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Besides a stoppage of drainage pathways, abnormally enlarged fluid pathways into the ear such as the vestibular aqueduct or cochlear aqueduct may also be associated with Meniere's like symptoms. Thus, the bottom line is that in most patients with Meniere's disease in 2015, the underlying cause of Meniere's disease is unknown. Figure 3a: Audiogram (hearing test) typical of early Meniere's disease on the right side (x=left, o=right). A longer discussion of the information to be gained in testing of persons with Meniere's disease is found here. When there is sulfa allergy, one may try amiloride by itself, or ethacrinic acid (edecrin).
Histamine injections (irrational treatment as histamine is broken down rapidly in the body).
What the author recommends in his practice in Chicago for medical prevention of Meniere's.
These are combined with symptomatic drugs such as meclizine, benzodiazepines, and antiemetics, to be taken during attacks. In persons with severe progressive bilateral disease, the author will generally recommend a trial of high dose steroids.
An emerging treatment that may eventually end up as a standard treatment is multiple injections of steroids into the middle ear. The big problem with this section is separating placebo's from slightly effective treatments. There are an immense number of placebo and probable or nearly placebo treatments for Meniere's disease.
The Meniett device's status at this writing (2010) seems most likely to be a slightly effective treatment. There is a separate page on this topic, but briefly, for intractable unilateral Menieres disease cases, we often presently advise a low dose gentamicin protocol. Persons who have intractable bilateral Meniere's disease are offered a trial of steroids with a safer immunosuppressant should this be effective, a modified gentamicin protocol, and very occasionally systemic aminoglycosides to deaden both ears. In our opinion, medical treatments, including a reasonable trial of anti-migraine drugs such as verapamil or venlafaxine, should precede use of any last resort treatment.
Hearing is thought to gradually decline throughout life, with roughly 50 dB loss in 10 years. Note that it is not the overall level of sodium that is important, but whether or not it fluctuates that is important in avoiding attacks of dizziness. Gu X, Fang ZM, Liu Y, Huang ZW, Zhang R, Chen X.Diagnostic advantages of intratympanically gadolinium contrast-enhanced magnetic resonance imaging in patients with bilateral Meniere's disease.
Sato G1, Sekine K, Matsuda K, Ueeda H, Horii A, Nishiike S, Kitahara T, Uno A, Imai T, Inohara H, Takeda N. Cases that are virus positive also are positive for TNF-alpha, suggesting a possible link to autoimmune inner ear disease. In otosclerosis, the most commonly affected portion of the bone around the inner ear (otic capsule) is the anterior oval window (about 96%). Disorders that can be mistaken for otosclerosis (a disease) include disorders of the inner ear bones (ossicles), and fluid within the middle ear. Examples of disorders of the inner ear bones other than otosclersosis include ossicular chain disruption and ossicular fixation.
In congenital ossicular fixation, the normal articulation of the small ossicular bones does not occur. Bedside: The otologic clinician should confirm audiological testing suggesting a conductive hearing loss with bedside examination.
VEMP testing can also be used to diagnose superior canal dehiscence and distinguish otosclerosis from SCD. The symptom of paracusis Willisii, where the patient perceives speech better in a noisy background, is said to be frequently present in otosclerosis as well as other causes of conductive hearing loss. Otosclerosis does not have to be treated, as there are no medications that have been shown to work, and it will progress or not independent of any treatment. Hearing aids are effective for conductive hearing loss and certainly are less risky than having ear surgery. Lictor (2013) reviewed all medical treatments and concluded that there is no medical treatment proven to work. At the present writing, fluoride therapy is no longer a recommended primary treatment for otosclerosis (NIH consensus), because of its effect on other bones including the possibility of increasing the risk of hip fractures. Lippy and Berenholtz suggest adding Caltrate and 30 mg of risedronate (a biphophonate -- Actonel) in persons who do not respond to Florical and Vitamin D.
The author's advice on stapedectomy (the author is not an otologic surgeon), is that the BAHA implanted hearing aid, as well as similar devices, seem better suited to treatment of otosclerosis and similar causes of conductive hearing loss than stapes surgery. Surgery should also not be performed in patients whose hearing loss is 70 dB or less unless their speech discrimination score is 80% or better. Riggs BL, Hodgson SF, O'Fallon WM, Chao EY, Wahner HW, Muhs JM, Cedel SL, Melton LJ 3rd.1990.
