Revisiting “Meniere’s Disease” as “Cervicogenic Endolymphatic Hydrops” and Other Vestibular and Cervicogenic Vertigo as “Spectrum of Same Disease”: A Novel Concept

Abstract

Vertigo and dizziness are one of the commonest and least understood symptom. Vestibular vertigo of Meniere’s disease and Benign Paroxysmal positional vertigo (BPPV) and cervicogenic dizziness are classified as separate entities. Cervicogenic dizziness is not considered the domain of Otolaryngologists, as it is mainly related to neck proprioceptors. Headache and neck pain, have been found to be associated with both Meniere’s disease and BPPV, so is cervicogenic dizziness. The present study was undertaken to study the association between cervical signs and symptoms in patients with Vestibular Vertigo of Meniere's disease, Benign Paroxysmal Positional Vertigo and cervicogenic dizziness. 132 patients complaining of vertigo and diagnosed with Meniere’s disease, BPPV or cervicogenic dizziness were examined for symptoms and signs related to neck, shoulder and muscle tightness and asymmetry. Most of the patients of Meniere’s Disease (80% for unilateral and 88.23% for bilateral), Benign Paroxysmal Positional Vertigo (75%for right sided BPPV, 66.67% for left sided BPPV) and cervicogenic dizziness (90%) had associated symptoms of neck pain or headache, and were found to be positive for neck tightness and/or asymmetry of shoulder. Headache was more common in patients with Meniere’s Disease. Vestibular Dizziness of Meniere’s Disease, Benign Paroxysmal Positional Vertigo and Cervicogenic Dizziness may be spectrum of the same disease with underlying myofascial problems. Meniere’s Disease of Idiopathic or primary type needs to be revisited as Cervicogenic Hydrops.

Introduction

Dizziness and vertigo are one of the commonest symptom in general and otolaryngology practice, attributed to various peripheral (vestibular) and central causes by Otorhinolaryngologists. Cervicogenic dizziness and vertigo as an entity is known only to physiotherapists and not mentioned in the list of vertigo syndromes in Otorhinolaryngology text books [1]. This has resulted in a gap in the current knowledge of the underlying pathophysiology of different vertigo syndromes and unsatisfactory treatment results. Meniere’s Disease and Benign Paroxysmal Positional vertigo (BPPV) are two leading causes of peripheral vestibular vertigo. Recently, it has been observed that there is considerable overlap among various vertigo syndromes, and also dizziness in patients with vertigo [2–4]. Since its original description by Prosper Meniere, Meniere’s Disease is still an enigma [5, 6]. Meniere’s disease is categorized under typical vertigo syndrome, and is a diagnosis of exclusion, said to be “Idiopathic or Primary Endolymphatic Hydrops” [7]. AAO-HNS guidelines had suggested that when there are recurrent attacks of spontaneous vertigo, each lasting from 20 min to a few hours (< 24), it is likely to be Ménière’s disease [8]. The current guidelines by Barany Society in collaboration with AAO-HNS, the Japan Society for Equilibrium Research, the EAONO, and the Korean Balance Society have modified the duration of episode of vertigo from 20 min to < 12 h [9]. This is often accompanied with auditory symptoms, in the form of low-frequency ipsilateral tinnitus and fluctuating hearing loss, preceded most often by a sense of pressure or fullness. Various syndromes like Probable and possible Meniere’s and Meniere’s variants have been described, the etiology of which cannot be explained. There is no satisfactory cure for recurrence and progressive course of Meniere’s disease. No form of pharmacotherapy is able to prevent progressive deafness and tinnitus, of Meniere’s Disease. Pharmacotherapy is only of temporary and partial relief for vertigo. BPPV is the most common cause of acute vertigo and is characterized by brief attacks of rotatory vertigo that are triggered by movements such as lying down or rolling over in bed or extending the neck, with each episode lasting only seconds. However, not so uncommonly, patients complain of persisting symptoms of disturbed gait, blurred vision or dizziness, which may be misleading. These features make these two clinical entities of vestibular vertigo confusing with unsatisfactory explanation to their clinical course and management, based on the current pathophysiologic basis.

