Hearing Loss Pattern in Endolymphatic Hydrops (Ménière's Disease)
The characteristic hearing loss pattern in endolymphatic hydrops (Ménière's disease) is a fluctuating low- to mid-frequency sensorineural hearing loss that, over time, progresses to involve all frequencies. 1
Initial Presentation
The hallmark audiometric finding in Ménière's disease is:
- Low- to mid-frequency sensorineural hearing loss that fluctuates in severity, documented on audiometry before, during, or after vertigo episodes 1
- The hearing loss is unilateral in most cases, though bilateral involvement can occur 1
- Fluctuation is a key diagnostic feature—hearing may improve between attacks early in the disease course 1, 2
Disease Progression Pattern
As Ménière's disease advances, the hearing loss pattern evolves:
- Initially affects low- to mid-frequencies with characteristic fluctuation 3, 4
- Eventually involves all frequencies as the disease progresses, losing its frequency-specific pattern 1, 3
- The hearing loss worsens over time despite treatment interventions, becoming more permanent and less fluctuating 4
- Progressive deterioration occurs even with medical management, as endolymphatic hydrops continues to develop longitudinally 5
Clinical Correlation
The hearing loss occurs alongside other characteristic features:
- Fluctuating aural symptoms including tinnitus and ear fullness in the affected ear 1
- Spontaneous vertigo attacks lasting 20 minutes to 12 hours 1, 3
- Physical examination and otoscopy are typically normal in sensorineural hearing loss, distinguishing it from conductive causes 2
Diagnostic Confirmation
Audiometry is essential for documenting the characteristic pattern:
- Pure tone audiometry demonstrates the low- to mid-frequency sensorineural pattern 2, 3
- Weber tuning fork test lateralizes to the unaffected ear in unilateral cases 2
- Serial audiograms are necessary to document fluctuation and progression 3, 4
Important Clinical Pitfall
Do not confuse early Ménière's disease with sudden sensorineural hearing loss (SSNHL). Ménière's disease is characterized by antecedent fluctuating hearing loss, whereas SSNHL typically occurs without prior fluctuation 1. The presence of fluctuating hearing loss in the history should raise suspicion for Ménière's disease rather than idiopathic SSNHL 1.