Usher syndrome is an autosomal recessive disorder that causes hearing loss, Retinitis Pigmentosa (RP) and vestibular dysfunction. It is clinically and genetically heterogeneous disorder which is clinically divided into three types i.e. type I, type II and type III. To date, there are about twelve loci and ten identified genes which are associated with Usher syndrome. A mutation in any of these ...

introduction: with prevalence figures close to 0.2% at birth, hearing loss (hl) is the most frequent sensory impairment in childhood. in developed countries, genetic causes account for more than 60% of congenital hl, most often resulting in non-syndromic deafness, which is usually autosomal recessive. hereditary nonsyndromic hearing loss (nshl) in iran is highly heterogeneous, rendering molecul...

hearing loss (hl) is the most common communication disorder affecting about 1/1000 births worldwide caused by environmental or genetic factors. about 30-50% is attributed to genetic factors and till now more than 85 genes have been implicated in non-syndromic hl. in iran, hl is second to intellectual disability as the most common disability, affecting 1 of every 166 persons. about 15 years ago ...

Introduction Usher syndrome (USH) represents a group of genetically heterogenous autosomal recessive disorders, characterized by combined vision and sensorineural hearing loss, in some cases vestibular dysfunction.1–3 It primarily affects the light-sensitive photoreceptor cells retina auditory hair cochlea. Vision loss all is progressive, manifests as retinitis pigmentosa (RP), gradual degenera...

Usher syndrome (USH) named after the British ophthalmologist Charles Usher [1] is the most common hereditary form of combined blindness and deafness [2]. It is a rare disorder with an incidence of 3.5/100,000 in Scandinavia [3] to 4.4/100,000 in the USA [4]. It shows an autosomal recessive mode of inheritance. According to clinical symptoms, USH is classified into three types: USH type I, USH t...