Audio-Vestibular Findings in Individuals with Takayasu’s Arteritis

Abstract

Takayasu’s Arteritis is defined as inflammatory disease of the large blood vessels with unknown cause mostly affecting the aorta and its main branches. The patients diagnosed with Takayasu’s arteritis are reported to have complaint of chronic dizziness. However, literature lacks detailed clinical evaluation of the auditory vestibular function in such patients. The current study documents the findings of auditory and vestibular evaluations in a rare syndrome. It was observed that patients with Takayasu’s arteritis had abnormal auditory and vestibular evoked potentials.

Introduction

Takayasu’s Arteritis is a chronic inflammatory disease of the large blood vessels. It is also described as granulomatous large vessel vasculitis. It is reported to mainly affect the large blood vessels like the aorta and its pulmonary and coronary branches [1]. The etiology of the syndrome is unknown; however, studies suggest the presence of an autoimmune reaction [2]. Takayasu’s arteritis is found to be more common among the Asian population and was first discovered in Japan [3]. The prevalence in India is not known, however, it was found to be 2.2 in Kuwait and 0.9 per million individuals in the United States [4]. The epidemiological studies identified that the prevalence of the thoracic aortal involvement in females and abdominal aortal branches involvement in males [5, 6]. The individuals with Takayasu’s arteritis (IWTA) are reported to mainly present with the complaint of giddiness or general dizziness, difficulty in vision, palpitation, numbness in the extremities, and hypertension [7, 8]. The symptom of dizziness was reported to be present. However, there are no reports of clinical vestibular evaluation.

Zhou, Ni [9] reported that dizziness is one of the most common complaints given by 74% of the IWTA. They made a consecutive sampling of 63 patients in the retrospective study. The other clinical manifestations of Takayasu’s arteritis were reported to be a visual abnormality, stroke, headache, and seizures. Recently, Bond, Nasr [10] also found similar symptoms with dizziness as the second most commonly reported after headache. This can be because of the selection criteria set by the authors to include patients with intracranial abnormalities with Takayasu’s arteritis. There is a dearth of literature about the auditory and vestibular skills in IWTS complaining of chronic giddiness.

The current case series will provide information about the ability to maintain body balance in IWTA, as it is found to be an outcome of good coordination between vision, vestibular system, and proprioceptive system [11]. The present case series aims to document the auditory and vestibular profile of the IWTA.

Case Report

Case A, 33 years old male reported spinning sensation lasting for 10 min, headache, blurring of vision and was referred to Audiology and Speech therapy department for detailed auditory and vestibular evaluation. He presented with a complaint of vertigo, six to seven episodes in a day after a sudden change in body position, predominantly after getting up from sitting posture. This was accompanied by nausea or vomiting. The client was medically diagnosed with Takayasu’s arteritis since 19 years of age and had undergone renal angioplasty at 20 years of age. The 2D Echocardiography test (2D Echo) revealed hypertensive heart disease and Type II diastolic function with 60% left ventricular ejection fraction (LVEF). The MRI brain showed severe stenosis of the left vertebral artery. Additionally, a 3D Aortic angiogram (Fig. 1) revealed the presence of stenosis in the origin of the superior mesenteric artery. On auditory evaluation, the client was found to have normal hearing sensitivity with normal middle ear functioning in both ears. He had abnormal morphology of the auditory evoked brainstem potential at higher presentation rates, done using click stimulus; absent distortion product otoacoustic emission (DP OAE) in both ears. The client was taken up for vestibular evaluation comprising behavioral and electrophysiological testing. The client was having significant balancing issues on the Rhomberg test, however, the findings of the clinical head impulse test, skewed deviation, smooth pursuit, positional and positioning maneuver were within normal limits. On an electrophysiological test like cervical vestibular evoked myogenic potential (cVEMP) the inter-aural amplitude was asymmetrical. However, the asymmetry was < 30% between the ears (Table 1). The cVEMP was recorded using a tone burst of 500 Hz. The ocular evoked myogenic potential (oVEMP) was present in both ears with an asymmetry of 18% between ears. The evoked potential was recorded using the Intelligent Hearing Systems (IHS) Duet system, with standard protocols.

Fig. 1.

a T2 Axial image showing severe stenosis of origin of Superior Mesenteric Artery trunk and b 3D Aortic angiogram showing the same (circle), respectively, in case A

Table 1.

