Rationale for Selective Neck Dissection in Tumors of the Upper Aerodigestive Tract

Relapsing polychondritis (RP) is characterized by inflammation and subsequent degeneration of cartilage. We report a 61-year-old woman who had RP with audio-vestibular manifestations. She was also diagnosed as having a myelofibrosis with myeloid metaplasia (MMM). Bilateral endolymphatic hydrops (EH) was confirmed by dominant –SP/AP of the electrocochleogram (ECochG). When thalidomide and prednisolone were prescribed for the treatment of MMM, symptoms of RP – including the inner ear dysfunction – were ameliorated. Isosorbide, one of the osmotic diuretics commonly used for the treatment of Meniere's disease (MD) in Japan, was also effective in keeping her free from inner ear dysfunction. This is the first report to confirm the existence of EH in a patient with RP with audio-vestibular manifestations. We suppose that an immunological imbalance due to MMM, in conjunction with a specific immunogenetic background, may have played a role in the pathogenesis of RP and the formation of EH in this patient.     

Results in caloric test,video head impulse test and inner ear MRI in patients with Ménière’s disease

ObjectiveOur aim was to elucidate relationships between results from the caloric test (c-test), video Head Impulse Test (vHIT) and inner ear gadolinium-enhanced MRI (ieMRI) in patients with endolymphatic hydrops (EH), especially patients with Ménière’s disease (MD).MethodsWe managed 1789 successive patients at the Vertigo/Dizziness Center in Nara Medical University from May 2014 to December 2018. After providing informed consent for vertigo/dizziness examinations, 281 patients were hospitalized to check their inner ear function for proper diagnosis and treatment. Then 76 participants underwent the c-test, vHIT and ieMRI. Among these 76 cases, 20 were diagnosed with MD (20/76; 26.3%) and 56 were non-MD (56/76; 73.7%) according to the 2015 diagnostic guideline of the International Classification of Vestibular Disorders. The MD group included 15 unilateral and 5 bilateral cases. The non-MD group included 22 benign paroxysmal positional vertigo, 10 vestibular neuritis, 8 sudden deafness with vertigo, 6 orthostatic dysregulation, 4 vestibular neuropathy and 6 others. Results in these examinations in the side of an active lesioned inner ear were representative in each peripheral case.ResultsTwenty-nine of the 76 patients (38.1%) showed discrepant results between the c-test (outside of normal range) and vHIT (within normal range). Twenty-two of 76 patients (28.9%) had a positive EH sign on ieMRI. The c-test/vHIT discrepancy percentage in MD (14/20; 70.0%) was significantly higher than that in non-MD (15/56; 26.8%) (p = 0.00179). The positive EH sign in ieMRI percentage in MD (15/20; 75.0%) was significantly higher than that in non-MD (7/56; 12.5%) (p = 0.0015). There was a significant positive relationship between the c-test/vHIT discrepancy and the positive EH sign (p = 0.00058) in all 76 cases combined. However, there was no significant relationship between c-test/vHIT discrepancy and positive EH sign (p = 0.13) in the 20 MD cases. Considering the 15 unilateral and 5 bilateral MD cases, the c-test/vHIT discrepancy was observed in 14 of the 25 affected ears. Positive signs of vestibular EH herniation into the cupula in the lateral semicircular canal was seen in 14 of the 25 MD ears. There was significant relationship between the c-test/vHIT discrepancy and EH herniation (p = 0.0012) in MD ears.ConclusionThe present results suggest that patients with MD could have inner ear EH significantly more often than those with non-MD. In cases with MD, a positive EH sign on ieMRI did not always indicate a c-test/vHIT discrepancy; both findings may occur due to herniation of vestibular EH adjacent to the lateral semicircular canal.     

Late recovery with cyclosporine-A of an auto-immune sudden sensorineural hearing loss

Sensorineural hearing loss of immune-mediated origin may be present as a symptom in systemic autoimmune diseases or may occur as a primary disorder without other organ involvement (auto-immune inner ear disease). The diagnosis of auto-immune inner ear disease is still predicated on clinical features and to date specific diagnostic tests are not available. We report a case of right-sided sudden hearing loss in a female patient in which the clinical manifestations, in addition to ANA positivity and hypocomplementaemia allowed us to hypothesize an auto-immune inner ear disease. The immunosuppressive treatment with cyclosporine-A was capable of a recovery of the hearing that, however, occurred progressively with normalization of the hearing function after 1 year of treatment. cyclosporine-A could be proposed as a therapeutic option in case of auto-immune inner ear disease allowing the suspension of corticosteroids that, at high dose, expose patients to potentially serious adverse events.     

