title: Unilateral auditory neuropathy spectrum disorder.
abstract: CONCLUSIONS
  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. 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.
  
  
  OBJECTIVE
  To identify the clinical characteristics of patients with UANSD.
  
  
  METHODS
  This was a retrospective study involving 14 patients diagnosed as having UANSD between 2004 and 2010 in the Chinese PLA Hospital.
  
  
  RESULTS
  In all, 50% of the cases were males (1:1 sex ratio) and the average age of onset was 4.1 years. Of the 14 affected ears with UANSD in these cases, 6 were left-sided, while 8 were right-sided. Of the 14 contralateral ears, 4 presented with sensorineural hearing loss, while the other 10 showed normal hearing. 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. ABRs were absent in the 14 affected ears, while the OAEs, and/or CM were present.
authors.affiliation: Department of Otolaryngology/Head and Neck Surgery, Chinese PLA Institute of Otolaryngology, Chinese PLA General Hospital, Beijing, China.
authors.forename: Qiu-Jing
authors.initials: QJ
authors.lastname: Zhang
authors.forename: Lan
authors.initials: L
authors.lastname: Lan
authors.forename: Wei
authors.initials: W
authors.lastname: Shi
authors.forename: Da-Yong
authors.initials: DY
authors.lastname: Wang
authors.forename: Yue
authors.initials: Y
authors.lastname: Qi
authors.forename: Liang
authors.initials: L
authors.lastname: Zong
authors.forename: Qian
authors.initials: Q
authors.lastname: Li
authors.forename: Hui
authors.initials: H
authors.lastname: Wang
authors.forename: Hai-Na
authors.initials: HN
authors.lastname: Ding
authors.forename: Na
authors.initials: N
authors.lastname: Li
authors.forename: Bing
authors.initials: B
authors.lastname: Han
authors.forename: Qiu-Ju
authors.initials: QJ
authors.lastname: Wang
mesh_terms: D001309:Auditory Threshold / Q000502:physiology; D002648:Child; D002675:Child, Preschool; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology*; D005260:Female; D005500:Follow-Up Studies; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D006801:Humans; D007223:Infant; D008297:Male; D017084:Otoacoustic Emissions, Spontaneous / Q000502:physiology*; D012189:Retrospective Studies; D000160:Vestibulocochlear Nerve Diseases / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology