Meniere's Disease

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Meniere’s Disease Diagnosis

Meniere’s disease is a cluster of auditory and vestibular symptoms that occur as a result of a cochlear pathology. The cluster of symptoms in the disorder include vertigo, tinnitus, aural fullness, and fluctuating sensorineural hearing loss. The following will review the audiometric, vestibular, and electrophysiological tools that can be used in diagnosing Meniere’s disease. This will be followed by a case study.

Background Information on Meniere’s disease

Meniere’s disease was first documented by Prosper Meniere in 1861, as a disorder that included intermittent bouts of vertigo and hearing loss. Meniere’s hypothesis was that there was a sort of “cerebral congestion” causing both the loss of hearing and balance equilibrium as a consequence. He localized this perceived congestion to the inner ear (Sajjadi & Paparella, 2008).

Through study of the anatomy and physiology of the inner ear and especially the endolymphatic system, researchers were able to pinpoint the likely culprit in this disorder as increased hydraulic pressure of the inner ear as a result of faulty endolymph circulation, production and removal. This is known as endolymphatic hydrops, which was first implicated as the cause of Meniere’s disease in 1943 by Altman and Fowler (Mancini, Catalani, Carru, & Monti, 2002; Sajjadi & Paparella, 2008).

The Mechanism for Endolymphatic Hydrops in Meniere’s Disease

The center of production for endolymph is a structure in the cochlea called the stria vascularis. Once the endolymph is created it is distributed throughout the scala media of the cochlea, and is then absorbed into the endolymphatic duct and sac (Roeser, Valente, & Hosford-Dunn., 2007). The endolymphatic sac of Meniere’s patients can malfunction, thus not allowing the endolymph to be reabsorbed. An endolymphatic sac blockage can cause the endolymph volume to increase along with the pressure within the cochlea (Mancini et al., 2002; Sajjadi & Paparella, 2008). This could lead to the perception of aural fullness, hearing loss, and tinnitus. The natural biochemical mechanism for relieving this endolymphatic sac blockage is to release hormones which cause an increase in endolymph, creating even more hydraulic pressure in the cochlea. Eventually there is a release of this endolymphatic sac blockage, allowing the pressure of the cochlea as well as the endolymph volume to return to baseline. This pressure release is responsible for the vertigo symptoms that occur during a full-blown Meniere’s attack (Sajjadi & Paparella, 2008).

Epidemiology of Meniere’s Disease

Tracking the prevalence and incidence of this disorder is difficult. It is diagnosed based on the presence of a cluster of symptoms which may not occur in unison, making it less likely for a physician to make the diagnosis. During case history formulation, a client may also forget to mention adjunct symptoms such as aural fullness or tinnitus without a physician’s prompting. Also, other disorders include dizziness as a main issue and are hard to differentiate from the vertigo experienced in Meniere’s. This is especially the case when the course of the disorder does not include the symptoms of hearing loss and tinnitus (Sajjadi & Paparella, 2008). Estimates of the prevalence of Meniere’s have been made and are as high as 513 per 100 000 in Finland and as low as 7.5 per 100 000 in France (Havia presenter & Kentala, 2004; Mancini et al., 2002). Incidence data from Sweden indicates Meniere’s affects 460 persons per million each year (Mancini et al., 2002; Stahle, Stahle, Arenberg. 1978).

Meniere’s disease appears to predominantly affect adults around the age of 40 years, with a range of onset between 20-60 years (Mancini et al., 2002). The disorder does affect a small number of children younger than 20 years, and these cases comprise around 3% of all Meniere’s cases. In addition, women seem to be slightly more likely to suffer from Meniere’s disease than men with a 1.3:1 preponderance (Sajjadi & Paparella, 2008). There is evidence that Meniere’s disease has a genetic component. Morrison (1995) found an autosomal dominant inheritance pattern with 61% penetrance for 41 families.

Diagnosis of Meniere’s Disease

The three main symptoms of this disorder are all part of the potential clinical caseload of an audiologist. Meniere’s sufferers may be seen by the audiologist for a battery of comprehensive testing including hearing, balance and tinnitus assessments. As such, an audiologist should be knowledgeable of the primary clinical concerns for Meniere’s disease as well as the course of the disorder.