Effect of fluoride treatment on the fracture rate in postmenopausal women with osteoporosis. When both conductive hearing loss and sensorineural hearing loss are present at the same time and at the same ear, it is referred to as mixed hearing loss or ‘combined –type’ hearing loss. Medication or surgery may be the answer to the conductive portion of the mixed hearing loss, but these interventions cannot treat the sensorineural portion of the hearing loss.
If you have any questions about mixed hearing loss or hearing loss in general, please contact our practice to speak with an audiologist. In our clinical practice, where we care for large numbers of Meniere's patients, the most common age of presentation is in the 6th decade. While in the past, it was felt that plumbing problems (hydrops) in the ear were responsible for the disease, the most current opinion is that hydrops is a marker for the Meniere's disease, rather than necessarily being responsible for the symptoms.
Recent evidence is against a relationship between the cochlear aqueduct and MD (Yilmazer et al, 2001). We ourselves hold this opinion, based in part on a study in which patients with Meniere's disease had a very high prevalence (about 25%) of autoimmune thyroid disease (Brenner et al, 2004). However, the evidence is against this being the main mechanism in human beings (see Kitamura et al, 1997; Nadol et al, 1995).
Those who present to the doctor with more severe hearing impairments at onset, usually do worse than those who have milder hearing impairments (Sato et al, 2014).


Although Ondansetron isn't as strong as phenergan or compazine, and doesn't always work, it also doesn't have much side effects either. All of the above diuretics have sulfa in them, which persons with sulfa allergies may be unable to tolerate. Loop diuretics such as Edecrin should be used in low doses and with caution because they are ototoxic. If there is a significant improvement in hearing, then there is an attempt to switch to Enbrel (an immunosuppressant with less side effects than steroids), with the thought that in this situation an autoimmune disorder is more probable.
There is nothing wrong with a placebo, if it provides some benefit (presumably psychological), and you can afford to do this. This treatment requires a surgical procedure (insertion of a ventilation tube) and purchase of a very expensive device (the pressure machine). Gentamicin is administered through the ear drum every month for a total of one or two administrations. The difficulty in pregnancy is that there should be an effort to avoid medications that might cause birth defects or otherwise injure the unborn child. Vertigo spells are generally thought to persist for decades, although with a gradual decline after roughly the first decade (Huppert et al, 2010).
It is not necessary or wise to lower your salt intake to amounts barely able to sustain life.
Psychological characteristics of patients with Meniere's disease compared with patients with vertigo, tinnitus or hearing loss. Hearing aids are generally used as a part of a comprehensive treatment program to successfully treat both the tinnitus and the hearing loss simultaneously. Overcoming balance and dizziness issues begins with undergoing regular hearing evaluations.If you’re concerned for yourself or a loved one, make an appointment with a certified hearing care provider or audiologist today. Although hearing loss is usually diagnosed in early adult life, the prevalence increases almost 7-fold with age (Niedermeyer et al, 2001).
This may be mediated by increased activity of the renin-angiotension system (Imauchi et al, 2008). There are some differences in opinion about this - -as about 100% of monozygotic twins match up with respect to otosclerosis (Fowler, 1966). The author's clinical experience suggests that this estimate may be an order of magnitude too high, as practically, otosclerosis is a rare diagnosis and getting rarer.
It can also involve the round window niche (30%), the internal auditory canal, and occasionally ossicles other than the stapes (Schuknecht and Kirchner 1974; Schucknecht, 1993). The reason for this is that mistakes can be made, and when there is a significant hearing loss, inexperienced audiologists may omit or use masking incorrectly. We have not found this to be useful and in fact find tymps to be almost universally normal (see tymps in figure towards top of this page). As mentioned above, VEMP's should be absent in conductive hearing loss, but present in sensorineural hearing loss. The main reason for this is to exclude alternatives (such as tumors or Menieres-- otosclerosis does not protect you from other ear diseases), and also to keep in touch with the audiologist in regards to when a hearing aid might be indicated. Hearing aid technology has undergone tremendous advances since the invention of surgical treatment for otosclerosis. Monical is a preparation that is absorbed in the intestine rather than in the stomach and may cause less side effects.
