Features include digestive signs (small bowel diverticula,
gastric motility loss, jejunoileal ulcerations, fat malabsorption, chronic
diarrhea), progressive neuropathy, hemiplegia, and progressive sensorineural
deafness (possibly due to cochleosaccular degeneration). Unexplained tachycardia and loss of the carotid sinus reflex may
occur.