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The Use of Vestibular Test Battery to Identify the Stages of Delayed Endolymphatic Hydrops
Mei-Chun Lin and Yi-Ho Young Otolaryngology -- Head and Neck Surgery 2012 147: 912 originally published online 29 June 2012 DOI: 10.1177/0194599812452993 The online version of this article can be found at: http://oto.sagepub.com/content/147/5/912
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The Use of Vestibular Test Battery to Identify the Stages of Delayed Endolymphatic Hydrops
Mei-Chun Lin, MD1, and Yi-Ho Young, MD1
Otolaryngology Head and Neck Surgery 147(5) 912918 American Academy of OtolaryngologyHead and Neck Surgery Foundation 2012 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/0194599812452993 http://otojournal.org
Abstract Objective. Patients with delayed endolymphatic hydrops (DEH) underwent a vestibular test battery to evaluate the residual function, assess their clinical stage, and predict outcome. Study Design. Case series with chart review. Setting. University hospital. Subjects and Methods. Twenty patients with DEH, 15 with ipsilateral type and 5 with contralateral type, were enrolled. All patients underwent audiometry and caloric, ocular vestibular-evoked myogenic potential (oVEMP), and cervical VEMP (cVEMP) tests. The DEH staging was based on vestibular test results. Stage 0 indicates that all 3 vestibular tests are normal, while stages I through III indicate abnormal results in tests 1 through 3, respectively. Results. Of the 20 DEH patients, 2 patients were stage 0, 12 patients were stage I, 4 patients were stage II, and 2 patients were stage III. The median frequency of vertigo in patients with stages II and III was 4 episodes monthly, significantly less than 15 episodes monthly in those with stages 0 and I. Ipsilateral and contralateral types did not differ significantly in the stage distribution. The percentages of abnormal cVEMP, oVEMP, and caloric test results for patients with ipsilateral type were 80%, 33%, and 13%, not significantly different from those for contralateral type. Conclusions. As a vertiginous attack may subside spontaneously for patients with long-term DEH, one must identify its clinical stage based on cVEMP, oVEMP, and caloric test results. In early stage DEH, most vestibular function remained relatively intact, leading to repeated vertiginous attacks. Conversely, subsidence of vertiginous episode can be anticipated in patients with late stage DEH.
psilateral delayed endolymphatic hydrops (DEH) refers to one ear with profound hearing loss. After several years or decades, episodic vertigo occurs, accompanied by nausea and vomiting.1,2 Contralateral DEH exhibits the same features as ipsilateral DEH and hearing loss in the opposite (better) ear fluctuates. Via a long-term follow-up study of cases with both ipsilateral and contralateral DEH, Kamie3 reported that 65% to 90% of cases had episodic vertigo that vanished within 5 to 10 years. According to the American Academy of Otolaryngology Head and Neck Surgery,4 Menieres disease staging was based on the arithmetic mean of pure tone thresholds at 0.5, 1, 2, and 3 kHz using the worst audiogram at 6 months before treatment. However, this staging system for Menieres disease cannot be applied to cases with ipsilateral DEH, since the latter manifests as profound sensorineural hearing loss on the lesion ear. Alternatively, the interaural amplitude difference (IAD) ratio (now termed asymmetry ratio) of the vestibularevoked myogenic potential (VEMP) test correlates with Menieres disease stage and can be used as another tool for assessing the stage of Menieres disease.5 By stimulating the ear with loud sound or bone vibration stimuli, VEMP can be recorded on contracted neck muscles, now called cervical VEMP (cVEMP), and on the extraocular muscles, termed ocular VEMP (oVEMP).6-8 These two recently developed electrophysiological tests expand the test battery for clinicians to assess utricular and saccular hydrops in patients with Menieres disease.9-11 Based on the hypothesis of efferent specificity proposed by Curthoys,12 cVEMP arises from the saccular macula, whereas oVEMP predominantly originates from the utricular macula. Thus, when using the inner ear monitoring system13 such as audiometry and cVEMP, oVEMP, and caloric tests to study the localization and prevalence of hydrops formation, it reveals that the declining function in the cochlea, saccule, utricle, and semicircular canals mimics the declining sequence of hydrops formation in temporal bone studies.14,15 As the disease progresses, canal
1 Department of Otolaryngology, National Taiwan University Hospital, Taipei, Taiwan
Keywords delayed endolymphatic hydrops (DEH), vestibular-evoked myogenic potential (VEMP), cervical VEMP (cVEMP), ocular VEMP (oVEMP)
Received March 23, 2012; revised May 14, 2012; accepted June 6, 2012.
