Inherited syndrome characterized by progressive
deafness, neuropathy, digestive and potential cardiac involvement.
Deafness, Mesenteric Diverticula of Small Bowel, and
Fewer than 10 cases reported in the literature.
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
Evaluate neurologic function,
particularly the severity of the neuropathy. Evaluate nutritional status and
hydration (clinical, laboratory, including albumin, electrolytes, creatinine
serum level, and urea). Perioperative cardiac monitoring (including invasive
blood pressure monitoring if necessary) may be considered. A rapid sequence
induction is recommended because of a high risk of
aspiration secondary to abnormal gastric motility. Avoid succinylcholine in
case of neuropathy.
Groll A, Hirschowitz BI: Steatorrhea and familial deafness in two
siblings. Clin Res 14:47, 1966.
Potasman I, Stermer E, Levy N, et al: The Groll-Hirschowitz syndrome. Clin Genet