Skip to main content
SpringerLink
Log in

Audiological evaluation in children affected by myelomeningocele

  • Original Paper
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Purpose

The aim of the present study is to evaluate the auditory system in children affected by myelomeningocele and comparing the results with clinical neurological conditions.

Materials and methods

Forty-three children, aged between 7 and 26 years, affected by myelomeningocele were investigated by means of subjective tonal audiometry and objective impedance audiometry (tympanometry and acoustic stapedial reflex).

Results

Audiological evaluation showed an alteration in 32 patients (74 %%). Nine patients presented a mild hearing loss: bilateral in six cases (three sensorineural, one mixed, and two conductive) and unilateral in three cases (two mixed and one conductive). One patient had moderate unilateral conductive deafness and, finally another one severe unilateral sensorineural. Almost all patients with deafness were affected by myelomeningocele and Chiari II. Stapedial-cochlear reflex investigation showed an alteration in 30 patients (70 %): 9 of these also showed deafness while the remaining 21 was normal hearing. In these 30 patients, we demonstrated the presence of myelomeningocele, hydrocephalus, and Chiari II malformation in 21 subjects (70 %).

Conclusion

Otoneurological evaluation is important in myelomeningocele not only at the birth but also in the follow-up. It could have an important prognostic role for neurological impairment.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

Abbreviations

MMC:

Myelomeningocele

VEMPs:

Vestibular Evoked Myogenic Potentials

EAEPs:

Early Auditory Evoked Potentials

ASR:

Acoustic Stapedial Reflex

UNHS:

Universal New-born Hearing Screening

References

  1. Inchingolo R, Maresca G, Cacaci S, Ausili E, Paolucci V, Bonomo L, Romagnoli C, Rendeli C (2013) Post-natal ultrasound morpho-dynamic evaluation of mild fetal hydronephrosis: a new management. Eur Rev Med Pharmacol Sci 17(16):2232–2239

    CAS  PubMed  Google Scholar 

  2. Rendeli C, Ausili E, Tabacco F, Caliandro P, Aprile I, Tonali P, Salvaggio E, Padua L (2005) Assessment of health status in children with spina bifida. Spinal Cord 43(4):230–235

    Article  CAS  PubMed  Google Scholar 

  3. Padua L, Rendeli C, Ausili E, Aprile I, Caliandro P, Tonali P, Salvaggio E (2004) Relationship between the clinical-neurophysiologic pattern, disability, and quality of life in adolescents with spina bifida. J Child Neurol 19(12):952–957

    PubMed  Google Scholar 

  4. Picciotti PM, Fiorita A, Calò L, Battista M, Paolucci V, Ausili E, Massimi L, Rendeli C (2012) Vestibular evoked myogenic potentials in children affected by myelomeningocele. Childs Nerv Syst 28(10):1761–1765

    Article  PubMed  Google Scholar 

  5. Luigetti M, Losurdo A, Dittoni S, Testani E, Colicchio S, Gnoni V, Farina B, Scarano E, Zampino G, Mariotti P, Rendeli C, Di Rocco C, Massimi L, Della Marca G (2010) Improvement of obstructive sleep apneas caused by hydrocephalus associated with Chiari malformation Type II following surgery. J Neurosurg Pediatr 6(4):336–339

    Article  PubMed  Google Scholar 

  6. Fujii M, Tomita T, McLone DG, Grant JA, Mori K (1996) Natural course of brainstem auditory evoked potentials in infants less than 6 months old with asymptomatic meningomyelocele. Pediatr Neurosurg 25(5):227–232

    Article  CAS  PubMed  Google Scholar 

  7. Fujii M, Tomita T, McLone DG, Grant JA, Stack CV, Mori K (1997) Developmental normo-maturation of brainstem auditory evoked potentials in children with asymptomatic meningo-myelocele during the first year of life. Childs Nerv Syst 13(3):147–153

    Article  CAS  PubMed  Google Scholar 

  8. Worley G, Erwin CW, Schuster JM, Park Y, Boyko OB, Griebel ML, Weidman ER, Radtke RA, Oakes WJ (1994) BAEPs in infants with myelomeningocele and later development of Chiari II malformation-related brainstem dysfunction. Dev Med Child Neurol 36(8):707–715

