Cochlear implants in young children with Usher's syndrome.

Usher's syndrome (US) is an autosomal recessive disorder characterized by congenital sensorineural hearing loss and retinitis pigmentosa. The majority of affected individuals have severe to profound sensorineural hearing loss and are therefore potential cochlear implant candidates. A goal of our otology program has been early identification and implantation of children with US. At our center, early diagnosis of US has been achieved by comprehensive ophthalmologic evaluation including electroretinography. Four of our 19 pediatric cochlear implant recipients with congenital sensorineural hearing loss were identified with US and implanted prior to the onset of signs or symptoms of visual loss. All children have received measurable benefit from cochlear implantation. In light of the dual sensory deficits present in US, the need for early diagnosis and cochlear implantation is of special importance in this population.