Ménière’s Disease: Do Not Consider Invasive Therapies Too Early

Definite Ménière’s disease (MD) is an episodic vertigo syndrome associated with low- to medium-frequency sensorineural hearing loss and fluctuating aural symptoms (hearing, tinnitus, and/or auricular plenitude sensation) in the affected ear. ¹ Duration of vertigo during a crisis is limited to a period of 20 minutes to 12 hours. ¹ The frequency of vertigo attacks and the difficulty in controlling the disease varies greatly from one patient to another.^1-3 Over the past few years, we have encountered several patients with MD for whom treatments with significant risks of complications like surgical labyrinthectomy or vestibular nerve section were proposed quickly by some medical centers. The purpose of this letter is to outline the progressive steps to follow in the management of MD.

As a first step, lifestyle recommendations including a low salt diet should be implemented.^2,3 Furthermore, conservative treatments like betahistine and/or diuretics should be tried because they have few side effects and do not impair hearing.^2,3 Betahistine is usually prescribed at a dosage of 48 mg/day. ⁴ However, its effects are dose and duration dependent. ⁵ Due to the poor bioavailability of betahistine which is rapidly degraded by monoamine oxidase (MAO) a/b, it has been proposed to gradually increase its dosages up to values of 480 mg/day if necessary.^4,5 Selegiline 5 mg/day is a type b MAO inhibitor that has been shown to increase the bioavailability of betahistine by approximately 80- to 100-fold. ⁴ The combination of betahistine and selegiline could be an alternative to a high dosage with betahistine but its effectiveness must still be confirmed in large cohorts of MD patients.^4,5

Subsequently, intratympanic steroid therapy could be offered as a second step to patients unresponsive to the first-line therapy.^2,3 Most important, other causes of dizziness associated with MD should be looked for before considering invasive therapies. Indeed, a common pathophysiology between MD and vestibular migraine (VM) has been suggested. ⁶ Noticeably, 50% of definite MD have migraine headaches and approximately 40% of definite MD are associated with VM. ⁶ For these reasons, migraine prophylactic therapy should be tried in case of failure of conservative treatments for MD whether associated with VM or not. ⁶ Persistent postural-perceptual dizziness (PPPD) should also be considered as a secondary cause of dizziness refractory to conservative MD treatments. ⁷ Implementation of vestibular rehabilitation and/or psychotherapy might significantly improve balance in these cases.^2,3,7

For refractory MD with useful hearing, endolymphatic sac surgery could be a nonablative option, although the level of evidence is very low. ² Vestibular nerve section should be considered as the last option to offer in cases of uncontrolled MD with serviceable hearing function or immediately in cases of Tumarkin drop attacks with useful hearing.^2,3 When the patient’s hearing is no longer useful, it is then preferable to offer intratympanic injections of gentamicin (ITG) which is usually associated with a good control of vertigo but carries a risk of worsening of hearing.^2,3 Surgical labyrinthectomy could only be proposed in case of failure of treatment with ITG with if possible cochlear implantation at the same time.^2,3 Finally, ablative treatments require good contralateral vestibular function, given the risk of developing bilateral vestibulopathy.^2,3

MD is therefore a complex situation since it can become bilateral and sometimes many years after the onset of a unilateral form.^1-3 Risk factors such as the presence of migraine, a young age at diagnosis of unilateral MD, and a familial history of MD should be taken into account in the decision to initiate ablative treatment. ⁸

Ablative treatments would thus remain reserved for refractory cases or severe forms such as Tumarkin drop attacks. Systematic detection and treatment of associated VM and/or PPPD could further limit these situations, and should thus be carefully looked for. The Figure 1 represents a proposal for a decision-making algorithm for the treatment of Ménière’s disease.

Figure 1.

Decision-making algorithm for the treatment of Ménière’s disease. MD, Ménière’s disease; VM, vestibular migraine; PPPD, persistent postural-perceptual dizziness; ITG, intratympanic injections of gentamicin.