Many Meniere’s sufferers will experience several attacks in a short period of time which can last for many weeks each. After a cluster of attacks, Meniere’s patients may then experience symptom free periods of time lasting months to even years (Roeser et al., 2007). There are some patients however who experience persistent and progressive vertigo over a period of months which lead to severe tinnitus and permanent flat sensorineural hearing loss. Therefore, it is important for the audiologist and physician to be prudent in documenting the timeline of symptoms so that appropriate treatment can be matched to the severity of Meniere’s symptoms (see Treatment section for further discussion). Only a small number Meniere’s sufferers will present with the full cluster of symptoms upon initial visit to a healthcare professional (Havia, Kentala, & Pyykkö, 2002; Sajjadi & Paparella, 2008) . Therefore, the importance of an in depth case history can not be overstated because of the intermittent nature of the disease. The course of the symptoms as well as any precipitating factors leading to an attack allows the clinician to understand the status of the disorder (Mancini et al., 2002; Sajjadi & Paparella, 2008).

The diagnosis of Meniere’s has become somewhat standardized, with the following as the American Academy of Otolaryngology criteria for Certain Meniere’s:

1. Recurrent vertigo that lasts longer than 20 minutes which is followed by a period of feeling nauseated, and off-balance.
2. Hearing loss which is fluctuating or sustained.
3. Either or both, tinnitus or a feeling of fullness in the ear (Sajjadi & Paparella, 2008).

There are other stages of progression for Meniere’s diagnosis that range from Possible Meniere’s where only vertigo is experienced to the Certain Meniere’s listed above.

Diagnostic Tools for Meniere’s Disease

Basic Audiometry and Tinnitus Matching

In a study of over 200 Meniere’s sufferers, Havia, Kentala, & Pyykko (2002) found that hearing loss was experienced by 64% of the cohort during Meniere’s attacks, with 45% experiencing permanent hearing loss over 4 years of the disorder. Unlike the debilitating vertigo that occurs in Meniere’s which tends to be intermittent, the sensorineural hearing loss can be a permanent symptom of this disorder, worsening during each Meniere’s attack and over time (Gates, 2006; Havia et al., 2002). The rate of hearing loss progression depends on the rate and severity of the Meniere’s attacks (See Figure below).

Figure 1- The progression of hearing loss measured as the pure tone average in Meniere’s disease patients over time (Havia et al, 2002).

For Meniere’s clients, the audiogram configuration appears at the first audiological assessment as a flat sensorineural hearing loss for 55% of clients and a low-frequency upward sloping hearing loss for 25% of patients (Havia et al., 2002). The sensorineural hearing loss that results from Meniere’s disease tends to occur first in the low frequencies, with 500 Hz being the most likely frequency of permanent loss (Havia et al., 2002; Roeser et al., 2007). This low frequency hearing loss may not be observed in patients that are not seen early on in the course of this disorder, or those patients who are not referred to an audiologist soon after Meniere’s symptoms are observed. It is over a period of years that the configuration of the hearing loss goes from being a low frequency upward sloping loss to a flat sensorineural loss (see Figure 2 below for the audiometric progression of sensorineural hearing loss in Meniere’s). Approximately 40% of Meniere’s sufferers will develop the disease in the other ear (Roeser et al., 2007).

Figure 2- Example of an up-ward sloping sensorineural hearing loss of a Meniere’s patient that might be observed upon initial audiometric assessment. This is followed by the flat configuration expected for a Meniere’s patient 5 years later.

Other audiological findings depend on the configuration of hearing loss as well as the severity of the cochlear damage as a result of the endolymphatic hydrops. This would be especially true for speech audiometry where a Meniere’s patient with normal thresholds will be expected to perform better than a Meniere’s patient with a moderate flat loss. Immitance measures should be observed for all Meniere’s patients. Acoustic reflexes should be present at normal or elevated stimulation levels depending on the amount of hearing loss. Otoacoustic emissions should also be evident for most Meniere’s patients when they are first diagnosed, disappearing as the sensorineural hearing loss progresses past a moderate flat sensorineural loss.

