High frequency hearing loss in Ullrich-Turner syndrome

A total of 38 patients with Ullrich-Turner syndrome underwent standard otol ogical and audiometric evaluation as well as high frequency audiological te sts. Some 26 (68.4%) patients had a history of middle ear infections, and t en (26.3%) had required otolaryngological surgery. Conventional audiometry (125-8000 Hz) demonstrated normal hearing in only 25 of the ears (33%); bet ween 500-4000 Hz, 16 ears (21.0%) had a mixed type and eight ears (10.5%) h ad conductive hearing loss. High frequency audiometry (8-18 kHz) revealed s ensorineural hearing loss in 98.7% of the ears. Our results for conventiona l audiometry are in accordance with the literature. Conclusion The detection of a high prevalence of hearing loss in the high f requency range brings a significant new perspective to the pursuit of the a etiology of ear and hearing problems in Ullrich-Turner syndrome. This patho logy seems to be a premature variant of presbycusis and it may underlie fut ure hearing impairment which will come to clinical attention only after it progresses to conventional testing frequencies. While further studies are u nderway to evaluate this aspect, routine otological and audiological follow -up of patients with Ullrich-Turner syndrome is warranted from the time of diagnosis.