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.

Keywords::

• Distortion product otoacoustic emission
• DPOAE
• ABR
• auditory brainstem response
• cochlear microphonics
• CM
• unilateral hearing loss

Acknowledgments

We thank the patients for their cooperation during this work. We also thank the technicians in the Clinical Auditory Medicine Centre at the Department of Otolaryngology Head and Neck Surgery, Chinese People's Liberation Army General Hospital for their technical support. This work was supported by grants from the National Natural Science Foundation of China, Key Program (grant nos 30830104 and 81120108009).

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.