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Bilateral Mondini dysplasia with normal hearing
1Department of Otorhinolaryngology, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Summary
This study reports a rare case of Mondini dysplasia with normal hearing in a patient experiencing unsteadiness. The unsteadiness was linked to semicircular canal hypofunction, possibly from cerebrospinal fluid otorrhea.
Area of Science:
- Otolaryngology
- Neuroscience
- Genetics
Background:
- Mondini dysplasia is a congenital inner ear malformation.
- It is typically associated with hearing loss and vestibular dysfunction.
- Normal hearing in the presence of Mondini dysplasia is considered rare.
Observation:
- A 37-year-old male presented with unsteadiness.
- Audiometry revealed normal hearing in both ears.
- CT scans showed enlarged vestibules and lateral semicircular canals, with normal cochlear and other semicircular canal morphology.
Findings:
- Caloric testing indicated severe left and moderate right canal paresis (CP).
- Active head rotation tests showed preserved head-eye coordination at lower frequencies (0.33, 0.67 Hz) but slight deterioration at 1.0 Hz.
- The patient's unsteadiness was attributed to bilateral semicircular canal hypofunction.
Implications:
- The findings suggest that insidious cerebrospinal fluid otorrhea, potentially from activities like judo wrestling, could cause semicircular canal hypofunction in patients with Mondini dysplasia.
- This case challenges the rarity of Mondini dysplasia with normal hearing, suggesting it may be underdiagnosed.
- Further research into the prevalence and clinical presentation of Mondini dysplasia with normal hearing is warranted.