Latencies and Amplitude findings of the physiological testing for Case A and Case B

Test	Test Indices	Case A		Case B
		Right ear	Left ear	Right ear	Left ear
Auditory Brainstem Response (19.3 Hz repetition rate)	Absolute latencies (msec)
	I	1.80	2.00	1.77	2.08
	III	3.83	4.1	3.67	4.00
	V	5.80	6.03	5.55	6.15
	Inter-peak Latencies (msec)
	III-I	2.03	2.10	1.90	1.92
	V-III	1.97	1.93	1.88	2.15
	V-I	4.00	4.03	3.77	4.08
	Relative Amplitude ratio (µV)
	V/I	1.52	3.97	2.43	1.16
cVEMP	Latency (msec)
	P1	15.6	15.00	Absent	Absent
	N1	25.8	24.00
	EMG corrected Amplitude (µV) P1-N1	12.72	8.84	Absent	Absent
	Asymmetry Ratio	17.99%		Absent
oVEMP	Latency (msec)
	N1	12.20	13.60	Absent	Absent
	P1	17.00	18.00
	N2	20.80	23.2
	EMG corrected Amplitude (µV) N1-P1	4.49	3.10	Absent	Absent
	Asymmetry Ratio	18.31%		Absent

EMG corrected Correction done for the ongoing muscle activity while testing, cVEMP Cervical vestibular evoked myogenic potential, and oVEMP Ocular vestibular evoked myogenic potential

Case B, a female 21-year-old had a complaint of inability to maintain balance and walk without support, severe headache, and difficulty in visual acuity for near and far-field objects. She was also diagnosed with Takayasu’s arteritis in 2006. The 2D echo showed the presence of turbulence in descending thoracic aorta with 60% of LVEF. Followed by a 3D aortic angiogram that revealed severe stenosis of the bilateral common carotid artery, bilateral vertebral artery, and bilateral subclavian artery (Fig. 2). On MRI Brain a chronic lacunar infarct in the left thalamocapsular region was observed. Like Case A, even she had normal findings on pure-tone audiometry, immittance audiometry, and additionally, normal morphology of the auditory evoked brainstem potential. The cVEMP was absent on both sides for a 500 Hz tone burst. Along with, absent DP OAE and oVEMP in both ears (Fig. 3). Case B also had remarkable difficulty in a behavioral test like sharpened Rhomberg. She was unable to maintain the tandem stance for even 2 s. She had severe difficulty in vision and hence was unable to perform the cerebellar test (finger-to-nose) she was advised to continue her treatment with the ophthalmologist. Her positional and positioning tests were normal. She had no spontaneous and gaze-evoked nystagmus with normal findings in clinical head impulse test, skewed deviation, and smooth pursuit. The absence of cVEMP and oVEMP was also observed. Both patients are presently enrolled in vestibular rehabilitation therapy and are on annual audiological follow-up.

Fig. 2.

3D Aortic angiogram shows long segment narrowing of thoracic Aorta and infra renal aorta (circles), respectively, in case B

Fig. 3.

a ABR findings of case A; b ABR findings of case B; c oVEMP findings of case A; d oVEMP findings of case B; e cVEMP findings from case A, done with tone burst 500 Hz; and f cVEMP findings from case B, done only with 500 Hz tone burst (patient-related limitations), respectively

Note: All the procedures were non-invasive and harmless. The testing procedures also complied with the declaration of Helsinki.

Discussion

The presence of normal hearing on the pure tone audiogram with absent OAEs in both cases reveals outer hair cell dysfunction. Also, abnormal ABR morphology at higher presentation rates (72 Hz repetition rate). This can be inferred to be the outcome of reduced blood supply due to stenosis of the branches of the aorta supplying the inner ear end organs.

The presence of vertigo in Case A and unsteadiness in case B can be attributed to the vertebrobasilar insufficiency caused by the stenosis of the anterior inferior cerebellar artery. As the stenosis of the anterior inferior cerebellar artery (AICA) is commonly seen in IWTA could cause the reduced blood supply to the vestibular end organs. In case A MR angiogram has also revealed an inability to visualize left AICA, this was observed as relatively reduced amplitudes in VEMP for the left ear.

The absence of cVEMP and oVEMP in case B can be attributed to the severe reduction in blood supply to the saccule and the utricle. This suggest pathology of the sacculo-collic pathways and oculo-utricular pathway dysfunction, respectively. Depending upon the place and the severity of the stenosis the outcome can vary from person to person. This was inferred from the absence of cVEMP in case B. More studies are required to report the findings in other IWTA.

Conclusion

The study highlights the importance of a detailed evaluation of a complaint of vertigo or dizziness in IWTA. The pathophysiology of the arteritis may vary depending upon the involved branch of the aorta and hence the evaluation will help in understanding the severity of the condition. This will add to the holistic management of IWTA. Additionally, more case–control studies are required to document any predictable deficits seen in IWTA.

Funding

No fund was obtained.

Declarations

Conflict of interest

The Authors declare that they do not have any conflict of interest.

Consent for Publication

Written consent was obtained from both cases.