Estimated incidence and characteristics of vestibular neuritis in Japan: A nationwide survey.

OBJECTIVE: To assess the annual incidence of vestibular neuritis (VN) in the Japanese population. METHODS: We conducted a mail-based survey targeting otolaryngologic clinics and hospitals across Japan to estimate the annual number of patients who were newly-diagnosed with VN during the one-year period of 2021. Using a stratified sampling method, we selected 1,107 departments and asked them to report the number of new patients with VN and their demographics. The total number of VN patients was estimated by multiplying the reported numbers by the reciprocal of the sampling rate and response rate. RESULTS: The overall survey response rate was 40.5 % (448 departments). The estimated number of newly-diagnosed VN patients in 2021 was 8,861 (95 % confidential interval [CI], 2,290-15,432) The annual incidence of VN was 7.05 per 100,000 population in Japan. The male-to-female ratio of VN patients was 0.96, and the mean age was 60.3 ± 16.1 years (range 11-94 years). CONCLUSIONS: The annual incidence of VN in Japan in 2021 had almost doubled and the mean age had become older compared to the previous study in 1993 (annual incidence; 3.5 per 100,000 per year; mean age: 45 years).

Isolated Otolith Dysfunction in Persistent Postural-Perceptual Dizziness.

The aims of this study were to investigate otolith dysfunction, especially isolated otolith dysfunction (with preserved semicircular canal function) in persistent postural-perceptual dizziness (PPPD) patients. Twenty-one patients who had been diagnosed with PPPD were enrolled in this study. The subjects filled out questionnaires [the Dizziness Handicap Inventory (DHI) and the Niigata PPPD Questionnaire (NPQ)] and underwent vestibular evoked myogenic potential (VEMP) tests, video head-impulse tests (vHIT), and stabilometry. Among the 21 subjects with PPPD, 9 showed isolated otolith dysfunction, 4 exhibited both otolith dysfunction and semicircular canal dysfunction, and 2 demonstrated isolated semicircular canal dysfunction. Six subjects exhibited normal VEMP and vHIT results. Concerning the subjects' questionnaire scores and stabilometric parameters, there were no significant differences among subgroups when the subjects were classified according to their VEMP and vHIT results while stabilometric parameters obtained in PPPD subjects were significantly increased than published data of healthy subjects. As precipitating conditions for PPPD, vestibular neuritis was the most frequent and the second most was idiopathic otolithic vertigo. In conclusion, the majority of PPPD patients had otolith dysfunction, and most of them showed isolated otolith dysfunction. Idiopathic otolithic vertigo can be a precipitating factor of PPPD. While otolith dysfunction may be associated with initiation of PPPD symptoms, PPPD symptoms are also considered to be associated with other dysfunctions of the sensory processing system.

Estimated prevalence and characteristics of bilateral vestibulopathy diagnosed in Japan: A nationwide survey.

OBJECTIVE: To assess the prevalence and annual incidence of bilateral vestibulopathy (BV) diagnosed in the Japanese adult population. METHODS: We conducted a mail-based survey targeting otolaryngologic clinics and hospitals across Japan to estimate the annual number of patients who were diagnosed as having bilateral vestibulopathy after vestibular function tests during a 12-month period ending March 2019. Using a stratified sampling method, we selected 1,106 departments and asked them to report the number of patients with BV and their demographics. The total number of patients was estimated by multiplying the reported numbers by the reciprocal of the sampling rate and response rate. RESULTS: The overall survey response rate was 51.4% (568 departments). The estimated number of patients diagnoses with BV in 2018 was 1,063 (95% confidential interval [CI], 127-1,998) which included 407 patients (95% CI: 134-680) newly-diagnosed with BV. The prevalence and annual incidence of BV in Japan were 0.84 and 0.32, respectively per 100,000 population in Japan. The male-to-female ratio of BV patients was 1.29, and the mean age was 63.7 ± 16.4 years (range 18-84 years). The most frequent etiologies of BV were Meniere's disease (11.4%), meningitis (3.4%), and ototoxic agents (3%). CONCLUSIONS: Patients who were diagnosed as having BV were extremely rare in Japan.

