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Auditory Brainstem Pathology in Autism Spectrum Disorder: A Review

Pillion, Joseph P., PhD*,†; Boatman-Reich, Dana, PhD‡,§; Gordon, Barry, MD, PhD‡,∥

Cognitive and Behavioral Neurology: June 2018 - Volume 31 - Issue 2 - p 53–78
doi: 10.1097/WNN.0000000000000154
Review Article

Atypical responses to sound are common in individuals with autism spectrum disorder (ASD), and growing evidence suggests an underlying auditory brainstem pathology. This review of the literature provides a comprehensive account of the structural and functional evidence for auditory brainstem abnormalities in ASD. The studies reviewed were published between 1975 and 2016 and were sourced from multiple online databases. Indices of both the quantity and quality of the studies reviewed are considered. Findings show converging evidence for auditory brainstem pathology in ASD, although the specific functions and anatomical structures involved remain equivocal. Two main trends emerge from the literature: (1) abnormalities occur mainly at higher levels of the auditory brainstem, according to structural imaging and electrophysiology studies; and (2) brainstem abnormalities appear to be more common in younger than older children with ASD. These findings suggest delayed maturation of neural transmission pathways between lower and higher levels of the brainstem and are consistent with the auditory disorders commonly observed in ASD, including atypical sound sensitivity, poor sound localization, and difficulty listening in background noise. Limitations of existing studies are discussed, and recommendations for future research are offered.

*Department of Audiology, Kennedy Krieger Institute, Baltimore, Maryland

Departments of Physical Medicine and Rehabilitation

Neurology, and

§Otolaryngology, Johns Hopkins University School of Medicine, Baltimore, Maryland

Cognitive Science Department, Johns Hopkins University, Baltimore, Maryland

Supported in part by the Therapeutic Cognitive Neuroscience Endowment and Fund (B.G.); the Adith and Benjamin Miller Family Endowment for Aging, Alzheimer’s Disease, and Autism (B.G.); the Murren Family Foundation (B.G.); and funding from the Saeed Family and the Binder Family.

The authors declare no conflicts of interest.

Correspondence: Joseph P. Pillion, PhD, Department of Audiology, Kennedy Krieger Institute, 801 N. Broadway, Baltimore, Maryland 21205 (e-mail:

Received August 11, 2017

Accepted May 8, 2018

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