Note that the small end of the prosthesis is no longer adjacent to bone, and also that the entire prosthesis has rotated about 45 degrees clockwise.
If the prosthesis migrates inward, then ear drum movement may directly stimulate the saccule utricle. This operation, which involves drilling out the bone of the mastoid, is rarely if ever offered. This may be due to increasing prevalence of measles immunization, catching up on the backlog of cases that existed before surgery was available, or just changes in how conductive hearing loss is treated. Devices like the BAHA have less risk of dizziness or failure, and more control over the outcome. Always test each patient at least twice before doing the surgery (because mistakes can happen). We ourselves would modify this to say that acoustic reflexes should be either absent or inverted. In this case, there is likely to be damage to the outer or middle ear as well as to the inner ear or auditory nerve. Typically, the sensorineural hearing loss is already present and the conductive hearing loss develops later and for an unrelated reason.
Many people who suffer from a mixed hearing loss will receive medical treatment for the conductive hearing but will have to use hearing aid to treat the remaining sensorineural component of their hearing loss. We think Meniere's disease should more properly be called Meniere's syndrome, but we will use the two terms as if they were the same. This probably reflects the age distribution of Meniere's in Chicago Illinois, which of course is also a function of the age of the population in Chicago. Enlarged vestibular aqueducts are one of the most commonly identified inner ear bony malformations in children with sensorineural hearing loss of unknown cause (Oh et al, 2001). On the other hand, Ruckenstein and others recently obtained a large number of autoimmune blood tests in a group of 40 patients with unilateral Meniere's disease including CBC, ANA, Anti-Sjoegren, RF, Complement, antiphospholipid antibody, western blot for heat shock protein, MHA (syphilis test), and Lyme. Rather, it seems that Meniere's disease damages both hair cells and nerve fibers in humans. Should MRI scanners become even more powerful in the future, this could be an important test. Oddly, there is sometimes a discrepency between OAE tests and audiometry in the low-frequency SNHL of Meniere's. Van Deelen and Huizing studied the use of diuretics in Meniere's disease in a double-blind, placebo controlled trial, and reported that it reduces vestibular complaints, but has no significant effect on hearing (1986). Because Menieres and Migraine are very often combined, this medication's main role may be to treat the migraine associated vertigo that can be confused with or accompany Menieres disease.
Of course this strategy should rationally be adjusted to the variability in hearing, as in any undertaking, one's confidence that a particular result has been obtained depends both on the size of the effect as well as the underlying variability in the effect. However, we don't think that placebos that are billed to health care insurance providers should be supported.
Bilaterality of the condition gradually increases with time, but in most the symptoms in the opposite ear are minor.


Renin blockers (tekturna -- Aliskiren) are available, as well as many angiotension convertase inhibitors (ACE), but to our knowledge, no one has attempted their use to block worsening of otosclerosis in pregnancy.
Nuclear inclusions similar to paramyxoviral nucleocapsids have been observed in otosclerosis osteoclasts (McKenna et al, 1986; Chole and McKenna, 2001).
Otosclerosis is thought to begin with otospongiosis, which is a localized softening of the normally very hard bone of the otic capsule (Shambough 1971).
The malleus can be fixed at the anteior nuchal ligament, or the head can be adherent to the medial-most portion of the ear canal.
Lippy and Berenholz) suggest that this condition can be diagnosed with a flat sensorineural hearing loss, good speech discrimination, absent acoustic reflexes, and a family history. It is best to confirm the conductive hearing loss both with the tuning fork test (512 hz) -- both Weber and Rinne, and with the VEMP test.
This is the same patient as above (right) where the Right ear was operated, and the left ear unoperated, but both clearly had stapedial otosclerosis. This being said, it is still difficult to see the logic and we ourselves have never seen a patient who has volunteered this observation. It would seem to us, that one would need to consider both the risk of hearing progression, and balance it against the risk of adverse effects on other bones, when prescribing. Cruise et al (2010) indicated that there is only low-quality evidence for sodium fluoride therapy being effective in otosclerosis.
