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Using Transtympanic Gadolinium and MRI in distinguishing between Meniere’s and Migraine Warren C. Swegal, MD and Syed F. Ahsan, MD Dept. of Otolaryngology, Henry Ford Health System, Detroit, MI Dr. Warren C. Swegal Dept. of Otolaryngology Henry Ford Health System Email: [email protected] Phone: 313-916-7086 Fax: 313-916-7263 Contact 1. Boismier TE, Disher MJ. Spontaneous vertigo and headache: endolymphatic hydrops or migraine? Ear Nose Throat J. 2001;80(12):881 2. Lee HJ, Jeon JH, Park S, Kim BG, Lee WS, Kim SH. Prevalence and clinical significance of spontaneous low-frequency air-bone gaps in Ménière's disease. Otol Neurotol. 2014 Mar;35(3):489-94. 3. Hong HR, Shim DB, Kim TS, Shim BS, Ahn JH, Chung JW, Yoon TH, Park HJ. Results of caloric and sensory organization testing of dynamic posturography in migrainous vertigo: comparison with Meniere's disease and vestibular neuritis. Acta Otolaryngol. 2013 Dec;133(12):1236-41 4. Rauch SD, Zhou G, Kujawa SG, Guinan JJ, Herrmann BS. Vestibular evoked myogenic potentials show altered tuning in patients with Ménière's disease. Otol Neurotol. 2004;25(3):333. 5. Orchik DJ, Shea JJ Jr, Ge X. Transtympanic electrocochleography in Menière's disease using clicks and tone-bursts. Am J Otol. 1993 May;14(3):290-4. Review. 6. Shi H, Li Y, Yin S, Zou J. The predominant vestibular uptake of gadolinium through the oval window pathway is compromised by endolymphatic hydrops in Ménière's disease. Otol Neurotol. 2014 Feb;35(2):315-22. 7. Claes G, Van den Hauwe L, Wuyts F, Van de Heyning P. Does intratympanic gadolinium injection predict efficacy of gentamicin partial chemolabyrinthectomy in Menière's disease patients? Eur Arch Otorhinolaryngol. 2012 Feb;269(2):413-8. 8. Hornibrook J, Coates M, Goh A, Gourley J, Bird P. Magnetic resonance imaging for Ménière's disease: correlation with tone burst electrocochleography. J Laryngol Otol. 2012 Feb;126(2):136-41. 9. Fukuoka H, Takumi Y, Tsukada K, Miyagawa M, Oguchi T, Ueda H, Kadoya M, Usami S. Comparison of the diagnostic value of 3 T MRI after intratympanic injection of GBCA, electrocochleography, and the glycerol test in patients with Meniere's disease. Acta Otolaryngol. 2012 Feb;132(2):141-5. References Objective: To describe the use of MRI imaging using Transtympanic Gadolinium (TT Gad) in distinguishing between Vertiginous Migraine and Meniere's Disease (MD) in a patient with vertigo. Study Design: Case Report Methods: Patient’s chart was reviewed for reports of vertigo, vestibular function tests and Imaging. The patient underwent TT Gad injection followed by MRI to assess for endolymphatic hydrops (EH). Audiogram before and after was assessed. Results: A 43 yo man presented with recurrent vertigo attacks lasting more than one hour with right side aural fullness, tinnitus, sensitivity to sound and temperature as well as left side headaches. Vestibular testing demonstrated reduced vestibular response on the left, possible EH on the right, and cervical Vestibular Evoked Myogenic Potential suggesting possible right side superior semicircular canal dehiscence. Patient was counseled about performing an MRI after TT Gad injection to rule in Meniere’s disease. MRI 3D FLAIR sequence ruled out EH and he was started on a Migraine diet. He was treated for migraines and his vertigo and headaches improved by over 90%. Discussion: This is an interesting report because using TT Gad and MRI was able to distinguish between MD and Migraine in a patient who presented with symptoms suggestive of both and when conventional vestibular testing gave inconclusive and a complex picture. This has the potential to save the patient from unnecessary treatment for MD and side effects as well as reduced the time to a diagnosis. Abstract Thus, it was recommended that the patient undergo an MRI using TT Gad to assess for EH. The injection was performed as an outpatient. - Magnevist gadolinium 1/8 th dilution in sterile water - 0.6mL injected through the posterior anterior quadrant - Patient was observed for 30 min, patient tolerated it well - The following day the patient underwent an MRI Results: - Limited visualization of lateral semicircular canals bilaterally