Skip to main content
Erschienen in: European Archives of Oto-Rhino-Laryngology 12/2018

26.09.2018 | Otology

Evaluation of saccular and inferior vestibular nerve function in children with auditory neuropathy spectrum disorder

verfasst von: Mohamed Mohamed EL-Badry, Reham Gamal, Amira Fawzy

Erschienen in: European Archives of Oto-Rhino-Laryngology | Ausgabe 12/2018

Einloggen, um Zugang zu erhalten

Abstract

Purpose

The aim of the current work was to evaluate the function of the saccule and inferior vestibular nerve in children with auditory neuropathy spectrum disorder (ANSD) by recording the cervical-evoked myogenic potentials (C-VEMP) on those children and to compare C-VEMP results in ANSD children of pre-lingual onset to those in ANSD children of post-lingual onset.

Methods

The study included 38 ANSD children of pre-lingual onset, 16 ANSD children of post-lingual onset, and 20 control children. All participant children were subjected to C-VEMP testing using 500 Hz tone burst stimuli.

Results

The vast majority of ANSD children of pre-lingual onset (35 out of 38; 92.1%) had bilateral intact C-VEMP response with C-VEMP parameters (amplitude, asymmetric ratio, latency, and inter-aural latency difference) that were not statistically different than those in the control children. Only three children had bilateral absent C-VEMP response. On the other hand, the majority of ANSD children of post-lingual onset (11 out of 16; 68.75%) had bilateral absent C-VEMP response. The remaining five children had bilateral intact C-VEMP response with C-VEMP parameters that were not statistically different than those in the control children.

