Beyond Vertigo- Perceptual Postural Phobic Dizziness (PPPD): Our experience

Abstract

Notwithstanding current understanding of vertigo, there are various clinical scenarios which are intriguing for clinicians, where patients have been too symptomatic but the presentation does not fit into any diagnosis. We stumbled upon a new entity during literature search known as Persistent Postural Perceptual Dizziness (PPPD). It fills the lacuna where we are often left wanting for diagnosis in the existing pool of knowledge. This case series has been prepared keeping in view the lack of data regarding PPPD in Indian population. For better understanding we present the illustration of our patients in this case series. We presented the details of three patients who were diagnosed as PPPD and managed effectively and followed up for one year. The nomenclature portrays the core concept of dizziness. The diagnostic criteria clearly define PPPD. It should not be used as escape or exclusion diagnosis. Our case series highlights various presentation of, not so uncommon, PPPD in Indian population. The case series has been brought out to address the deficiency of knowledge in dealing with intriguing vertigo. Careful thorough history is important to reach a diagnosis and avoids unwarranted vestibular sedatives. It highlights that proper counselling and vestibular rehabilitation can help the patients overcome their chronic disability.

Introduction

Vertigo is one of the common presentation and dizziness or giddiness are common terms used to explain symptoms in Otolaryngology clinic. It is functionally incapacitating for majority with significant limitation of daily activities. The persistent perceived unsteadiness and fear of fall triggers a stiff posture known as “high-risk postural control”. The patient’s orientation adapts to visual cues and somatosensory inputs known as “visual-somatosensory dependence” rather than vestibular stimulation [1]. The underlying anxiety or depressive tendency or apprehension of falling which perpetuates these physiological effects are under-reported [2, 3].

Notwithstanding current understanding of vertigo, there are various clinical scenarios which are intriguing for clinicians, where patients have been too symptomatic but the presentation does not fit into any diagnosis. We also faced such thought-provoking presentations which forced us to foray into literature search for plausible explanation. We stumbled upon a new entity during literature search known as Persistent postural perceptual dizziness (PPPD), which would be discussed subsequently. The presentation of our patients fits very well into this newly diagnosed syndrome. It fills the lacuna where we are often left wanting for diagnosis in the existing pool of knowledge. For better understanding we present the illustration of our patients in this case series. This case series has been prepared keeping in view the lack of data regarding PPPD in Indian population.

Case 1

A 24 year old female patient, health professional, presented with complaints of three incidences of vertigo. She suffered first episode in 2016 after swing ride at an adventure park when she developed dizziness and unsteadiness. There was no feeling of rotatory sensation or spinning vertigo of either self or surrounding. There was no hearing loss, nausea, vomiting or aural fullness. The symptoms persisted for couple of days. The management was conservative with initial bed rest and tab cinnarizine for approx. 45 days. The symptoms started resolving after initial one and half months. The fear of imbalance and fall gripped her which affected daily and professional activities. The symptoms resolved gradually over next two months.

In 2018 there was second similar incidence of dizziness and unsteadiness just before professional exam with no other inciting event reported. She did not develop any other complaints. After consultation of Otolaryngologist she was labelled as Meniere’s disease without any nystagmus or hearing loss and started on tab Betahistine for one month. She became asymptomatic after three to four months.

The third episode was in September 2020 when symptoms recurred, when while working on a revolving chair she was rotated vigorously by a colleague and presented to our center. She complained of persistent feeling of unsteadiness and non-rotatory vertigo, lightheadedness and wobbling sensation. The symptoms got aggravated by sitting upright from lying position or walking, with difficulty in crossing road amidst moving traffic or moving up or down the stairs at fast pace. The patient did not have nausea or vomiting, hearing loss, otalgia, otorrhoea, tinnitus or aural fullness. She had history of waxing and waning of symptoms throughout the day. There is no history of ear or neurological disease, ear surgery, head injury or exposure to ototoxic drugs.

The general and ear examination was normal. The clinical hearing tests did not reveal any hearing loss. On otoneurological examination there was no spontaneous or gaze evoked nystagmus. The Head impulse test (HIT), positional test (Dix Hallpike test and supine roll test) and Unterberger stepping gait test were normal. Romberg’s test and cerebellar signs were negative. There was no sensory-motor deficit and gait was normal.

Pure tone audiometry (PTA) showed normal hearing thresholds. Impedance audiometry showed ‘A’ type curve and normal acoustic reflex bilaterally. High Resolution Computed Tomography (HRCT) of temporal bone (no. 0769/2020, dated 25/09/2020) ruled out third window in labyrinth or perilymphatic fistula. Contrast enhanced magnetic resonance imaging (CE-MRI), angiography & venography (MRA & MRV) was done (no. 182049266/68) which ruled out any intracranial space occupying lesion or vascular cause.