on T2-eighted images, likely a susceptibility artifact (Figure 2) - Homogenous distribution of injected gadolinium on perilymphatic inversion recovery on right ear - Membranous labyrinth in continuity on Endolymphatic sequence No evidence of endolymphatic hydrops Case Details The patient’s initial audiogram (Figure 1) demonstrated a right sided conductive hearing loss. At this point multiple etiologies were discussed with the patient. However it was felt that the 2 most likely etiologies included Meniere’s Disease vs. Vertiginous Migraines with semicircular canal dehiscence also a possibility. The patient was referred for further testing including videonystagmography (VNG), electrocochleography (ECOG), cervical vestibular-evoked myogenic potential (cVEMP) testing, and rotational chair testing to better delineate the cause of vertigo. (Table 1) However, when taken together, balance function testing was inconclusive although Rotary Chair test demonstrated vestibular compensation. Clinical Course Vertigo can be a challenging patient complaint. However there are multiple modalities to help delineate the route cause of this symptom. Our patient’s clinical picture did not lead to a definitive diagnosis. Prior research would suggest that treating the patient for MD and switching to treat migraines is reasonable. 1 However, this approach may cause a delay in diagnosis and could potential lead to future non-compliance. Thus further testing was pursued. The audiogram, with the low frequency hearing loss may be suggestive of meniere’s. 2 The VNG was showed vestibular weakness on the left and cVEMP demonstrated hypersensitivity on the right, opposite of what would be expected in either MD or migraines. 3,4 However, ECOG demonstrated EH, suggestive of MD. 5 Recently, MRI with TT Gad has been shown to be safe and effective at assessing for EH. 6,7 Furthermore, there is limited evidence to suggest it may be more effective than ECOG at identifying EH. 8,9 In this case, MRI with TT Gad did not demonstrate EH when ECOG did. The MRI findings allowed the patient to be diagnosed correctly and treated appropriate. The success of the migraine treatment further confirms that this patient suffered from Vertiginous Migraines and not MD. Discussion In a patient who’s clinical picture lead to several different diagnoses, and who’s test results did not support a single diagnosis, MRI with TT Gad was able to rule out Meniere’s disease as the cause of his symptoms. This prevented unnecessary trial and error medical treatment and a delay in diagnosis. Furthermore, this case demonstrated the relative feasibility of the MRI with TT Gad, potentially paving the way for it to be used in other disease models. Conclusions Test Details Right Ear Left Ear VNG Warm Caloric 53 degree/sec 6 Degree/sec Cold Caloric 9 Degree/sec 3 Degree/sec Summary 75% Weakness on Left cVEMP Tone Burst Threshold 60dBnHl 90dBnHL Summary Hypersensitivity on Right (Normal 75-100nHL) ECOG SP/AP Ratio 0.77 0.38 Summary Consistent with EH on right (Normal <0.5) HPI: The patient is a 43 yo male who presented with episodic vertigo for 3-4 years with sound hypersensitivity. - Attacks were preceded by Right side aural fullness, pressure sensation, tinnitus, and occasionally headaches - Episodes lasted over 1 hour but less than 4 hours - He denied hearing loss or autophony - Increased frequency of attacks with higher salt intake PMHx: Sinusitis, GERD, Anxiety, Migraines Physical Exam: - Otoscopic exam benign, TMs mobile - Cranial Nerves Grossly intact - Hennebert’s and Tullio’s negative - Negative Head thrust, Dix Hallpike and Fukuda tests - Non-specific imbalance during Romberg MRI with TT Gad Figure 1. Initial Audiogram Table 1. Selected results from balance function testing. - 1 week after the MRI, the patient denied any major effects from the TT-Gad. He denied otalgia, otorrhea, tinnitus or vertigo. - 2 months after MRI, patient had seen a neurologist and was being treat for with valproate for the migraines, no vertigo - 5 months after MRI, headaches 90% improved, no vertigo - 8 months after MRI, no vertigo, Audiogram normal (Figure 3) Post Scan Follow up Figure 2. MRI with TT Gad Figure 3. Audiogram 8 Months post injection

