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.