The patient was diagnosed as PPPD. She was counselled about the nature of disease and treatment being mainly vestibular rehabilitation and medication as last resort. However, she was started on tab cinnarizine 25 mg once at night for seven days to overcome the initial discomfort of symptoms. She was advised vestibular rehabilitation initially with brisk walks on even ground followed by long walks and finally moving up and down the stairs. She responded well to the habituation and desensitization exercises. At the end of four to six weeks she responded well and had significant improvement. She could perform all daily activities and professional work as before without fear of precipitating dizziness.

She was kept on follow up every two weeks for one year. She is presently asymptomatic.

We had similar 2 other cases who reported in our OPD over the last one year. The demographics, clinical presentation and management of case 2 and 3 is as mentioned in Table 1.

Table 1.

Case 2 and 3 diagnosed as PPPD

	Case no 2	Case no 3
Age & Sex	47 years old female	52 years old female
Profession	–	Clerk in government office
Clinical presentation	Diagnosed case of Recurrent BPPV. 3 episodes in 6 months. Each episode managed with Canalolith repositioning manueouvres and vestibular sedatives. She reported to our centre post BPPV for persistent sensation of unsteadiness for last 3 months. The symptoms eased while sitting and worsened while walking. She was symptomatic for most days with few asymptomatic days in between.	Unsteadiness while walking (on road, park or malls) almost on a daily basis for past 12 years. She was distressed by her symptoms. No other ENT complaints. The detailed history revealed that she had suffered from vestibular neuronitis 12 years back which was managed by vestibular sedatives for a month.
Ear examination	Normal	Normal
Otoneurological examination	No abnormality detected	No abnormality detected
Audiological evaluation	Normal	Normal
Vestibular evaluation	No abnormality detected	cervical Vestibular Evoked Myogenic Potential (cVEMP), Vestibular Neuronography (VNG) and Elelctrocochleography (ECochG) were normal
Imaging: CE-MRI Brain & Inner ear	Normal	Normal
Management	Counselling, Vestibular rehabilitation exercise	Counselling, Vestibular rehabilitation exercise
Outcomes	Symptoms resolved over next 3 months and now she is asymptomatic for last 6 months.	Symptoms resolved over 6 months. Presently she is asymptomatic.

Discussion

PPPD is a relatively newer diagnostic addition. It was first defined in 2017 and was subsequently recognized by Barany Society and was added to International classification of vestibular disorders (ICVD) and International classification of diseases (ICD-11) [4, 5]. The nomenclature portrays its core criteria of persistent dizziness which is non-spinning type of vertigo exacerbated by postural stimulation and perceptual response to space motion stimuli [4]. The epidemiological studies from tertiary centers reflect that approx. 25% of patients with vestibular symptoms could receive no diagnosis [6]. The recent studies have concluded that with introduction of PPPD, the number of undiagnosed cases have reduced to less than 2% [7]. Thus, proving unarguably that the diagnosis has addressed many undiagnosed cases and served to fill significant gap in clinical neurotologic practice.

The epidemiology of PPPD per se is unavailable as specific studies have not been conducted [4]. The estimated prevalence of PPPD, based on the epidemiological data, has been shown to be 15–20% [8, 9]. Similarly, the incidence of PPPD, obtained from the studies with prospective follow up of patients with acute or episodic or chronic vestibular disorders with PPPD like symptoms, can be found in approximately 25% of patients after 3–12 months of follow up. The average duration of PPPD presentation to ENT specialist has been four and half years [9]. The duration of presentation in our patients ranged from 04 months to 12 years.

The age distribution has been observed from adolescence to late adulthood [9, 10]. The age of presentation in our case series ranged from 24 to 52 years. A predominance of female gender has been observed in the case reports of PPPD [10]. In our case series all three patients are females.

It has strict diagnostic criteria laid down for diagnosis as depicted in Table 2 [4].

Table 2.