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Using Transtympanic Gadolinium and MRI in distinguishing between Meniere’s and Migraine

Warren C. Swegal, MD and Syed F. Ahsan, MD

Dept. of Otolaryngology, Henry Ford Health System, Detroit, MI

Dr. Warren C. Swegal Dept. of Otolaryngology Henry Ford Health System Email: [email protected] Phone: 313-916-7086 Fax: 313-916-7263

Contact 1. Boismier TE, Disher MJ. Spontaneous vertigo and headache: endolymphatic hydrops or migraine? Ear Nose Throat J. 2001;80(12):881 2. Lee HJ, Jeon JH, Park S, Kim BG, Lee WS, Kim SH. Prevalence and clinical significance of spontaneous low-frequency air-bone gaps in Ménière's disease. Otol Neurotol. 2014 Mar;35(3):489-94. 3. Hong HR, Shim DB, Kim TS, Shim BS, Ahn JH, Chung JW, Yoon TH, Park HJ. Results of caloric and sensory organization testing of dynamic posturography in migrainous vertigo: comparison with Meniere's

disease and vestibular neuritis. Acta Otolaryngol. 2013 Dec;133(12):1236-41 4. Rauch SD, Zhou G, Kujawa SG, Guinan JJ, Herrmann BS. Vestibular evoked myogenic potentials show altered tuning in patients with Ménière's disease. Otol Neurotol. 2004;25(3):333. 5. Orchik DJ, Shea JJ Jr, Ge X. Transtympanic electrocochleography in Menière's disease using clicks and tone-bursts. Am J Otol. 1993 May;14(3):290-4. Review. 6. Shi H, Li Y, Yin S, Zou J. The predominant vestibular uptake of gadolinium through the oval window pathway is compromised by endolymphatic hydrops in Ménière's disease. Otol Neurotol. 2014

Feb;35(2):315-22. 7. Claes G, Van den Hauwe L, Wuyts F, Van de Heyning P. Does intratympanic gadolinium injection predict efficacy of gentamicin partial chemolabyrinthectomy in Menière's disease patients? Eur Arch

Otorhinolaryngol. 2012 Feb;269(2):413-8. 8. Hornibrook J, Coates M, Goh A, Gourley J, Bird P. Magnetic resonance imaging for Ménière's disease: correlation with tone burst electrocochleography. J Laryngol Otol. 2012 Feb;126(2):136-41. 9. Fukuoka H, Takumi Y, Tsukada K, Miyagawa M, Oguchi T, Ueda H, Kadoya M, Usami S. Comparison of the diagnostic value of 3 T MRI after intratympanic injection of GBCA, electrocochleography, and the

glycerol test in patients with Meniere's disease. Acta Otolaryngol. 2012 Feb;132(2):141-5.

References

Objective: To describe the use of MRI imaging using Transtympanic Gadolinium (TT Gad) in distinguishing between Vertiginous Migraine and Meniere's Disease (MD) in a patient with vertigo. Study Design: Case Report Methods: Patient’s chart was reviewed for reports of vertigo, vestibular function tests and Imaging. The patient underwent TT Gad injection followed by MRI to assess for endolymphatic hydrops (EH). Audiogram before and after was assessed. Results: A 43 yo man presented with recurrent vertigo attacks lasting more than one hour with right side aural fullness, tinnitus, sensitivity to sound and temperature as well as left side headaches. Vestibular testing demonstrated reduced vestibular response on the left, possible EH on the right, and cervical Vestibular Evoked Myogenic Potential suggesting possible right side superior semicircular canal dehiscence. Patient was counseled about performing an MRI after TT Gad injection to rule in Meniere’s disease. MRI 3D FLAIR sequence ruled out EH and he was started on a Migraine diet. He was treated for migraines and his vertigo and headaches improved by over 90%. Discussion: This is an interesting report because using TT Gad and MRI was able to distinguish between MD and Migraine in a patient who presented with symptoms suggestive of both and when conventional vestibular testing gave inconclusive and a complex picture. This has the potential to save the patient from unnecessary treatment for MD and side effects as well as reduced the time to a diagnosis.