There is another entity called cervicogenic vertigo. Vestibular vertigo and cervicogenic vertigo have been identified as separate entities. It has been stated that when aural symptoms are there, consider it as vestibular vertigo and when neck pain is there, without aural symptoms, consider it as cervicogenic vertigo. This confusion occurred because of otolaryngologists and physiotherapists acting independently and not reaching to any consensus. Term ‘cervical vertigo’ was first described by Ryan and Cope in 1955, and is often a difficult diagnosis [10]. True rotatory vertigo, as seen in conditions like Meniere’s Disease and BPPV is rarely observed in vertigo of cervical origin, which has also been named proprioceptive vertigo, cervicogenic vertigo, and cervical dizziness. Hence, it is now generally termed cervicogenic dizziness [11]. Cervicogenic dizziness is characterized by the presence of imbalance, unsteadiness, disorientation, neck pain, limited cervical range of motion (ROM), and may be accompanied by a headache [11, 12]. Diagnosis of cervical vertigo can be challenging and controversial, and it is made only after other potential causes for dizziness or vertigo have been excluded [11]. The symptom of neck pain for diagnosis of cervical vertigo is very important. It has been proposed that if a patient has a chief complaint of vertigo, but it is not accompanied by neck pain, a diagnosis of cervical vertigo may first be excluded [11].

Is there any association between vertigo syndromes of vestibular and cervicogenic type? With this in mind, the current study was undertaken to study the association between cervical signs and symptoms in patients with Vestibular Vertigo of Meniere's disease, Benign Paroxysmal Positional Vertigoand cervicogenic dizziness.

Methods

Design and Participants

This prospective study was carried out between February 2017 and July 2019, in the Otorhinolaryngology department in collaboration with Physiotherapy Department of a rural tertiary hospital of central India. The study was conducted on 132 consecutive patients complaining of vertigo, attending “Neuro-otology and vertigo clinic” of the hospital, in which the diagnosis of Meniere’s disease or BPPV was made. The study was approved by the Institutional Ethics Committee and prior written informed consent was taken from the participants.

Data Collection and Analysis

A detailed history in relation to vestibular symptoms, type and pattern of giddiness, recurrence of symptoms, audiological symptoms, symptoms of cervicogenic dizziness for neck pain, headache were noted. History of trauma, domestic violence, occupation, etc. was noted for precipitation of myofascial problems. Otorhinolaryngological and vestibular examination findings, including Dix–Hall Pike tests, diagnostic Semont’s manoeuvre, Spontaneous nystagmus, were noted for the diagnosis of type of vestibular vertigo, along with findings of examination of neck and shoulder for any evidence of muscle tightness, asymmetry, etc. were recorded. Findings of Pure Tone Audiometry was also noted for confirmation of Meniere’s disease. Haematological investigations, Blood sugar, Thyroid Profile, Lipid Profile, Renal function and Liver Function tests were done to rule out secondary hydrops. Diagnosis of Meniere’s disease was made based on AAO-HNS guidelines, Benign Paroxysmal positional vertigo was diagnosed on the basis of results of Dix–Hall Pike test and diagnostic Semont’s manoeuvre. When there was history of neck pain with no vestibular symptoms and main complaint was unsteadiness, it was considered cervicogenic dizziness. Correlation was done between vestibular vertigo of Meniere’s Disease, Benign paroxysmal positional vertigo and cervicogenic dizziness with symptoms and signs related to neck, shoulder and muscle tightness.

Participants with neck pain were subjected to myofascial release form of manual therapy in multiple sessions to achieve what we call “Structural Rehabilitation” of the body, in order to relieve them of pressure jacket in the neck [13]. The protocol for manual therapy was tailored according to patient assessment of body symmetry and tightness.

Standardization of Methods Used

The Myofascial release form of manual therapy treatment comprised of a total of 3–4 sessions per week. The therapy was continued till clinical and objective improvement in posture, tightness, etc. The treatment sessions involved release of the fascia deep to the muscles by deep digital pressure of the fascia deep to muscles, around shoulder, or even distant areas like biceps or triceps.

Digital Pressure—Amount and Time Duration

Time factor was important in our technique, for obtaining significant and long lasting results. When pressure is applied into myofascia, initial sensation is that of softening and some motion, due to release of the elastic and muscular component. However, this is short of the actual release (contrary to other forms of therapy, which stop here). This is just the first stage of a multistep process that is required for long-lasting results. After the release of elastic and muscular component, one then encounters the collagenous barrier which feels as if one has hit a brick wall. This collagenous barrier does not release from force. It is important to wait at the collagenous barrier with gentle but firm pressure for a minimum of 40 to 70 s before the collagenous barrier responded by softening, reducing the enormous pressure on pain-sensitive structures and resulting in increase in motion. It is also important to apply correct amount of pressure, which should be neither too much, nor too less, in order to cause the hypothesized cellular and biomechanical changes. The sensation of release of the collagenous barrier, gives an idea that the release was proper. Our technique was based on our subjective sensation of perception of release and improved range of motion and patient’s feedback.

