Usher syndrome: reason for combined hearing and vision loss
نویسندگان
چکیده
منابع مشابه
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...
متن کاملCombined Intratympanic and Systemic Steroid Therapy for Poor-Prognosis Sudden Sensorineural Hearing Loss
Introduction: The aim of this study was to evaluate the efficacy of combined intratympanic and systemic steroid therapy compared with systemic steroid therapy alone in idiopathic sudden sensorineural hearing loss (ISSNHL) patients with poor prognostic factors. Materials and Methods: Seventy-seven patients with sudden sensorineural hearing loss (SSNHL) who had at least one poor prognostic fa...
متن کاملA small molecule mitigates hearing loss in a mouse model of Usher syndrome III
Usher syndrome type III (USH3), characterized by progressive deafness, variable balance disorder and blindness, is caused by destabilizing mutations in the gene encoding the clarin-1 (CLRN1) protein. Here we report a new strategy to mitigate hearing loss associated with a common USH3 mutation CLRN1(N48K) that involves cell-based high-throughput screening of small molecules capable of stabilizin...
متن کاملHearing aid fitting for visual and hearing impaired patients with Usher syndrome type IIa
OBJECTIVES Usher syndrome is the leading cause of hereditary deaf-blindness. Most patients with Usher syndrome type IIa start using hearing aids from a young age. A serious complaint refers to interference between sound localisation abilities and adaptive sound processing (compression), as present in today's hearing aids. The aim of this study was to investigate the effect of advanced signal pr...
متن کاملDevelopment of a case-mix funding system for adults with combined vision and hearing loss
BACKGROUND Adults with vision and hearing loss, or dual sensory loss (DSL), present with a wide range of needs and abilities. This creates many challenges when attempting to set the most appropriate and equitable funding levels. Case-mix (CM) funding models represent one method for understanding client characteristics that correlate with resource intensity. METHODS A CM model was developed ba...
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ژورنال
عنوان ژورنال: Journal of Otolaryngology-ENT Research
سال: 2018
ISSN: 2379-6359
DOI: 10.15406/joentr.2018.10.00337