Audiologic and Vestibular Findings in Wolfram Syndrome
نویسندگان
چکیده
منابع مشابه
Radiological findings in Wolfram syndrome.
OBJECTIVE To determine the precise radiologic findings in Wolfram syndrome (WFS) patients using objective techniques in order to better define the reference population for the clinical evaluation. METHODS Sixteen patients (6 males and 10 females) with WFS found in 4 families were included in this study. Fourteen patients with WFS-2 came from 3 families while 2 patients with WFS-1 from one fam...
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متن کاملWolfram syndrome.
Wolfram syndrome is a rare neurodegenerative and genetic disorder, which should be suspected in patients with young onset non-immune insulin dependent diabetes mellitus and optic atrophy. Patients are most likely to develop diabetes insipidus, deafness, urinary tract, and neurological abnormalities. 60% of the people with Wolfram syndrome die at age 35, usually due to central respiratory center...
متن کاملAudiologic Findings in Hyperacusic and Nonhyperacusic Subjects
This study compared pure-tone threshold data, acoustic reflex threshold data, and loudness growth data for a group of 25 hyperacusic male subjects vs. a group of 13 nonhyperacusic male subjects. Pure-tone thresholds and acoustic reflex thresholds were obtained in 5-dB steps, using revised HughsonWestlake procedures. Loudness growth functions were obtained with a fractionation (method of adjustm...
متن کاملDiabetes and Neurodegeneration in Wolfram Syndrome
OBJECTIVE To describe the diabetes phenotype in Wolfram syndrome compared with type 1 diabetes, to investigate the effect of glycemic control on the neurodegenerative process, and to assess the genotype-phenotype correlation. RESEARCH DESIGN AND METHODS The clinical data of 50 patients with Wolfram syndrome-related diabetes (WSD) were reviewed and compared with the data of 24,164 patients wit...
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ژورنال
عنوان ژورنال: Ear & Hearing
سال: 2013
ISSN: 0196-0202
DOI: 10.1097/aud.0b013e3182944db7