Tinnitus Evaluation

Tinnitus is an additional auditory problem that is very common for Meniere’s disease sufferers. Tinnitus is the perception of sounds such as ringing, rushing or buzzing that have no source in the auditory external environment. This symptom can be annoying and in some cases debilitating. Tinnitus can be persistent or intermittent and this symptom is directly affected by aversive psychological states. The more people dislike the ringing in their ears the more terrible the symptom seems to become (Henry, Dennis & Schechter, 2005). The cause of tinnitus can be cochlear malfunction: the de-coupling of the OHCs from the tectorial membrane, causing discordant stimulation of the coupled inner hair cells (IHCs) by disrupted basilar mechanical transduction (Jerger et al, 1993). There are many theories of the etiology of tinnitus which vary as much as the patients experiencing this symptom (see Henry, Dennis & Schechter (2005) for an extensive review). The similarity among many theories is that tinnitus is observed when the peripheral or central auditory system is compromised either through a pathology like Meniere’s or through trauma like that experienced with excess noise or ototoxicity.

The tinnitus experienced by Meniere’s patients tends to be a low frequency buzzing or roaring and this symptom is experienced more by those with the most severe hearing losses (Havia et al., 2002). Tinnitus persists beyond the Meniere’s attacks and for many Meniere’s sufferers the tinnitus experienced is severe and debilitating. Havia et al. (2002) observed that 30% of their Meniere’s patients classified their tinnitus as severe; in addition, the patients who rated their tinnitus as severe had the most severe vertigo and were most likely to suffer “drop attacks”. The tinnitus can be localized to the ear affected by Meniere’s; however, there are some clients that will perceive the tinnitus to be delocalized or central despite a unilateral pathology (Havia et al., 2002).

The diagnosis of tinnitus is made during initial interview and case history, and to a lesser extent on tinnitus pitch and loudness matching, minimum masking level (MML), loudness discomfort level, and residual inhibition (see Henry et al, 2005 for tinnitus assessment protocol). The assessment of the pitch quality of the tinnitus might help the clinician decide whether there is a particular locus of the basilar membrane affected. Also, tinnitus maskability (MML) and residual inhibition should be assessed to objectify the severity of the tinnitus and as a way to identify possible treatment options. The only true standard for assessing tinnitus severity is by client case history questioning. Questionnaires can provide the patient an opportunity to highlight the quality and severity of their tinnitus symptoms. Tinnitus questionnaires are best when they are standardized and used as part of a clinical protocol (Roeser et al., 2007).

Electro-Physiologic Measures

Magnetic resonance imaging (MRI) scans and auditory brainstem response (ABR) testing can be used in assessing a Meniere’s case by providing a differential diagnosis between this disorder and retrocochlear pathology. For example, the aural fullness, hearing loss, tinnitus and balance issues associated with Meniere’s are also symptoms of having a tumor on the vestibular nerve (Roeser et al., 2007; Sajjadi & Paparella, 2008). An MRI of the internal auditory meatus allows the radiologist and otolaryngologist to determine if a mass is present as would be the case for an acoustic neuroma. An ABR could be ordered as a less expensive and less sensitive test for diagnosing a vestibular neuroma. Unlike an acoustic neuroma patient, a Meniere’s patient’s ABR would show normal absolute wave I, III and V latencies, as well as normal interwave latencies, and normal wave morphology (Roeser et al., 2007).

Electrocochleography (ECochG)

ECochG has been used recently to diagnose endolymphatic hydrops. This is a measurement of the electrical activity of the cochlea when click stimuli of different phases are presented. The compound action potential (AP) of the auditory nerve and the summating potential (SP) is measured at the tympanic membrane or the promontory of the cochlea. The summating potential is observed throughout the duration of the stimulus and is a DC voltage. The AP is a measurement of IHC depolarization output and is an alternating current voltage response with the most negative peak marked as N1 (see Roeser et al, 2007. chapter 19 for in depth discussion of ECochG). Meniere’s sufferers tend to have a longer AP-N1 latency difference between the condensation and rarefaction stimulus conditions when compared to a normals. In addition, the amplitude or area ratio of the SP/AP is significantly higher for those with endolymphatic hydrops (Roeser et al., 2007). ECochG is sensitive for Meniere’s diagnosis only when the patient is experiencing cochlear hydrops either directly before or during a Meniere’s attack.

