Objective: On the basis of previous systematic investigation on a Chinese pedigree with dominantly inherited auditory neuropathy, we aims to further search the deafness-causing genes (mutations) involved in this family. Methods: Whole-exome sequencing was conducted in three affected members and one spouse to determine the candidate genes related to the pedigree, and cosegregation analysis was performed on other members of the family. Finally, fifty normal-hearing individuals acting as controls were recruited for candidate variant detection. Results: A total of 41 variations were identified by whole-exome sequencing analysis. These variations were verified in the kernal pedigree members and 2 normal-hearing individuals by PCR-Sanger sequencing. The results showed that there was only ALOX15B gene co-segregated with the phenotype of the family. This gene mutation, however, was also detected in 2 of 50 normal-hearing individuals, which excluded the causative effect of ALOX15B mutation in this family. Conclusion:Whole-exome sequencing combined with Sanger sequencing does not reveal novel gene mutation in this family, which excludes the causative effects of Indels or mutations in coding sequence.