Characterization of Sensorineural Hearing Loss in Patients With MYH9-Related Disease: A Systematic Review

Abstract

Objectives: To review the characteristics and progression of hearing loss in MYH9-related disease (MYH9-RD) patients and present a unique case of bilateral non-simultaneous sudden sensorineural hearing loss (SNHL) in an MYH9-RD patient. MYH9-RD is a rare autosomal dominant platelet disorder. Patients with this disorder have a variable risk of developing SNHL. Methods: A comprehensive literature search for scientific articles in PubMed, Scopus, and Web of Science that reported hearing loss outcomes in MYH9-RD patients. Results: Initial search yielded 270 studies. Eight studies with a total of 23 patients met inclusion criteria and were used for data analysis. MYH9-RD patients typically present with progressive bilateral SNHL affecting predominantly the high frequencies. Mean age of hearing loss onset was 17.1 years, progressing to severe-profound SNHL over a mean period of 14.4 years.