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. Copyright 2009 Wiley-Liss, Inc.
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. Copyright 2009 Wiley-Liss, Inc.