Long-term effect of transtympanic intermittent pressure therapy using a tympanic membrane massage device for intractable meniere's disease and delayed endolymphatic hydrops.

BACKGROUND: A 12-month follow-up study showed that middle ear pressure treatment with a transtympanic membrane massage (TMM) device had a similar effect to a Meniett device. OBJECTIVES: The effects of pressure treatment with a TMM device were retrospectively compared to the effects of treatment with a Meniett device in patients with Meniere's disease (MD) and delayed endolymphatic hydrops (DEH) who were followed for a minimum of 24 months. MATERIALS AND METHODS: Twenty-seven patients were treated with the TMM device and 14 patients were treated with a Meniett device. The insertion of a transtympanic ventilation tube was necessary for the Meniett device but not for the TMM device. RESULTS: In patients treated with the TMM and Meniett devices, the frequency of vertigo significantly improved at 19-24 months after treatment. The distribution of vertigo at 19-24 months after treatment did not differ between the patients treated with the two types of devices. Pressure treatment for 8 months or more was suitable to achieve remission. CONCLUSIONS AND SIGNIFICANCE: Middle ear pressure treatment for 8 months or more with a TMM or Meniett device was equally effective and provided minimally invasive treatment options for intractable MD and DEH.

Effects of transtympanic intermittent pressure therapy using a new tympanic membrane massage device for intractable Meniere's disease and delayed endolymphatic hydrops: a prospective study.

BACKGROUND: The retrospective study showed that the effect of the middle ear pressure treatment by the tramstympanic membrane massage (TMM) device was similar to that of the Meniett device. OBJECTIVES: The new TMM device named EFET device was prospectively evaluated in patients with Meniere's disease (MD) and delayed endolymphatic hydrops (DEH) and we compared the effects to the Meniett device. MATERIALS AND METHODS: 23 ears of 19 patients were treated with an EFET device, and 17 ears of 15 patients were treated with the Meniett device. All patients suffering from intractable MD and DEH were treated for 4 months. The insertion of a transtympanic ventilation tube was necessary for the Meniett device, but not the EFET device. RESULTS: In patients treated by the EFET and Meniett devices, the frequency of vertigo significantly improved after treatment. The distribution of vertigo outcomes at 4 months after treatment did not differ between patients treated with the both devices. CONCLUSIONS AND SIGNIFICANCE: Middle ear pressure treatment by the EFET device is effective and provides minimally invasive options for intractable MD and DEH like the Meniett device.

Effects of External Auditory Meatus Occlusion on Ocular Vestibular Evoked Myogenic Potentials Induced by Bone Conducted Sound.

To facilitate more reliable recordings of the ocular vestibular evoked myogenic potentials (oVEMP) induced by bone-conducted sound using the B81 bone conduction transducer, we preliminarily studied the effects of external auditory meatus occlusion using an earplug on such oVEMP. Eight healthy volunteers (four males and four females, 26-48 years of age, mean age: 34. 5 years) and 14 patients with vestibular disease (2 males and 12 females, 18-59 years of age, mean age: 41.5 years) were enrolled. oVEMP testing was performed using a B81 placed on the temple. Tone bursts (500 Hz, rise/fall time: 2 ms, plateau time: 2 ms, and 70 dB nHL) were presented at a rate of 5.1 Hz. N1-P1 amplitudes were measured and analyzed. Occlusion resulted in significantly larger N1-P1 amplitudes [mean ± SE (SD): 12.3 ± 1.67 (6.71) µV vs. 9.55 ± 1.55 (6.21) µV; p = 0.020, paired t-test]. While four patients did not exhibit any response on either side in the absence of occlusion, all of them showed unilateral or bilateral responses when occlusion was employed. In any patient occlusion did not result in loss of oVEMP responses. External auditory meatus occlusion using an earplug could allow more reliable recordings of bone conduction transducer-induced oVEMP.

Association of cervical vestibular-evoked myogenic potential tuning property test results with MRI findings of endolymphatic hydrops in Meniere's disease.

