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Background <p>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.</p> Procedure <p>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.</p> Results <p>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 (<i>n</i> = 1) to severe (<i>n</i> = 2) and profound (<i>n</i> = 4). CNS-PCs were found in five patients and were significantly associated with hearing loss (<i>p</i> = 0.018). One patient without ear lesions developed progressive sensorineural hearing loss due to neurodegeneration (ND).</p> Conclusions <p>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.</p>

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Hearing loss in Langerhans cell histiocytosis: close association with central nervous system consequences

  • 期刊论文

  • 发表日期：

    2026年1月19日

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  • Akira Kaino,
  • Kenichi Sakamoto,
  • Kunihiko Moriya,
  • Shinya Osone,
  • Toshihiko Imamura,
  • Kazuko Kudo,
  • Yoko Shioda,
  • Shinsaku Imashuku,
  • Akira Morimoto

摘要

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.