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Hearing loss in Langerhans cell histiocytosis: close association with central nervous system consequences
Akira Kaino1, Kenichi Sakamoto2,3, Kunihiko Moriya4
1Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.
Insights
Hearing loss is a permanent consequence of Langerhans cell histiocytosis (LCH). This study found hearing loss significantly associated with central nervous system (CNS) complications in pediatric LCH patients.
Area of Science:
- Pediatric Oncology
- Otolaryngology
- Neurology
Background:
- Hearing loss is a known permanent consequence of Langerhans cell histiocytosis (LCH).
- The specific characteristics and associations of hearing loss with central nervous system (CNS) complications in LCH are not well understood.
Purpose of the Study:
- To investigate the characteristics of hearing loss in pediatric patients with LCH.
- To determine the association between hearing loss and central nervous system (CNS)-related permanent consequences in LCH.
Main Methods:
- Retrospective analysis of 317 pediatric patients with multisystem or multifocal bone LCH.
- Data collected from the Japan LCH Study Group -96 or -02 trials.
- Evaluation of hearing loss incidence, type, and association with LCH characteristics and CNS complications.
Main Results:
- Hearing loss occurred in 2.8% of patients, lower than previously reported.
- Hearing loss was significantly associated with ear lesions and CNS-PCs (p=0.018).
- Seven patients had sensorineural or mixed-type hearing loss, often severe or profound and unilateral.
Conclusions:
- Patients with LCH-related hearing loss require close monitoring for CNS complications.
- Individuals with LCH-associated neurodegeneration (ND) need careful surveillance for sensorineural hearing loss development.
Background:
Hearing loss is a recognized permanent consequence (PC) of Langerhans cell histiocytosis (LCH). However, its characteristics and association with central nervous system (CNS)-related PCs remain unclear.
Procedure:
This study retrospectively analyzed the data of 317 pediatric patients with multisystem or multifocal bone LCH enrolled in the Japan LCH Study Group -96 or -02 trial.
Results:
Hearing loss was identified in nine patients (2.8%), a lower incidence than previously reported. It was significantly associated with ear lesions, but not with craniofacial bone involvement at the time of diagnosis. Detailed information was available for seven patients: Three had sensorineural hearing loss, and four had mixed-type hearing loss without hearing improvement. At the last follow-up, hearing loss was unilateral in six cases and bilateral in one, with severity ranging from moderate (n = 1) to severe (n = 2) and profound (n = 4). CNS-PCs were found in five patients and were significantly associated with hearing loss (p = 0.018). One patient without ear lesions developed progressive sensorineural hearing loss due to neurodegeneration (ND).
Conclusions:
Patients with hearing loss should be closely monitored for CNS-PCs, and those with LCH-associated ND should be carefully monitored for the development of sensorineural hearing loss.
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