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Updated: Apr 22, 2026

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In Vivo Morphometric Analysis of Human Cranial Nerves Using Magnetic Resonance Imaging in Menière's Disease Ears and Normal Hearing Ears
Published on: February 21, 2018
8.9K
Cochlear Neural Degeneration Is Widespread in Ménière's Disease: A Temporal Bone Study
Charanjeet Kaur1,2,3, Pei-Zhe Wu4, Andreas H Eckhard5,6,7
1Eaton-Peabody Laboratories, Massachusetts Eye and Ear, 243 Charles St., Boston, MA, 02114, USA. Charanjeet_Kaur@meei.harvard.edu.
Summary
Ménière
Area of Science:
- Otolaryngology
- Neuroscience
- Pathology
Background:
- Ménière's disease is characterized by endolymphatic hydrops.
- Patients with Ménière's disease exhibit poor word recognition disproportionate to hearing loss.
- The cochlear pathology underlying these symptoms is not fully understood.
Purpose of the Study:
- To characterize cochlear pathology in Ménière's disease.
- To compare Ménière's cochlear pathology with age-matched controls and unaffected contralateral ears.
- To evaluate the relationship between hydrops severity, clinical endotypes, and tissue degeneration.
Main Methods:
- Analysis of 97 human cochleas (43 Ménière's, 10 contralateral, 44 controls).
- Quantitative histopathology of hair cells, spiral ganglion cells, peripheral axons, stria vascularis, and spiral ligament fibrocytes.
- Assessment of endolymphatic hydrops, disease duration, and endolymphatic sac phenotype.
Main Results:
- Ménière's ears showed significant cochlear and saccular hydrops.
- Reduced hair cells and profound loss of peripheral axons were observed in Ménière's ears.
- Hydrops severity strongly correlated with cochlear neural degeneration.
- Contralateral ears showed strial and fibrocyte degeneration without hydrops.
- Disease duration and endolymphatic sac phenotype had minor effects on histopathology.
Conclusions:
- Profound cochlear neural degeneration is the primary cause of poor word recognition in Ménière's disease.
- Strial and spiral ligament atrophy in clinically unaffected ears suggest early lateral wall damage.
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