Hearing loss in the RBF/DnJ mouse, a proposed animal model of Usher syndrome type IIa
S Pieke-Dahl, KK Ohlemiller, JA McGee, EJ Walsh… - Hearing research, 1997 - Elsevier
The Usher syndromes (US) are a group of inherited disorders that feature autosomal
recessive neurosensory hearing loss or deafness with retinitis pigmentosa (RP). Moderate to
severe non-progressive high frequency hearing loss with RP and normal vestibular function
describes Usher syndrome type IIa, which has been localized to 1q41. Severe retinal
degeneration in the inbred mouse strain RBF/DnJ is caused by rd3, a recessive gene
located on mouse chromosome 1 distal to akp1 in a region which is orthologous to human …
recessive neurosensory hearing loss or deafness with retinitis pigmentosa (RP). Moderate to
severe non-progressive high frequency hearing loss with RP and normal vestibular function
describes Usher syndrome type IIa, which has been localized to 1q41. Severe retinal
degeneration in the inbred mouse strain RBF/DnJ is caused by rd3, a recessive gene
located on mouse chromosome 1 distal to akp1 in a region which is orthologous to human …