If it migrates outward, a fistula can arise at the oval window, from which the prosthesis was dislodged.
Because it involves creating an otic capsule fistula, similar to that seen in superior canal dehiscence, it has many untoward effects.
The surgeon should be prepared, if necessary, to repair the ear drum prior to the operation, treat all infections prior to the operation, and remove exostoses at the time of the ear surgery. BTEs are compatible with a wide range of ear molds, making these devices a favorable choice, as they must be replaced regularly as the child grows. It is a little older than the typical age of presentation of vestibular migraine which is in the 5th decade, much older than the reported highest prevalence age for non-vestibular migraine (about 35), and similar to the age of presentation of BPPV. The vestibular aqueduct extends from the medial wall of the vestibule to the posterior surface of the petrous temporal bone.
Only elevations in the antiphospholipid antibody test were found 27% of patients, above the expected 6-9%. Studies of humans are handicapped by the possibility that Meniere's is the final common pathway of a variety of illnesses of the ear. Potential reasons are an exitatory nystagmus, Bechterew's phenomenon (recovery nystagmus), Meniere's involving the opposite ear, and stronger vestibular responses on the side with hydrops due to hydrodynamic factors associated with hydrops. Another new MRI approach involves injecting gadolinium through the ear drum, and then a MRI scan (Gu et al, 2014). The same cannot always be said for meclizine, lorazepam and klonazepam, phenergan or compazine. Much room for improvement in these algorithms is needed -perhaps involving daily hearing monitoring using an internet protocol. The treatments briefly discussed below are (probably) not placebos, but they are high in cost (because they are surgical), and low in benefit (because the literature suggests low or no efficacy). This would be consistent with the observation that it is of variable penetrance, as one would expect a single gene to be more consistent in behavior. Measles virus RNA has been found in the stapes of otosclerotic persons (Potocka-Baklazek et al, 2014). On the other hand, about 40% of stapes from otosclerosis patients are negative for measles (Karosi et al, 2007), and it is also unclear why measles should affect the temporal bone, but not affect other bones in the body. To our knowledge, nobody has yet attempted to apply this finding with anti-TNF drugs such as entanercept. There appear to be three stages of otosclerosis -resorptive osteoclastic stages with signs of inflammation, followed by an osteoblastic stage involving immature bone, followed by mature bone formation. OPH is found in high concentration in the perilymph (Zehnder et al, 2005), and thus lack of OPG might be thought of as a possible explanation for the the otospongiosis part of otosclerosis. To us, this clinical picture would seem rather difficult to distinguish from any other type of sensorineural hearing loss.
VEMP's should be absent in a conductive hearing loss but present in sensorineural hearing loss.
Another possible explanation is that persons with sensorineural hearing loss have great problems hearing in noise, perhaps because of loss of outer hair cells, but this is simply not the case in conductive hearing loss.
There is not much one can do about a failed stapes procedure other than take more CT scans adding on more radiation, and then do more surgery.
As with sensorineural and conductive hearing losses, only a thorough diagnostic hearing and medical evaluation can identify a specific cause. Most persons with enlarged vestibular aqueducts with ear disorders have hearing loss, but occasionally there is an association with vestibular problems (Shessel and Nedzelski, 1992).
This technique is plausible, but we are doubtful that the diagnostic gain justifies injecting gadolinium dye into the middle ear. Kundu (2006) were unable to come to a conclusion as to efficacy as no papers published up to 2006 were adequate for meta-analysis. As the treatments for cochlear otosclerosis vary from doing nothing to an unproven medical treatment (sodium fluoride), we would favor simply diagnosing such patients as having sensorineural hearing loss. There may be a genetic link between enlarged vestibular aqueduct and Pendred syndrome, which is a congenital thyroid disorder. They concluded that it is unlikely that autoimmune etiologies play a significant role in their population of unilateral Meniere's (Ruckenstein, 2002). This is called Schwartz sign -- it is not a reliable sign and should not be relied upon (Lippy and Berenholz, 2008). Another interpretation of their data is that they may simply not have had an appropriate marker for autoimmune involvement, as well as unilateral Meniere's may have less autoimmune cause than bilateral.



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