Corresponding Author: Yi-Ho Young, MD, Department of Otolaryngology, National Taiwan University Hospital, 1 Chang-te St, Taipei, Taiwan Email: youngyh@ntu.edu.tw
Lin and Young paresis is noted in 50% of Menieres patients after the first decade.16,17 Restated, the fluctuation of endolymphatic hydrops can be reflected by functional deterioration and recovery of the cochlea, saccule, utricle, and semicircular canals. Thus, in this study, DEH patients underwent a vestibular test battery to evaluate residual vestibular function, assess their clinical stage, and predict outcome.
913 placed on the sternum. During recording, the subject was instructed to look upward at a small fixed target . 2 m from the eyes, with a vertical visual angle of approximately 30o above horizontal. The EMG signals were amplified and bandpass filtered between 1 and 1000 Hz. The stimulation rate was 5 Hz. The duration of analysis of each response was 50 ms, and 30 responses were averaged for each run. The operator held the vibrator by hand so that the axis of the connected bakelite cap perpendicularly delivered a repeatable tap on the subjects skull at Fz (midline of the hairline). The initial peak driving voltage was about 8 V, equivalent to 128 dB force level (FL). The input signal was a half cycle 500 Hz sine wave, driven by a custom amplifier. The initial negative-positive biphasic waveform comprised peaks nI and pI. Consecutive runs were performed to confirm the reproducibility of peaks nI and pI, and oVEMPs were deemed to be present. Conversely, oVEMPs were deemed to be absent when the biphasic waveform was not reproducible. At our laboratory, the norm for the latency of peak nI was 11.4 6 0.8 (mean 6 SD) ms. Those with the nI latency exceeding 13.0 ms were defined as delayed response. The asymmetry ratio (%) was defined as the difference of the amplitude nI pI on each ear divided by the sum of amplitude nI pI of both ears, that is, (larger amplitude smaller amplitude / larger amplitude 1 smaller amplitude) 3 100. Those with asymmetry ratio . 40% were interpreted as reduced responses.19
cVEMP Test
Each subject was in a supine position. Two active electrodes were placed on the upper half of the sternocleidomastoid (SCM) muscles; one reference electrode was positioned on the suprasternal notch, and a ground electrode was situated on the forehead. The other settings were the same as in the oVEMP test, except that the vibrator delivered a repeatable tap on the subjects head at inion.19 To measure background muscle activity, subjects were given feedback of the level of EMG activity in their SCM muscles during data collection and were required to keep a background muscle activity of at least . 50 mV.20 The subjects elevated their heads during testing. A total of 50 responses were averaged and recorded bilaterally. The first positive and second negative polarities of biphasic waveform were termed waves p13 and n23, respectively. Consecutive runs were performed to confirm the reproducibility of peaks p13 and n23, and cVEMP responses were termed present. The latencies of p13, n23, and amplitude p13 n23 were measured. At our laboratory, the norm for the latency of p13 was 14.4 6 1.3 (mean 6 SD) ms, and we defined when the latency of peak p13 exceeding 17.0 ms as delayed cVEMPs. In addition, those with asymmetry ratio . 33% were defined as reduced response.
Caloric Test
Bithermal caloric test was conducted with electronystagmographic recorders (Sanei 1B21, Tokyo, Japan). Canal paresis was defined when the mean slow phase velocity (SPV) \ 17o/s, or as a greater than 25% difference between maximum slow phase velocity measurements for each ear, when compared with the sum of SPVs from each ear. If no caloric response was elicited, the subject underwent ice water (0oC, 10mL) caloric testing to further confirm the caloric areflexia.
