (45%) were diagnosed with posterior semicircular canal BPPV, 46 patients (15%) were diagnosed with lateral semicircular canal (LSCC) canalolithiasis, 126 patients (40%) were diagnosed with LSCC cupulolithiasis, and 1 patient (0%) was diagnosed with anterior semicircular canal BPPV. While 66 and 63% of the patients with posterior semicircular canal BPPV and LSCC canalolithiasis recovered after one session of CRM, only 32% of the patients with LSCC cupulolithiasis recovered after one session of CRM.The proportion of LSCC cupulolithiasis was higher in the elderly, and treatment efficacy by CRM is lower in LSCC cupulolithiasis than other subtypes of BPPV. High prevalence of LSCC cupulolithiasis may be explained by a delay between onset of BPPV and patient's presentation to the tertiary referral hospital or pathophysiology of ageotropic positional nystagmus other than otoconial attachment on the LSCC cupula in the elderly.

Although the etiology of benign paroxysmal positional vertigo (BPPV) is idiopathic in most cases, the association of osteoporosis or vitamin D deficiency with BPPV has attracted much interest recently. While it is generally accepted that osteoporosis or vitamin D deficiency is related to the occurrence and/or recurrence of BPPV, the difference in serum vitamin D level and bone mineral density (BMD) among different subtypes of BPPV has not been investigated. We aimed to compare T-score of BMD score and serum 25-hydroxyvitamin D level among idiopathic BPPV patients with a different subtype. This study included 117 consecutive patients with idiopathic BPPV (26 men and 91 women; mean age, 55 ± 11 years; age range, 25 to 78 years) who underwent blood sampling for serum 25-hydroxyvitamin D level measurement and bone mineral densitometry of the anterior-posterior lumbar spine and femur between April 2018 and February 2019. Among 117 patients, 49 were diagnosed with posterior semicircular canal (PSCC) BPPV, 24 were diagnosed with lateral semicircular canal (LSCC) canalolithiasis, and 44 were diagnosed with LSCC cupulolithiasis. The mean T-score of BMD was −1.5 ± 0.9 in PSCC BPPV, −1.5 ± 1.3 in LSCC canalolithiasis, and −1.5 ± 1.1 in LSCC cupulolithiasis, which was not significantly different (p = 0.998, One-way ANOVA test). The mean level of 25-hydroxyvitamin D was 22.5 ± 10.6 ng/ml in PSCC BPPV, 26.8 ± 16.0 ng/ml in LSCC canalolithiasis, and 25.4 ± 9.6 ng/ml in LSCC cupulolithiasis, which was not significantly different (p = 0.262, One-way ANOVA test). The proportion of osteoporosis/osteopenia or vitamin D deficiency/insufficiency did not show significant difference among idiopathic BPPV patients with different subtypes, and findings of this study indicate that either serum level of vitamin D or T-score of BMD is not a distinguishable characteristic among different subtypes of BPPV.

Objective To demonstrate characteristic nystagmus findings in acute otitis media (AOM) complicated by serous labyrinthitis and discuss the mechanism of direction-changing positional nystagmus (DCPN) in this condition. Patients A patient with AOM complicated by serous labyrinthitis on the left side. Intervention Video nystagmography and 3D fluid attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI). Main outcome measures Characterize positional nystagmus in a head-roll test observing the change of nystagmus direction in process of time and compare findings of temporal bone 3D FLAIR MRI. Results A previously healthy 50-year-old man who complained of acute otalgia, hearing loss, and vertigo was diagnosed with AOM complicated by serous labyrinthitis on the left side. A head-roll test performed on the day when vertigo developed showed persistent geotropic DCPN. While pre- and postcontrast T1-weighted MRI showed no signal abnormality in both inner ears, 10-minute delay postcontrast 3D FLAIR image showed enhancement in the inner ear on the left side. Four-hour-delay postcontrast 3D FLAIR images showed more conspicuous enhancement of the whole cochlea, vestibule, and semicircular canals on the left side. Conclusions In AOM complicated by serous labyrinthitis, density of perilymph may increase due to direct penetration of cytokines and other inflammatory mediators from the middle ear into perilymph and breakdown of blood-labyrinth barrier that causes vascular leakage of serum albumin into perilymph. The density difference between perilymph and endolymph makes the semicircular canal gravity sensitive. A buoyant force is also generated by gravity, causing indentation of endolymphatic membrane in the ampulla and cupula displacement. Thus, at the early stage of serous labyrinthitis, a head-roll test may elicit persistent geotropic DCPN, of which the direction can be changed over time.

