Vestibular hypersensitivity to sound in superior canal dehiscence: Large evoked responses in the legs produce little postural sway

• Published in: Clinical neurophysiology 2008-07, Vol.119 (7), p.1674-1682
• Main Authors: Rosengren, S.M, Halmagyi, G.M, Colebatch, J.G
• Format: Article
• Language: English
• Subjects: Adult; Aged; Aged, 80 and over; Biological and medical sciences; Ear Canal - physiopathology; Ear Diseases - physiopathology; Electrodiagnosis. Electric activity recording; Electromyography; Evoked Potentials - physiology; Female; Fundamental and applied biological sciences. Psychology; Humans; Investigative techniques, diagnostic techniques (general aspects); Leg - physiology; Male; Medical sciences; Middle Aged; Motor control and motor pathways. Reflexes. Control centers of vegetative functions. Vestibular system and equilibration; Nervous system; Neurology; Posture - physiology; Reflex - physiology; Sound - adverse effects; Spinal Cord - physiology; Superior canal dehiscence; Sway; Tullio phenomenon; VEMP; Vertebrates: nervous system and sense organs; Vestibular; Vestibule, Labyrinth - physiology
• ISSN: 1388-2457; 1872-8952
• DOI: 10.1016/j.clinph.2008.03.021

Abstract Objective Patients with superior canal dehiscence (SCD) typically have enhanced sound-evoked vestibular reflexes, such as vestibulo-collic and vestibulo-ocular reflexes. We wished to investigate whether sound-evoked lower limb EMG responses and postural sway are also enhanced in this condition. Methods Eight patients with CT confirmed SCD (11 affected ears) and 8 age-matched normal controls participated. Three sound-evoked responses were measured; vestibulo-collic reflexes (i.e. vestibular-evoked myogenic potentials, VEMPs), lower limb vestibulo-spinal reflexes and body sway (centre of pressure in mm). Sound stimuli were 500 Hz air-conducted tone bursts of varying lengths (VEMPs: 2 ms; vestibulo-spinal: 20 ms; sway: 1 s and 200 ms) set at fixed levels above each subject’s VEMP threshold. Results SCD patients had very large VEMP and vestibulo-spinal responses following high intensity stimulation, but at the matched intensity of 15 dB above threshold amplitudes were similar in both SCD patients and controls. The amplitude of both responses increased linearly with increasing stimulus intensity in both groups. Large (∼20 mm), stereotyped sway responses were present in only one (atypical) patient with high intensity stimulation. Small (∼2 mm) sway responses were present in the remaining patients, and began immediately following the vestibulo-spinal responses. Conclusions Despite the presence of large vestibular reflexes, there is usually very little body sway in response to loud sounds in SCD patients. Significance Large short-latency vestibulo-spinal reflexes in SCD do not necessarily evoke large sway responses.