Abstract

Thus, it was recommended that the patient undergo an MRI using TT Gad to assess for EH. The injection was performed as an outpatient. - Magnevist gadolinium 1/8th dilution in sterile water - 0.6mL injected through the posterior anterior quadrant - Patient was observed for 30 min, patient tolerated it well - The following day the patient underwent an MRI Results: - Limited visualization of lateral semicircular canals bilaterally on

T2-eighted images, likely a susceptibility artifact (Figure 2) - Homogenous distribution of injected gadolinium on perilymphatic

inversion recovery on right ear - Membranous labyrinth in continuity on Endolymphatic sequence No evidence of endolymphatic hydrops

Case Details

The patient’s initial audiogram (Figure 1) demonstrated a right sided conductive hearing loss. At this point multiple etiologies were discussed with the patient. However it was felt that the 2 most likely etiologies included Meniere’s Disease vs. Vertiginous Migraines with semicircular canal dehiscence also a possibility. The patient was referred for further testing including videonystagmography (VNG), electrocochleography (ECOG), cervical vestibular-evoked myogenic potential (cVEMP) testing, and rotational chair testing to better delineate the cause of vertigo. (Table 1) However, when taken together, balance function testing was inconclusive although Rotary Chair test demonstrated vestibular compensation.

Clinical Course

Vertigo can be a challenging patient complaint. However there are multiple modalities to help delineate the route cause of this symptom. Our patient’s clinical picture did not lead to a definitive diagnosis. Prior research would suggest that treating the patient for MD and switching to treat migraines is reasonable.1 However, this approach may cause a delay in diagnosis and could potential lead to future non-compliance. Thus further testing was pursued. The audiogram, with the low frequency hearing loss may be suggestive of meniere’s.2 The VNG was showed vestibular weakness on the left and cVEMP demonstrated hypersensitivity on the right, opposite of what would be expected in either MD or migraines.3,4 However, ECOG demonstrated EH, suggestive of MD.5 Recently, MRI with TT Gad has been shown to be safe and effective at assessing for EH.6,7 Furthermore, there is limited evidence to suggest it may be more effective than ECOG at identifying EH.8,9 In this case, MRI with TT Gad did not demonstrate EH when ECOG did. The MRI findings allowed the patient to be diagnosed correctly and treated appropriate. The success of the migraine treatment further confirms that this patient suffered from Vertiginous Migraines and not MD.

Discussion

In a patient who’s clinical picture lead to several different diagnoses, and who’s test results did not support a single diagnosis, MRI with TT Gad was able to rule out Meniere’s disease as the cause of his symptoms. This prevented unnecessary trial and error medical treatment and a delay in diagnosis. Furthermore, this case demonstrated the relative feasibility of the MRI with TT Gad, potentially paving the way for it to be used in other disease models.

Conclusions

Test Details Right Ear Left Ear

VNG Warm Caloric 53 degree/sec 6 Degree/sec

Cold Caloric 9 Degree/sec 3 Degree/sec

Summary 75% Weakness on Left

cVEMP Tone Burst Threshold

60dBnHl 90dBnHL

Summary Hypersensitivity on Right (Normal 75-100nHL)

ECOG SP/AP Ratio 0.77 0.38

Summary Consistent with EH on right (Normal <0.5)

HPI: The patient is a 43 yo male who presented with episodic vertigo for 3-4 years with sound hypersensitivity. - Attacks were preceded by Right side aural fullness, pressure

sensation, tinnitus, and occasionally headaches - Episodes lasted over 1 hour but less than 4 hours - He denied hearing loss or autophony - Increased frequency of attacks with higher salt intake PMHx: Sinusitis, GERD, Anxiety, Migraines Physical Exam: - Otoscopic exam benign, TMs mobile - Cranial Nerves Grossly intact - Hennebert’s and Tullio’s negative - Negative Head thrust, Dix Hallpike and Fukuda tests - Non-specific imbalance during Romberg

MRI with TT Gad

Figure 1. Initial Audiogram

Table 1. Selected results from balance function testing.

- 1 week after the MRI, the patient denied any major effects from the TT-Gad. He denied otalgia, otorrhea, tinnitus or vertigo.

- 2 months after MRI, patient had seen a neurologist and was being treat for with valproate for the migraines, no vertigo

- 5 months after MRI, headaches 90% improved, no vertigo - 8 months after MRI, no vertigo, Audiogram normal (Figure 3)

Post Scan Follow up

Figure 2. MRI with TT Gad

Figure 3. Audiogram 8 Months post injection