To analyze acute vertigo showing spontaneous nystagmus with negative video head impulse test (vHIT).Retrospective chart analysis.Tertiary referral hospital.Over 16 months, 155 patients were identified with acute vertigo with spontaneous nystagmus. Of these 155, 30 (19.4%) were enrolled in this study because they did not show gain loss or catch-up saccades in both sides of the horizontal vHIT. Results of vestibular function tests (videonystagmography, horizontal vHIT, caloric test, and cervical vestibular-evoked myogenic potential [cVEMP]) and pure tone audiometry were analyzed. For all cases, magnetic resonance imaging with diffusion-weighted imaging was checked.Patients consisted of 17 with Ménière's disease and 7 with sudden sensorineural hearing loss with vertigo (SSNHL_V), and only 3 patients were finally diagnosed as having acute vascular stroke. Except for the loss of hearing on the lesion side, the direction of nystagmus or cVEMP asymmetry showed very different results. All 7 patients with SSNHL_V did not have canal paresis in the caloric test, but cVEMP amplitude was smaller on the lesion side for 6 patients.For patients with acute vertigo presenting spontaneous nystagmus with negative horizontal vHIT, it is important not only to focus on the diagnosis of acute vascular stroke but also to evaluate hearing because of the high possibility of Ménière's disease or SSNHL_V.

Accurate lateralization is important to improve treatment outcomes in horizontal semicircular canal (HSCC) benign paroxysmal positional vertigo (BPPV). To determine the involved side in HSCC-BPPV, the intensity of nystagmus has been compared in a head-roll test (HRT) and the direction of nystagmus was evaluated in a bow and lean test (BLT). The aim of this study is to compare the results of a BLT with those of a HRT for lateralization of HSCC-canalolithiasis and cupulopathy (heavy cupula and light cupula), and evaluate treatment outcomes in patients with HSCC-canalolithiasis. We conducted retrospective case reviews in 66 patients with HSCC-canalolithiasis and 63 patients with HSCC-cupulopathy. The affected side was identified as the direction of bowing nystagmus on BLT in 55 % (36 of 66) of patients with canalolithiasis, which was concordant with the HRT result in 67 % (24 of 36) of cases (concordant group). Lateralization was determined by comparison of nystagmus intensity during HRT in 30 patients who did not show bowing or leaning nystagmus. The remission rate after the first treatment was 71 % (17 of 24) in the concordant group and 45 % (5 of 11) in the discordant group. Both bowing and leaning nystagmus were observed in all patients with cupulopathy, and the side of the null plane was identified as the affected side. In conclusion, bowing and/or leaning nystagmus were observed in only 55 % of patients with HSCC-canalolithiasis, and the first treatment based on the result of BLT alone was effective in only 45 % of the patients in whom the BLT and HRT were discordant, which may suggest that the usefulness of BLT in lateralizing the HSCC-canalolithiasis may be limited.