PURPOSE: To examine the concordance between cervical vestibular-evoked myogenic potential (cVEMP) tuning property test results and MRI findings of endolymphatic hydrops (EH). METHODS: Fourteen subjects (age 24-76 years) that had been diagnosed with unilateral definite Meniere's disease (MD) (N = 8) or unilateral probable MD (N = 6) were enrolled. All of the subjects underwent cVEMP tests (using 500 Hz and 1000 Hz tone bursts), pure-tone audiometry, and gadolinium-enhanced 3 T-MRI. To examine tuning properties of cVEMP, the 500-1000 Hz amplitude ratio was calculated as the SLOPE. The results of the cVEMP tuning property test results were compared with EH-related MRI findings. RESULTS: EH positivity in both the cochlea and vestibule on MRI was observed in 7 of the 8 affected ears of definite MD and 3 of the 6 affected ears of probable MD, although it was only in one of the 14 unaffected ears. EH(+) or no response (NR) in cVEMP tuning property test was significantly associated with EH positivity on MRI, while EH(-) in the cVEMP tuning property test was significantly associated with EH-negativity on MRI (p = 0.0016 Fisher's exact test test). CONCLUSION: EH(+) or NR in the cVEMP tuning property test correspond well to EH positivity in the MRI findings. The cVEMP tuning property test is useful for screening and following up EH.

Cervical vestibular evoked myogenic potential with chirp sounds.

BACKGROUND: There are only a few reports concerning cervical vestibular evoked myogenic potential (cVEMP) using chirp sound, and clinical indications/advantages of it are still unclear. OBJECTIVE: To compare cVEMP using CE-chirp LS® with cVEMP using 500 Hz and 1000 Hz tone bursts (TB) and to investigate clinical indications/advantages of CE-chirp LS® for recording cVEMP. METHODS: Sixteen patients with vestibular disorders (2men and 14 women) (18∼62, mean 42.9 years of age) were enrolled in this study. Participants underwent cVEMP testing using 500 Hz and 1000 Hz tone bursts (TB) and CE-chirp LS®. Response rate of P1-N1, corrected/normalized amplitude of P1-N1, latencies of P1 and N1, asymmetry ratio, and correlation of P1 latency to SLOPE in tuning property test (an index of endolymphatic hydrops) were compared. RESULTS: Corrected/normalized amplitude of P1-N1 to CE-chirp LS® was smaller than corrected/normalized amplitude of P1-N1 to 500 Hz TB. Peak latencies to CE-chirp LS® were the shortest among the 3 types of stimulation. EH-positive ears according to the tuning property test had tendency of prolonged P1 latencies to CE-chirp LS®. CONCLUSION: CE-chirp LS® is applicable for recording cVEMP with a similar diagnostic accuracy to TB. Prolongation of P1 latency in CE-chirp LS® might be an indicator of endolymphatic hydrops in the saccule.

Vestibular Migraine Patients Show Lack of Habituation in Auditory Middle Latency Responses to Repetitive Stimuli: Comparison With Meniere's Disease Patients.

Objectives: To compare habituation in auditory middle latency response (AMLR) to repetitive stimuli of vestibular migraine (VM) patients with Meniere's disease (MD) patients and healthy controls (HC) and to assess usefulness of AMLR for diagnosis of VM. Subjects: Thirteen unilateral definite MD patients (2 men, 11 women, mean age 50.6), 13 definite VM patients (3 men, 10 women, mean age 45.5), and 8 HC subjects (2 men, 6 women, mean age 37.1) were enrolled. Methods: The electrodes were placed on the vertex and the spinal process of the fifth cervical vertebra. Binaural click stimulation (0.1 ms, 70 dBnHL) was presented. A total of 800 responses were averaged. Averaged responses were divided into four sets (S1 to S4) according to the temporal order. No, Po, Na, and Pa were identified, and amplitudes and latencies were measured. Results: Concerning latencies, HC subjects showed a tendency of shorter latencies. However, there was no clear effect of repetitive stimulation. Concerning No-Po amplitudes, no significant differences were observed. Raw amplitudes of Na-Pa showed statistically significant differences in S1 and S2 among the groups (p < 0.01 one-way ANOVA). Differences were shown in MD vs. VM and HC vs. VM in S1 (smaller in VM) (p < 0.01 Bonferroni's test) and in MD vs. VM in S2 (smaller in VM) (p < 0.01 Bonferroni test). Relative amplitudes of Na-Pa to S1 showed statistically significant differences in S4 (p < 0.01 one-way ANOVA). Differences were shown in MD vs. VM and HC vs. VM (larger in VM) (p < 0.01 Bonferroni's test). Differences of Na-Pa amplitudes in S2 to S4 from Na-Pa amplitude in S1 were significant in S4 of VM patients (Dunnett's test). Conclusions: VM patients showed lack of habituation (potentiation) of Na-Pa amplitude in AMLR to repetitive stimuli while MD patients and HC subjects showed habituation. Observation of lack of habituation has high diagnostic accuracy for differential diagnosis of VM from MD.