Diagnostic criteria for PPPD

a) One or more symptoms of dizziness, unsteadiness or non-spinning vertigo which are present on most days for 3 months or more -Symptoms may last for prolonged (hours long) period of time, but severity may wax and wane -Symptoms need not be present throughout the day

b) Persistent symptoms occur without specific provocation -Exacerbated by three factors: upright posture, active or passive motion without regard to direction or position, and exposure to moving stimuli or complex visual patterns

c) Precipitated by conditions that cause vertigo, unsteadiness, dizziness, or problems with balance including acute, episodic or chronic vestibular syndromes, other neurological or medical illnesses or psychological distress, -When the precipitant is acute or episodic, symptoms settle into pattern of criterion A, but they may occur intermittently at first, and then consolidate into a persistent course -When the precipitant is a chronic syndrome, symptoms may develop slowly at first and worsen gradually

d) Symptoms cause significant distress or functional impairment

e) Symptoms are not better accounted for by another disease or disorder

All the criteria mentioned ibid (a) to (e), should be fulfilled for confirmed diagnosis of PPPD. It is evident from the defining criteria that there are no physical findings, vestibular tests or pathognomonic investigations or imaging findings for its diagnosis. It needs to be stressed that PPPD is neither a diagnosis of exclusion nor it should be used as an escape diagnosis [11]. In doubtful cases, follow up may help to arrive at a diagnosis [4]. All the 3 reported cases met the diagnostic criteria of PPPD.

The detailed history of probable precipitating factors should be obtained which can be vestibular disorders, peripheral or central, being most frequent cause (~ 25–30%), followed by episodes of vestibular migraine (15–20%), panic or anxiety attacks (15% each), concussive brain injuries or whiplash injury (10–15%) and autonomic disorders (7%) being the prominent ones. Others factors being drugs, cardiac events, etc. (accounting cumulatively to ~ 3%) [9, 12]. In our patients the precipitating factors were clearly evident. The first patient had suffered recurrent vertiginous episodes over past four years. She developed symptoms of PPPD after probable episodes of anxiety in form of swing ride, stress of appearing in exam and vigorous rotation. She never had any symptoms of peripheral vestibular vertigo. The second patient had recurrent episodes of BPPV as precipitating factor. The third patient had developed symptoms of PPPD after an episode of vestibular neuritis. The literature suggests that peripheral vestibular disorder is the most frequent precipitating cause, however, interestingly in our case series the first patient with confirmed diagnosis had stress as etiology and the latter two cases had peripheral vestibular cause.

It is imperative to note that there are no specific investigations to diagnose PPPD. However, the investigations are mandatory to rule out underlying cause. It is highlighted that any positive clinical finding or investigation during management does not exclude PPPD, rather it is indicative of ongoing precipitating condition or co-existing vestibular disorder. In all our patients the audiological, vestibular and radiological investigations were normal.

The treatment strategies for PPPD include counselling, vestibular rehabilitation, cognitive behavioral therapy and medications. The clear communication of diagnosis, pathophysiology and importance of rehabilitation is the foundation of management [13]. The treatment primarily aims to retrain the brain. The rehabilitative measures include wide variety of exercises and starts with basic walking to more complex exercises. The patients will at the outset become aware of dizziness but should be encouraged to remain calm and proceed with efforts [14]. Cognitive behavioral treatment (CBT) starts with patient education also termed as “psychoeducation”. Treatment should be aimed at reducing the handicap rather than just reducing the symptoms [15]. CBT has shown better improvement in patients when compared to exercise alone group [16]. Selective serotonin reuptake inhibitors (SSRI) and Serotonin norepinephrine reuptake inhibitors (SNRI) have been recommended for chronic functional dizziness with or without psychiatry comorbidity. However, evidence level of most of studies is low [17, 18]. The use of transcranial and non-invasive electrical stimulation of prefrontal cortex and vagus nerve are innovative approaches currently in experimental stage [19, 20].

All the three reported patients were managed with counselling and vestibular rehabilitation exercised. The pathophysiology was explained and they were reassured through investigations about no structural abnormalities. They were encouraged to carry on daily activities with caution though advised against driving. Rehabilitation was initiated with short slow walks followed by brisk walks on plain ground and graduated to moving up and down the stairs. Initially the patients were reluctant, as expected, but gradually after few consultations with reassurances they proceeded. The vestibular sedatives were used only in two patients for initial sense of dizziness. The patients were themselves reluctant to use oral medication stating that symptoms were just bearable. They showed significant improvement at the end of approx. 4–5 months and were asymptomatic upon subsequent follow up.

Conclusion

Our case series highlights various presentation of, not so uncommon, PPPD in Indian population. The case series has been brought out to address the deficiency of knowledge in dealing with intriguing vertigo. Careful thorough history is important to reach a diagnosis and avoids unwarranted vestibular sedatives. It highlights that proper counselling and vestibular rehabilitation can help the patients overcome their chronic disability.

Declarations

Conflict of interest

The authors have no relevant financial or non-financial interests to disclose.

Human and Animal Rights

The study is a purely observational study with no intervention on individual participants and no deviation of standard of care. The Institutional Ethics Committee has confirmed that no ethical approval is required.

Informed Consent

Informed consent was obtained from all individual participants included in the study. The personal identity, whatsoever, of patients has not been revealed in any form in the study. No images of patients have been used in the study.