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Assessment of cochlear implantation outcome in patients with enlarged vestibular aqueduct syndrome



Theoverall outcome of cochlear implantation (CI) in patients with enlarged vestibular aqueduct (EVA) was comparable to other CI users. However, there were various concerns regarding surgical issues that may affect postoperative outcome. Moreover, exact timing for CI is still a matter of debate owing to the fluctuating nature of the disorder.


This study aimed to investigate speech and language outcome in patients with EVA who had undergone CI.

Patients and Methods

This prospective study included 48 participants who underwent CI. The participants’ age ranged from 4 to 28 years. The participants were divided according to preoperative radiological studies of petrous temporal bone into isolated EVA, EVA-IPII (incomplete partition type II), and control groups. Patients’ records were revised for preoperative investigations as well as available intraoperative data. Aided hearing thresholds, auditory skills, and aided speech perception abilities were all assessed postoperatively in all participants.


No major surgical complications were found in patients with EVA. Speech and language development in EVA group was comparable to that of control group.


CI outcomes in patients with EVA regarding surgical issues, auditory benefit, and speech perception abilities are generally good.


  1. 1

    Madden C, Halsted M, Benton C, Greinwald J, Choo D. Enlarged vestibular aqueduct syndrome in the pediatric population. Otol Neurotol 2003; 24:625–632.

    PubMed  Google Scholar 

  2. 2

    Gopen Q, Zhou G, Whittemore K, Kenna M. Enlarged vestibular aqueduct: review of controversial aspects. Laryngoscope 2011; 121:1971–1978.

    PubMed  Google Scholar 

  3. 3

    Varghese CM, Scampion P, Das VK, Gillespie J, Umapathy D. Enlarged vestibular aqueduct in two male siblings. Dev Med Child Neurol 2002; 44:706–711.

    CAS  PubMed  Google Scholar 

  4. 4

    Walsh RM, Ayshford CA, Chavda SV, Proops DW. Large vestibular aqueduct syndrome. ORL J Otorhinolaryngol Relat Spec 1999; 61:41–44.

    CAS  PubMed  Google Scholar 

  5. 5

    Au G, Gibson W. Cochlear implantation in children with large vestibular aqueduct syndrome. Am J Otol 1999; 20:183–186.

    CAS  PubMed  Google Scholar 

  6. 6

    Zalzal GH, Tomaski SM, Vezina LG, Bjornsti P, Grundfast KM. Enlarged vestibular aqueduct and sensorineural hearing loss in childhood. Arch Otolaryngol Head Neck Surg 1995; 121:23–28.

    CAS  PubMed  Google Scholar 

  7. 7

    Jackler RK, De La Cruz A. The large vestibular aqueduct syndrome. Laryngoscope 1989; 99:1238–1242. Discussion 42–43.

    CAS  PubMed  Google Scholar 

  8. 8

    Pritchett C, Zwolan T, Huq F, Phillips A, Parmar H, Ibrahim M, et al. Variations in the cochlear implant experience in children with enlarged vestibular aqueduct. Laryngoscope 2015; 125:2169–2174.

    PubMed  Google Scholar 

  9. 9

    Tse KS, Chu KM, Chiu LF, Fan TW, Tsang T, Kwan TL. Congenital inner ear malformations. Hong Kong J Radiol 2011; 14:118–125.

    Google Scholar 

  10. 10

    Chen X, Liu B, Liu S, Mo L, Liu H. The development of auditory skills in infants with isolated large vestibular aqueduct syndrome after cochlear implantation. Int J Pediatr Otorhinolaryngol 2011; 75:943–947.

    PubMed  Google Scholar 

  11. 11

    Lee KH, Lee J, Isaacson B, Kutz JW, Roland PS. Cochlear implantation in children with enlarged vestibular aqueduct. Laryngoscope 2010; 120:1675–1681.

    PubMed  Google Scholar 

  12. 12

    Sennaroglu L. Cochlear implantation in inner ear malformations-a review article. Cochlear Implants Int 2009; 11:4–41.

    PubMed  Google Scholar 

  13. 13

    Dewan K, Wippold FJ2nd, Lieu JE. Enlarged vestibular aqueduct in pediatric sensorineural hearing loss. Otolaryngol Head Neck Surg 2009; 140:552–558.

