Two tests overcome the challenge of atypical presentations of Menière’s disease


By Jacinta Bowler

10 Feb 2022

Dr Miriam Welgampola

Some patients with Menière’s disease are presenting with atypical symptoms and it could be causing diagnoses to be missed and treatment, according to a team of Sydney neurologists.

Published in Otology & Neurotology, their review of 375 patients seen over 10 years at Royal Prince Alfred Hospital in Sydney with either definite or probable Menière’s disease found that atypical symptoms included disequilibrium (13%), imbalance (3%), drop-attacks (3%) and unexplained vomiting (1%).

“We found, like many other authors, that the most common presentation of Menière’s disease was an attack with spinning vertigo lasting hours and very rarely extended to a day,” said Dr Miriam Welgampola, Associate Professor in Neurology at the Central Clinical School at the University of Sydney.

“But there was a very small proportion of patients who don’t do this. They might present with rocking and tilting vision … or they might present with no vertigo at all but severe nausea, or they may present only with hearing loss,” she told the limbic.

Although these patients might present differently, Dr Welgampola’s team said observation of ictal nystagmus in these atypical cases may help reach a confident diagnosis, and their review described which audio-vestibular tests were most effective.

Surprisingly, the team found that two outdated tests – the caloric test and the vestibular evoked myogenic potential (VEMP) test were two of the tests with the highest sensitivity.

The caloric test found abnormal results in 70% of cases, while the VEMP test showed abnormal results in approximately 30% of cases. The researchers explain that neither test is a ‘magic bullet’, but both tests together will help diagnose the majority of patients.

The team are also working on a pair of video glasses used in the clinic that was able to provide 90% sensitivity and specify. The test is done at home when patients begin to have an attack, providing more detailed data and a much higher likelihood of diagnosing the problem. These glasses are currently still in development.

Although there’s no cure for Menière’s disease, Dr Welgampola said that with a diagnosis and appropriate treatment those with Menière’s disease can return to the workforce and ensure their quality of life stays high. But it’s important to make sure even those cases with abnormal symptoms are properly diagnosed.

“The biggest thing is that early Menière’s disease can present without any particular diagnostic features,” she said.

“My take home message is that in the patient with recurrent severe vertigo, even if the hearing test is normal, don’t forget about Menière’s disease. Keep looking, and that might mean to keep repeating the hearing test.”

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