Simultaneous Presentation of Definite Vestibular Migraine and Definite Ménière's Disease: Overlapping Syndrome of Two Diseases.

Objectives: To review the clinical records of patients that exhibited the clinical features of both vestibular migraine (VM) and Ménière's disease (MD) during each episodic vertigo attack and to discuss the possible pathophysiology of such combination of symptoms. Subjects: Ten patients that were selected according to criteria based on a combination of the diagnostic criteria for definite MD and VM (9 females and one male, age: 22-54 years) were enrolled. They were required to show features of both diseases in each vertigo attack. Methods: The patients' medical histories and pure-tone audiometry, cervical vestibular evoked myogenic potential (cVEMP), video head-impulse test (vHIT), and caloric test results were examined. cVEMP was recorded using 500 and 1,000 Hz short tone bursts (125dBSPL, air-conducted), 500 Hz-1,000 Hz cVEMP slope, an index of endolymphatic hydrops in the saccule was calculated using normalized amplitudes of p13-n23. For performing vHIT, each subject was seated 1.5 m in front of a target and asked to keep watching it as their head was passively rotated by the examiner. Their eye movements were evaluated using video-oculography while their head movements were recorded using inertial sensors. Results: The patients were predominantly female. On average, the onset of migrainous headaches occurred 9 years earlier than the onset of vertigo attacks. All of the patients but one had migraines with auras. Five of the 10 patients had a family history of vertigo attacks accompanied by both migrainous and auditory symptoms. The patients mainly displayed hearing loss at low frequencies. Nine patients exhibited 500-1,000 Hz cVEMP slope < -19.9, which was suggestive of endolymphatic hydrops. None of the patients who underwent vHIT showed abnormal canal function. One patient showed unilaterally decreased caloric responses. Conclusions: These patients presented with simultaneous MD and VM signs/symptoms might be referred to "VM/MD overlapping syndrome (VM/MD-OS)" as a new clinical syndrome.

Is Alteration of Tuning Property in Cervical Vestibular-Evoked Myogenic Potential Specific for Ménière's Disease?

OBJECTIVE: The aim of this study is to show sensitivity and specificity of cervical vestibular-evoked myogenic potential (cVEMP) tuning property test to Ménière's disease (MD) in comparison with healthy controls (HC) and patients with other vestibular diseases. SUBJECTS: Totally 92 subjects (50 women and 42 men, 20-77 years of age) were enrolled in this study. Subjects were composed of 38 definite unilateral MD patients, 11 unilateral benign paroxysmal positional vertigo patients, 14 vestibular migraine patients, 19 unilateral vestibular neuritis patients, and 10 HC. METHODS: The subjects underwent cVEMP testing to 500 and 1,000 Hz short tone bursts (125 dBSPL). The corrected amplitudes of the first biphasic responses (p13-n23) (cVEMP) were measured. Then, a tuning property index (the 500-1,000 Hz cVEMP slope) was calculated. RESULTS: The area of under the ROC curve (AUC) was 0.75 in comparison with other vestibular disease patients, while AUC was 0.77 in comparison with other vestibular disease patients plus HC. The best cutoff point of the 500-1,000 Hz cVEMP slope was -19.9. Sensitivity of the tuning property test to MD was 0.74, while specificity was 0.76 to other vestibular disease patients. CONCLUSION: The tuning property test of cVEMP is useful as a screening test of MD.

