Presentation
Right facial asymmetry since the age of one year and six months and right sensorineural hearing loss. History of fall prior to the facial asymmetry. Otherwise, prenatal and early life is unremarkable
Patient Data
Small right 7th and 8th cranial nerves, and lack of visualisation of the cochlea, and vestibule.
Moderate mucosal thickening of the left maxillary sinus.
The right petrous temporal bone demonstrated a right labyrinthitis ossificans. Normal middle ear cavity, ossicles, external auditory canal, and mastoid air cells.
Case Discussion
The child presented with long-standing clinical facial asymmetry (which is not radiologically evident) and right sensorineural hearing loss. MRI demonstrated small right 7th and 8th cranial nerves and HRCT temporal bone showed right labyrinthitis ossificans.
Labyrinthitis ossificans is the most common cause of acquired sensorineural hearing loss in children. In this case, it could either be related to a trauma she sustained early in life or an autoimmune disease of the inner ear. Other aetiology include meningitis, otomastoiditis, and sickle cell disease.
A differential diagnosis in this case is Goldenhar syndrome. It is a complex congenital anomaly developing from first and second brachial arch structures. It is characterised by a wide range of physical abnormalities that involve the eye, ear, and vertebrae.
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