OUT OF COMMITTEE: Equilibrium | Persistent Postural-Perceptual Dizziness (PPPD): More Than a Diagnosis of Exclusion
PPPD should be suspected in patients describing non-spinning vertigo or unsteadiness exacerbated by prolonged or repeated high-velocity movements.
Adam C. Thompson-Harvey, MD, and Erika McCarty Walsh, MD
Managing patients with chronic dizziness can be frustrating for clinicians, especially when the diagnosis is unclear. Whether you encounter these patients in a comprehensive otolaryngology clinic, on a call from the emergency department for consultation, or during resident clinic, the following case is not uncommon:1
A 53-year-old female presents to your outpatient clinic with dizziness. She previously has been evaluated by her primary care provider, an otolaryngologist, and a neurologist without a clear diagnosis. Her symptoms started about 18 months ago after she woke up one morning and experienced a brief, intense sensation of “room spinning” dizziness. She noticed this sensation whenever she would get up in the morning or lay down at night to sleep. Over time, her symptoms changed. She no longer has “spinning” dizziness but now feels constantly dizzy every day. She reports feeling unstable and swaying as if she is on a boat. When she walks, she feels like she is “walking on marshmallows.” She has not had any falls but is fearful of falling. Any movement worsens her dizziness, and she feels best when sitting quietly. She describes good days and bad days but cannot identify any pattern. She was sent for vestibular rehabilitation but stopped therapy sessions after six weeks because the sessions exacerbated her symptoms. Audiogram reveals mild bilateral high-frequency sensorineural hearing loss with preserved word discrimination. Prior videonystagmography showed normal ocular motor function and symmetric caloric function. She has no significant medical problems and denies a history of migraine. She now avoids social activity, travel, and daily tasks because of her debilitating symptoms.
Based on this patient’s ongoing symptoms and course of workup, she would meet the diagnostic criteria for persistent postural-perceptual dizziness (PPPD, or 3PD). In 2017 the Committee for the Classification of Vestibular Disorders of the Bárány Society published the diagnostic criteria for PPPD. Outlined as a specific chronic vestibular disorder, PPPD diagnostic criteria are met if the patient has:
- Non-spinning dizziness or unsteadiness at least 50% of the time for three months
- Worse when upright, in motion, and with visual stimulation
- Symptoms precipitated by conditions causing acute, episodic, or chronic vestibular syndromes, other neurologic or medical illnesses, or psychological distress
- Examples include benign paroxysmal positional vertigo (BPPV), vestibular neuritis, vestibular migraine, traumatic brain injury, panic attacks, or anxiety
- Not better accounted for by another disease or disorder2
It should be noted that evidence of another active illness or vestibular disorder does not necessarily exclude a diagnosis of PPPD, so clinical judgment must be exercised to determine the best attribution of the patient’s vestibular symptoms to all identified illnesses. Staab et al. emphasized that PPPD is not a “diagnosis of exclusion” and should not be diagnosed in patients with only nonspecific chronic vestibular symptoms.2 Further, abnormal findings on physical examination or laboratory testing do not exclude a diagnosis of PPPD but may indicate a precipitating condition or comorbid condition. Often, more history is needed to establish the diagnosis. Thus, clinicians should expect to observe patients over time and properly screen for other diseases until the diagnostic picture is clear.3 Although patients with audio-vestibular complaints are often seen by other providers (e.g., neurology, psychiatry), otolaryngologists should be prepared to diagnose PPPD if the criteria above are met.4
PPPD should be suspected in patients describing non-spinning vertigo or unsteadiness exacerbated by prolonged or repeated high-velocity movements.5 Most patients feel best when still, but low-level motion (e.g., walking or riding a bicycle) may also be preferred over an upright, stationary position. After cessation of triggers, symptoms usually do not return to baseline and may last for hours. PPPD typically develops after acute symptoms of precipitating conditions remit, with symptoms settling into a chronic course. Difficulty with full field visual flow, including observing high-speed traffic passing on a highway or large crowds of milling people, is typical. Symptoms may be exacerbated by scrolling on a computer screen or smartphone due to difficulty with precise visual focus. Self-report questionnaires (e.g., Generalized Anxiety Disorders Scale) can help detect psychiatric morbidity in patients who cope with PPPD in maladaptive ways or have underlying anxiety and depressive disorders.6
As there are no validated questionnaires, findings on physical examination, laboratory testing, or diagnostic imaging that are pathognomonic of PPPD, overlapping diagnostic entities can coexist and confuse the clinical picture. Sarna et al. reported nearly half of their subjects with PPPD had migraine, and 17% met diagnostic criteria for definite vestibular migraine.7 Nonetheless, PPPD may be preceded by episodic vestibular disorders such as Ménière disease, vestibular migraine, and BPPV that cause distinct bouts of vestibular symptoms.8,9 This diagnostic dilemma is resolved by carefully examining the clinical history and assessing patients’ vestibular compensation status. Persistent non-vertiginous dizziness and unsteadiness provoked by upright posture, patients’ movements, and exposure to visual motion stimuli plus physical exam and laboratory evidence of reasonable compensation (e.g., no spontaneous nystagmus or abnormal responses to head thrust, headshake, or stepping tests) indicate that PPPD is the only active diagnosis.