Endonasal flap suture-dacryocystorhinostomy (eFS-DCR): a new surgical technique for nasolacrimal duct obstruction (NLDO).

CONCLUSION: Endonasal flap suture-dacryocystorhinostomy (eFS- DCR) is a new and valuable technique for the treatment of nasolacrimal duct obstruction (NLDO). This technique could improve the success rate of endonasal DCR in comparison to external DCR. OBJECTIVE: The standard procedure for NLDO is DCR. DCR can be performed via either an external or endonasal approach. External DCR is now regarded as the gold standard in the treatment of NLDO because of its higher success rate. However, we report a new endonasal surgical technique, eFS-DCR, and assess its efficacy by comparison with the standard endonasal DCR. METHODS: We prospectively investigated a series of 62 consecutive patients with NLDO undergoing endonasal DCR. There were two surgical intervention groups: group 1 (24 patients, 28 sites) undergoing endonasal DCR without eFS; and group 2 (38 patients, 47 sites) undergoing eFS-DCR. Study end points were success rate, the ostium diameter (OD) of the lacrimal sac after DCR, and the duration of stent placement. We compared the two groups on these three points. RESULTS: The success rate was 78.6% (22 of 28 sites) for group 1 (DCR without eFS) and 97.9% (46 of 47 sites) for group 2 (eFS-DCR). The ostium size in patients in group 2 was larger than that in group 1. The median time for the duration of stent placement was 42.5 days for group 1 and 31.5 days for group 2. There was a statistical difference (p < 0.01) in all these parameters between the two groups.

Application of hyperdry amniotic membrane patches without fibrin glue over the bony surface of mastoid cavities in canal wall down tympanoplasty.

CONCLUSION: Fibrin glue might be an inessential bioadhesive for attachment of hyperdry amniotic membrane (AM) patches in canal wall down (CWD) tympanoplasty. OBJECTIVE: To clarify the pliability and adherence capability of human hyperdry AM, the feasibility of fixing hyperdry AM without fibrin glue to the bony surface of the mastoid cavity was evaluated in CWD tympanoplasty. METHODS: This was a retrospective chart review. In seven ears of seven patients, the AM was simply attached over the bony surface of the mastoid cavity without fibrin glue (AMG(-) group). In 22 ears of 20 other patients, hyperdry AM (11 ears of 11 patients, AMG(+) group) or temporal fascia (11 ears of 9 patients, TFG(+) group) was attached over the bony surface of the mastoid cavity with fibrin glue. Times for graft epithelization were compared among the three groups. RESULTS: Complete epithelization of the mastoid cavity took place in all patients in all three groups. The mean time to complete epithelization of the graft in the AMG(-) and AMG(+) groups was significantly faster than that in the TFG(+) group (p < 0.05) and was not significantly different between the two AM groups (p > 0.05).

Intermittent pressure therapy of intractable Meniere's disease and delayed endolymphatic hydrops using the transtympanic membrane massage device: a preliminary report.

CONCLUSION: Middle ear pressure treatment by the tympanic membrane massage (TMM) device as well as the Meniett device is effective and provides minimally invasive options for intractable vertigo in patients with Meniere's disease (MD) and delayed endolymphatic hydrops (DEH). OBJECTIVE: The effects of the TMM device were evaluated according to the criteria of the Japan Society for Equilibrium Research (1995) in patients with MD and DEH and compared to those in patients treated with the Meniett device. METHODS: Twelve ears of 10 patients (MD 8; DEH 2) were treated with the TMM device, while 16 ears of 15 patients (MD 11; DEH 4) were treated with the Meniett device. All the patients had failed to respond to medical treatment including diuretics before each pressure treatment, and were followed up for more than 12 months after treatment. Tympanotomy is necessary before treatment for the Meniett device, not but for the TMM device. RESULTS: With both devices, the frequency of vertigo after treatment was significantly lower than before treatment (p < 0.05). The time course of vestibular symptoms with the TMM device was not significantly different from that with the Meniett device (p > 0.05). No complications were directly attributable to treatment with the TMM device.

