نتایج جستجو برای: autosomal recessive non syndromic hearing loss

تعداد نتایج: 1782138  

2014
Zehra Agha Zafar Iqbal Maleeha Azam Humaira Ayub Lisenka E. L. M. Vissers Christian Gilissen Syeda Hafiza Benish Ali Moeen Riaz Joris A. Veltman Rolph Pfundt Hans van Bokhoven Raheel Qamar

Intellectual disability (ID) is a major health problem mostly with an unknown etiology. Recently exome sequencing of individuals with ID identified novel genes implicated in the disease. Therefore the purpose of the present study was to identify the genetic cause of ID in one syndromic and two non-syndromic Pakistani families. Whole exome of three ID probands was sequenced. Missense variations ...

Journal: :journal of sciences islamic republic of iran 0
m.r. noori-daloii

the incidence of pre-lingual hearing loss (hl) is about 1 in 1000 neonates. more than 60% of cases are inherited. non-syndromic hl (nshl) is extremely heterogeneous: more than 130 loci have been identified so far. the most common form of nshl is the autosomal recessive form (arnshl). in this study, a cohort of 36 big arnshl pedigrees with 4 or more patients from 7 provinces of iran was investig...

2010
Lawrence Copelovitch Bernard S. Kaplan

We describe a 7-month-old male with atypical features of autosomal recessive distal renal tubular acidosis (dRTA) with sensorineural hearing loss. Uncharacteristically, he presented with mild acidosis, hypokalaemia and hypocalciuria as well as unilateral sensorineural hearing loss. Subsequent investigations led to the discovery of both hyperoxaluria and beta2-microglobulinuria, thereby expandin...

Journal: :Journal of medical genetics 1992
J R Ward C Saad de Owens I A Sierra

Two sibs are reported with upper limb defect, developmental delay, central hearing loss, unilateral poorly developed antihelix, and bilateral choroid coloboma. The inheritance is probably autosomal recessive.

Journal: :Bulletin de la Societe belge d'ophtalmologie 2004
J A Witters J De Zaeytijd M Leys B P Leroy

A 39-year old man presented 13 years ago with a history of progressive loss of vision and photophobia. A full ophthalmological and ENT work-up during several years of follow-up, including psychophysical as well as electrophysiological tests, revealed a progressive cone dystrophy in combination with sensorineural hearing loss. His younger sister presented with very similar features and underwent...

Journal: :Vision Research 2012
Ander Anasagasti Cristina Irigoyen Olatz Barandika Adolfo López de Munain Javier Ruiz-Ederra

With a worldwide prevalence of about 1 in 3500-5000 individuals, Retinitis Pigmentosa (RP) is the most common form of hereditary retinal degeneration. It is an extremely heterogeneous group of genetically determined retinal diseases leading to progressive loss of vision due to impairment of rod and cone photoreceptors. RP can be inherited as an autosomal-recessive, autosomal-dominant, or X-link...

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