Cochlear nerve aplasia in a child with negative neonatal hearing screening test
DOI:
https://doi.org/10.34631/sporl.1010Keywords:
cochlear nerve, hearing loss, otoacoustic emissions, cochlear implant, auditory brainstem implantAbstract
Otoacoustic emissions (OAE) are sounds produced by a healthy cochlea and are used as a neonatal hearing screening test with good sensitivity and specificity, differentiating the ears with normal hearing from those with hearing loss. However, this test does not exclude a neural hearing loss.
The authors present a case of a 3-year-old male referred to an otorhinolaryngology consultation for recurrent otitis media. In the study carried out with complementary diagnostic tests, unilateral hearing loss of the left ear was detected, which motivated the study with magnetic resonance imaging, which in turn revealed aplasia of the left cochlear nerve.
The aplasia or hypoplasia of the cochlear nerve is an extremely rare congenital malformation, and may not be detected in neonatal hearing screening.
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