Looking at a missing cochlea: cochlear aplasia or labyrinth ossification?

Abstract

Radiological imaging can reveal crucial information when dealing with single-side deafness. In early childhood, this condition is a challenging task for any clinician due to poor reliability of the patient at audiological testing. Moreover, clinical symptoms of unilateral hearing loss are blurred, even if it can severally affect quality of life, especially in scholastic age. Audiological evaluation, characterization of pathology onset and identification of rehabilitation options are consequently difficult to establish. We report the case of a 6-year-old girl who was found to have unilateral, right-sided, hearing loss. Both CT and MRI showed absent anterior labyrinth raising concerns about a congenital or acquired aetiology. The present paper showcases the main radiological and clinical clues that drive the diagnosis and help in defining both the timing and the possible causes of hearing loss in an emblematic case of unrecognized single-side deafness.

Keywords:

• Audiology
• otology
• single-side deafness
• CT
• MRI
• cochlear aplasia
• labyrinth ossification

Disclosure statement

No potential conflict of interest was reported by the authors.