For this blog we will be looking at Meniere’s disease. Remember audiograms will differ depending where they are along the pathology pathway. Meniere’s causes a progressive hearing loss in sufferers. I will try and give examples of audiograms early on the disease pathway and later on when the hearing loss has progressed. Once again these are examples, of audiograms taken from clients. Meniere’s disease is very individualistic and the how the different symptoms present and how they affect the sufferer will vary from individual to individual. Because of how complicated Meniere’s can be this will be a very long blog!
Meniere’s
According to the American National Institute on Deafness and Other Communication Disorders ‘Meniere’s disease is a disorder of the inner ear that causes severe dizziness (vertigo), ringing in the ears (tinnitus), hearing loss, and a feeling of fullness or congestion in the ear. Meniere’s disease usually affects only one ear, but in 15% to 25% of people with the disorder, both ears may be affected. Attacks of dizziness may come on suddenly or after a short period of tinnitus or muffled hearing. Some people have single attacks of dizziness separated by long periods of time. Others may experience many attacks close together over several days. Some people with Ménière’s disease have vertigo so extreme that they lose their balance and fall’. https://www.nidcd.nih.gov/health/menieres-disease (I have even seen cases where the individual is unable to get out of bed and function normally, the vertigo is so persistent and severe).
We rarely see it in individuals under 18 years of age. It is most likely to occur in adults between the ages of 40-60 years of age. No one is quite sure what causes Meniere’s disease. Where it occurs in more than one family member it is thought genetic variations may play a part in the origin. About 7-10% of those affected have a family history of the condition. Incidence of Meniere’s is between 1:1000 and 1:2000 of the population. (Meniere’s UK)
What happens in the ear
When you see a picture of the cochlear you will see that it contains a snail like bit called the cochlea and two upwards facing semi-circular canals these are the organs of balance NIDCD explains it as follows ‘(the semi circular canals and otolithic organs) and the cochlea and has two sections: the bony labyrinth and the membranous labyrinth. The membranous labyrinth is filled with a fluid called endolymph that stimulates receptors in the balance organs as the body moves. The receptors then send signals to the brain about the body’s position and movement. In the cochlea, the fluid is compressed in response to sound vibrations. This stimulates sensory cells that send signals to the brain. In people with Meniere’s disease, a build up of endolymph in the labyrinth called endolymphatic hydrops disrupts normal balance and hearing signals between the inner ear and the brain. This disruption is also associated with vertigo and other Meniere’s disease symptoms’ https://www.nidcd.nih.gov/health/menieres-disease.
We think this endolymphatic hydrops causes an increase in the pressure in the endolymphatic space. It is quite likely that this increased pressure is what causes the fullness and reduced hearing. Meniere’s UK explains it quite nicely ‘The sudden release in pressure accounts for the sudden attacks of vertigo. Repeated episodes of high pressure and sudden releases of that pressure damage the delicate structures of the inner ear and the balance structures of the semi-circular canals. This cumulative damage results in a decline in hearing levels over time.’
Symptoms of Meniere’s
Meniere’s is very individualistic; symptoms vary between people and over time. The main symptom is attacks of vertigo with nausea and vomiting. These attacks can last from a few minutes to 24 hours (or longer in some people). During the attack people may also suffer from tinnitus, a feeling of fullness in the affected ear and hearing loss. After the attack the hearing may improve, but generally never to where it was previously. The tinnitus and fullness may go.
For some tinnitus is there all the time with Meniere’s but worsens during the attack. As Meniere’s disease effects the low frequencies initially, many sufferers indicate that the tinnitus sound is often more low frequency in pitch like a rumbling.
Periods of remission between attacks can vary from days to months or even years. For some the vertigo attacks can be severely debilitating causing them to be bed bound; as just raising their head will make the dizziness worse and induce nausea and vomiting. Whilst other individuals may be less affected. As it progresses the vertigo may become less severe. In the later stages tinnitus is often more prominent and the fluctuating hearing loss worsens. Some individuals with Meniere’s will not just have a significant hearing loss but also find that what they are hearing, even with hearing aids in, is severally distorted and may also suffer from sound tolerance issues whilst others may not have this issue at all.
Some people use some of these symptoms as forewarning as to when an attack is about to occur. I have had many clients over the years say that a sudden change in the ear fullness or increase in the tinnitus alerts them to the fact that an attack is imminent.
Lastly whilst stress is not a trigger to Meniere’s we know that it can increase the frequency and intensity of attacks.
Diagnosing Meniere’s disease
GPs cannot diagnose Meniere’s disease. Only Ear, Nose and Throat Surgeons/Otolaryngologists are able to diagnose the disorder after evaluating all the information from testing and medical history. There is no one test that allows for diagnosis. The client’s history, hearing tests, speech understanding tests, blood tests, MRI scans and vestibular testing procedures which check the balance mechanism will be used in order to get a diagnosis. For further information on these visit Visiting ENT, diagnosis and testing | Ménière’s Society
Intervention Options
Hearing Aids
Hearing aids can be helpful for suffers, and many will find it improves their hearing. However, the sufferer needs to have a clear understanding of the limitations also. Speech in noise understanding will still be difficult, and hearing aids will need to be adjusted as the hearing loss changes and in the later stages, discrimination becomes further impeded. You may wish to consider a CROS aid for the affected Meniere’s ear, particularly in the mid to late stages of the disease. For those with hyperacusis issues or severe distortion then hearing aids may be very limited in their benefit or may not work at all for that ear. Hearing aids may also help with the tinnitus. (remember tinnitus therapy is also available on the NHS if this is needed). I would always suggest trying a hearing aid, but both of you must have a clear understanding and discussion about the limitations and benefits and the issues that may occur.
Surgery
Where the balance/vertigo is severely affecting the client’s quality of life is then surgical intervention may be the only option. There are different options here such as intratympanic injections and endolymphatic sac surgery. Surgery | Ménière’s Society
Drugs/Therapy
Medications can be used to help manage vertigo symptoms Medication | Ménière’s Society. Some people also find adjusting their diet and reducing their salt intake helps with symptoms. Also managing stress is very important in helping control the symptoms. Controlling Your_Symptoms_(Nov_2023).pdf. There are also balance rehabilitation exercises that help you manage your symptoms Balancing_Retraining_(Nov_2023).pdf
Audiograms
The “classic” picture of Meniere’s hearing loss is that there is a fluctuating, gradually progressive sensorineural hearing loss. It starts with low frequency hearing loss, and eventually progresses to become “peaked” (both low and high tone), and then flat. Audiograms and description from Hearing loss in Meniere’s disease
Early Meniere’s Disease Left ear : Low Freq are reduced
Mid stage Meniere’s Disease Right Ear: Low Freq worse but flattening out
Late-stage Meniere’s Disease Right ear: Hearing aid likely to be ineffective CROS/BICROS better option