The collected data was analyzed with IBM, SPSS (IBM Corp., Statistics for Windows, version 23.0, Armonk, NY). Chi square test was used to evaluate the level of significance and P value of < 0.05 was considered significant.

Results

Results of 132 patients, in the age group of 15 years to 75 years, with regards neck related signs and symptoms were tabulated, after reaching to diagnosis of Meniere’s disease, BPPV or cervicogenic dizziness. Maximum patients with Meniere’s disease were in the age group of 30–40 years. Females outnumbered males, in the ratio of 2:1. Many patients with neck pain, neck malalignment and tightness had some past history of trauma, with fascia tightness in some part of the body. The neck malalignment and shoulder asymmetry were associated with fascia tightness, for which myofascial release form of manual therapy was given to the patients (Structural rehabilitation), with resultant significant improvement in neck alignment and symptoms of dizziness. The results of treatment by “Structural Rehabilitation” will be published soon in detail, along with objective parameters, like radiologic improvement, etc. The etiological hypothesis and treatment concept has been copyrighted by us.

Table 1 shows that most of the patients of Meniere’s Disease (80% for unilateral and 88.23% for bilateral), Benign Paroxysmal Positional Vertigo (75% for right sided BPPV, 66.67% for left sided BPPV) and cervicogenic dizziness (90%) had associated symptoms of neck pain and/ or headache, and were found to be positive for neck tightness and/or asymmetry of shoulder. Headache was more common in patient with Meniere’s Disease. BPPV immediately following head trauma and viral infection, were the cases with negative history and examination findings for neck and shoulder related symptoms and signs.

Table 1.

Clinical profile of vertigo patients

Patient S. No	Clinical diagnosis	Aural symptoms	Neck symptoms/headache	Neck signs	Number of patients
1	Unilateral Meniere’s	Episodic vertigo, tinnitus, hearing loss, aural fullness	Headache, Neck pain	Neck tightness, Neck malalignment, Shoulder asymmetry	20/25 (80%)
2	Bilateral Meniere’s	Episodic vertigo, tinnitus, hearing loss, aural fullness	Headache, Neck pain	Neck tightness, Neck malalignment, Shoulder asymmetry	26/30 (86.67%)
3	Right sided BPPV	Right sided Positional vertigo	Neck pain +	Neck tightness, Neck malalignment, Shoulder asymmetry	16/22 (72.73%)
4	Left Sided BPPV	Left sided Positional vertigo	Neck Pain +	Neck tightness, Neck malalignment, Shoulder asymmetry	10/15 (66.67%)
5	Cervicogenic Dizziness	Nil	Neck Pain +	Neck tightness Neck malalignment, Shoulder asymmery	36/40 (90%)
Total			Headache, Neck Pain	Neck tightness, Neck malalignment, shouder asymmetry	108/132 (81.8%) P < 0.001

Discussion

We found strong association of headache, neck pain, and/or neck tightness, neck malalignment, shoulder asymmetry in patients with Meniere’s disease, cervicogenic dizziness and also to a lesser degree in patients with BPPV. Meniere’s disease is considered to be “Idiopathic or Primary Endolymphatic Hydrops”. We have given the hypothesis, that “Meniere’s disease should be revisited as Cervicogenic hydrops” and that different “ cervicogenic and vestibular vertigo are spectrum of the same disease”, based on our present study observations and unpublished data of over 15 years, This happens due to underlying myofascial problems, which further result from postural habits, specific activities or lack of activity, and compensations for prior injuries. These in turn result in chronic stress on neck with neck spasm, limitation of full range of movements, and changes occur in circulation of two important fluids related to inner ear function, namely blood and cerebrospinal fluid. Forward head posture and other forms of head/neck misalignment can cause change in circulation of blood and cerebrospinal fluid.

The main underlying pathophysiology appears to be ischemia of inner ear which occurs due to compression of vertebral artery resulting from cervical problems, and giving rise to aural symptoms in Meniere’s disease and other syndromes of cervicogenic vertigo. The CSF pressure may increase due to neck spasms with increase in endolymphatic pressure. This could be explained on the basis of explanation given by a study which has observed an increase in the perilymphatic pressure induced by the infusion of artificial cerebrospinal fluid into the subarachnoid space resulting in an increase in the endolymphatic pressure, but no pressure difference between the endolymph and the perilymph was evident, regardless of the patency of the vestibular aqueduct [14]. This study found that the endolymphatic pressure is dependent on the perilymphatic pressure and there is no pressure difference between them. This hypothesis is then able to explain the increase in the amount of endolymph in Meniere’s disease.

