I. Hore et al., Langerhans' cell histiocytosis - A rare cause of sudden onset unilateral sensorineural hearing loss, J LARYNG OT, 113(12), 1999, pp. 1098-1100
Langerhans' cell histiocytosis is a rare disorder of unknown aetiology in w
hich pathological Langerhans' cells accumulate and destroy local tissue. We
report a 38-year-old female who presented with a sudden onset of left sens
orineural hearing loss. Magnetic resonance imaging (MRI) revealed a contras
t-enhancing lesion in the left mastoid and a second lesion in the hypothala
mus. Following left mastoid exploration and biopsy a definitive diagnosis o
f Langerhans' cell histiocytosis was made and the patient was treated with
external beam radiotherapy. Subsequent right femur and right mastoid involv
ement were successfully treated with steroids and cytotoxic chemotherapy. A
t one year follow-up the patient had residual left-sided sensorineural hear
ing loss with normal hearing in the right ear. To our knowledge, Langerhans
' cell histiocytosis has not been previously reported as a cause of unilate
ral sudden onset sensorineural hearing loss. It should be considered in the
differential diagnosis of this condition.