    Article  CAS  PubMed  Google Scholar 

  9. Nishimura T, Mori K, Uchida Y, Ohira T, Tamura K (1991) Brain stem auditory-evoked potentials in meningomyelocele. Natural history of Chiari II malformations. Childs Nerv Syst 7(6):316–326

    Article  CAS  PubMed  Google Scholar 

  10. Mori K, Nishimura T (1995) Electrophysiological studies on brainstem function in patients with myelomeningocele. Pediatr Neurosurg 22(3):120–131

    Article  CAS  PubMed  Google Scholar 

  11. Lütschg J, Meyer E, Jeanneret-Iseli C, Kaiser G (1985) Brainstem auditory evoked potentials in meningomyelocele. Neuropediatrics 16(4):202–204

    Article  PubMed  Google Scholar 

  12. Liu XZ, Pandya A, Angeli S, Telischi FF, Arnos KS, Nance WE, Balkany T (2005) Audiological features of GJB2 (connexin 26) deafness. Ear Hearing 26:361–369

    Article  PubMed  Google Scholar 

  13. Taylor MJ, Boor R, Keenan NK, Rutka JT, Drake JM (1996) Brainstem auditory and visual evoked potentials in infants with myelomeningocele. Brain Dev 18:99–104

    Article  CAS  PubMed  Google Scholar 

  14. Paludetti G, Conti G, DI Nardo W, DE Corso E, Rolesi R, Picciotti PM, Fetoni AR (2012) Infant hearing loss: from diagnosis to therapy Official Report of XXI Conference of Italian Society of Pediatric Otorhinolaryngology. Acta Otorhinolaryngol Ital 32(6):347–370

    PubMed Central  CAS  PubMed  Google Scholar 

  15. Satzer D, Guillaume DJ (2014) Prognostic value of newborn hearing screening in patients with myelomeningocele. J Neurosurg Pediatr 14(5):495–500

    Article  PubMed  Google Scholar 

  16. Vinck A, Nijhuis-van der Sanden MW, Roeleveld NJ, Mullaart RA, Rotteveel JJ, Maassen BA (2010) Motor profile and cognitive functioning in children with spina bifida. Eur J Paediatr Neurol 14(1):86–92

    Article  PubMed  Google Scholar 

  17. Dennis M, Barnes MA (2010) The cognitive phenotype of spina bifida meningomyelocele. Dev Disabil Res Rev 16(1):31–39

    Article  PubMed Central  PubMed  Google Scholar 

  18. Koehler J, Schwarz M, Boor R, Hölker C, Hopf HC, Voth D, Urban PP, Ermert A (2000) Assessment of brainstem function in Chiari II malformation utilizing brainstem auditory evoked potentials (BAEP), blink reflex and masseter reflex. Brain Dev 22(7):417–420

    Article  CAS  PubMed  Google Scholar 

  19. Nishimura T, Mori K (1996) Blink reflex in meningomyelocele, with special reference to its usefulness in the evaluation of brainstem dysfunction. Childs Nerv Syst

Download references

Financial disclosure

This work has no financial support.

The authors do not have any financial interest in companies or other entities that have an interest in the information in the contribution.

Conflict of interest

The authors declare that they have no conflict of interest.

Author information

Authors and Affiliations

  1. Department of Otolaryngology Head and Neck Surgery and Paediatrics, Rome, Italy

    Pasqualina Maria Picciotti, M. Battista, M. Pandolfini & G. Paludetti

  2. Catholic University of Sacred Heart, Rome, Italy

    E. Ausili, C. Romagnoli & C. Rendeli

  3. Department of Otolaryngology, Catholic University of Sacred Heart, Largo A. Gemelli 8, 00168, Rome, Italy

    Pasqualina Maria Picciotti

Authors
  1. Pasqualina Maria Picciotti
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. M. Battista
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. M. Pandolfini
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. G. Paludetti
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. E. Ausili
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. C. Romagnoli
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. C. Rendeli
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Pasqualina Maria Picciotti.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Picciotti, P.M., Battista, M., Pandolfini, M. et al. Audiological evaluation in children affected by myelomeningocele. Childs Nerv Syst 31, 2321–2324 (2015). https://doi.org/10.1007/s00381-015-2898-9

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-015-2898-9

Keywords

Search

Navigation