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Unilateral auditory neuropathy spectrum disorder: retrocochlear lesion in disguise?

Published online by Cambridge University Press:  18 November 2014

A Mohammadi ,
P Walker  and
K Gardner-Berry
A Mohammadi*
Affiliation:
Department of Surgery, Hunter New England Area Health Service, New Lambton Heights, New South Wales, Australia
P Walker
Affiliation:
Paediatric Otolaryngology, John Hunter Children's Hospital, New Lambton Heights, New South Wales, Australia Department of Surgery, University of Newcastle, Callaghan, New South Wales, Australia Department of Paediatrics, University of Newcastle, Callaghan, New South Wales, Australia
K Gardner-Berry
Affiliation:
National Acoustic Laboratories, Sydney Cochlear Implant Centre, Gladesville, New South Wales, Australia
*
Address for correspondence: Dr Aydin Mohammadi, Department of Surgery, John Hunter Hospital, Lookout Road, New Lambton Heights, NSW 2305, Australia E-mail: [email protected]
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Abstract

Objective:

To investigate whether the aetiology for hearing impairment in neonates with unilateral auditory neuropathy spectrum disorder could be explained by structural abnormalities such as cochlear nerve aplasia, a cerebellopontine angle tumour or another identifiable lesion.

Methods:

In this prospective case series, 17 neonates were diagnosed with unilateral auditory neuropathy spectrum disorder on electrophysiological testing. Diagnostic audiology testing, including auditory brainstem response testing, was supplemented with computed tomography and/or magnetic resonance imaging.

Results:

Ten of the neonates (59 per cent) showed evidence for cochlear nerve aplasia. Of the remaining seven, four were shown to have another abnormality of the temporal bone on imaging. Only three neonates (18 per cent) were not diagnosed with cochlear nerve aplasia or another lesion. Three computed tomography scans were reported as normal, but subsequent magnetic resonance imaging revealed cochlear nerve aplasia.

Conclusion:

Auditory neuropathy spectrum disorder as a unilateral condition mandates further investigation for a definitive diagnosis. This series demonstrates that most neonates with unilateral auditory neuropathy spectrum disorder had pathology as visualised on computed tomography and/or magnetic resonance imaging scans. Magnetic resonance imaging is an appropriate first-line imaging modality.

Keywords

Auditory NeuropathyAuditory Dys-synchronyCochlear NerveAplasiaCochlear Microphonic PotentialsEvoked Potentials, Auditory, Brain Stem
Type
Main Articles
Information
The Journal of Laryngology & Otology , Volume 129 , Issue S1 , January 2015 , pp. S38 - S44
Copyright
Copyright © JLO (1984) Limited 2014 

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References

1Zhang, QJ, Lan, L, Shi, W, Wang, DY, Qi, Y, Zong, L et al. Unilateral auditory neuropathy spectrum disorder. Acta Otolaryngol 2012;132:72–9Google Scholar
2Berlin, CI, Morlet, T, Hood, LJ. Auditory neuropathy/dyssynchrony: its diagnosis and management. Pediatr Clin North Am 2003;50:331–40CrossRefGoogle ScholarPubMed
3Berlin, CI, Hood, LJ, Morlet, T, Wilensky, D, St John, P, Montgomery, E et al. Absent or elevated middle ear muscle reflexes in the presence of normal otoacoustic emissions: a universal finding in 136 cases of auditory neuropathy/dys-synchrony. J Am Acad Audiol 2005;16:546–53Google ScholarPubMed
4Kileny, P, Zwolan, T. Diagnostic audiology. In: Flint, P, Haughey, B. Lund, V. Niparko, J. Richardson, M. Robbins, K et al. , eds. Cummings Otolaryngology - Head and Neck Surgery, 5th edn.Philadelphia: Mosby Elsevier, 2010;1887–903CrossRefGoogle Scholar
5Shaffer, LA, Withnell, RH, Dhar, S, Lilly, DJ, Goodman, SS, Harmon, KM. Sources and mechanisms of DPOAE generation: implications for the prediction of auditory sensitivity. Ear Hear 2003;24:367–79CrossRefGoogle ScholarPubMed
6Withnell, RH. Brief report: the cochlear microphonic as an indication of outer hair cell function. Ear Hear 2001;22:75–7CrossRefGoogle ScholarPubMed
7Dallos, P. Comments on the differential-electrode technique. J Acoust Soc Am 1969;45:999–1007CrossRefGoogle Scholar
8Price, GR. Correspondence between cochlear microphonic sensitivity and behavioural threshold in the cat. J Acoust Soc Am 1971;49:1899–901CrossRefGoogle ScholarPubMed
9Patuzzi, RB, Yates, GK, Johnstone, BM. The origin of the low-frequency microphonic in the first cochlear turn of guinea-pig. Hear Res 1989;39:177–88CrossRefGoogle ScholarPubMed
10Lefebvre, P, Legatt, A. Assessment of central auditory function. In: Van De Water, T, Hinrich, S, eds. Otolaryngology: Basic Science and Clinical Review. New York: Thieme, 2006;363–7Google Scholar
11Bielecki, I, Horbulewicz, A, Wolan, T. Prevalence and risk factors for auditory neuropathy spectrum disorder in a screened newborn population at risk for hearing loss. Int J Pediatr Otorhinolaryngol 2012;76:1668–70CrossRefGoogle Scholar
12Mittal, R, Ramesh, AV, Panwar, SS, Nilkanthan, A, Nair, S, Mehra, PR. Auditory neuropathy spectrum disorder: its prevalence and audiological characteristics in an Indian tertiary care hospital. Int J Pediatr Otorhinolaryngol 2012;76:1351–4CrossRefGoogle Scholar
13Kirkim, G, Serbetcioglu, B, Erdag, TK, Kerim, C. The frequency of auditory neuropathy detected by universal newborn hearing screening program. Int J Pediatr Otorhinolaryngol 2008;72:1461–9CrossRefGoogle ScholarPubMed
14Warren, FM 3rd, Wiggins, RH 3rd, Pitt, C, Harnsberger, HR, Shelton, C. Apparent cochlear nerve aplasia: to implant or not to implant? Otol Neurotol 2010;31:1088–94CrossRefGoogle ScholarPubMed
15Colletti, V, Carner, M, Fiorino, F, Sacchetto, L, Miorelli, V, Orsi, A et al. Hearing restoration with auditory brainstem implant in three children with cochlear nerve aplasia. Otol Neurotol 2002;23:682–93CrossRefGoogle ScholarPubMed
16Jeong, S, Kim, L. Auditory neuropathy spectrum disorder: predictive value of radiologic studies and electrophysiologic tests on cochlear implant outcomes and its radiologic classification. Acta Otolaryngol 2013;133:714–21Google Scholar
17Walton, J, Gibson, W, Sanli, H, Prelog, K. Predicting cochlear implant outcomes in children with auditory neuropathy. Otol Neurotol 2008;29:302–9CrossRefGoogle ScholarPubMed
18Adunka, O, Jewells, V, Buchman, C. Value of computed tomography in the evaluation of children with cochlear nerve deficiency. Otol Neurotol 2007;28:597–604CrossRefGoogle ScholarPubMed