Progressive cochlear ossification secondary to Langerhans cell histiocytosis requiring sequential bilateral cochlear implantation: a case report

OBJECTIVES: Langerhans cell histiocytosis (LCH) is a rare disorder that may involve the temporal bone and, in rare cases, result in sensorineural hearing loss. This report highlights the importance of close radiological surveillance and timely cochlear implantation in the setting of progressive otic capsule ossification.

No actionable change for routine practice; this case highlights that Langerhans cell histiocytosis should be considered in the differential for progressive cochlear ossification, and early implantation before complete ossification may preserve surgical options — but evidence is limited to a single case.

This is an exceptionally rare aetiology of cochlear ossification, and documenting successful sequential bilateral cochlear implantation expands the known surgical options for clinicians encountering this unusual presentation.

1. 01Langerhans cell histiocytosis (LCH) is a rare immune-cell disorder that can infiltrate and ossify the cochlea (the inner-ear hearing structure).
2. 02Progressive cochlear ossification from LCH led to profound bilateral hearing loss requiring sequential bilateral cochlear implantation.
3. 03Case report format: single patient, so findings cannot be generalised.
4. 04Temporal bone involvement by LCH is extremely uncommon, making this one of a small number of published cases with CI as treatment.
5. 05Early recognition of LCH-related temporal bone disease may allow timely cochlear implantation before full ossification forecloses insertion.

PMID

42217228

DOI

10.1080/14670100.2026.2670091.

Journal

Cochlear Implants International

Publication type

case_report

Evidence level

4

Sample size

1

Population

Single patient with progressive cochlear ossification secondary to Langerhans cell histiocytosis involving the temporal bone

Intervention

Sequential bilateral cochlear implantation