Autoimmune vestibular dysfunction: preliminary report

Since 1981, 28 patients have been diagnosed with autoimmune inner ear disease. This preliminary, retrospective study reviews clinical and laboratory manifestations of autoimmune vestibular dysfunction. Sixteen of 28 patients presented with dizziness (7 vertigo, 8 lightheadedness, 1 ataxia). Twelve of these patients presented with Meniere's syndrome. Seven of 16 dizzy patients underwent formal vestibular testing. Five had reduced vestibular response in the involved ear(s); one patient had positional nystagmus only; and one had normal results. Of 17 patients with adequate follow-up, 11 presented with dizziness. Hearing improved and dizziness resolved in two patients following treatment. Hearing stabilized and dizziness resolved in two. Hearing stabilized but lightheadedness (without vertigo) persisted in five. Hearing was lost and oscillopsia resulted in two patients. Preliminary conclusions are vestibular dysfunction in autoimmune inner ear disease is nonspecific; bilateral vestibular dysfunction is more common; Meniere's syndrome is relatively common; medical management usually is helpful; surgery at this time does not seem appropriate; vestibular treatment results tend to parallel auditory treatment results.     

Deterioration of hearing in a cochlear implantee with relapsing polychondritis

We report on a rare case of cochlear implantation in a patient affected by relapsing polychondritis (RP), which over time induced cochlear fibrosis/ossification and deterioration of previously excellent hearing performance. The clinical course was determined by CT scan, electrophysiology, and speech perception data. We conclude that RP is a severe autoimmune connective disorder that can cause profound sensorineural hearing loss. Cochlear implantation in these patients can provide excellent performance. Continuation of therapy may improve prognosis, but relapses involving inner ear structures can determine fibrosis/ossification of the modiolus and interfere with cochlear implant use.     

Evaluation of changes in endolymphatic hydrops volume after medical treatments for Meniere's disease using 3D magnetic resonance imaging

ObjectiveTo elucidate the relationship between vertigo and EH volume after medical treatment, we investigated changes in endolymphatic hydrops (EH) volume using inner ear magnetic resonance imaging (ieMRI) in relation to clinical results for vertigo and hearing after administration of the anti-vertiginous medications betahistine, adenosine triphosphate (ATP), isosorbide (ISO), and saireito (SAI) for Meniere's disease (MD).MethodsWe retrospectively enrolled 202 consecutive patients diagnosed with unilateral MD from 2015 to 2021 and assigned them to four groups: Group I (G-I), symptomatic oral medication with betahistine only (CONT); Group II (G-II), inner ear vasoactive oral medication (ATP); Group III (G-III), osmotic diuretic oral medication (ISO); and Group IV (G-IV), kampo oral medication (SAI). In total, 172 patients completed the planned one-year-follow-up, which included the assessment of vertigo frequency, hearing improvement, and changes in EH using ieMRI (G-I, n=40; G-II, n=42; G-III, n=44; G-IV, n=46). We constructed 3D MRI images semi-automatically and fused the 3D images of the total fluid space (TFS) of the inner ear and endolymphatic space (ELS). After fusing the images, we calculated the volume ratios of the TFS and ELS (ELS ratios).ResultsOne year after treatment, vertigo was controlled with zero episodes per month in 57.5% (23/40) of patients in G-I, 78.6% (33/42) in G-II, 81.8% (36/44) in G-III, and 82.6% (38/46) in G-IV (statistical significance: G-I<G-II=G-III=G-IV). Hearing improved by > 10 dB in 5.0% (2/40) of patients in G-I, 16.7% (7/42) in G-II, 18.2% (8/44) in G-III, and 21.7% (10/46) in G-IV (statistical significance: G-I=G-II=G-III=G-IV). ELS ratios were significantly reduced after treatment only in the vestibule for G-II, G-III, and G-IV when compared with G-I. Especially among patients with complete control of vertigo after treatment, ELS ratios were significantly reduced after treatment in the vestibule and total inner ear for G-II; in the cochlea, vestibule, and total inner ear for G-III; and in the cochlea, vestibule, and total inner ear for G-IV compared with G-I. However, there were no significant findings in the relationship between hearing results and changes in ELS ratios.ConclusionThese results indicate that daily administration of anti-vertiginous medications including ATP, ISO, and SAI could be an effective treatment option for patients with MD at an early stage before it becomes intractable. Treatments to reduce EH might offer better control of vertigo rather than improve hearing.     

