Overview of Peripheral Vestibulopathies

Educational Objectives

The goal of this program is to improve the management of peripheral vestibulopathies. After hearing and assimilating this program, the clinician will be better able to:

1. Diagnose benign paroxysmal positional vertigo and Meniere disease.
2. Develop treatment strategies for peripheral vestibulopathies.

Summary

Introduction: vestibular testing helps to differentiate between central and peripheral etiologies; the timing and duration of symptoms is crucial in diagnosis; nystagmus is difficult to perceive and can be diagnosed with videonystagmography (VNG) quantitatively

Differential diagnosis based on timing of symptoms

Brief excitation of the labyrinth (sec to min): consider benign paroxysmal positional vertigo (BPPV), superior canal dehiscence syndrome (SCDS), hyperventilation-induced vertigo, and perilymphatic fistula (PLF)

Symptoms from minutes to hours: consider Meniere disease (MD), vertebrobasilar ischemia, and PLF

Symptoms for >24 hr: consider vestibular neuronitis (VN; acute unilateral vestibulopathy), migraines, labyrinthitis, and vestibular schwannoma (VS); in VS, brief episodes of dizziness occur for several weeks followed by stabilization; physical therapy (PT) is beneficial

Rare peripheral vestibulopathies: causes include stroke, trauma, infectious issues, and multiple sclerosis

Chronic bilateral vestibular loss (CBVL): is common in patients with neurofibromatosis type 2; patients undergo multiple PT sessions without progress; familial and autoimmune issues, chemotherapy, radiation, and toxins may also cause CBVL

Benign paroxysmal positional vertigo: in BPPV, otoconia or calcium carbonate crystals in each semicircular canal get dislodged; turning the head sends an abnormal signal to the brain and leads to dizziness; it is thought to be canalithiasis and managed with stone repositioning (but this may not always be the case)

Causes: include head injury, history of VN, prolonged supine positioning, and migraines; the canals are filled with a viscous fluid that causes the stones to get lodged in the canals; the maneuvers provide temporary relief, but the symptoms may persist and not improve (cupulolithiasis); multiple stones can lodge and block the entire canal leading to “canal jam”; these require multiple new maneuvers repeatedly; it was historically managed with canal obliteration (associated with ≈5% risk for permanent hearing loss); at present, it is managed similar to an atypical migraine; persistent PT is beneficial

Crystal repositioning maneuvers: the Epley maneuver and the Semont maneuver are used to treat posterior canal BPPV; the modified Semont maneuver is beneficial for patients with cupulolithiasis; Brandt-Darof exercises are the first-line options for individuals with cervical instability or neck issues; other maneuvers — include Lempert, Gufoni, or Casani maneuvers for the lateral canal BPPV, and Yacovino, a modified maneuver for the superior canal BPPV; PT is beneficial for patients with multi-canal problems and those who do not improve with home maneuvers

BPPV diagnostic tests: include Dix-Hallpike maneuver (for the posterior canal BPPV; most common test), supine head roll test (for the lateral or horizontal canal BPPV), Epley maneuver (for the posterior canal BPPV), and barbecue or log roll maneuver (for the lateral canal BPPV); children with BPPV are likely to develop migraines later in life

Meniere disease: characterized by multiple episodes of vertigo associated with low- to mid-frequency sensorineural hearing loss with fluctuating ear symptoms, eg, tinnitus and fullness; it is attributed to the accumulation of the viscous fluid in the cochlear duct, and it usually involves one ear; the endolymphatic sac is swollen in MD

Diagnosis: Kakigi et al (2008) found that endolymphatic hydrops can be visualized using magnetic resonance imaging (MRI) with gadolinium injected into the inner ear in patients with MD; no clear correlation exists between the presence of endolymphatic hydrops (in MRI and histopathology) and MD symptoms; MD is associated with several comorbidities, ie, brain disorders, migraine, anxiety, depression, autoimmune disorders, rheumatoid arthritis, and psoriasis; 15% of patients with MD may have an allergy etiology; family aggregation has been found in 6% to 9%; patients with MD in one ear are at risk of developing it in the other ear as well

Treatment: there are no US Food and Drug Administration (FDA) approved diagnostic tests or treatments for MD; electrocochleography is used for diagnosis but has limited diagnostic value; a low-salt diet, diuretics, eg, triamterene plus hydrochlorothiazide (Dyazide), betahistine, intratympanic steroid injections, and endolymphatic sac decompression are used to treat MD; clipping the endolymphatic duct increases pressure and is effective for controlling symptoms (Saliba et al [2015]); intratympanic gentamicin and labyrinthectomy can also be used; aggressive options, eg, vestibular nerve sections, are becoming obsolete

MD vs migraine: ≈50% of patients with MD have a history of migraines; vestibular testing cannot differentiate between MD and migraines; the lack of unilateral low-frequency hearing loss with episodic vertigo for >1 yr favors the diagnosis of vestibular migraine; patients with definite MD who fail conventional medical therapy respond to verapamil and nortriptyline (first line options for migraines)

Diagnostic criteria: MD — individuals presenting with symptoms lasting 20 min to 12 hr (definite) or ≤24 hr (probable) in addition to unilateral hearing loss (low frequency), tinnitus, and fullness in the affected ear can be diagnosed with definite or probable MD; vestibular migraine — vertigo is generally 24 hr (similar to MD); it is also episodic and usually associated with sensitivity to light or sound; not every episode has to have a migraine

Vestibular neuronitis: is inflammation of one of the balance nerves without hearing loss; it can also affect hearing (labyrinthitis); it is caused by the herpes simplex virus type 1; vertigo lasts for hours to days and improves slowly and steadily; recurring episodes after symptom improvement rules out VN; asking the patient which direction the world is spinning, and the slow phase eye movement can help identify the affected side; steroids may speed up recovery and reduce inflammation; vestibular rehabilitation therapy (VRT) can accelerate the process

