Sensorineural hearing loss owing to deficient G proteins in patients with pseudohypoparathyroidism: results of a multicentre study

European Journal of Clinical Investigation
T KochR D Hesch

Abstract

Pseudohypoparathyroidism (PHP) is a rare disorder that might be caused by an hereditary defect in the G protein system. These membrane-bound proteins are responsible for the transduction of biological signals through the outer cell membrane. The investigation of 22 patients with PHP showed a symmetric sensorineural hearing loss in 63.6% of the subjects. In erythrocyte membrane preparations from blood samples of 15 of these patients, we measured the concentration of the stimulatory Gs protein and the inhibitory Gi protein by means of the Western blot analysis using selective antibodies against alpha-subunits of G proteins. In nine of the 15 cases (60%), we found a distinct decrease in the amount of the Gs protein with a partial preponderance of the Gi protein. These patients had a considerable symmetric sensorineural hearing loss. Pathophysiological mechanisms and the possible role of G proteins in the inner ear are discussed.

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Citations

May 31, 2019·Current Opinion in Pediatrics·Emily L Germain-Lee
Jan 1, 1991·European Archives of Oto-rhino-laryngology : Official Journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : Affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery·T Koch, B Gloddek
Jun 23, 2006·Journal of Pediatric Endocrinology & Metabolism : JPEM·Jean-Louis WémeauVirginie Vlaeminck-Guillem
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