A nationwide multicenter study of the Cochlin tomo-protein detection test: clinical characteristics of perilymphatic fistula cases

Abstract

Objective: To investigate the positive rate for the Cochlin tomo-protein (CTP: an inner ear-specific protein) detection test among patients with inner ear-related clinical manifestations and evaluate the clinical characteristics of definite perilymphatic fistula (PLF).

Methods: We have performed an ELISA-based CTP detection test using middle ear lavage (MEL) samples from 497 cases of suspected PLF enrolled from 70 clinical centers nationwide between 2014 and 2015. In addition to the CTP-positive rate, audio-vestibular symptoms were compared between CTP-positive and -negative cases.

Results: 8–50% of patients in category 1 (trauma, middle and inner ear disease cases), and about 20% of those in categories 2, 3 and 4 (external origin antecedent events, internal origin antecedent events, and without antecedent event, respectively) were positive for CTP. In category 1 cases, the earlier tested samples showed a higher CTP-positive rate, whereas no differences were observed in categories 2, 3 or 4. The characteristic clinical features in the earlier tested cases were nystagmus and fistula sign in CTP test-positive cases in category 1, and streaming water-like tinnitus in those in categories 2, 3 and 4.

Conclusion: The present study clarified that CTP detection test-positive patients exist at considerable rates among patients with inner ear-related manifestations.     

内耳疾病继发BPPV的发病机制和临床特征的研究进展

良性阵发性位置性眩晕(BPPV)是最常见的外周性前庭功能障碍疾病之一,可分为特发性BPPV和继发性BPPV。外源性疾病被认为是继发性BPPV的最重要危险因素。除外伤和内耳手术病史外,常见内耳疾病也会导致继发性BPPV,如梅尼埃病(MD)、突发性感音神经性听力损失(SSNHL)、前庭神经炎(VN)和前庭性偏头痛(VM)等,继发性BPPV也可发生于内耳疾病的某个阶段,且临床表现相似。系统回顾国内外相关文献,总结并分析常见内耳疾病继发BPPV的发病机制和临床特征,以作临床诊疗参考。     

Audio-vestibular evaluation in patients with Behçet's syndrome

A prospective controlled clinical study was carried out at the Department of Ophthalmology and ENT, In?nü University Medical Faculty, Turgut Ozal Medical Center, Research Hospital, to evaluate the audio-vestibular involvement in patients with Beh?et's syndrome compared with controls. Twenty-five consecutive patients with Beh?et's syndrome (mean age +/- SD, 34.96 +/- 8.50) and 20 age- and sex-matched healthy volunteers (hospital staff) as control subjects (mean age +/- SD, 34.45 +/- 9.16) were included in this study. Beh?et's patients were divided into two groups according to the number of criteria, complete (all four major criteria) and incomplete (three major criteria without ocular involvement). The groups were compared with each other or controls regarding inner ear involvement. Audiometric pure-tone thresholds at 125 to 8000 Hz were obtained in all subjects in both groups, and pure tone average (PTA) hearing thresholds were calculated for the middle, high and low frequencies. In addition, short increment sensitivity index (SISI), tone decay and BERA examinations were performed in all Beh?et's patients. Sensorineural hearing loss (SNHL) was present in six of 25 patients with Beh?et's syndrome. Two Beh?et's patients had unilateral total SNHL, two had bilateral moderate level SNHL, one had bilateral low-frequency SNHL and one bilateral high frequency SNHL. In two, BERA, and in five SISI, examination disclosed inner ear involvement. In control subjects, the past medical history was normal and there was no consistent audio-vestibular complaint. Their PTA thresholds were all in the normal range. Otoscopic examination findings were normal, with intact, mobile tympanic membranes in both groups. The present study showed that audio-vestibular involvement is not infrequent in Beh?et's syndrome compared with age- and sex-matched healthy controls, and it is under-estimated. All Beh?et's patients should regularly be followed by an otolaryngologists and be given information about the possibility of inner ear involvement. According to our results, hearing loss occurs more often in older patients and also in the complete form of Beh?et's syndrome.     

