Abstract: Objective To analyze the audiological characteristics of the cochlear implanted patients with large vestibular aqueduct syndrome(LVAS) and investigate the effects of the age of final diagnosis of LVAS on the postoperative results and the value of early diagnosis. Methods One hundred and twelve patients with LVAS who received cochlear implants between the year of 2000 and 2008 at the Cochlear Implant Center of Beijing Tongren Hospital were investigated in this study. The audiological results were assessed by using Infant-toddle Meaningful Auditory Integration Scale(IT-MAIS) or Meaningful Auditory Integration Scale(MAIS) questionnaire or Nijmegen Cochlear Implantation Questionnaire(NCIQ) depending on the age of the patients. Results 1 The average age of onset of hearing loss(HL) was(2.1±1.76) years old. The average age of final diagnosis of LVAS was(7.0±7.34) years old. There is a significant delay from the onset to final diagnosis of LVAS. 2 In the preoperatively diagnosed group(group A) of 50(44.6%) patients, the average age of HL onset was 2.0 years old, and both the average age of final diagnosis of LVAS and the average age of operation were 9.0 years old. In the non-preoperatively diagnosed group(group B) of 62 patients(55.4%), the average age of HL onset was 1.5 years old, and the average age of final diagnosis of LVAS and the average age of implantation were 2.3 and 4.0 years old, respectively. 3 One hundred and twelve patients suffered from profound sensorineural hearing losses. There was no significant difference in the speech communication ability between group A and B. 4 For 18 adult patients, the scores of NCIQ were significantly different between the group A and B(P=0.029); For the 16 patients under the age of 7 years old, there was no significant difference in the ability of MAIS questionnaire between the two groups during the first two months following implantation. Conclusion The final diagnosis of LVAS was much delayed from the onset of HL. Early diagnosis of LVAS may contribute to the protection of the residual hearing and benefit for auditory rehabilitation after cochlear implantation.

Key words: large vestibular aqueduct syndrome, hearing loss, cochlear implants, questionnaire