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Dissection of the Endolymphatic Sac from Mice
Published on: March 29, 2021
Enlarged vestibular aqueduct concomitant with endolymphatic hydrops.
Po-Yu Chen1, Yu-Fen Wang2, Yi-Ho Young3
1Departments of Otolaryngology, National Taiwan University Hospital Yun-Lin Branch, Yun-Lin, Taiwan.
Acta Oto-Laryngologica
|May 11, 2026
Summary
Enlarged vestibular aqueduct (EVA) often causes childhood hearing loss. This study found 57% of patients with large vestibular aqueduct syndrome (LVAS) also have endolymphatic hydrops (EH), identified via MRI.
Area of Science:
- Otolaryngology
- Pediatric Audiology
- Radiology
Background:
- Enlarged vestibular aqueduct (EVA) is a common cause of childhood sensorineural hearing loss (SNHL).
- Large vestibular aqueduct syndrome (LVAS) is a specific inner ear anomaly.
- Endolymphatic hydrops (EH) is a condition of fluid imbalance within the inner ear.
Purpose of the Study:
- To determine the prevalence of endolymphatic hydrops (EH) in patients diagnosed with large vestibular aqueduct syndrome (LVAS).
Main Methods:
- A cohort of 20 patients with congenital SNHL underwent comprehensive inner ear testing.
- Seven patients (12 ears) with diagnosed LVAS were included.
- Hydrops MRI was utilized to assess for the presence of EH.
Main Results:
- Inner ear test abnormalities varied, with audiometry at 100% and ocular VEMP at 58%.
- Hydrops MRI revealed EH in 57% of LVAS patients (4 out of 7), affecting both cochlea and vestibule.
- One patient undergoing sac and shunt surgery for LVAS experienced profound deafness and vertigo.
Conclusions:
- Hydrops MRI is effective for simultaneously detecting EVA and EH.
- A significant proportion (57%) of patients with LVAS exhibit concurrent EH.
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