oVEMP Test
The subject was in a sitting position. Surface potentials, predominantly electromyographic (EMG) activities, were recorded (Smart EP 3.90, Intelligent Hearing Systems, Miami, Florida, USA). Two active electrodes were placed around 1 cm below the center of the 2 lower eyelids. The other 2 reference electrodes were positioned about 1 to 2 cm below the active ones, and 1 ground electrode was
Statistical Methods
Comparison of the abnormal test results between the 2 types was analyzed by Fishers exact test. Comparison of the
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Table 1. Clinical Information of 20 Patients with Delayed Endolymphatic Hydrops
Four-Tone Average(dB) Case No. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 Age, y 68 68 57 42 25 61 45 57 42 52 34 58 68 48 32 34 55 40 29 55 Sex F M F F F M M F F F M M F F F M F F M M Side R R R R R R R L L L L L L L L L L L L L Type I I I I I I I I I I I I I I I C C C C C Right so so so so 93 so so 24 4 18 11 36 23 6 12 so so so 103 so Left 28 38 19 26 12 28 13 so so so 94 so 90 so so 53 64 19 51 17
abnormal test results between the 2 sides was analyzed by McNemar test. Frequency of vertigo between the stages was compared by Mann-Whitney U test. A significant difference indicates the P value \ .05.
shown in 17 ears and canal paresis was observed in 3 ears (15%). For the opposite unaffected ears, the cVEMP, oVEMP, and caloric tests identified abnormal responses in 55%, 30%, and 30% of the ears, respectively (Table 2). There were no significant differences in the abnormal percentage of the 3 tests between the affected and unaffected ears (P . .05, McNemar test).
DEH Staging
The DEH staging was based on cVEMP, oVEMP, and caloric test results. Stage 0 indicates that all 3 test results of the affected ear are normal; stage I indicates that 1 of 3 test results are abnormal (Figure 1); stage II indicates that 2 test results are abnormal (Figure 2); and stage III indicates that no test result is normal. Of the 20 DEH patients, 2 patients were classified as stage 0, 12 patients were stage I, 4 patients were stage II, and 2 patients were stage III (Table 2).
Audiometry
All patients had profound hearing loss (. 90 dB) in at least one ear. For the better hearing ear, the 4-tone average at 0.5, 1.0, 2.0, and 3.0 kHz was \ 26 dB in 12 ears, 26 to 40 dB in 5 ears, and 41 to 70 dB in 3 ears (Table 1).
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both ears, termed bilateral DEH, the time of onset could be different for each ear. In other words, 2 situations are supposed in bilateral DEH; namely, both ears are affected by the ipsilateral type, or 1 ear is affected by the ipsilateral type while the other ear is affected by the contralateral type.3 Nevertheless, such a case has rarely been observed. As the recently developed inner ear test battery including audiometry and cVEMP, oVEMP, and caloric tests investigates the inner ear function completely,13 it may assist clinicians in evaluating inner ear involvements in DEH patients with various inner ear insults.
Discussion
The mechanism of DEH is a sufficiently major labyrinthine insult that causes total deafness while vestibular function is preserved, producing delayed atrophy or fibrous obliteration of the endolymph resorption system.2 Thus, clinical expressions of DEH vary with involvement severity. The most severe involvement results in immediate total loss of auditory and vestibular function, whereas the mildest involvement causes delayed malfunction of endolymph resorption, resulting in episodic vertigo (ie, ipsilateral type) and/or fluctuating hearing loss (ie, contralateral type). Clinically, it is usually difficult to determine the ear causing vertigo when ipsilateral DEH occurs in patients with bilateral profound hearing loss unless it is accompanied by tinnitus or aural fullness during episodic vertigo. When the vestibular involvement occurs in
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Figure 1. Case No. 5, female, 25 years, delayed endolymphatic hydrops, right, stage I, ipsilateral type. Caloric test results are normal. The oVEMP test shows normal (nI pI) responses, bilaterally. The cVEMP test shows absent responses on the right ear, but normal (p13 n23) responses on the left ear.
Figure 2. Case No. 13, female, 68 years, delayed endolymphatic hydrops, left, stage II, ipsilateral type. Caloric test results are normal. Both oVEMP and cVEMP tests show normal responses on the right ear, but absent responses on the left ear.