Objectives To investigate the incidence of nystagmus in patients with sudden sensorineural hearing loss (SSNHL) without dizziness, and to evaluate the correlation of the presence of nystagmus with hearing recovery in those patients. Study design Retrospective study. Setting Tertiary referral academic medical center. Patients Thirty-eight patients with SSNHL who did not complained of dizziness were enrolled. Main outcome measure The presence of nystagmus was evaluated by recording eye movements, and if present, nystagmus was classified as direction-fixed or positional nystagmus. Vestibular function tests were performed, including caloric test. Results Of 33 idiopathic SSNHL patients without dizziness, nystagmus was observed in 22 patients (67%), of which 14 patients exhibited direction-fixed nystagmus, and 8 patients exhibited direction-changing nystagmus. Among the 14 patients with direction-fixed nystagmus, 9 displayed the paretic type, and 5 displayed the irritative type. Direction-changing nystagmus (n = 8) was defined as that in which the direction of nystagmus was changed in a supine head-roll test, and the geotropic type and apogeotropic type were observed in two and six patients, respectively. The mean initial pure tone threshold was 58.2 ± 28.1 dB and 57.3 ± 20.0 dB in SSNHL patients with and without nystagmus, respectively, which was not significantly different (p = 0.925). When hearing improvement was compared according to the presence of nystagmus, 39% (8 of 21) of patients with nystagmus were found to belong in the good prognosis group, and 72% (8 of 11) of patients without nystagmus were found to belong in the good prognosis group, which showed marginal statistical significance (p = 0.063). Conclusion Nystagmus was observed in two-thirds of idiopathic SSNHL patients without dizziness, and the pattern of nystagmus was either direction fixed or direction changing. Because hearing recovery was worse in patients with nystagmus than those without it, the diagnosis of nystagmus, even in SSNHL patients without dizziness, may be important in evaluating the hearing prognosis.

Background Hemotympanum refers to both the presence of blood in the middle ear cavity and to ecchymosis of the tympanic membrane (TM), and a systematic study of intra-TM (iTM) hemorrhage without bleeding in the middle ear cavity has not been conducted. The goals of our study were to analyze the causes of iTM hemorrhage without TM perforation or bleeding in the middle ear cavity, and to demonstrate the clinical characteristics of the disease. Methods This Case series study included five patients with iTM hemorrhage between August 2014 and August 2017. An iTM hemorrhage was diagnosed when otoendoscopic examination demonstrated minor bleeding behind the intact TM, a hemorrhage was observed between the TM annulus and the epidermal layer, and temporal bone computed tomography revealed thickening of the TM without soft tissue density within the tympanic cavity or temporal bone fracture. Initial symptoms, and serial findings of otoendoscopy and pure tone audiometry (PTA) were investigated. Results iTM hemorrhage developed due to blunt head trauma in two patients, descent barotrauma during scuba diving in two patients, and spontaneous epistaxis in one patient. Otalgia and ear fullness were the most common symptoms, but PTA showed no or minimal conductive hearing loss in all patients. Conclusions An iTM hemorrhage may develop after blunt head trauma, barotrauma due to scuba diving, or spontaneous epistaxis; otological symptoms included otalgia, tinnitus, and aural fullness. An iTM hemorrhage resolved spontaneously without specific treatment, usually within 1 month.

Clinical features in the course of conversion differed between patients with SSNHL and cupulopathy, which indicates that the pathophysiology of persistent geotropic or apogeotropic DCPN and the mechanism of the change in nystagmus direction may differ between the two groups.The aim of this study is to investigate clinical characteristics of 10 patients with persistent DCPN who exhibited a conversion of nystagmus direction between geotropic and apogeotropic, and discuss possible mechanisms.Using video-oculography, serial examinations of nystagmus in a head-roll test were performed.Of these 10 patients, five had sudden sensorineural hearing loss (SSNHL) and the remaining five had cupulopathy. In SSNHL, direction of nystagmus changed from geotropic to apogeotropic in three patients and from apogeotropic to geotropic in two patients. In cupulopathy, persistent apogeotropic DCPN always preceded persistent geotropic DCPN. The change in nystagmus direction occurred earlier in patients with cupulopathy (1 or 2 days after vertigo onset) than in patients with SSNHL (4-23 days after vertigo onset). While the null plane was consistently identified on one side, regardless of the nystagmus direction in cupulopathy, it was not always identified on the side of hearing loss in SSNHL.

