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

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. Seventeen of the 23 patients received cochlear implant (CI) at a mean age of 37.9 years. In comparison, the study patient presented initially with bilateral progressive SNHL as a teenager and developed bilateral non-simultaneous sudden SNHL, first in her right ear at the age of 31 and 7 months later in her left ear at the age of 32. She is now a successful bilateral CI user. Conclusions: This is the first systematic investigation of the relationship between MYH9-RD patients and SNHL. Hearing loss in MYH9 -RD patients is generally characterized as progressive SNHL. However, the study patient presented with the unique feature of bilateral non-simultaneous sudden SNHL, potentially expanding the hearing loss sequela associated with this disorder.