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Cochlear implants in young children with Usher's syndrome
N M Young1, J C Johnson, M B Mets
1Division of Pediatric Otolaryngology, Children's Memorial Medical Center, Chicago, Illinois, USA.
Insights
Early cochlear implantation benefits children with Usher syndrome (US), a genetic disorder causing hearing loss and vision impairment. Prompt diagnosis through ophthalmologic evaluation is key for successful intervention before vision loss occurs.
Area of Science:
- Ophthalmology
- Audiology
- Genetics
Background:
- Usher syndrome (US) is an autosomal recessive disorder causing congenital sensorineural hearing loss and retinitis pigmentosa.
- Individuals with US often have severe to profound hearing loss, making them candidates for cochlear implantation.
- Early identification and intervention are crucial for managing dual sensory deficits.
Observation:
- A pediatric otology program focused on early identification and cochlear implantation for children with US.
- Comprehensive ophthalmologic evaluations, including electroretinography, were used for early US diagnosis.
- Four out of 19 pediatric cochlear implant recipients with congenital hearing loss were diagnosed with US.
Findings:
- These four children with US were implanted before any visual symptoms manifested.
- All implanted children demonstrated measurable benefits from cochlear implantation.
- Early diagnosis facilitated timely intervention for Usher syndrome patients.
Implications:
- Early cochlear implantation in Usher syndrome patients can significantly improve auditory outcomes.
- Ophthalmologic screening is vital for identifying US in children with congenital hearing loss.
- Addressing both hearing and vision impairments early is critical for individuals with Usher syndrome.
Abstract:
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.