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Posterior Semicircular Canal Approach for Inner Ear Gene Delivery in Neonatal Mouse

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Isolated lateral semicircular canal aplasia: Functional consequences.

G Michel¹, F Espitalier¹, A-S Delemazure²

¹Service d'ORL et de chirurgie cervico-faciale, hôpital Hôtel-Dieu, CHU, 1, place A.-Ricordeau, BP 1005, 44093 Nantes cedex 01, France.

European Annals of Otorhinolaryngology, Head and Neck Diseases

|September 22, 2015

Summary

Isolated lateral semicircular canal aplasia, though appearing isolated on imaging, can present with functional inner ear deficits. Comprehensive vestibular testing is crucial even for incidental findings.

Keywords:

Semicircular canals Sensorineural hearing loss Tinnitus Vestibular labyrinth

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Area of Science:

Otolaryngology
Neuroscience
Developmental Biology

Background:

Lateral semicircular canal aplasia is a rare inner ear malformation.
Often associated with other vestibular and cochlear abnormalities within congenital syndromes.
This case highlights isolated aplasia without typical vestibular symptoms.

Observation:

A 20-year-old man presented with unilateral tinnitus and moderate sensorineural hearing loss.
MRI revealed isolated aplasia of the right lateral semicircular canal.
Videonystagmography showed right hyporeflexia, and vestibular evoked myogenic potentials were absent on the right.

Findings:

Despite isolated imaging findings, functional tests indicated widespread inner ear dysfunction.
Evidence of cochlear, semicircular canal, and otolithic system compromise was observed.
These functional deficits suggest a potential developmental disorder of the membranous labyrinth.

Implications:

Functional investigations are essential for isolated semicircular canal aplasia.
Even incidental findings warrant thorough vestibular assessment.
This case underscores the importance of excluding broader labyrinthine lesions.