    PubMed  PubMed Central  Google Scholar 

  14. 14

    Wu CM, Sun YS, Liu TC. Long-term speech perception of cochlear implantation in children with large vestibular aqueduct syndrome: how we do it. Clin Otolaryngol 2008; 33:472–475.

    PubMed  Google Scholar 

  15. 15

    Vassoler TM, Bergonse Gda F, Meira Junior S, Bevilacqua MC, Costa Filho OA. Cochlear implant and large vestibular aqueduct syndrome in children. Braz J Otorhinolaryngol 2008; 74:260–264.

    PubMed  Google Scholar 

  16. 16

    Incesulu A, Adapinar B, Kecik C. Cochlear implantation in cases with incomplete partition type III (X-linked anomaly). Eur Arch Otorhinolaryngol 2008; 265:1425–1430.

    PubMed  Google Scholar 

  17. 17

    Berrettini S, Forli F, Bogazzi F, Neri E, Salvatori L, Casani AP, et al. Large vestibular aqueduct syndrome: audiological, radiological, clinical, and genetic features. Am J Otolaryngol 2005; 26:363–371.

    PubMed  Google Scholar 

  18. 18

    Nowak KC, Messner AH. Isolated large vestibular aqueduct syndrome in a family. Ann Otol Rhinol Laryngol 2000; 109:40–44.

    CAS  PubMed  Google Scholar 

  19. 19

    Bent JP3rd, Chute P, Parisier SC. Cochlear implantation in children with enlarged vestibular aqueducts. Laryngoscope 1999; 109(Pt 1):1019–1022.

    PubMed  Google Scholar 

  20. 20

    Harker LA, Vanderheiden S, Veazey D, Gentile N, McCleary E. Multichannel cochlear implantation in children with large vestibular aqueduct syndrome. Ann Otol Rhinol Laryngol Suppl 1999; 177:39–43.

    CAS  PubMed  Google Scholar 

  21. 21

    Bianchin G, Polizzi V, Formigoni P, Russo C, Tribi L. Cerebrospinal fluid leak in cochlear implantation: enlarged cochlear versus enlarged vestibular aqueduct (common cavity excluded). Int J Otolaryngol 2016; 2016:6591684.

    PubMed  PubMed Central  Google Scholar 

  22. 22

    Ko HC, Liu TC, Lee LA, Chao WC, Tsou YT, Ng SH, et al. Timing of surgical intervention with cochlear implant in patients with large vestibular aqueduct syndrome. PLoS One 2013; 8:e81568.

    PubMed  PubMed Central  Google Scholar 

  23. 23

    Hazmi M, Ab Aziz A, Asma A. Familial pattern of large vestibular aqueduct syndrome in a Chinese family. Excli J 2013; 12:118–121.

    PubMed  PubMed Central  Google Scholar 

  24. 24

    M Robbins A, J Renshaw J, W Berry S. Meaningful Auditory Integration Scale. Evaluating meaningful auditory integration in profoundly deaf children; 1991. pp. 144–150.

  25. 25

    Soliman S, El-Mahallawy T. Simple speech test material as a predictor for speech reception threshold in preschool children. Cairo: Ain Shams university; 1984.

    Google Scholar 

  26. 26

    Soliman S. speech discrimination audiometry using Arabic phonetically balanced words. Ain shams Med J 1976; 27:27–30.

    Google Scholar 

  27. 27

    Soliman S, Abd El-Hady M, Saad M, Kolkaila E. Development and study of Arabic word intelligibility by picture identification (WIPI) in normal hearing Egyptian children. Sci J Al-Azhar Univ Med Fac Girls 1987; 5:6.

    Google Scholar 

  28. 28

    El Kholi W. Development of an Arabic pediatric speech perception test battery. Egypt J Otolaryngol 2001; 18:1–16.

    Google Scholar 

  29. 29

    El Danasoury I, El Kholi W, Morgan A. The Arabic low verbal early speech perception test: development and application. Sci J Al-Azhar Med Fac 2006; 27:1187–1201.

    Google Scholar 

  30. 30

    Boothroyd A. Auditory perception of speech by subjects with sensorineural hearing loss. J Speech Hear Res 1984; 27:134–144.

    CAS  PubMed  Google Scholar 

  31. 31

    Talaat M. Electrical and speech outcome measures for the evaluation of cochlear implantees. Alexandria: University of Alexandria; 2009.

    Google Scholar 

  32. 32

    Kotby M, Khairy A, Barakah M, Rifaie N, Elshobary A. Language testing of Arabic speaking children. Proceedings of the XXIII World Congress of the International Association of Logopedics and Phoniatrics. 1995; Cairo.