Objectives/Hypothesis Persistent direction-changing positional nystagmus (DCPN) during the supine head-roll test is a typical finding of cupulopathy. The aim of this study was to introduce a simple method of evaluating patients with cupulopathy (light cupula and heavy cupula), which is performed in a seated position, and investigate its diagnostic utility and use for lateralization. Study Design Retrospective case series. Methods Using video-oculography, nystagmus during head roll in the leaning and bending head positions while seated upright was evaluated in 26 patients with cupulopathy (five light cupula and 21 heavy cupula). The diagnosis of cupulopathy was confirmed with the supine head-roll test. Results Spontaneous nystagmus while seated upright was directed toward the ipsilesional side in heavy cupula and the contralesional side in light cupula. The first null point was identified when the head was slightly bent in the pitch plane in all 26 patients. Head rolling elicited a persistent geotropic DCPN in light cupula patients and persistent apogeotropic DCPN in heavy cupula patients during both leaning and bending head positions. In both light and heavy cupula, nystagmus disappeared when the head was turned slightly toward the affected side in both leaning (second null point) and bending (third null point) head positions. Conclusions Cupulopathy and its affected side can be diagnosed by identifying the three null points while seated upright. Level of Evidence 4. Laryngoscope, 125:1921–1925, 2015

To characterize the clinical features of patients who simultaneously developed ipsilateral sudden sensorineural hearing loss (SSNHL) and positional vertigo showing geotropic direction-changing positional nystagmus (DCPN) on a supine head-roll test, and to address the possible pathophysiology of the disease.Retrospective case series review.We conducted a retrospective case series study in 17 patients with SSNHL and simultaneous positional vertigo showing geotropic DCPN.All 17 patients showed persistent geotropic DCPN without latency in the supine head-roll test. The intensity of nystagmus was stronger when the patient's head was turned to the lesion side of SSNHL in 15 of 17 patients. We sought to identify a null plane in nine patients, and the null plane was identified on the same side as the SSNHL in all nine patients, which was thought to be caused by the "light cupula" of the lateral semicircular canal in the same ear as the SSNHL.A light cupula mechanism may be one of the causes of positional vertigo in patients with SSNHL. In these cases, persistent geotropic DCPN is observed in the supine head-roll test and the null plane can be identified on the same side as the hearing loss.

Objectives To describe vertical and horizontal components of head-shaking nystagmus (HSN) in various vestibular disorders. Design Retrospective case review. Setting Tertiary care academic referral centre. Participants Head-shaking nystagmus was assessed in 66 vestibular neuritis (VN) patients at acute (

Objectives To investigate the relationship between the threshold and the interaural amplitude difference ratio (IADR) in cervical vestibular evoked myogenic potential (cVEMP) testing and pursuit the clinical significance of the parameters. Materials and methods cVEMP responses were recorded while the SCM contraction was controlled using a pressure cuff. The intensities of the sound stimulation decreased from 95 dB nHL by 5 dB, until no responses were evoked. Thresholds, interaural threshold difference (ITD), amplitudes, and interaural amplitude difference ratio at the stimulation of 95 dB nHL were calculated and the relationship between them was examined. Results All subjects showed cVEMP responses bilaterally. Thresholds measured were overall 76 dB nHL and most (92%) ears showed the ITD of 0 or 5 dB. The amplitudes of cVEMP responses showed a positive correlation with the sound intensities, and more specifically with the sound intensity above each threshold value. There was no significant difference in IADR values by the ITD. Conclusions Based on our study, the ITD is less than 10 dB in most normal subjects and estimation of threshold should be added to cVEMP testing for probing vestibular asymmetry. Getting a threshold might be helpful in determining whether the abnormal interaural amplitude difference ratio is related to the abnormal ITD.