Lin and Young prevail. Thus, function of the utricle and semicircular canals warrants further investigation. As it stimulates the semicircular canals, the caloric test has been utilized to analyze the rotational vestibulo-ocular reflex (VOR) system in individual ears for over 100 years. In contrast, the recently developed oVEMP test assesses the translational VOR by activating the otolithic maculae. As saccular neurons have a strong projection to neck muscles and a weak projection to the oculomotor system, neural connections in the sacculo-ocular system are relatively weak compared with neural connections in the utriculo-ocular and sacculo-collic reflexes.23 Thus, the oVEMP test may reflect utricular macula activity. Unlike the substantial damage to the pars inferior (cochlea and saccule) in DEH patients, the pars superior (utricle and canals) was relatively intact, as evidenced in a temporal bone study by the normal appearance of the utricular macula, crista ampullaris, and semicircular ducts except for dilatation of the utricle.2 These findings may explain why abnormal percentages of oVEMP (45%) and caloric (15%) tests are less than that of the cVEMP (70%) test. Even in the opposite unaffected ears, similar deteriorated results are also observed (Table 2), indicating that primary labyrinthine insult may cause potential defects in both ears. Once both ears showed deteriorated functions in vestibular test battery, which ear having tinnitus or aural fullness during vertiginous episode was considered to be the cause of vertigo. In our previous report,14 abnormal percentages of cVEMP, oVEMP, and caloric tests in Menieres ears were 45%, 25%, and 20%, respectively. In this study, those in DEH cases were 70%, 45%, and 15%, respectively. Notably, the declining sequence of abnormal percentages of cVEMP, oVEMP, and caloric tests in DEH cases mimics that in Menieres cases, and this further supports that DEH and Menieres disease may, at least in part, share the same pathophysiology. In late stage Menieres disease, severe dilatation or collapsed ampullary walls may interfere with cupular movement, resulting in a poor caloric response.17 The vestibular sense organs may also undergo severe atrophic change. After repeated ruptures of the labyrinthine membrane, partial or total collapse of the membranous labyrinth ensues. This condition is coined as vestibular atelectasis.24 These histopathological findings explain why caloric areflexia was observed in late stage Menieres disease, leading to spontaneous relief of vertigo. As only 15% of DEH ears in this study showed canal paresis, one may expect that most patients had preserved canal function, leading to frequent vertiginous attacks. Conversely, most vestibular function deteriorates in late stage DEH patients, which may cause relief of vertiginous episode. Hence, investigating residual vestibular function to identify the clinical stage of DEH is necessary. However, one may question whether a physician can base the decision on whether someone is at a late stage of DEH according to the number of episodes of vertigo he or she has. As some DEH patients with episodic vertigo may visit other hospitals for help, the true numbers of episodic
917 vertigo may be higher than estimation. Nevertheless, this disadvantage can be overcome with the aid of caloric, oVEMP, and cVEMP tests, which may help evaluate the residual vestibular function in deaf ears. It takes a short time (approximately 20 minutes) for both oVEMP and cVEMP tests, and the fee for the vestibular test battery (including ENG, oVEMP test, and cVEMP test) at our hospital costs US$70, which is significantly less than a routine MR imaging (US$700). Thus, it would be practical to make the inner ear test battery a routine examination in DEH patients.
Clinical Relevance
As episodic vertigo disappeared in 90% of DEH patients within 10 years,3 vertiginous attacks may subside spontaneously. For those with intractable vertigo, surgical labyrinthectomy or intratympanic gentamicin injections may be an alternative treatment for abolition of the vestibular function.18 Our study is useful because when a surgeon can identify the DEH stage, surgical intervention may be reserved in late stage DEH patients. For patients with profound deafness and recalcitrant vertigo, surgical labyrinthectomy or gentamicin injection should be mentioned as a treatment option, and this may be reasonable early in the disease process since many DEH patients ultimately lose significant vestibular function.
Conclusion
As a vertiginous attack may subside spontaneously for patients with long-term DEH, one must identify its clinical stage based on cVEMP, oVEMP, and caloric test results. In early stage DEH, most vestibular function remained relatively intact, leading to repeated vertiginous attacks. Conversely, subsidence of vertiginous episode can be anticipated in patients with late stage DEH, and surgical intervention may be set aside. Author Contributions
Mei-Chun Lin, performed VEMP and wrote paper; Yi-Ho Young, supervised study.
Disclosures
Competing interests: None. Sponsorships: None. Funding source: None.
References
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