Auditory dysfunction in type 2 Stickler Syndrome

ConclusionsAuditory impairment in type 2 Stickler Syndrome is predominantly associated with cochlear hearing loss of varying severities across affected individuals. The impact on hearing thresholds can be seen across the frequency range, suggesting a contribution of defective collagen throughout the cochlea. Self-report questionnaires showed that difficulties understanding speech, and spatial information in sound (such as that used for localisation), were worse than a young, normal-hearing population but comparable to a non-syndromic cohort with similar audiometric thresholds. Therefore, it is likely that hearing loss in type 2 Stickler Syndrome arises in the auditory periphery, without significant central processing deficits.

http://link.springer.com/10.1007/s00405-020-06306-y

Source: European Archives of Oto-Rhino-Laryngology - September 7, 2020 Category: ENT & OMF Source Type: research