Videonystamography (VNG)

Vertigo is the false perception of movement such as the feeling of spinning or having the room spin around you and may be caused by either peripheral or central sites of pathology. VNG is an objective measure of vertigo which records the changes in nystagmus beat rate and direction under varying positional and visual conditions. VNG examines the integrity of the central (brainstem and cortical areas) and peripheral (semicircular canals) balance centers. Tests of eye movement such as in smooth pursuit or optokinetics will assess the central sites of balance. Tests such as the Dix-Hallpike or bithermal caloric directly test the semicircular canals of the cochlea isolating the cochlea as a source of dizziness (Roeser et al., 2007; Sajjadi & Paparella, 2008).

A VNG assessment done during a Meniere’s attack may show normal nystagmus patterns during visual tasks but abnormal caloric and potentially abnormal Dix- Hallpike nystagmus, with beat amplitude, rate and direction to the affected side. The observation of unilateral weakness and directional preponderance in the caloric test would confirm the vertigo experienced as well as give ear specific balance information. However, many Meniere’s patients may be assessed well after an attack, leading to normal video nystagmography findings. Moreover, almost half of those with Meniere’s disease will have normal nystagmography and calorics, even when they are assessed during an attack (Sajjadi & Paparella, 2008). Therefore, VNG results should be considered part of a diagnostic test battery that also considers patient reports of dizziness as confirmation of vertigo systems.

Treatment Options for Meniere’s Disease

It is important for the audiologist and physician to be prudent in documenting the timeline of symptoms so that appropriate treatment can be matched to the severity of Meniere’s symptoms. The recommendation for those experiencing their primary bout of Meniere’s is a change in diet: cessation of salt, caffeine, and alcohol consumption. Tobacco use is also advised against. If this primary treatment suggestion fails to affect the rate and severity of Meniere’s symptoms, the next approach is medical intervention starting with diuretics or steroids. Diuretics have not been very successful in treating Meniere’s, but because they are a safe option with few side-effects, physicians should consider these first. Steroids such as prednisone have been shown to reduce acute vertigo during Meniere’s attacks but seem to have no beneficial affect on hearing. If after 3-6 months Meniere’s attacks have not been attenuated, surgical intervention may be indicated to reduce cochlear hydrops through endolyphmatic sac correction or the perception of vertigo symptoms through gentamycin injection or vestibular sectioning (Sajjadi & Paparella, ). Please see Figure 3 below for an outline of the current treatment protocol recommended by the American Association of otolaryngologists.

Figure 3- The current treatment protocol for managing a patient with diagnosed Certain Meniere’s Disease (Sajjadi & Paparella, 2008).

Case Study from Goto, Nakai, Kunihiro & Ogawa, (2008)

“A 51-year-old male patient has suffered from fluctuating right sensorineural hearing loss with vertigo since 1994. In May 2002, he was first admitted to our hospital due to a severe vertigo attack accompanied by right sensorineural hearing loss. Spontaneous nystagmus toward the right side was observed. Since April 2004, he has experienced vertigo spells with right-sided tinnitus a few times per month that are intractable to conventional medical therapy.” (Goto, Nakai, Kunihiro & Ogawa, 2008)

Audiometric Test Battery:

AUDIOGRAM #1 April - 2002

• This patient presented with a moderate low frequency peaked sensorineural hearing loss in April, 2002. This configuration of hearing loss is consistent with the low frequency hearing loss of Meniere’s Disease. Treatment: steroid therapy and diet restrictions were implemented to reduce the frequency of cochlear hydrops.

AUDIOGRAM #2 April - 2006

• Over a period of 4 years his audiogram progressed to a flat sensorineural hearing loss in the right ear. This is consistent with the typical progression of hearing loss with Meniere’s. Hearing sensitivity was not affected by steroid therapy and diet limitations.