The HSN test is a simple examination that can be easily performed by clinicians, however only a few studies have analyzed the 2-dimensional characteristics of HSN in patients with MD. The objective of the study was to characterize different types of 2-dimensional head-shaking nystagmus (HSN) in patients with Meniere's disease (MD). Sixty-five patients with definite MD were enrolled. HSN was considered pathologic, if slow-phase velocity (SPV) was ⩾ 4°/s and was classified as paretic or recovery according to the direction, and as monophasic or biphasic according to the presence of direction change. HSN was categorized as pure horizontal, mixed or pure vertical. When vertical SPV was larger than horizontal SPV, HSN was categorized as perverted. Forty-four patients (68%) had pathologic HSN and 28 patients (43%) had pathologic canal paresis. Monophasic-paretic HSN was the most common and followed by biphasic-paretic HSN, monophasic-recovery HSN and biphasic-recovery HSN. Delayed-peak monophasic-paretic HSN, which was not observed in vestibular neuritis, was found in 6 patients with MD. Thirty-three patients (51%) had a vertical component, which was monophasic and downbeat in 32 (97%). Every pathologic HSN in horizontal plane had higher SPV in horizontal plane than that of vertical plane. Perverted HSN was found in 4 patients, of whom 3 had pure vertical and one had mixed type HSN. Our data showed that HSN is a sensitive detector of vestibular dysfunction. HSN showed diverse types with a new type of delayed-peak HSN. Vertical components of HSN were observed in about half, but they were negligible compared to horizontal components. Weak perverted HSN (vertical SPV ⩽ 4°/s) could be found in patients with MD.

Objective The purpose of the present study was to investigate the effect of dopamine as a possible regulator of epithelial Na + channel (ENaC) in the Reissner's membrane (RM). Methods RM was freshly dissected from the gerbil cochlea, and short-circuit current ( I sc ) was measured using the voltage-sensitive vibrating probe technique. The dopamine receptor expression was examined using immunohistochemistry. Results The results showed that dopamine induced activation of the amiloride-sensitive I sc , but not after pre-treatment with amiloride. The D 1 -like receptor antagonist SCH-23390, but not the D 2 -like receptor antagonist sulpiride, decreased the stimulatory effect of dopamine on RM. The effect of dopamine on Na + transport via ENaC was still observed after blockade of the Na + –K + -ATPase by ouabain. D 1 receptor immunoreactivity was observed in RM, stria vascularis and spiral ganglion. Conclusion Na + transport in RM is activated by dopamine possibly via D 1 -like receptors, and intracellular mechanisms other than cAMP-mediated pathway may be involved.

Objectives Children differ from adults in the expression of dizziness symptoms and the causes of dizziness. In several studies, benign paroxysmal vertigo of childhood (BPVC) and vestibular migraine (VM) were seen exclusively in children with vertigo, but the age threshold used to define ‘children’ varies, and there are few reported studies about adolescents with dizziness. In this study, we investigated the prevalence of vestibular and balance disorders according to age category in a multi-center study (otolaryngology departments of 11 hospitals) of children and adolescents. Methods Children and adolescents aged under 18 who visited the otolaryngology departments of 11 hospitals for dizziness were included. We classified the patients into three categories: preschool (up to and including 6-year-olds), school age (7- to 12-year-olds), and adolescents (13- to 18-year-olds). These patients were reviewed retrospectively based on their clinical charts. Results In the preschool age group, BPVC was most common, followed by VM. In the school-age group, BPVC and VMs were most common, followed by psychogenic vertigo and benign paroxysmal positional vertigo (BPPV). In adolescents, VM was the most common, and Meniere's disease, cardiogenic vertigo, and BPVC, which are seen primarily in adults, were also seen in some adolescents. Conclusion In children and adolescents with dizziness, VM and BPVC were the most common diseases, and prevalence of disease by age showed differing distributions. These findings will help in diagnosing and managing children and adolescents with vertigo.

