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Outcomes of long-term audiological rehabilitation in charge syndrome
Insights
CHARGE syndrome often involves hearing loss, impacting communication. Early intervention and tailored hearing rehabilitation, including hearing aids or cochlear implants, can offer benefits despite complex disabilities.
Area of Science:
- Audiology
- Genetics
- Pediatrics
Background:
- CHARGE syndrome is a complex genetic disorder associated with various health issues, including significant hearing impairment.
- Hearing loss in CHARGE syndrome can range in severity and type, often complicated by other disabilities.
Purpose of the Study:
- To evaluate the long-term audiological characteristics and hearing rehabilitation outcomes in children with CHARGE syndrome.
- To assess the impact of hearing aids and cochlear implants on communication and language development in this population.
Main Methods:
- Retrospective analysis of a pediatric patient database from audiology departments.
- Hearing assessment using auditory brainstem responses (ABRs), electrocochleography, and behavioral audiometry (VRA, play audiometry).
- Monitoring of auditory-perceptual outcomes, communication skills, and language development post-rehabilitation.
Main Results:
- Hearing rehabilitation outcomes varied due to the heterogeneous disabilities present in CHARGE syndrome, such as developmental and intellectual delays, and visual impairment.
- Severe impairment in oral expressive language was common, even after extended follow-up.
- Retrocochlear auditory dysfunction, including auditory neuropathy/dyssynchrony, was noted in some cases.
Conclusions:
- Early identification of sensorineural hearing loss is crucial for children with CHARGE syndrome.
- Carefully planned and individualized hearing rehabilitation strategies are essential.
- Alternative and augmentative communication methods may be necessary due to persistent language impairments.
Abstract:
The aim of this paper is to assess the long-term audiological features and outcomes of hearing rehabilitation in a large group of individuals with CHARGE Syndrome. The study has been conducted retrospectively, on a paediatric patient database, at the Audiology Department of the University Hospitals of Ferrara and Padua. The study sample included 31 children presenting with different degrees of hearing impairment associated with CHARGE syndrome. Hearing was assessed using auditory brainstem responses (ABRs) and/or electrocochleography, or conditioned audiometry (visual reinforcement audiometry [VRA] or play audiometry). Auditory-perceptual outcomes in terms of communication skills and expressive language were also recorded. The effects of hearing rehabilitation (with hearing aids or cochlear implants) in this group of children and language outcomes after rehabilitation were monitored during long-term follow-up. The outcomes of rehabilitation measures differed in relation to the heterogeneous and often severe disabilities associated with CHARGE syndrome, e.g. developmental delay, intellectual delay, visual impairment, thin 8(th) nerve with retrocochlear auditory dysfunction (as described in cases of auditory neuropathy/dyssynchrony). Oral expressive language was severely impaired in most cases, even after lengthy follow-up, suggesting the need for alternative augmentative communication modes. The early identification of sensorineural hearing loss, and carefully planned rehabilitation treatments, can be of some benefit in children with CHARGE syndrome.
