Longitudinal hearing loss in Wolfram syndrome

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Pseudoexfoliation Syndrome and Sensorineural Hearing Loss

Introduction: Pseudoexfoliation syndrome (PXS) occurs due to the deposition of extracellular fibrillar materials on the anterior chamber of the eye. This syndrome has been considered to be part of a systemic disease with the potential involvement of the inner ear called sensoroneural hearing loss (SNHL).  In this study, we aimed on evaluating SNHL within PXS patients in Iran to compare them wi...

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Hearing loss in Turner syndrome.

OBJECTIVE To address the characteristics of hearing loss in patients with Turner syndrome (TS), we evaluated hearing levels of patients with TS and analyzed causative factors. STUDY DESIGN Thirty-three patients with TS (8 to 40 years of age) were studied through the use of audiological measurements, and causative factors were explored. RESULTS Twenty cases (35 of 66 ears tested) showed high...

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Wolfram 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...

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sensorineural hearing loss in pseudoexfoliation syndrome

purpose: to determine hearing thresholds at sound frequencies important for speech comprehension in subjects with ocular pseudoexfoliation (pxf) and to compare them with that of controls without pxf. methods: eighty-three subjects with ocular pxf and 83 age and sex matched controls without pxf were enrolled in this case-control study. pure tone audiometry (bone conduction) was performed at 1, 2...

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HEARING LOSS IN HEMODIALYSIS PATIENTS

Inner ear cells are sensitive to some metabolic and hormonal disorders, but the relation between chronic renal failure (CRF), regular dialysis treatment (RDT) and sensorineural hearing loss (SNHL) is still a debatable field. The object of this paper was to verify the presence of SNHL in patients submitted to hemodialysis with different duration due to CRF and in those submitted to conservat...

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ژورنال

عنوان ژورنال: Orphanet Journal of Rare Diseases

سال: 2018

ISSN: 1750-1172

DOI: 10.1186/s13023-018-0852-0