Conclusion The patients with serous labyrinthitis caused by acute otitis media (AOM) exhibited various patterns of nystagmus in which direction-fixed irritative-type nystagmus was the most common pattern. Differential effects on inner ear function by toxic or inflammatory mediators may be responsible for the various manifestation of nystagmus. Objective This study aimed to investigate nystagmus patterns in patients with serous labyrinthitis, and discuss possible mechanisms. Methods From October 2011 to March 2014, 13 consecutive patients with serous labyrinthitis were included. Eye movements of the patients were serially examined using video-nystagmography, and patterns of nystagmus were investigated. Results The most commonly observed pattern was direction-fixed nystagmus (nine of 13 patients). Of these, eight showed irritative-type, and one showed paretic-type. Direction of nystagmus, although the intensity gradually decreased, was not changed during the course of treatment. One patient showed direction-changing spontaneous nystagmus, which changed into paretic-type direction-fixed nystagmus 1 day after myringotomy. Three patients exhibited persistent direction-changing positional nystagmus in a supine head-roll test. Of them, two showed apogeotropic and one showed geotropic type. In all 13 patients, vertigo and hearing loss were improved after the treatment.

Vestibular abnormality was found in 84% of patients with Meniere's disease (MD) and 66% of those with migrainous vertigo (MV), even in the interictal period. Although MV does not have proven pathology like endolymphatic hydrops of MD, MV had high vestibular abnormality, suggesting that comorbid vestibular abnormality can be a cause of vertigo and needs to be pursued.MD and MV are common disorders causing recurrent vertigo. We determined the vestibular abnormality rates using vestibular tests with objective measurements.Results of caloric, head-shaking nystagmus (HSN), and vibration-induced nystagmus (VIN) tests were analyzed in 45 patients with MD and 76 with MV.The abnormal rates in MD were significantly higher than those in MV. Of 45 MD patients, 21 (47%), 32 (71%), and 24 (53%) exhibited abnormal caloric, HSN, and VIN results, respectively. Fourteen (31%) MD patients had migraine associated with vertigo, but the association was not accompanied by high rates of abnormal results in the tests. Of 76 MV patients, 19 (25%), 38 (50%), and 24 (32%) exhibited abnormal caloric, HSN, and VIN results, respectively. Overall, 84% of patients with MD and 66% with MV exhibited abnormal results on at least one test. Abnormal rates were highest in HSN, followed by VIN and the caloric test.

Conclusion: The up-regulation of osmotic stress protein 94 (OSP94) in the murine endolymphatic hydrops (EH) models suggests that OSP94 might be involved in cellular adaptation in response to ionic and osmotic stress in the murine inner ear. Objectives: The purpose of the present study was to investigate the expression of OSP94 in cochlear tissues of the murine EH models and control animals. Methods: Nine adult BALB/c mice were treated with both intratympanic injection of lipopolysaccharide and intraperitoneal administration of aldosterone to induce EH. Nine mice were used as control animals. The expression level of OSP94 in the EH and control groups was compared using immunohistochemistry and real-time RT-PCR. Results: Immunohistochemical staining of tissues in the EH group showed an up-regulation of OSP94 expression in the cochlea, especially in the stria vascularis and Reissner's membrane. Quantitative real-time PCR analysis also showed that transcription of the OSP94 gene in the cochlea was si...

Objective To demonstrate positional nystagmus during a head-roll test in two patients with lateral semicircular canal (LSCC) cupulolithiasis who presented with spontaneous detachment of otoliths from the LSCC cupula, and to confirm that otoliths may adhere to both the utricle and canal sides of the cupula. Patients and methods Using video nystagmography, positional nystagmus was evaluated in two patients with LSCC cupulolithiasis who showed the change of nystagmus direction during a head-roll test. Results Both patients presented with persistent left-beating and right-beating nystagmus when the head was bent forward and backward, respectively. This suggests the presence of either cupulolithiasis on the right side or light cupula on the left side of the LSCC. In Case 1, transformation from cupulolithiasis to canalolithiasis occurred, implicating the detachment of otoliths from the canal side of the right LSCC cupula. In Case 2, vigorous right-beating nystagmus was followed by persistent left-beating nystagmus when the head was rolled to the left. Following this, direction-fixed left-beating nystagmus was observed at all positions, which may indicate that otoliths attached on utricle side of the right LSCC cupula were detached and fell into the utricle under the influence of gravity. A conversion of nystagmus direction may be explained by an expression of short-term adaptation of vestibular tone. Conclusions Otoliths can be attached to either side of the cupula in LSCC cupulolithiasis. Because it is difficult to determine the attached side at the time of diagnostic maneuver, the therapeutic approach should include maneuvers designed to detach otolith particles from both the utricle and canal side.

