How Common is Meniere’s Disease?

Patient Presentation
A 15 year old male came to clinic after a 1 week history of rhinorrhea and cough. He denied any fever, chills, nausea, or sore throat. He denied actual acute ear pain but said it felt fuller with pressure and was more difficult to hear from with his left ear. He had a history of seasonal allergic rhinitis during the fall and was not taking any of his medications at this time.

The pertinent physical exam showed a healthy male with normal vital signs and growth parameters. His nose had moderate clear rhinorrhea. His left ear had serous fluid with bubbles behind the tympanic membrane which was dull. There was no erythema or purulence noted. His right ear had only a small rim of serous fluid noted. The rest of his examination was normal.

The diagnosis of left sided serous otitis media was made and the natural history and symptomatic treatment were reviewed. He said, “It’s a good thing I don’t feel dizzy. I read a book about space flight and the first American in space was grounded for a while because he got hearing loss and dizziness. He got better too and went on one of the Apollo missions. He even golfed on the moon.” The pediatrician encouraged his reading and wish to possibly pursue an aerospace career.

Discussion
Meniere’s disease (MD) was described in 1861 by Dr. Prosper Meniere as an otologic balance disorder caused by semicircular canal problems. It is also known as endolymphatic hydrops.

MD causes vertigo, hearing loss, tinnitus and aural fullness. Usually, at least initially, the symptoms are unilateral. Attacks start suddenly and can last for 20 minutes to several hours. Its prevalence increases with age. It is more common in females than males (73-84 per 100,000 compared to 56 per 100,000 in the US). It is more common in Caucasians (91 per 100,000) and is more common in less populated areas such as the Midwest of the US.

Alan Shephard was the first American and second human in space in 1961. He was grounded for a period of time reportedly due to MD. He eventually was restored to flight status, and was crew commander for Apollo XIV. He loved to golf and therefore had a specially modified club to hit golf balls on the moon as well.

Learning Point
Although least common in the pediatric age range, MD does occur. Prevalence is estimated at 3 per 100,000. Vertigo occurs in children and benign paroxysmal positional vertigo and vestibular migraine account for the large majority of the patients. However there are several other entities that together account for 10-15% of the cases. MD in one study was the 3rd most common reason for vertigo/inner ear problem and accounted for 1.5% in a European study. Other literature estimates it at 0.4-2.9%.

MD and vestibular migraine can have similar symptoms. Attacks can last hours and be intermittent occurring over long time periods. Headache may not occur. Nystagmus may not be noted by caregivers and patients. Patients may also not be able to verbalize mild hearing loss/fullness or tinnitus. As it is difficult for children to describe their symptoms which may develop over long periods of time. One author “…suggested the young children with idiopathic recurrent vertiginous attacks of more than 20 min, accompanied by fluctuating low-tone hearing loss may have definite MD.”

Although some physical examination signs can be performed in the outpatient office, these are done infrequently and may not be performed in a standard manner or may not be able to be interpreted by a generalist physician. One example would be caloric testing. Patients may require other specialties (audiometry, otolaryngology, neurology) to more fully evaluate patients especially for differentiating among the various problems causing vertigo. Even more specialized testing may be needed including ocular or cervical vestibular evoked myogenic potentials. Additional testing may include causes of congenital hearing problems including syphilis, and MRI of the head to evaluate for embryopathic anomalies. Both pediatric and adult patients may be seen to show similar abnormal vestibular function, but adults have worse audiological function when diagnosed. Studies of pediatric MD have shown a genetic predisposition with 5-15% of patients having an autosomal dominant inheritance pattern. About 1/3 of pediatric patients have some type of positive family history. Over time the rates of bilateral disease in pediatric patients increases. There is no standardized treatment for pediatric MD but different treatments have been tried including diuretics anti-nausea, steroids and surgery.

Questions for Further Discussion
1. What causes vertigo in children? A review can be found here.
2. What are causes of ataxia? A review can be found here.
3. How common is syncope? A review can be found here.
4. What are some potential treatments for motion sickness? A review can be found here.

Related Cases

    Disease: Acute Serous Otitis Media | Ear Disorders | Meniere’s Disease

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Meniere’s Disease, Hearing Loss and Dizziness and Vertigo.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Simo H, Yang S, Qu W, Preis M, Nazzal M, Baugh R. Meniere’s disease: importance of socioeconomic and environmental factors. American Journal of Otolaryngology. 2015;36(3):393-398. doi:10.1016/j.amjoto.2015.01.009

Wang C, Wu CH, Cheng PW, Young YH. Pediatric Meniere’s disease. International Journal of Pediatric Otorhinolaryngology. 2018;105:16-19. doi:10.1016/j.ijporl.2017.11.029

Gedik-Soyuyuce O, Gence-Gumus Z, Ozdilek A, Ada M, Korkut N. Vestibular disorders in children: A retrospective analysis of vestibular function test findings. International Journal of Pediatric Otorhinolaryngology. 2021;146:110751. doi:10.1016/j.ijporl.2021.110751

Alan Shepard. In: Wikipedia. 2024. Accessed August 26, 2024. https://en.wikipedia.org/w/index.php?title=Alan_Shepard&oldid=1241612336

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa