High incidence of hearing loss in long-term survivors of multisystem Langerhans cell histiocytosis.
BACKGROUND
Ear involvement in the acute phase of Langerhans cell histiocytosis (LCH) is commonly seen and well documented, but the long-term sequelae are less well described, particularly in relation to hearing loss.


METHODS
We investigated 40 patients with biopsy-proven multisystem LCH >5 years from the end of treatment, using detailed audiological assessment and CT/MRI imaging of the petrous temporal bones.


RESULTS
The incidence of ear involvement in the acute phase of disease was 70%. Fifteen of the 39 patients tested (38%) had residual permanent hearing loss at long-term follow-up.


CONCLUSIONS
The incidence of hearing loss is much higher than has previously been reported in LCH, and may reflect a referral bias of young (<2 years) and more complex patients to our tertiary centre. However, the hearing loss appears to be highly specific to this patient group when compared to other long-term survivors of childhood cancers, probably due to the propensity of LCH to involve the ears. We therefore recommend audiology testing as an important part of long-term follow-up for patients with multisystem LCH.
Nanduri
Vasanta
V
Department of Paediatrics, Watford General Hospital, Watford, UK. vasanta.nanduri@whht.nhs.uk
Tatevossian
Ruth
R
Sirimanna
Tony
T
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D034381:Hearing Loss / Q000209:etiology*; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications*; D006801:Humans; D007223:Infant; D008297:Male; D017741:Survivors