Das Cogan-Syndrom

Classical Cogan's syndrome is a disease of the inner ear with participation of the eyes, typically involving keratitis. If no objectively assessable optical symptoms are present, a diagnosis is difficult. Additional nonspecific symptoms can, combined with inner ear participation, suggest Cogan's syndrome. Between 2001 and 2003, we documented the course of two patients with Cogan's syndrome. As indicated in the literature, organ related symptoms could be related to a generalized vascular illness. In addition to the usual otological symptoms with cochleovestibular dysfunction and symptoms of typical and atypical ocular manifestations, other non-specific changes were found. Interstitial keratitis was diagnosed in one patient while in the second objective ophthalmological symptoms failed. The symptoms could be improved by systemic and local administration of corticosteroids and immunosuppressive therapy, however, progression of inner ear deafness could not be stopped in one patient and a cochlear implant was necessary.     

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.     

Standardisierte Messungen der Schallübertragung verschiedener Mittelohrprothesen

The sound transmission properties of three different commercially available middle ear implants and the prototype of a complete middle ear prosthesis (CoMEP) were evaluated with a special measurement system that permits standardized conditions. This system uses a mechanical middle ear model (MMM) which approximates the impedances of the tympanic membrane and inner ear. The implants were fitted under defined conditions into the MMM. Displacement of the artificial stapes footplate of the MMM was measured with an optical probe. The measurements of different middle ear prostheses showed that the mass of the implant was the most important factor for optimum high frequency transmission. The lightest implant (4 mg, titanium) showed the best results. The CoMEP revealed the highest sensitivity because of a slight enlargement of the diameter of the artificial tympanic membrane. These findings show that the CoMEP is able to restore sound transmission to a normal range. In a second experiment one of the implants was fitted into the MMM with different forces. The increase of stiffness produced a slightly better high frequency transmission at the expense of low frequency sensitivity. Hence, the fitting of the implant may not be too stiff because of its loss of mobility within a significant frequency range.     

Wideband tympanometry findings in inner ear malformations

ObjectiveThe deficits in the cochlea which is at the one end of the ear sound transfer system, may effect middle ear functions. Wideband typanometry (WBT) is frequently used to evaluate these transfer functions which play a crucial role in setting the impedance matching between the external ear and the cochlea. To this end, the aim of this study was to investigate the ear transfer functions in inner ear malformations via WBT, and to question whether these functions change depending on the types of inner ear malformation.MethodsThis prospective case-control study was conducted in a university hospital. One hundered-fifty-seven ears (aged 3–37 years) under the groups of cochlear hypoplasia, incomplete partition I, incomplete partition II, cochlear aplasia and complete labyrinthine aplasia were evaluated. In the control group, 30 ears with normal hearing were enrolled and WBT was carried out. Tympanometric peak pressure, equivalent middle ear volume, static admittance, tympanogram width, resonance frequency, average wideband tympanometry and absorbance measurements were analyzed.ResultsThe inner ear malformation groups demonstrated statistically significant differences than the control group and from each other in terms of traditional tympanometric parameters and WBT test parameters (p < 0.05). The most remarkable difference was between the group of complete labyrinthine aplasia and the control group, most probably because of complete labyrinthine aplasia’s structural effects. However, on some parameters, incomplete partition II and the control group showed similarities. In absorbance measurements, there was significant difference between all patient groups and the control group, especially at high frequencies (p < 0.05). The largest difference was between the control group and the group of complete labyrinthine aplasia which has revealed the lowest absorbance values (p < 0.05). In averaged-wideband tympanogram analysis, all patient groups obtained a lower amplitude peak than the control group; complete labyrinthine aplasia group had the flattest peaked amplitude, while the incomplete partition II group had a near-normal curve.ConclusionThe results of the study revealed the distinctive effects of inner ear malformations in middle ear transfer functions. It is concluded that the absence of inner ear structures causes negative effects on energy absorbance and the other transfer functions of the middle ear. WBT may provide additional information on diagnosis of patients with inner ear malformations.     

Clinical analysis of 15 patients with relapsing auricular polychondritis

Relapsing polychondritis (RP) is a rare autoimmune disorder of unknown etiology. The disease is characterized by episodic inflammation and destruction of cartilaginous and connective tissue structures, including the ear, eye, nose, larynx, trachea, bronchi, joints, skin, heart valves, and aorta. As the symptoms of RP are diverse and complex, it is easily misdiagnosed. The aim of this paper was to improve the understanding of the clinical features of RP, thereby facilitating its early diagnosis. Fifteen patients with RP were analyzed retrospectively and the relevant literature reviewed. The number of patients presenting with auricular chondritis was 13, while two presented with polyarthritis. Among them, the treatment of 2 RP patients with respiratory tract involvement failed and 1 patient died. Eleven patients with RP (73 %) were initially misdiagnosed. RP involves cartilage and connective tissue. The prognosis for patients with respiratory tract involvement is poor. RP causes episodic and progressive inflammation of cartilage throughout the body and is associated with a variety of clinical manifestations. Early diagnosis of RP depends on a thorough understanding of its clinical features.     

