Vestibular impairment in Charcot-Marie-Tooth disease.

OBJECTIVE: To find out if Charcot-Marie-Tooth (CMT) patients, who have peripheral vestibular as well as peripheral somatosensory impairment, have worse postural balance than those who do not. METHODS: We studied 32 patients with various CMT phenotypes and genotypes. Vestibular function was measured with the video head impulse test (vHIT) which tests vestibulo-ocular reflex (VOR) gain from each of the six semicircular canals in response to rapid head rotations. Postural balance was evaluated with a battery of four postural tests with emphasis on the modified clinical test of sensory integration in balance (mCTSIB). RESULTS: Half of the 32 patients had some impairment of vestibular function ranging from mild, affecting only 1-2 semicircular canals, to almost total affecting all 6 semicircular canals. Their mCTSIB scores correlated with VOR gain from the vertical rather than from the lateral semicircular canals. The worse the vertical VOR gain the worse the mCTSIB score. CONCLUSION: We propose that any CMT patient could have clinically inapparent vestibular impairment that can be easily measured with the vHIT. This vestibular impairment could be contributing to their imbalance and could respond to a focused vestibular rehabilitation program.

Vestibular impairment in chronic inflammatory demyelinating polyneuropathy.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a common, treatable, autoimmune peripheral neuropathy considered to produce imbalance by weakness and proprioceptive impairment rather than vestibular impairment. We measured semicircular canal vestibular function in 21 CIDP patients (15M/6F) by the video head impulse test and postural stability with a battery comprising the modified Clinical Test of Sensory Integration and Balance, the Berg Balance Scale, the Dynamic Gait Index, the Fall Efficiency Scale, and the International Cooperative Ataxia Rating Scale. Of the 21 patients, 16 had vestibular impairment, ranging from mild-affecting just a single semicircular canal, to severe-affecting all 6 canals. Although the severity of the vestibular impairment did not correlate either with the severity of the postural imbalance or of the peripheral neuropathy, our data show that vestibular impairment is an additional challenge to balance that some CIDP patients will face.

Convergence spasm due to aquaporin-positive neuromyelitis optica spectrum disorder.

A female 27 presented with nausea and diplopia for 1 week. On examination she had normal vertical gaze but would develop convergence with miosis whenever she made horizontal saccades. Pupils were 6 mm and unreactive to light. MRI showed extensive hyperintensity in the dorsal midbrain and thalamus. Spinal MRI and CSF were both normal. Serum aquaporin-4-antibody was positive. She was treated with steroids and plasmapheresis and after 3 months convergence spasm resolved but pupils remained unreactive. Neuromyelitis optica often presents with brainstem signs, rarely a dorsal midbrain syndrome. Convergence spasm is occasionally of organic neurologic origin.

Comparison of Audiological Findings in Patients with Vestibular Migraine and Migraine.

OBJECTIVE: The aim of this study was to investigate and compare the auditory findings in vestibular migraine (VM) and migraine patients without a history of vertigo. METHODS: This study was conducted on 44 patients diagnosed with definite VM and 31 patients diagnosed with migraine who were followed and treated between January 2011 and February 2015. Also, 52 healthy subjects were included in this study as a control group. All participants underwent a detailed otorhinolaryngological examination followed by audiological evaluation, including pure tone audiometry, speech reception threshold, speech recognition score, and acoustic immitancemetry. RESULTS: In the VM group, there were 16 patients (36.4%) with tinnitus, while in the other groups we did not observe any patients with tinnitus. The rate of tinnitus in the VM group was significantly higher in comparison to other groups (p<0.05). None of the groups had any patients with permanent or fluctuating sensorineural hearing loss. CONCLUSION: We conclude that patients with VM should be closely and longitudinally followed up for the early detection of other otological symptoms and possible occurrence of sensorineural hearing loss in the long term.