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. 2010 Jul;31(5):780-8.
doi: 10.1097/mao.0b013e3181d8d528.

Imaging characteristics of children with auditory neuropathy spectrum disorder

Joseph P Roche  1 , Benjamin Y Huang, Mauricio Castillo, Marc K Bassim, Oliver F Adunka, Craig A Buchman
Affiliations

Imaging characteristics of children with auditory neuropathy spectrum disorder

Joseph P Roche et al. Otol Neurotol. 2010 Jul.

Abstract

Objective: To identify and define the imaging characteristics of children with auditory neuropathy spectrum disorder (ANSD).

Design: Retrospective medical records review and analysis of both temporal bone computed tomographic (CT) and magnetic resonance images (MRI) in children with a diagnosis of ANSD.

Setting: Tertiary referral center.

Patients: One hundred eighteen children with the electrophysiologic characteristics of ANSD with available imaging studies for review.

Interventions: Two neuroradiologists and a neurotologist reviewed each study, and consensus descriptions were established.

Main outcome measures: The type and number of imaging findings were tabulated.

Results: Sixty-eight (64%) MRIs revealed at least 1 imaging abnormality, whereas selective use of CT identified 23 (55%) with anomalies. The most prevalent MRI findings included cochlear nerve deficiency (n = 51; 28% of 183 nerves), brain abnormalities (n = 42; 40% of 106 brains), and prominent temporal horns (n = 33, 16% of 212 temporal lobes). The most prevalent CT finding from selective use of CT was cochlear dysplasia (n = 13; 31%).

Conclusion: Magnetic resonance imaging will identify many abnormalities in children with ANSD that are not readily discernable on CT. Specifically, both developmental and acquired abnormalities of the brain, posterior cranial fossa, and cochlear nerves are not uncommonly seen in this patient population. Inner ear anomalies are well delineated using either imaging modality. Because many of the central nervous system findings identified in this study using MRI can alter the treatment and prognosis for these children, we think that MRI should be the initial imaging study of choice for children with ANSD.

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Figures

Figure 1
Figure 1
A child with right-sided auditory neuropathy spectrum disorder (ANSD) evaluated with MRI using 3D constructive interference in the steady state (CISS) sequence of the bilateral internal auditory canals (IAC). Axial plane images demonstrate normal right (A) and left (B) IAC morphology. The oblique-sagittal reconstructed plane demonstrates normal facial (black arrow head) and cochlear (white arrow) nerves on the left (C). The oblique-sagittal image through the right IAC (D) demonstrates a normal facial nerve (arrowhead) and absent cochlear (white arrow) nerve. In both C & D, the vestibular nerves are normal.
Figure 1
Figure 1
A child with right-sided auditory neuropathy spectrum disorder (ANSD) evaluated with MRI using 3D constructive interference in the steady state (CISS) sequence of the bilateral internal auditory canals (IAC). Axial plane images demonstrate normal right (A) and left (B) IAC morphology. The oblique-sagittal reconstructed plane demonstrates normal facial (black arrow head) and cochlear (white arrow) nerves on the left (C). The oblique-sagittal image through the right IAC (D) demonstrates a normal facial nerve (arrowhead) and absent cochlear (white arrow) nerve. In both C & D, the vestibular nerves are normal.
Figure 1
Figure 1
A child with right-sided auditory neuropathy spectrum disorder (ANSD) evaluated with MRI using 3D constructive interference in the steady state (CISS) sequence of the bilateral internal auditory canals (IAC). Axial plane images demonstrate normal right (A) and left (B) IAC morphology. The oblique-sagittal reconstructed plane demonstrates normal facial (black arrow head) and cochlear (white arrow) nerves on the left (C). The oblique-sagittal image through the right IAC (D) demonstrates a normal facial nerve (arrowhead) and absent cochlear (white arrow) nerve. In both C & D, the vestibular nerves are normal.
Figure 1
Figure 1
A child with right-sided auditory neuropathy spectrum disorder (ANSD) evaluated with MRI using 3D constructive interference in the steady state (CISS) sequence of the bilateral internal auditory canals (IAC). Axial plane images demonstrate normal right (A) and left (B) IAC morphology. The oblique-sagittal reconstructed plane demonstrates normal facial (black arrow head) and cochlear (white arrow) nerves on the left (C). The oblique-sagittal image through the right IAC (D) demonstrates a normal facial nerve (arrowhead) and absent cochlear (white arrow) nerve. In both C & D, the vestibular nerves are normal.
Figure 2
Figure 2
A child with bilateral auditory neuropathy spectrum disorder (ANSD) evaluated with 3D constructive interference in the steady state (CISS) MRI. This image demonstrates a normal left IAC (3.1mm; white arrow) and a stenotic right IAC (1.4mm; white arrowhead).
Figure 3
Figure 3
A child with bilateral auditory neuropathy spectrum disorder (ANSD) evaluated with MRI. This T2-weighted axial MRI image demonstrates bilateral prominent extra-axial CSF (*) anterior to the temporal lobes.
Figure 4
Figure 4
A child with bilateral auditory neuropathy spectrum disorder (ANSD) evaluated with MRI. T1 weighted sequence demonstrates (A) bilateral hydrocephalus of the lateral ventricles and (B) cerebellar hypoplasia (arrow). Lateral ventricle hydrocephalus is additionally visualized (*). Severe pontine hypoplasia is additionally present.
Figure 4
Figure 4
A child with bilateral auditory neuropathy spectrum disorder (ANSD) evaluated with MRI. T1 weighted sequence demonstrates (A) bilateral hydrocephalus of the lateral ventricles and (B) cerebellar hypoplasia (arrow). Lateral ventricle hydrocephalus is additionally visualized (*). Severe pontine hypoplasia is additionally present.
Figure 5
Figure 5
A child with bilateral auditory neuropathy spectrum disorder (ANSD) evaluated with MRI. This T1-weighted sagittal image demonstrates a Dandy-Walker spectrum variant (arrow), with associated dysplastic appearing pons and corpus callosum agenesis (*).
Figure 6
Figure 6
A child with bilateral auditory neuropathy spectrum disorder (ANSD) evaluated with MRI. (A) 3D constructive interference in the steady state (CISS) sequence demonstrates right cerebellar hypoplasia. (B), FLAIR sequence demonstrates bilateral periventricular signal abnormality and cysts consistent with periventricular leukomalacia (arrows).
Figure 6
Figure 6
A child with bilateral auditory neuropathy spectrum disorder (ANSD) evaluated with MRI. (A) 3D constructive interference in the steady state (CISS) sequence demonstrates right cerebellar hypoplasia. (B), FLAIR sequence demonstrates bilateral periventricular signal abnormality and cysts consistent with periventricular leukomalacia (arrows).
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