Primary tumors arising from the ear lobule are uncommon, and hemangiomas in this region are extremely rare. The authors report a case of an arteriovenous hemangioma in the left ear lobule. The mass was reddish-colored, soft, mobile, nonpulsatile, and not tender on palpation. There was no recurrence 22 months after surgical excision.

A congenital bony defect of the external auditory canal (EAC) may cause temporomandibular joint (TMJ) herniation into the EAC. This rare condition is known as persistent foramen tympanicum. We report 2 cases of symptomatic TMJ herniation through a patent foramen tympanicum. The patients complained of mastication-induced tinnitus. Otoscopic examination revealed that a whitish protruding mass at the anterior wall of the EAC bulged when the patients closed their mouths. After conservative managements such as avoidance of chewing foods on the affected side for 2 months, mastication-induced tinnitus disappeared and the size of protruding mass was decreased in one of the patients. Surgical repair of the bony defect has been the treatment of choice. In cases when symptoms are trivial and patients do not want surgical treatment, however, conservative management would be a sound alternative.

Background The light cupula is a condition wherein the cupula of the semicircular canal has a lower specific gravity than its surrounding endolymph. It is characterized by a persistent geotropic direction-changing positional nystagmus in the supine head-roll test, and the identification of a null plane with slight head-turning to either side. Case presentation This study describes a case of recurring light cupula that occurred alternately on both sides. At the first episode, a null plane was identified on the right side, which led to the diagnosis of a light cupula on the right side. At the second episode, a null plane was identified on the left side, leading to the diagnosis of a light cupula on the left side. Conclusion This is the first case report of recurring light cupula alternately involving both sides. Although the pathophysiology is not entirely understood yet, the light cupula should be considered as one of causes of recurrent positional vertigo. Electronic supplementary material The online version of this article (doi:10.1186/s12901-015-0015-z) contains supplementary material, which is available to authorized users.

Patients with herpes zoster oticus (HZO) may exhibit diverse symptoms regarding cochleovestibular dysfunction. This study investigated the clinical manifestations of HZO by comparing symptoms associated with dysfunctions of the 7th and 8th cranial nerves (CN VII and VIII, respectively). This study is a retrospective case series. Eighty-one patients with HZO who had dysfunction of CN VII or VIII were included in this study. Electroneuronography (ENoG) values were compared among patient groups with facial weakness. Patients with ipsilateral facial weakness (62 of 81) were more common than those without. Among 81 patients, those with facial weakness, hearing loss, and vertigo were most common, and only 1 patient had vertigo without hearing loss or facial weakness. Most patients with vertigo also had hearing loss (28 of 30), and patients without hearing loss did not have vertigo (19 of 21). While patients with vertigo had worse ENoG values than those without vertigo, ENoG values were not significantly different between patients with and without hearing loss. In conclusion, various clinical manifestations of CN VII and VIII dysfunction are possible in patients with HZO. Patients with vertigo had worse ENoG values than those without, which may indicate that vertigo reflects more severe facial nerve degeneration in HZO patients with facial weakness.

A primary mass lesion arising from the tympanic membrane is extremely rare. The authors report a patient of extensive fibrosis within the tympanic membrane in a 65-year-old woman who did not have a history of ear infection. The patient complained of slow progressing hearing loss on the left side without otorrhea or otalgia. Otoscopic examination showed a thick tympanic membrane with whitish mass-like bulge, and pure-tone audiometry revealed conductive hearing loss. The lesion was totally resected, and final findings of a histopathologic examination revealed extensive fibrosis with subsiding inflammation within the tympanic membrane. Fibrosis within the tympanic membrane may present with slow progressing conductive hearing loss and should be considered a possible differential diagnosis of mass lesions in the tympanic membrane.