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Prevalence of Otological Disease in Turner Syndrome: A Systematic Review

Supplemental Digital Content is available in the text Introduction: Girls and women with Turner syndrome (TS) present with multiple ear and hearing problems, ranging from external morphologic abnormalities to sensorineural or conductive hearing loss. The exact pathophysiology behind these otological diseases is not yet completely understood. The aim of this study is to provide a systematic review on the prevalence of otological disease in TS. Methods: We conducted a systematic review according to the PRISMA guidelines. A database search was performed in PubMed, Embase, Web of Science, and Cochrane library. Results: The prevalence of otological disease as external ear deformities (20–62%), recurrent otitis media (24–48%), and hearing loss (36–84%) is high in TS. The auditory phenotype in TS is complex and seems to be dynamic with CHL due to middle ear disease at young age and sensorineural hearing loss later in life. Conclusion: This systematic review of the literature confirms that otological disease is definitely part of the widely variable phenotype in Turner patients. Strong evidence is lacking on the exact prevalence numbers, emphasizing the need for more prospective data gathering. Growing insights in its pathophysiology will help in the understanding and management of hearing problems in TS across lifespan.