[Effect of S-1 in a patient with metastatic eyelid sebaceous carcinoma].

We report a 68-year-old female with a history of repeated right cervical lymph node metastasis secondary to eyelid sebaceous carcinoma. She had undergone right neck dissection due to lymph node metastasis at the department of surgery in an other local hospital. After the orbital exenteration, she underwent multiple operations, including 2 right partial parotidectomy and then total parotidectomy, 4 right cervical lymph node excisions and 1 left upper neck dissection due to lymph node metastasis. Adjuvant chemotherapy with S-1(at a dose of 80 mg per day)alone was applied as tumor dormancy therapy on an outpatient basis. Any adverse events during S-1 medication were observed. New metastatic lesions were not found until 29 months after the beginning of chemotherapy with S-1 alone. Although the effectiveness of S-1 for eyelid sebaceous carcinoma has not been demonstrated, S-1 might be useful in patients with recurrent eyelid sebaceous carcinoma based on our patient's recurrence-free survival.

Long-term effects of the Meniett device in Japanese patients with Meniere's disease and delayed endolymphatic hydrops reported by the Middle Ear Pressure Treatment Research Group of Japan.

CONCLUSION: The Meniett device is a minimally invasive and safe treatment that may be used to provide longer-term reduction of vestibular symptoms in patients with delayed endolymphatic hydrops (DEH) as well as those with Meniere's disease (MD). OBJECTIVE: The effects of the Meniett device were evaluated in patients with a diagnosis of definite MD or DEH according to the 1995 AAO-HNS criteria. METHODS: Twenty-nine ears of 28 patients with MD and 5 ears of 5 patients with DEH (ipsilateral type 4, contralateral type 1) were treated with the Meniett device by the Middle Ear Pressure Treatment Research Group of Japan. All of the patients had failed to respond to medical treatment including diuretics before the pressure treatment. RESULTS: Sixteen (57%) patients with MD and all five (100%) patients with DEH remained entirely free from vertigo spells; nine (32%) patients with MD responded with a significant decrease in the frequency of vertigo spells. In regard to hearing, 25 ears (74%: MD, n = 21; ipsilateral DEH, n = 4) had stable hearing levels; only 4 ears (12%: MD, n = 3; contralateral DEH, n = 1) showed a significant hearing improvement. No complications were attributable to the Meniett device.

Prognosis of inner ear function in pneumolabyrinth: case report and literature review.

Pneumolabyrinth, the presence of air within the labyrinth, is a rare radiologic finding associated with a well-known perilymphatic fistula (PLF). As there are only 24 published case reports in medical literature, multiple treatment options for the pneumolabyrinth are available. We report a case of a 20-year-old man with a pneumolabyrinth secondary to tympanic membrane and ossicular trauma. Despite conservative treatment, sensorineural hearing loss (SNHL) had progressed rapidly, and his profound deafness had not recovered after surgical exploration with closure of the PLF. Functional prognostic factors of SNHL are revised and compared with this case.

Mumps virus may damage the vestibular nerve as well as the inner ear.

We report a case of mumps deafness with acute vestibular symptoms secondary to both retrolabyrinthine and inner ear dysfunction. To our knowledge, no such case has been reported elsewhere. The patient was a 6-year-old girl in whom mumps deafness was initially diagnosed. Two days after onset of the hearing impairment, severe vertigo developed. Neurotologic examinations revealed spontaneous right-beating nystagmus, left canal paresis, absence of vestibular-evoked myogenic potentials in the left ear, and absence of responses to a left galvanic body sway test (GBST). The vertigo resolved 7 days after onset. However, the left hearing impairment progressed to complete deafness. Six months later, left canal paresis was still present, but bilateral GBST responses were normal, suggesting that retrolabyrinthine vestibular function had been restored. Mumps virus may affect retrolabyrinthine function as well as the inner ear.