Conclusions

The pathology of ANSD spares the saccule and inferior vestibular nerve in the vast majority of ANSD children of pre-lingual onset, while it involves them in the majority of ANSD of post-lingual onset reflecting different site(s) of lesion between the two ANSD categories. Such results have important clinical implications as regards to the management of ANSD in children.
Literatur
1.
Zurück zum Zitat Lee JS, McPherson B, Yuen KC, Wong LL (2001) Screening for auditory neuropathy in a school for hearing impaired children. Int J Pediatr Otorhinolaryngol 61:39–46CrossRef Lee JS, McPherson B, Yuen KC, Wong LL (2001) Screening for auditory neuropathy in a school for hearing impaired children. Int J Pediatr Otorhinolaryngol 61:39–46CrossRef
2.
Zurück zum Zitat Sininger YS (2002) Identification of auditory neuropathy in infants and children. Semin Hear 23:193–200CrossRef Sininger YS (2002) Identification of auditory neuropathy in infants and children. Semin Hear 23:193–200CrossRef
3.
Zurück zum Zitat Talaat HS, Kabel AH, Samy HM, El-Badry MM (2009) Prevalence of auditory neuropathy (AN) among infants and young children with severe to profound hearing loss. Int J Pediatr Otorhinolaryngol 73:937–939CrossRef Talaat HS, Kabel AH, Samy HM, El-Badry MM (2009) Prevalence of auditory neuropathy (AN) among infants and young children with severe to profound hearing loss. Int J Pediatr Otorhinolaryngol 73:937–939CrossRef
4.
Zurück zum Zitat Bielecki I, Horbulewiczt A, Wolan T (2012) Prevalence and risk factors for auditory neuropathy spectrum disorder in a screened population at risk for hearing loss. Int J Pediatr Otorhinolaryngol 76:1668–1670CrossRef Bielecki I, Horbulewiczt A, Wolan T (2012) Prevalence and risk factors for auditory neuropathy spectrum disorder in a screened population at risk for hearing loss. Int J Pediatr Otorhinolaryngol 76:1668–1670CrossRef
5.
Zurück zum Zitat Varga R, Kelley PM, Keats BJ, Starr A, Leal SM, Cohn E et al (2003) Non-syndromic recessive auditory neuropathy is the result of mutation in the otoferlin (OTOF) gene. J Med 40:45–50 Varga R, Kelley PM, Keats BJ, Starr A, Leal SM, Cohn E et al (2003) Non-syndromic recessive auditory neuropathy is the result of mutation in the otoferlin (OTOF) gene. J Med 40:45–50
6.
Zurück zum Zitat Sininger Y, Oba S (2001) Patients with auditory neuropathy: who are they and what can they hear? In: Sininger A, Starr A (eds) Auditory neuropathy: a perspective on hearing disorders. Singular/Thomson Learning, San Diego, pp 15–23 Sininger Y, Oba S (2001) Patients with auditory neuropathy: who are they and what can they hear? In: Sininger A, Starr A (eds) Auditory neuropathy: a perspective on hearing disorders. Singular/Thomson Learning, San Diego, pp 15–23
7.
Zurück zum Zitat Star A, Zeng FG, Michalewski HJ, Moser T (2008) Perspectives on auditory neuropathy: disorder of inner hair cell, auditory nerve, and their synapse. In: Basbaum AI, Kaneko A, Shephered GM, Dallos P, Oertel D (eds) The senses: a comprehensive reference, audition, vol 3. Academic Press, San Diego, pp 397–412CrossRef Star A, Zeng FG, Michalewski HJ, Moser T (2008) Perspectives on auditory neuropathy: disorder of inner hair cell, auditory nerve, and their synapse. In: Basbaum AI, Kaneko A, Shephered GM, Dallos P, Oertel D (eds) The senses: a comprehensive reference, audition, vol 3. Academic Press, San Diego, pp 397–412CrossRef
8.
Zurück zum Zitat Soliman S (1987) Low frequency sensorineural hearing loss: a syndrome. Audiology 26:332–338CrossRef Soliman S (1987) Low frequency sensorineural hearing loss: a syndrome. Audiology 26:332–338CrossRef
9.
Zurück zum Zitat Starr A, Picton TW, Sininger Y, Hood LJ, Berlin CI (1996) Auditory neuropathy. Brain 119:741–753CrossRef Starr A, Picton TW, Sininger Y, Hood LJ, Berlin CI (1996) Auditory neuropathy. Brain 119:741–753CrossRef
10.
Zurück zum Zitat Berlin CI, Hood LJ, Morlet T, Wilensky D, Li L, Mattingly KR et al (2010) Multi-site diagnosis and management with 260 patients with Auditory Neuropathy/Auditory Dys-synchrony (Auditory Neuropathy Spectrum Disorder). Int J Audiol 49:30–43CrossRef Berlin CI, Hood LJ, Morlet T, Wilensky D, Li L, Mattingly KR et al (2010) Multi-site diagnosis and management with 260 patients with Auditory Neuropathy/Auditory Dys-synchrony (Auditory Neuropathy Spectrum Disorder). Int J Audiol 49:30–43CrossRef
11.
Zurück zum Zitat Versino M, Colnaghi S, Callieco R, Cosi V (2001) Vestibular evoked myogenic potentials: test–retest reliability. Funct Neurol 16:299–309PubMed Versino M, Colnaghi S, Callieco R, Cosi V (2001) Vestibular evoked myogenic potentials: test–retest reliability. Funct Neurol 16:299–309PubMed
12.
Zurück zum Zitat Akin FW, Murnane OD (2004) Vestibular evoked myogenic potentials. Insight in practice: clinical topics in otoneurology, pp 1–7 Akin FW, Murnane OD (2004) Vestibular evoked myogenic potentials. Insight in practice: clinical topics in otoneurology, pp 1–7