No standardized approach to treating PPPD currently exists, and only a minority of patients may experience spontaneous resolution of symptoms.10 Studies support a multidisciplinary approach to 3PD, with the use of cognitive behavioral therapy (CBT), vestibular rehabilitation therapy, selective serotonin uptake inhibitors (SSRIs), and/or serotonin-norepinephrine reuptake inhibitors (SNRIs) having a role in PPPD management.11-14 Vestibular rehabilitation combined with CBT, and possibly supported by medication, can help patients escape a cycle of maladaptive balance control, recalibrate vestibular systems, and regain independence in everyday life.15-17
In summary, clinicians diagnosing PPDD should be aware that an unidentified underlying medical condition is usually driving the diagnosis. Until all diagnostic criteria are met, clinicians should remain open-minded when considering the diagnosis, and, when met, PPPD serves as a framework to develop an optimal treatment solution.
- Staab JP, Ruckenstein MJ. Expanding the differential diagnosis of chronic dizziness. Arch Otolaryngol Head Neck Surg. 2007;133:170-176.
- Staab JP, Eckhardt-Henn A, Horii A, et al. Diagnostic criteria for persistent postural-perceptual dizziness (PPPD): Consensus document of the Committee for the Classification of Vestibular Disorders of the Bárány Society. J Vestib Res. 2017;27:191-208.
- Powell G, Derry-Sumner H, Rajenderkumar D, Rushton SK, Sumner P. Persistent postural perceptual dizziness is on a spectrum in the general population. Neurology. 2020;94:e1929-e1938.
- Zachou A, Anagnostou E. Diagnostic value of key signs in persistent postural-perceptual dizziness. J Neurol. 2020;267:1846-1848.
- Kabaya K, Tamai H, Okajima A, et al. Presence of exacerbating factors of persistent perceptual-postural dizziness in patients with vestibular symptoms at initial presentation. Laryngoscope Investig Otolaryngol. 2022;7:499-505.
- Azzi JL, Khoury M, Séguin J, et al. Characteristics of persistent postural perceptual dizziness patients in a multidisciplinary dizziness clinic. J Vestib Res. 2022;32:285-293.
- Sarna B, Risbud A, Lee A, Muhonen E, Abouzari M, Djalilian HR. Migraine features in patients with persistent postural-perceptual dizziness. Ann Otol Rhinol Laryngol. 2021;130:1326-1331.
- Neff BA, Staab JP, Eggers SD, et al. Auditory and vestibular symptoms and chronic subjective dizziness in patients with Ménière’s disease, vestibular migraine, and Ménière’s disease with concomitant vestibular migraine. Otol Neurotol. 2012;33:1235-1244.
- Tropiano P, Lacerenza LM, Agostini G, Barboni A, Faralli M. Persistent postural perceptual dizziness following paroxysmal positional vertigo in migraine. Acta Otorhinolaryngol Ital. 2021;41:263-269.
- Huppert D, Strupp M, Rettinger N, Hecht J, Brandt T. Phobic postural vertigo—a long-term follow-up (5 to 15 years) of 106 patients. J Neurol. 2005;252:564-569.
- Trinidade A, Goebel JA. Persistent postural-perceptual dizziness—a systematic review of the literature for the balance specialist. Otol Neurotol. 2018;39:1291-1303.
- Axer H, Finn S, Wassermann A, Guntinas-Lichius O, Klingner CM, Witte OW. Multimodal treatment of persistent postural-perceptual dizziness. Brain Behav. 2020;10:e01864.
- Min S, Kim JS, Park HY. Predictors of treatment response to pharmacotherapy in patients with persistent postural-perceptual dizziness. J Neurol. 2021;268:2523-2532.
- Waterston J, Chen L, Mahony K, Gencarelli J, Stuart G. Persistent postural-perceptual dizziness: precipitating conditions, co-morbidities and treatment with cognitive behavioral therapy. Front Neurol. 2021;12:795516.
- Herdman D, Norton S, Murdin L, Frost K, Pavlou M, Moss-Morris R. The INVEST trial: a randomised feasibility trial of psychologically informed vestibular rehabilitation versus current gold standard physiotherapy for people with persistent postural perceptual dizziness. J Neurol. 2022:1-11.
- Popkirov S, Stone J, Holle-Lee D. Treatment of persistent postural-perceptual dizziness (PPPD) and related disorders. Curr Treat Options Neurol. 2018;20:50.
- Yu YC, Xue H, Zhang YX, Zhou J. Cognitive behavior therapy as augmentation for sertraline in treating patients with persistent postural-perceptual dizziness. Biomed Res Int. 2018;2018:8518631.