Hearing loss in patients with congenital coagulation disorders

Hearing status was studied in 22 subjects (10 adults and 12 children) with confirmed congenital blood coagulation disorder. Each subject was assessed using an extensive audiological test battery. For the adult subjects this included pure-tone audiometry, tympanometry and acoustic reflex measures, speech audiometry, staggered spondaic word test, synthetic sentence identification test and masking level difference test. The children's tests included pure-tone audiometry, tympanometry, acoustic reflex measures and paediatric speech intelligibility testing. Test results were compared to normative data. An increased prevalence in adults of both sensorineural hearing loss above 4000 Hz and possible central auditory dysfunction localised in the higher brain stem or cortical structures were the main findings of the research.