13.
Zurück zum Zitat Zhou G, Kenna MA, Stevens K, Licameli G (2009) Assessment of saccular function in children with sensorineural hearing loss. Arch Otolaryngol Head Neck Surg 135(1):40–44CrossRef Zhou G, Kenna MA, Stevens K, Licameli G (2009) Assessment of saccular function in children with sensorineural hearing loss. Arch Otolaryngol Head Neck Surg 135(1):40–44CrossRef
14.
Zurück zum Zitat Singh S, Gupta RK, Kumar P (2012) Vestibular evoked myogenic potentials in children with sensorineural hearing loss. Int J Pediatr Otorhinolaryngol 76:1308–1311CrossRef Singh S, Gupta RK, Kumar P (2012) Vestibular evoked myogenic potentials in children with sensorineural hearing loss. Int J Pediatr Otorhinolaryngol 76:1308–1311CrossRef
15.
Zurück zum Zitat Sazgar AA, Yazdani N, Rezazdeh N, Yazdi AK (2010) Vestibular evoked myogenic potential (VEMP) in patients with auditory neuropathy: auditory neuropathy or audiovestibular neuropathy? Acta Otolaryngol 130:1130–1134CrossRef Sazgar AA, Yazdani N, Rezazdeh N, Yazdi AK (2010) Vestibular evoked myogenic potential (VEMP) in patients with auditory neuropathy: auditory neuropathy or audiovestibular neuropathy? Acta Otolaryngol 130:1130–1134CrossRef
16.
Zurück zum Zitat Sujeet KS, Niraj KS, Animesh B, Rajeshwari G, Sharanya R (2014) Cervical vestibular evoked myogenic potentials and caloric test results in individuals with auditory neuropathy spectrum disorders. J Vestib Res 24(4):313–323PubMed Sujeet KS, Niraj KS, Animesh B, Rajeshwari G, Sharanya R (2014) Cervical vestibular evoked myogenic potentials and caloric test results in individuals with auditory neuropathy spectrum disorders. J Vestib Res 24(4):313–323PubMed
17.
Zurück zum Zitat Akdogan O, Selcuk A, Ozcan I, Dere H (2008) Vestibular nerve functions in children with auditory neuropathy. Int J Pediatr Otorhinolaryngol 72(3):415–419CrossRef Akdogan O, Selcuk A, Ozcan I, Dere H (2008) Vestibular nerve functions in children with auditory neuropathy. Int J Pediatr Otorhinolaryngol 72(3):415–419CrossRef
18.
Zurück zum Zitat Rosengren SM, Welgampola MS, Colebatch JG (2010) Vestibular evoked myogenic potentials: past, present and future. Clin Neurophysiol 121:636–651CrossRef Rosengren SM, Welgampola MS, Colebatch JG (2010) Vestibular evoked myogenic potentials: past, present and future. Clin Neurophysiol 121:636–651CrossRef
19.
Zurück zum Zitat Todt L, Goepel F, Ernst A (2008) Loss of saccular function after cochlear implantation: the diagnostic impact of intracochlear electrically elicited vestibular evoked myogenic potentials. Audiol Neurotol 13:186–192 Todt L, Goepel F, Ernst A (2008) Loss of saccular function after cochlear implantation: the diagnostic impact of intracochlear electrically elicited vestibular evoked myogenic potentials. Audiol Neurotol 13:186–192
20.
Zurück zum Zitat Licameli G, Zhou G, Kenna MA (2009) Disturbance of vestibular function attributable to cochlear implantation in children. Laryngoscope 119(4):740–745CrossRef Licameli G, Zhou G, Kenna MA (2009) Disturbance of vestibular function attributable to cochlear implantation in children. Laryngoscope 119(4):740–745CrossRef
21.
Zurück zum Zitat Starr A, Michalewski HJ, Zeng FG, Fujikawa-Brooks S, Linthicum F, Kim CS et al (2003) Pathology and physiology of auditory neuropathy with a novel mutation in the MPZ Gene (Tyr145-Ser). Brain 126:1604–1619CrossRef Starr A, Michalewski HJ, Zeng FG, Fujikawa-Brooks S, Linthicum F, Kim CS et al (2003) Pathology and physiology of auditory neuropathy with a novel mutation in the MPZ Gene (Tyr145-Ser). Brain 126:1604–1619CrossRef
22.
Zurück zum Zitat Nash R, Veness J, Wyatt M, Raglan E, Rajput K (2014) Vestibular function in children with auditory neuropathy spectrum disorder. Int J Pediatr Otorhinolaryngol 78:1269–1273CrossRef Nash R, Veness J, Wyatt M, Raglan E, Rajput K (2014) Vestibular function in children with auditory neuropathy spectrum disorder. Int J Pediatr Otorhinolaryngol 78:1269–1273CrossRef
23.
Zurück zum Zitat Watson SR, Colebatch JG (1998) Vestibulocollic reflexes evoked by short-duration galvanic stimulation in man. J Physiol 513(Pt 2):587–597CrossRef Watson SR, Colebatch JG (1998) Vestibulocollic reflexes evoked by short-duration galvanic stimulation in man. J Physiol 513(Pt 2):587–597CrossRef
Metadaten
Titel
Evaluation of saccular and inferior vestibular nerve function in children with auditory neuropathy spectrum disorder
verfasst von
Mohamed Mohamed EL-Badry
Reham Gamal
Amira Fawzy
Publikationsdatum
26.09.2018
Verlag
Springer Berlin Heidelberg
Erschienen in
European Archives of Oto-Rhino-Laryngology / Ausgabe 12/2018
Print ISSN: 0937-4477
Elektronische ISSN: 1434-4726
DOI
https://doi.org/10.1007/s00405-018-5149-3

Weitere Artikel der Ausgabe 12/2018

European Archives of Oto-Rhino-Laryngology 12/2018 Zur Ausgabe

Update HNO

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert – ganz bequem per eMail.