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Vestibular dysfunction in adult patients with osteogenesis imperfecta
K Kuurila1, E Kentala, S Karjalainen
1Department of Otorhinolaryngology-Head and Neck Surgery, Vaasa Central Hospital, Finland. kaija.kuurila@vshp.fi
American Journal of Medical Genetics. Part A
|July 3, 2003
Summary
Vertigo is common in osteogenesis imperfecta (OI), often linked to inner ear issues. Basilar impression (BI) may contribute, but further research is needed to understand all causes of vertigo in OI patients.
Area of Science:
- Otolaryngology
- Genetics
- Neurology
Background:
- Osteogenesis imperfecta (OI) is a genetic disorder characterized by brittle bones.
- Progressive hearing loss is a common OI symptom.
- Vertigo is frequently observed in OI patients, similar to conditions like otosclerosis and basilar impression (BI).
Purpose of the Study:
- To investigate the cause, frequency, and characteristics of vertigo in patients with osteogenesis imperfecta (OI).
Main Methods:
- A study involving 42 OI patients.
- Evaluations included interviews, clinical and audiological examinations, electronystagmography (ENG), and lateral skull radiography.
- Audiometry and auditory brain-stem response (ABR) were used to assess hearing and neural pathways.
Main Results:
- Hearing loss affected 59.5% of patients; 21% had basilar impression (BI).
- Vertigo was reported by 52.4% of patients, often as short-duration rotational or floating sensations.
- Vertigo prevalence was higher in patients with hearing loss and tended to be higher in those with BI, though not statistically significant. ENG abnormalities were found in 33.3% without clear correlation to vertigo or BI.
Conclusions:
- Vertigo is a common symptom in OI patients.
- Inner ear pathology is a likely cause in many cases, with BI explaining it in some.
- The pathogenesis of vertigo in OI requires further investigation, as some patients experience it without identifiable BI or hearing loss.
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