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Published on: May 23, 2013
Audiological findings in patients with oculo-auriculo-vertebral spectrum
Pricila Sleifer1, Natalya de Souza Gorsky2, Thayse Bienert Goetze3
1Department of Health and Human Communication, Universidade Federal do Rio Grande do Sul, Porto Alegre, Rio Grande do Sul, Brazil.
Oculo-auriculo-vertebral spectrum, or Goldenhar syndrome, frequently causes conductive hearing loss, particularly in the right ear. Early audiological assessment is crucial for speech therapy and intervention in affected individuals.
Area of Science:
- Genetics and developmental biology
- Audiology and otolaryngology
Background:
- Oculo-auriculo-vertebral spectrum (OAVS), also known as Goldenhar syndrome, involves developmental abnormalities of the first and second branchial arches.
- Key features include craniofacial, ocular, auricular, and vertebral anomalies, with auricular abnormalities often linked to hearing impairment.
Purpose of the Study:
- To investigate the audiological profile of patients diagnosed with OAVS.
- To analyze hearing loss patterns using pure-tone and speech audiometry.
Main Methods:
- A cross-sectional study involving 10 patients with OAVS, exhibiting clinical signs in at least two affected areas.
- Comprehensive audiological evaluations, including liminal pure-tone audiometry and speech audiometry, were performed on all participants.
Main Results:
- All 10 patients presented with ear abnormalities, predominantly affecting the right side.
- Conductive hearing loss was the most prevalent type (10 ears), followed by sensorineural (5 ears) and mixed hearing loss (1 ear).
- Hearing impairment severity ranged from mild to moderate, with one instance of profound hearing loss.
Conclusions:
- Individuals with OAVS show a high incidence of conductive hearing loss, often moderate and affecting the right ear.
- Understanding auditory thresholds in OAVS is vital for effective speech therapy and timely intervention for hearing-related issues.
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