ArticleCureus2024
A Three-Year Follow-Up of a Patient With Large Vestibular Aqueduct Syndrome Who Underwent Bilateral Endolymphatic Duct Blockage Surgery.
Article in Cureus, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Large vestibular aqueduct syndrome (LVAS) is a congenital malformation characterized by an abnormally large vestibular aqueduct, diagnosed primarily via CT scans. Patients with LVAS often experience progressive hearing loss and recurrent vertigo, with treatment strategies mirroring those for Meniere's disease. Traditional surgical interventions such as endolymphatic sac decompression (ESD) are common; however, the efficacy of endolymphatic duct blockage (EDB) as an alternative still remains under investigation. We present the case of a female in her 20s who was diagnosed with Meniere's disease after presenting with hearing loss and recurrent vertigo since the age of 17. Despite conservative treatments and tympanostomy tube insertion, her symptoms worsened, prompting surgical intervention. CT scans revealed bilaterally enlarged vestibular aqueducts, which suggested LVAS. The patient underwent EDB combined with ESD on both ears, starting with the more affected right ear. The surgical approach involved mastoidectomy, exposure of the enlarged endolymphatic duct, placement of a 4 mm titanium clip followed by drainage of the endolymphatic duct. Over a three-year follow-up, the patient experienced no recurrence of dizziness or tinnitus. However, hearing in the right ear gradually deteriorated, suggesting the necessity of future cochlear implant surgery. The patient has returned to work and maintained stable vertigo control post-bilateral surgery. This case introduces a new surgical method of combining EDB with ESD. Three years post-surgery, the method has resulted in potentially stabilized hearing in the better ear while effectively managing vertigo. It may be a solution to the challenge of vertigo in LVAS. The combined EDB and ESD procedure shows possibility for vertigo management in LVAS patients, along with the potential to preserve hearing when performed before significant deterioration. Despite hearing loss progression in the initially worse ear, the patient's quality of life improved significantly, highlighting the procedure's viability as a treatment option.
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