Diagnosis of Auditory Neuropathy (AN) in Child Neurology

Abstract

Discrepancy of normal hearing and auditory brainstem response (ABR) abnormality has been a focus of attention since the early stages of the introduction of ABR to clinical medicine. The concept of auditory neuropathy (AN) seems to be in chaos because AN should be a syndrome with various etiologies and clinical features with mutual discrepancy in neurophysiological findings of ABR and otoacoustic emission (OAE). In this situation, we have found three patients with almost no ABR and normal OAE in our child neurology clinic. Among these three patients, the pure type of AN was diagnosed only in one patient, and for the other two patients the diagnoses were “fluctuating hearing loss, episodic headache, and stroke with platelet hyperaggregability” and “alternating hemiplegia of childhood (AHC). ” Thus, we would like to recommend that patients who have pure auditory nerve symptoms and are suspected of localized pathology in the auditory nerve should be named as having “auditory nerve disease (AND) ” and patients with other complicated physical symptoms should be diagnosed as “auditory neuropathy (AN) ” as the special symptom of the underlying diseases.