Dizziness and vertigo in conditions associated with neck spasm also occurs due to increased proprioceptive inputs from the neck due to pressure on neck receptors Brown, 1992 demonstrated that there were close connections between the cervical dorsal roots and the vestibular nuclei with the neck receptors (such as proprioceptors and joint receptors), which played a role in eye-hand coordination, perception of balance, and postural adjustments via an experimental study in animals [15]. With such close connections between the cervical receptors, especially from receptors in the joints and ligaments of first three cervical vertebrae, and balance function, it is understandable that mechanical derangements of the cervical spine can give rise to vertigo, mainly dysbalance [16, 17]. The existence of neck reflexes and their role in reflexly orienting the limbs in relation to the head body angle was described by Magnus [15]. In fact, unilateral local anesthesia of the upper dorsal cervical roots induces ataxia and nystagmus in animals, and ataxia without nystagmus in humans.

Few authors had previously found neck pain and headache to be significantly associated with conditions of vestibular vertigo like Meniere’s Disease and Benign Paroxysmal Positional Vertigo [18–22]. Headache has been found to be associated in as many as 70% of patients with Meniere’s Disease, varying in intensity from slight to severe. Most of them complain of occipital and neck headache. Earlier studies have also found significant association between symptoms of cervical spine disorders, such as head and neck/shoulder pain in patients with Meniere’s disease as compared to controls, in the ipsilateral ear Bjorne et al., 1998 reported a strong association between head neck movements in the atlanto-occipital and atlanto-axial joints and triggered attacks of vertigo [23]. Also, 29% of the patients could influence their tinnitus by mandibular movements. Signs of cervical spine disorders, such as limitations in side-bending and rotation movements, were also significantly more frequent in the patient group than in the control group. They also observed tenderness to palpation of the transverse processes of the atlas and the axis, the upper and middle trapezius, and the levator scapulae muscle in a significant number of patients with Meniere’s disease as compared to controls. Further studies by Bjorne et al., 2003, showed a clear association with of Meniere’s Disease with Temporomandibular Joint Disorders (TMD) and Cervical Spine Disorders (CSD) [24]. They observed that a coordinated treatment of TMD and CSD in patients with Meniere's disease is an effective therapy for symptoms of this disease. They proposed that these three ailments appeared to be caused by the same stress, nervousness, and muscular tension.

Our results show a strong association between vestibular vertigo of Meniere’s Disease and BPPV with neck symptoms and signs. BPPV immediately following head trauma and viral infection, was not found to be associated with cervical symptoms and signs. So neck may be the root cause of vestibular vertigo. Cervicogenic dizziness is said to be a separate entity. However, there is a missing link between vestibular dizziness and cervicogenic dizziness. We found benefit by myofascial form of manual therapy in our patients and we call this “structural rehabilitation”. Our hypothesis for etiology and management of vertigo syndromes is summarized in Fig. 1.

Fig.1.

Revisiting “Meniere’s Disease” as “Cervicogenic Endolymphatic Hydrops” and other Vestibular and Cervicogenic Vertigo as “Spectrum of same disease”—a novel concept and treatment by “Structural Rehabilitation”

Conclusion

• We are the first one to propose that Vestibular Dizziness of Meniere’s Disease, Benign Paroxysmal Positional Vertigo and Cervicogenic Dizziness may be spectrum of the same disease with underlying myofascial problems.

• Meniere’s Disease of Idiopathic or primary type needs to be revisited as Cervicogenic Endolymphatic Hydrops.

• Our study highlights the fact that most of vestibular disorders like Meniere’s disease and Benign paroxysmal positional vertigo, have underlying postural problems with symptoms and signs of neck pain, headache, neck tightness and asymmetry of shoulders.

• Postural problems with underlying myofascial problems are the real culprit which lead to a series of events with end organ affection at inner ear level causing vestibular symptoms.

• Understanding of the actual cause of the problem can prevent the recurrence of the symptoms in both BPPV and Meniere’s disease and prevent the progression of symptoms in Meniere’s Disease.

• Concept of “Structural Rehabilitation” by “Myofascial release form of manual therapy” has a great potential to revolutionize the treatment of vestibular and cervicogenic dizziness.