Related Experiment Videos
Labyrinthine involvement in Langerhans' cell histiocytosis
V R Nanduri1, J Pritchard, W K Chong
1Department of Haematology/Oncology, Great Ormond Street Hospital for Children, London, UK. V.Nanduri@ich.ucl.ac.uk
Insights
Langerhans
Area of Science:
- Otorhinolaryngology
- Pediatric Oncology
- Histiocytosis
Background:
- Langerhans' cell histiocytosis (LCH) is a rare clonal proliferative disorder of Langerhans' cells.
- While LCH commonly affects the ear, inner ear involvement is infrequent due to the protective bony labyrinth.
- Previous literature contains limited case reports on inner ear LCH.
Observation:
- This report details two pediatric cases of LCH with inner ear (labyrinthine) invasion.
- One patient presented with acute hearing loss secondary to cochlear invasion in unilateral mastoid LCH.
- The second patient, with multi-system LCH, had labyrinthine involvement detected via imaging before symptom onset.
Findings:
- Inner ear invasion by LCH can result in profound and permanent hearing loss.
- Early diagnosis and intervention are crucial for preserving hearing function in affected children.
- Labyrinthine involvement in LCH necessitates prompt systemic treatment.
Implications:
- Prompt medical intervention, including steroids and potentially chemotherapy, is vital to prevent irreversible deafness.
- This study highlights the importance of considering inner ear involvement in pediatric LCH cases.
- Further research is needed to understand the pathophysiology and optimize treatment strategies for inner ear LCH.
Background:
Langerhans' cell histiocytosis, a rare condition caused by the proliferation of abnormal Langerhans' cells ('LCH cells') and an accompanying granulomatous infiltrate, can affect several organs including the ear. External and middle ear involvement are common with a reported incidence as high as 61%. The bony labyrinth is resistant to erosion by the granulation tissue, thereby protecting the cochlea and vestibular structures. Probably for this reason, involvement of the inner ear is rare, with few case reports in the literature.
Patients:
We report two girls, one with bilateral and the other with unilateral mastoid involvement, in whom there was invasion of the labyrinth. The first girl had 'single system' LCH affecting only bone and developed an acute hearing loss due to invasion of the cochlea, while the second had both bone and skin involvement and labyrinthine involvement was diagnosed on imaging prior to the onset of labyrinthine symptoms.
Conclusion:
Inner ear involvement can lead to permanent deafness, which may be prevented by early institution of treatment. Threatened inner ear involvement requires urgent systemic medical therapy with steroids, possibly combined with chemotherapy.