Meniere’s disease

Meniere’s disease is a chronic disorder of the inner ear characterized by episodes of vertigo, fluctuating hearing loss, tinnitus, and aural fullness. It results from abnormal fluid accumulation in the inner ear, known as endolymphatic hydrops, affecting both auditory and vestibular functions. Early recognition and proper management are essential to reduce the impact on quality of life.

Etiology

The exact cause of Meniere’s disease is not fully understood, but it is generally classified into primary (idiopathic) and secondary forms based on the underlying origin.

Idiopathic Causes (Primary Meniere’s Disease)

• Unknown origin with characteristic clinical features
• Likely multifactorial with genetic and environmental contributions

Secondary Causes

Secondary Meniere’s disease occurs due to identifiable conditions that affect inner ear function or fluid dynamics.

• Viral infections, such as vestibular neuritis or herpes simplex virus
• Autoimmune inner ear disease
• Trauma or head injury affecting the labyrinth
• Allergic reactions impacting the inner ear
• Vascular disorders impairing cochlear or vestibular blood supply

Pathophysiology

The hallmark of Meniere’s disease is endolymphatic hydrops, an excessive accumulation of endolymph within the membranous labyrinth. This fluid imbalance leads to dysfunction in both the auditory and vestibular systems.

• Increased endolymph volume distends the membranous labyrinth, disrupting normal cochlear and vestibular function.
• Distortion of the cochlear hair cells leads to fluctuating sensorineural hearing loss and tinnitus.
• Disturbance of vestibular hair cells and semicircular canals causes episodic vertigo, imbalance, and nausea.
• Chronic hydrops may result in permanent hearing loss and progressive vestibular dysfunction over time.

Clinical Features

Auditory Manifestations

Meniere’s disease primarily affects hearing, presenting with fluctuating symptoms.

• Fluctuating Sensorineural Hearing Loss: Usually affects low to mid frequencies and may progress over time.
• Tinnitus: Ringing, buzzing, or roaring sound in the affected ear.
• Aural Fullness: Sensation of pressure or fullness in the ear, often preceding attacks.

Vestibular Manifestations

• Spontaneous Vertigo: Sudden episodes lasting minutes to hours, often severe and disabling.
• Nausea and Vomiting: Associated with vertigo attacks due to vestibular dysfunction.
• Imbalance: Unsteady gait or postural instability between episodes.

Other Symptoms

• Headache and motion sensitivity during attacks
• Psychological impact, including anxiety and fear of attacks

Diagnostic Evaluation

Clinical Criteria

Diagnosis of Meniere’s disease is primarily clinical, based on the combination of characteristic symptoms and audiometric findings.

• Recurrent spontaneous vertigo episodes lasting 20 minutes to 12 hours
• Documented fluctuating sensorineural hearing loss
• Tinnitus or aural fullness in the affected ear

Laboratory and Audiometric Tests

• Pure tone audiometry to evaluate hearing thresholds and monitor progression
• Electrocochleography (ECoG) to detect endolymphatic hydrops
• Vestibular function tests such as caloric testing, vestibular-evoked myogenic potentials (VEMP), and videonystagmography (VNG)

Imaging Studies

• MRI to rule out vestibular schwannoma or other retrocochlear pathology
• High-resolution CT to identify structural anomalies of the temporal bone if suspected

Management and Treatment

Conservative Measures

Initial management of Meniere’s disease focuses on lifestyle modifications and medical therapy to reduce the frequency and severity of attacks.

• Dietary modifications, including a low-salt diet to reduce endolymphatic pressure
• Diuretics to manage fluid balance within the inner ear
• Vestibular suppressants such as meclizine or dimenhydrinate during acute vertigo episodes
• Lifestyle adjustments including stress management, avoidance of known triggers, and adequate sleep

Interventional Procedures

For patients with refractory symptoms despite conservative therapy, targeted interventions may be considered.

• Intratympanic corticosteroid injections to reduce inner ear inflammation
• Intratympanic gentamicin injections for selective vestibular ablation in severe cases
• Endolymphatic sac decompression or shunt surgery to relieve hydrops

Surgical Management

Surgery is reserved for patients with debilitating symptoms not controlled by medical or interventional therapy.

• Labyrinthectomy for complete destruction of vestibular function in the affected ear, typically used in non-serviceable hearing
• Vestibular nerve section (neurectomy) to selectively cut vestibular nerve fibers while preserving hearing in suitable candidates

Complications

Meniere’s disease can lead to both sensory and functional complications due to recurrent vestibular and auditory disturbances.

• Permanent sensorineural hearing loss in the affected ear
• Chronic imbalance, increasing the risk of falls and injury
• Psychological effects including anxiety, depression, and fear of vertigo attacks
• Side effects from long-term use of medications such as vestibular suppressants or diuretics

Prognosis

The prognosis of Meniere’s disease varies among patients and depends on disease severity, response to treatment, and frequency of attacks. While some patients experience long periods of remission, others may develop progressive hearing loss and chronic vestibular dysfunction.

• Natural Course: Symptoms often fluctuate, with intermittent vertigo episodes and periods of relative stability.
• Factors Influencing Prognosis: Early diagnosis, adherence to treatment, and control of fluid balance improve outcomes.
• Long-Term Outcomes: Progressive hearing loss may occur in the affected ear, while balance function may stabilize with treatment or compensation.

Prevention and Monitoring

Preventive strategies and regular monitoring are essential to reduce attack frequency, detect progression, and manage comorbidities.

• Adherence to dietary restrictions, such as low-salt intake, to minimize fluid retention
• Stress management and avoidance of known triggers for vertigo attacks
• Regular audiometric and vestibular evaluations to monitor hearing and balance function
• Early intervention when symptoms worsen or new complications develop

References

1. Merchant SN, Adams JC, Nadol JB Jr. Pathophysiology of Meniere’s syndrome: Are symptoms caused by endolymphatic hydrops? Otol Neurotol. 2005;26(1):74-81.
2. Nakashima T, Pyykko I, Arroll MA, et al. Meniere’s disease. Nat Rev Dis Primers. 2016;2:16028.
3. Committee on Hearing and Equilibrium. Guidelines for the diagnosis and evaluation of therapy in Meniere’s disease. Otolaryngol Head Neck Surg. 1995;113(3):181-185.
4. Schuknecht HF. Pathology of Meniere’s Disease. Ann Otol Rhinol Laryngol. 1993;102(1):8-20.
5. American Academy of Otolaryngology–Head and Neck Surgery Foundation. Meniere’s Disease. [Internet]. 2021 [cited 2025 Oct 7]. Available from: https://www.entnet.org/content/menieres-disease
6. Minor LB. Meniere’s Disease. Continuum (Minneap Minn). 2014;20(5):1228-1240.
7. Spiegel JH, Kheradmand A. Diagnosis and Management of Meniere’s Disease. Curr Opin Otolaryngol Head Neck Surg. 2018;26(5):371-378.