Superior semicircular canal dehiscence syndrome: an abnormal opening occurs in the bone overlying the superior semicircular canal; this creates a mobile window within the labyrinth; the patient may hear their eyeballs move, hear themselves eat, hear their footfalls on the floor, or hear their bones in their neck move (bone conduction hyperacusis); pulsatile tinnitus, dizziness, low-frequency hearing loss, vertigo, and chronic disequilibrium occur; the vestibular evoked myogenic potential (VEMP) test can help identify patients most affected by the dizziness; individuals with SCDS may be symptomatic or asymptomatic

Treatment: mild symptoms are managed conservatively by avoiding changes to pressure and observation; one can obliterate, plug, or resurface it; SCDS is often treated as an atypical migraine to avoid surgery; acetazolamide (Diamox) can be used to decrease the intracranial pressure; middle fossa craniotomy can also be performed

Bilateral vestibular hypofunction (BVH): is characterized by bilateral chronic vestibular loss; it causes unsteadiness when walking or standing and worsens in darkness (but no symptoms when sitting or lying down); it can be quantified with laboratory testing; treatment is challenging; amiodarone increases risk for BVH; vestibular therapy can be initiated with caution; vestibular implants are effective (Chow et al [2021])

Vestibular paroxysmia: MRI may show anterior inferior cerebellar artery loop resting on the vestibular nerve; vestibular “drop attacks” may occur

Diagnostic testing: VNG is the most common test; caloric testing, positional testing, and ocular testing assess the lateral canal and not the entire system; video head impulse testing enables assessing all 3 semicircular canals; the VEMP test (ocular and cervical) helps to evaluate the saccule and the utricle; sinusoidal harmonic acceleration test in a vestibular rotary chair helps to assess the vestibular ocular reflex; platform posturography is used in some rehabilitation centers; electrocochleography can be used for MD diagnosis and cochlear implants

Final points: meclizine can be used in emergencies but it does not enable the peripheral system to compensate; for symptoms that last >24 hr, one should consider migraines and central causes, eg, persistent postural perceptual dizziness, in the differential diagnosis

Readings

Basura GJ, Adams ME, Monfared A, et al. Clinical practice guideline: Ménière’s disease. Otolaryngol Head Neck Surg. 2020;162(2_suppl):S1-S55. doi:10.1177/0194599820909438; Chow MR, Ayiotis AI, Schoo DP, et al. Posture, Gait, Quality of life, and hearing with a vestibular implant. N Engl J Med. 2021;384(6):521-532. doi:10.1056/NEJMoa2020457; Fife TD, Colebatch JG, Kerber KA, et al. Practice guideline: Cervical and ocular vestibular evoked myogenic potential testing: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology. Neurology. 2017;89(22):2288-2296. doi:10.1212/WNL.0000000000004690; Huang HH, Chen CC, Lee HH, et al. Efficacy of vestibular rehabilitation in vestibular neuritis: A systematic review and meta-analysis. Am J Phys Med Rehabil. 2024;103(1):38-46. doi:10.1097/PHM.0000000000002301; Kakigi A, Nishimura M, Takeda T, et al. Effects of gadolinium injected into the middle ear on the stria vascularis. Acta Otolaryngol. 2008;128(8):841-845. doi:10.1080/00016480701769776; Saliba I, Gabra N, Alzahrani M, et al. Endolymphatic duct blockage: A randomized controlled trial of a novel surgical technique for Ménière’s disease treatment. Otolaryngol Head Neck Surg. 2015;152(1):122-129. doi:10.1177/0194599814555840; Schenck AA, Kruyt JM, van Benthem PP, et al. Effectiveness of endolymphatic duct blockage versus endolymphatic sac decompression in patients with intractable Ménière’s disease: Study protocol for a double-blinded, randomised controlled trial. BMJ Open. 2021;11(8):e054514. Published 2021 Aug 10. doi:10.1136/bmjopen-2021-054514; Strupp M, Mandalà M, López-Escámez JA. Peripheral vestibular disorders: An update. Curr Opin Neurol. 2019;32(1):165-173. doi:10.1097/WCO.0000000000000649; Traboulsi H, Teixido M. Qualitative analysis of the Dix-Hallpike maneuver in multi-canal BPPV using a biomechanical model: Introduction of an expanded Dix-Hallpike maneuver for enhanced diagnosis of multi-canal BPPV. World J Otorhinolaryngol Head Neck Surg. 2017;3(3):163-168. Published 2017 Jun 8. doi:10.1016/j.wjorl.2017.01.005.

Disclosures

For this program, members of the faculty and planning committee reported nothing relevant to disclose.

Acknowledgements

Dr. Hunter was recorded at the 7th Annual New Jersey Neurovascular and Neurosciences Symposium, held November 14, 2024, in Mount Laurel, NJ, and presented by Thomas Jefferson University. For information on upcoming CME activities from this presenter, please visit Jefferson.edu. Audio Digest thanks the speakers and presenters for their cooperation in the production of this program.

CME/CE INFO

Accreditation:

The Audio- Digest Foundation is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.

The Audio- Digest Foundation designates this enduring material for a maximum of 1.00 AMA PRA Category 1 Credits™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

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Lecture ID:

NE160402

Expiration:

This CME course qualifies for AMA PRA Category 1 Credits™ for 3 years from the date of publication.

Instructions:

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Estimated time to complete this CME/CE course:

Approximately 2x the length of the recorded lecture to account for time spent studying accompanying learning materials and completing tests.

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