Endolymphatic hydrops in patients with tinnitus as the major symptom

Tinnitus is one of the symptoms of Meniere’s disease. The relationship between a clinical presentation of subjective tinnitus or ear fullness and endolymphatic hydrops (EH) has not yet been explored. We studied 15 patients with symptoms of tinnitus as their major complaint, with or without hearing loss, who were evaluated using magnetic resonance imaging (MRI). The mean age of the subjects was 59 years (range 35–79 years). Nine were women and six were men. Patients were divided into two groups based on whether they had fluctuating or stable tinnitus. These groups were subdivided in the presence or absence of accompanying sensation of ear fullness. MRI was performed 4 h after intravenous gadolinium administration. Overall, 30 ears were evaluated. EH in the cochlea was present in 14 of 25 symptomatic ears (56 %) in patients with tinnitus as the major complaint. Significant hydrops was present in 7 of 14 ears and mild hydrops in the other ears. Patients with fluctuating tinnitus had EH more frequently than patients with stable tinnitus. Furthermore, the presence of ear fullness also correlated with the presence of EH in the cochlea. However, there was no significant relationship between EH in the cochlea and age, sex, duration of tinnitus, hearing level or the configuration of the audiogram. Our study revealed that patients who had tinnitus as their major symptom often had EH. Using MRI to identify this covert early EH in patients who have tinnitus as their major symptom may broaden the treatment options for tinnitus.     

MRI performed after intratympanic gadolinium administration in patients with Ménière’s disease: correlation with symptoms and signs

The objective of the study was to compare the outcomes of a series of diagnostic parameters in Ménière’s disease (MD) patients with the extent of endolymphatic hydrops (EH) as shown by magnetic resonance imaging (MRI) performed after intra-tympanic gadolinium administration using 18 patients (13 males and 5 females, age 25–78 years, median age 54.3 years) with definite MD. A 0.6-ml solution of Gadobutrol (1 mmol/ml) diluted 1:7 in saline was injected through the inferior-posterior quadrant of the tympanic membrane, using a 22-gauge spinal needle. The patient was kept with the head rotated 45° contralaterally for 30 min after the injection. Twenty-four hours later, three-dimensional fluid-attenuated inversion recovery MRI, using a 3-Tesla unit, was performed. Prevalence and extension of EH in MD patients was evaluated and correlated with age, duration and stage of the disease, frequency of attacks, time interval from the last attack, functional level scale, tinnitus, aural fullness, caloric stimulation, electrocochleography, and vestibular evoked myogenic potentials. All patients showed impaired enhancement of the inner ear of variable degree with the vestibular portion of the labyrinth more frequently involved than the cochlea. Abnormal vestibular evoked myogenic potentials, duration, and stage of the disease were significantly correlated to the number of inner ear sites involved. Modern imaging makes possible the identification of the endolymphatic hydrops in MD patients, improving diagnostic accuracy. The role of hydrops in the clinical manifestations and its correlation with most of the diagnostic parameters remain, however, not completely clear.     

Relapsing polychondritis -- a case report and review of the literature

PATIENT: A case of a 54-year-old woman with a three month history of recurrent bilateral chondritis of the auricles, cochlear and vestibular inner ear damage and conjunctivitis is described. The diagnosis was based only on clinical criteria (auricular chondritis, conjunctivitis, inner ear damage). Antinuclear antibodies, ANCA and HLA-DR 4 antigen were negative. The only laboratory abnormality was an elevated erythrocyte sedimentation rate. The patient has been treated successfully with Methotrexate 20 mg 1 x /week and Prednisone 15 mg/die for 4 month now. DISCUSSION: The relapsing polychondritis (RP) is a rare, multisystemic and potentially fatal disease. The pathogenesis and optimal therapeutic approach is poorly understood. The disease is characterized by episodic inflammation of cartilage such as auricular, nasal and laryngotracheal. Many other proteoglycan-rich structures like inner ear, eye, kidney and blood vessels, may be involved as well. RP has an equal sex prevalence. The majority of cases appear between 40 and 60 years. Auricular inflammation is the most common feature. Effectiveness of non-steroidal anti-inflammatory drugs, dapsone, immunosuppressive drugs and prednisone has been described. The overall survival rates were 74 % at 5 years and 55 % at 10 in one 1986 series. CONCLUSION: The most common clinical presentation of RP regularly involves ENT-structures. Therefore ENT-specialists should be familiar with this disease. A close interdisciplinary cooperation is essential for therapy and follow-up, because pulmonary and cardiac involvement are limiting prognostic factors.     

