caa a22 4500 475785401 CHVBK 20180406123656.0 cr unu---uuuuu 170329e20000901xx s 000 0 eng 10.1007/PL00008338 doi (NATIONALLICENCE)springer-10.1007/PL00008338 High frequency hearing loss in Ullrich-Turner syndrome [Elektronische Daten] [Neslihan Güngör, Bilgehan Böke, Erol Belgin, Ergül Tunçbilek] A total of 38 patients with Ullrich-Turner syndrome underwent standard otological and audiometric evaluation as well as high frequency audiological tests. Some 26 (68.4%) patients had a history of middle ear infections, and ten (26.3%) had required otolaryngological surgery. Conventional audiometry (125-8000 Hz) demonstrated normal hearing in only 25 of the ears (33%); between 500-4000 Hz, 16 ears (21.0%) had a mixed type and eight ears (10.5%) had conductive hearing loss. High frequency audiometry (8-18 kHz) revealed sensorineural hearing loss in 98.7% of the ears. Our results for conventional audiometry are in accordance with the literature. Conclusion The detection of a high prevalence of hearing loss in the high frequency range brings a significant new perspective to the pursuit of the aetiology of ear and hearing problems in Ullrich-Turner syndrome. This pathology seems to be a premature variant of presbycusis and it may underlie future hearing impairment which will come to clinical attention only after it progresses to conventional testing frequencies. While further studies are underway to evaluate this aspect, routine otological and audiological follow-up of patients with Ullrich-Turner syndrome is warranted from the time of diagnosis. Springer-Verlag Berlin Heidelberg, 2000 Key words Hearing loss nationallicence Audiometry nationallicence High frequency audiometry nationallicence Ullrich-Turner syndrome nationallicence Güngör Neslihan Departments of Paediatrics and Clinical Genetics, Hacettepe University Hospital, Ankara, Turkey, TR aut Böke Bilgehan Department of Otolaryngology, Hacettepe University Hospital, Ankara, Turkey, TR aut Belgin Erol Department of Otolaryngology, Hacettepe University Hospital, Ankara, Turkey, TR aut Tunçbilek Ergül Departments of Paediatrics and Clinical Genetics, Hacettepe University Hospital, Ankara, Turkey, TR aut https://doi.org/10.1007/PL00008338 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/PL00008338 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Güngör Neslihan Departments of Paediatrics and Clinical Genetics, Hacettepe University Hospital, Ankara, Turkey, TR aut NATIONALLICENCE 700 1- Böke Bilgehan Department of Otolaryngology, Hacettepe University Hospital, Ankara, Turkey, TR aut NATIONALLICENCE 700 1- Belgin Erol Department of Otolaryngology, Hacettepe University Hospital, Ankara, Turkey, TR aut NATIONALLICENCE 700 1- Tunçbilek Ergül Departments of Paediatrics and Clinical Genetics, Hacettepe University Hospital, Ankara, Turkey, TR aut Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer