ABRs were absent in the 14 affected ears, while the OAEs, and/or CM were present. The degree of hearing loss in the 14 affected ears varied, including moderate in 1, moderately severe in 4, severe in 5, and profound in 4. CClinical test, RResearch test, OOMIM, GGeneReviews, ClinVar. Hearing loss resulting from disorders of the central nervous system auditory pathways. Of the 14 contralateral ears, 4 presented with sensorineural hearing loss, while the other 10 showed normal hearing. A group of experts 4 thus decided to modify the term AN to ANSD-Auditory Neuropathy Spectrum Disorder to avoid a precise terminology being able to describe various and different dysfunctions, even if with common involvement of the 8 th nerve. Central hearing loss (68467004) Central deafness (68467004) Monarch Initiative: MONDO:0020707. Of the 14 affected ears with UANSD in these cases, 6 were left-sided, while 8 were right-sided. In all, 50% of the cases were males (1:1 sex ratio) and the average age of onset was 4.1 years. This was a retrospective study involving 14 patients diagnosed as having UANSD between 20 in the Chinese PLA Hospital. To identify the clinical characteristics of patients with UANSD. Abnormal auditory brainstem response (ABR) and preserved otoacoustic emissions (OAEs) and/or cochlear microphonics (CM) were important features to differentiate it from common sensorineural deafness and central nerve hearing loss. The majority of the patients with unilateral auditory neuropathy spectrum disorder (UANSD) were pediatric and mostly showed a great degree of hearing loss when diagnosed.
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