ArticleBrain communications2026
Abnormal auditory neural activity in individuals with spinal muscular atrophy.
Article in Brain communications, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Spinal muscular atrophy (SMA) is an autosomal recessive disorder characterized by hypotonia, progressive muscle weakness and atrophy caused by progressive loss of motor neurons in the spinal cord and lower cranial nerve nuclei. The disease has also been associated with sensory neuropathies affecting the visual, somatosensory and auditory pathways. This case-control study investigated auditory neural function and perceptual ability in children and young adults with SMA. Twenty individuals with genetically confirmed SMA of varying severity (Types 1-3) participated. Sixteen children/young adults aged 6-20 years and 16 age-, gender- and hearing-level matched controls underwent a battery of peripheral and central auditory assessments. These included sound detection measurement, otoacoustic emission and auditory brainstem response testing and speech perception evaluation. In addition, four infants (3-28 months) treated with disease-modifying therapies underwent auditory brainstem response assessment. Despite normal cochlear responses and normal sound detection, most child or young adult participants presented with clinically abnormal auditory neural function and significant speech perception deficits. Auditory brainstem response amplitudes were reduced and latencies increased relative to matched controls (
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