  33. 33

    Kirkpatrick LA, Feeney BC. A simple guide to IBM SPSS statistics for version 20.0. Student ed. Belmont: Wadsworth, Cengage Learning 2013.

    Google Scholar 

  34. 34

    Kotz S, Balakrishnan N, Read CB, Vidakovic B. Encyclopedia of statistical sciences. 2nd ed., Hoboken: Wiley-Interscience 2006.

    Google Scholar 

  35. 35

    Valvassori GE, Clemis JD. The large vestibular aqueduct syndrome. Laryngoscope 1978; 88:723–728.

    CAS  PubMed  Google Scholar 

  36. 36

    Valvassori GE, Clemis JD. Abnormal vestibular aqueduct in cochleovestibular disorders. Adv Otorhinolaryngol 1978; 24:100–105.

    CAS  PubMed  Google Scholar 

  37. 37

    Pryor SP, Madeo AC, Reynolds JC. SLC26A /PDS genotype-phenotype correlation in hearing loss with enlargement of the vestibular aqueduct (EVA): evidence that Pendred syndrome and non-syndromic EVA are distinct clinical and genetic entities. J Med Genet 2005; 42:159–165.

    CAS  PubMed  PubMed Central  Google Scholar 

  38. 38

    Campbell C, Cucci RA, Prasad S, Green GE, Edeal JB, Galer CE, et al. Pendred syndrome, DFNB4, and PDS/SLC26A4 identification of eight novel mutations and possible genotype-phenotype correlations. Hum Mutat 2001; 17:403–411.

    CAS  PubMed  Google Scholar 

  39. 39

    Rah YC, Kim AR, Koo JW, Lee JH, Oh SH, Choi BY. Audiologic presentation of enlargement of the vestibular aqueduct according to the SLC26A4 genotypes. Laryngoscope 2015; 125:E216–E222.

    CAS  PubMed  Google Scholar 

  40. 40

    Zhou G, Gopen Q. Characteristics of vestibular evoked myogenic potentials in children with enlarged vestibular aqueduct. Laryngoscope 2011; 121:220–225.

    PubMed  Google Scholar 

  41. 41

    Yang CJ, Lavender V, Meinzen-Derr JK, Cohen AP, Youssif M, Castiglione M, et al. Vestibular pathology in children with enlarged vestibular aqueduct. Laryngoscope 2016; 126:2344–2350.

    PubMed  Google Scholar 

  42. 42

    Pradhananga R, Natarajan K, Devarasetty A, Kameswaran M. Cochlear Implantation in Isolated Large Vestibular Aqueduct Syndrome: Report of Three Cases and Literature Review. Int Arch Otorhinolaryngol 2015; 19:359–363.

    PubMed  Google Scholar 

  43. 43

    Takhellambam BS, Venkataramani N, Natarajan K, Kumari A. Cochlear implant in patients with incomplete partition type II: surgical considerations. Int J Otorhinolaryngol Head Neck Surg 2017; 3:1081–1086.

    Google Scholar 

  44. 44

    Berrettini S, Forli F, De Vito A, Bruschini L, Quaranta N. Cochlear implant in incomplete partition type I. Acta Otorhinolaryngol Ital 2013; 33:56–62.

    CAS  PubMed  PubMed Central  Google Scholar 

  45. 45

    Slattery WH3rd, Luxford WM. Cochlear implantation in the congenital malformed cochlea. Laryngoscope 1995; 105:1184–1187.

    PubMed  Google Scholar 

  46. 46

    Buchman CA, Teagle HF, Roush PA, Park LR, Hatch D, Woodard J, et al. Cochlear implantation in children with labyrinthine anomalies and cochlear nerve deficiency: implications for auditory brainstem implantation. Laryngoscope 2011; 121:1979–1988.

    PubMed  Google Scholar 

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Correspondence to Amira M. Al-Mahy MSc.

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Sobhy, O.A., Attallah, M.B., Abdou, R.M. et al. Assessment of cochlear implantation outcome in patients with enlarged vestibular aqueduct syndrome. Egypt J Otolaryngol 34, 316–325 (2018).

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  • cerebrospinal fluid gusher
  • cochlear implantation
  • enlarged vestibular aqueduct syndrome
  • speech outcome
  • vestibular aqueduct