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Acoustic Brainstem Response (ABR)

• He was sent for a right sided neurological ABR in June, 2002. All interwave latencies and absolute latencies were normal indicating normal auditory brainstem functioning. A retrocochlear pathology as a cause for his vertigo, asymmetrical hearing loss and tinnitus is unlikely based on his ABR results.

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Tinnitus Case History- April, 2002

• He reports right sided tinnitus. He reports that his tinnitus is persistent but worsens during his bouts of vertigo and he considers his tinnitus to be debilitating during quiet moments especially right before going to sleep. He states that his tinnitus is a roaring (low frequency sound) which is consistent with Meniere’s disease. Steroid treatment diminished his tinnitus for a few months following his first hospital visit but worsened over the past two years.

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ECochG assessment- May, 2002

• An Abnormally long AP-N1 latency difference between rarefaction versus condensation click stimuli was noted for the right ear. In addition, the SP/AP ratio was abnormal for the right ear. Both of these findings are indicative of the endolymphatic hydrops present in Meniere’s.

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VNG assessment- May, 2002:

• Spontaneous right beating nystagmus was observed during the visual gaze task. From the bithermal calorics assessment a unilateral weakness was observed for the right ear consistent with cochlear hydrops present in Meniere’s Disease.

Timeline and Frequency of Vertigo Bouts

This patient was seen every three months to track his Meniere’s symptoms. It appears that he follows a clustered course for the disorder, having 1-3 bouts of vertigo in one month followed by many months without an attack. The frequency of vertigo bouts has increased in the past 2 years where he has experienced 3-6 bouts every three months instead of 1-3. His vertigo has not been helped by steroid therapy.

Overall, the vertigo and tinnitus symptoms are worsening over time, and are not responding to medical treatments. A more aggressive treatment may be indicated to treat vertigo such as gentamycin injection. The risk of losing even more hearing because of gentamycin therapy has made this treatment option unfavorable for the patient. He has refused gentamycin injection. The last treatment option is for the client to attend autogenic training (AT) and cognitive behavioral therapy (CBT). He has agreed to do AT and CBT. The treatment began November, 2006 and the number of vertigo attacks diminished significantly over the subsequent 5 months (see below).

Note: AT is a set of relaxation techniques that are used in a prescribed way. AT is much like yoga with deep breathing and “mind-centering” exercises.

References

• Gates, G. A. (2006). Ménière's disease review 2005. Journal of the American Academy of Audiology, 17(1), 16-26.
• Goto, Nakai, Kunihiro & Ogawa, (2008). Case report: a case of intractable Meniere's disease treated with autogenic training. Biopsychosocial Medicine, 2 (3)
• Havia presenter, M., & Kentala, E. (2004). The prevalence of ménière’s disease in finland. Otolaryngology - Head and Neck Surgery, 131(2), P154-P154.
• Havia, M., Kentala, E., & Pyykkö, I. (2002). Hearing loss and tinnitus in meniere's disease. Auris Nasus Larynx, 29(2), 115-119.
• Henry JA, Dennis KC, Schechter MA. (2005). General review of tinnitus: prevalence, mechanisms, effects, and management. Journal of speech, language, and hearing research Oct; 48(5):1204-35.
• Jastreboff, P., & Hazell, J. (1993). A neurophysiological approach to tinnitus: clinical implications. British Journal of Audiology; 27, pp. 7-l7
• Mancini, F., Catalani, M., Carru, M., & Monti, B. (2002). History of meniere's disease and its clinical presentation. Otolaryngologic Clinics of North America, 35(3), 565-580.
• Morrison AW (1995). Anticipation in Meniere’s disease. Journal of Laryngolology Otology; 109: 499–502
• Roeser, Valente, & Hosford-Dunn. (2007). In Brandenburg (Ed.), Audiology diagnosis (2nd Edition ed.). New York, NY.: Thieme Medical Publishers Inc.
• Sajjadi, H., & Paparella, M. M. (2008). Meniere's disease. The Lancet, 372(9636), 406-414.
• Stahle J, Stahle C, Arenberg IK (1978). Incidence of Meniere’s disease. Arch Otolaryngol;104:99–102