Conflict of Diagnosis: Auditory Neuropathy Disorder or Cochlear Nerve Deficiency?

According to the Magnetic Resonance Imaging (MRI), when the diameter of the internal acoustic canal is narrow than the 5 mm or the diameter of the cochlear nerve canal is narrow than the 1.8 mm or the cochlear nerve is narrow than the facial nerve, cochlear nerve deficiency could be considered.

In the present study, audiological results of 7 children with auditory neuropathy spectrum disorder were evaluated. All children were diagnosed with auditory neuropathy spectrum disorder due to the auditory brainstem response (ABR) testing. Although the hearing thresholds showed differences among the children, all children diagnosed with cochlear nerve aplasia or hypoplasia on MRI.

It was recommended that in case of a cochlear microphonics in ABR testing, cochlear nerve deficiency should be excluded via MRI during the follow up.