6533b834fe1ef96bd129cb00

RESEARCH PRODUCT

Therapy Strategies for Usher Syndrome Type 1C in the Retina

Uwe WolfrumKerstin Nagel-wolfrumTimor Baasov

subject

Pediatricsmedicine.medical_specialtyRetinabusiness.industryUsher syndromemacromolecular substancesDegeneration (medical)medicine.diseaseProfound hearing lossmedicine.anatomical_structurenervous systemRetinitis pigmentosaotorhinolaryngologic diseasesmedicineDeaf blindnessVestibular dysfunctionAge of onsetbusiness

description

The Usher syndrome (USH) is the most common form of inherited deaf-blindness with a prevalence of ~ 1/6,000. Three clinical subtypes (USH1–USH3) are defined according to the severity of the hearing impairment, the presence or absence of vestibular dysfunction and the age of onset of retinitis pigmentosa (RP). USH1 is the most severe subtype with congenital severe to profound hearing loss and onset of RP before puberty. Currently only the amelioration of the hearing deficiency is implemented, but no treatment of the senso-neuronal degeneration in the eye exists.

yearjournalcountryeditionlanguage
2014-01-01
https://doi.org/10.1007/978-1-4614-3209-8_93