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Mixed hearing loss in Larsen syndrome
C S Stanley1, J W Thelin, J H Miles
1Department of Surgery, University of Missouri School of Medicine, Columbia.
Clinical Genetics
|May 1, 1988
Summary
Children with Larsen syndrome can experience mixed bilateral hearing loss. This may be caused by abnormalities in the small bones of the middle ear (ossicles).
Area of Science:
- Genetics
- Otolaryngology
- Pediatrics
Background:
- Larsen syndrome is a rare genetic disorder characterized by multiple joint dislocations and distinctive facial features.
- Hearing loss is a potential, though not widely recognized, comorbidity in Larsen syndrome.
Observation:
- A case study of a child with classical Larsen syndrome presenting with mixed bilateral hearing loss.
- Initial audiological evaluation revealed a significant conductive component to the hearing loss.
Findings:
- Ventilating tube placement successfully addressed the sensorineural component but a residual conductive loss persisted.
- This persistent conductive loss strongly suggests an underlying ossicular abnormality as the cause.
Implications:
- Ossicular chain abnormalities should be considered in the audiological workup of children with Larsen syndrome.
- Further research is warranted to determine the prevalence and specific nature of ossicular involvement in Larsen syndrome.
- This finding may inform earlier diagnosis and management strategies for hearing impairment in affected children.