Hearing loss in patients with enlarged vestibular aqueduct: air-bone gap and audiological Bing test

The Bing test is based on the principle that occlusion of the external auditory meatus improves the perception of bone-conducted sounds unless there is a conductive hearing impairment. An air-bone gap has been reported in patients with large vestibular aqueduct (LVA) syndrome without apparent middle ear dysfunction. We therefore performed the Bing test on nine patients with this syndrome to evaluate whether it is associated with an air-bone gap or middle ear dysfunction. Bone conduction thresholds did not change significantly during the Bing test in any patient. Because an air-bone gap is observed in patients with abnormal communication between the inner ear and cerebrospinal fluid through the LVA, dehiscent superior canal, or dilated inner ear meatus; we propose that a 'three windows' model (in which the abnormal communication provided by the enlarged endolymphatic duct and sac in LVA acts as the 'third window' for sound conductance) might explain the air-bone gap in such patients.     

Preceding profound deafness and co-factors promote development of endolymphatic hydrops in preliminary patients with delayed endolymphatic hydrops

Conclusion: Profound deafness (PD) is unlikely to be the only cause of endolymphatic hydrops (EH) in patients with delayed endolymphatic hydrops (DEH). Further studies in large patient cohorts are required to determine the etiologic co-factors involved in the development of EH in patients with PD and DEH.

Objectives: DEH usually occurs in patients with sustained unilateral PD who then develop episodic vertigo or fluctuating hearing loss. The latency can vary markedly between PD and development of DEH, but the factors affecting inner ear homeostasis and causing EH during this latency period remain unclear. The study aimed to assess the differences in EH and functional status of the inner ear between patients with PD only and those with DEH.

Method: Two PD and 10 DEH patients who had developed PD in early childhood were enrolled. Gadolinium-enhanced magnetic resonance imaging of the inner ear was performed in all patients to assess the degree of EH in the vestibule and cochlea.

Results: There was no evidence of EH in the affected ears of patients with PD alone. DEH patients, however, showed significant EH in either the cochlea or vestibule, suggesting that EH development and DEH onset were strongly correlated.     

Glycerol pure tone audiometry and glycerol vestibular evoked myogenic potential: representing specific status of endolymphatic hydrops in the inner ear

This prospective study attempts to explore the effect of glycerol on vestibular evoked myogenic potential (VEMP) in patients with definite unilateral Meniere’s disease (MD) and to investigate whether the test reflects different pathologic states of endolymphatic hydrops (EH) in the inner ear by comparing the results of glycerol pure tone audiometry (PTA). Twenty-eight patients with definite unilateral MD were studied. As a control group, 11 patients with vestibular neuritis were selected. Sixteen ears of normal volunteers were enrolled to acquire normal range of the test. Each MD patient underwent glycerol testing measure with both conventional PTA and VEMP testing. Eleven vestibular neuritis patients also underwent glycerol-VEMP testing. The difference ratio was adopted to compare the changes in p1-n1 amplitude and latency of VEMP after the administration of glycerol (1.3 g/kg). Significant changes in the VEMP wave after the glycerol loading were seen in amplitude, but not in latency with MD patients. On the affected side, the amplitude of the p1-n1 biphasic wave significantly increased in 11 of 28 (39.3%) ears and decreased in 2 (7.1%) ears. On the unaffected side, 5 (17.9%) ears showed significant increase in amplitude. Significant changes in PTA were noted after administration of glycerol in 14 of 28 (50%) patients with MD. However, there was no statistically significant correlation between the test results. Glycerol administration had no significant effect on VEMP in patients with vestibular neuritis. The amplitude of VEMP in some patients with MD was increased, but the latency was not influenced by oral administration of glycerol. The severity of EH appears to vary in different parts of the inner ear. Glycerol-VEMP test results in patients with vestibular neuritis suggest that the VEMP reflects potential abnormality in the sacculocollic pathway, while glycerol-VEMP identifies the existence of EH in the saccule. VEMP and PTA after glycerol administration provide potential status of MD.