Evaluation of iron chelator administration, and cochlear implantation for superficial siderosis

Evaluation of iron chelator administration, and cochlear implantation for superficial siderosis - ECHACISS

Status

Recruiting

Phases

Unknown

Study type

Interventional

Source

JPRN

Registry ID

JPRN-jRCTs031180438

Enrollment

Registered

2019-03-27

Start date

2019-04-01

Completion date

Unknown

Last updated

2026-06-29

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

Superficial siderosis siderosis, cerebellar ataxia, hearing loss, superficial siderosis

Interventions

1. Administration of Deferiprone orally 30mg/kg after every meal for 36 months. 2. Artificial cochlear implantation for severe hearing disturbance

iron chelator administration, cochlear implantation

Eligibility

Sex/Gender

All

Inclusion criteria

Inclusion criteria: 1) Patients who manifest a triad (hearing loss, cerebellar ataxia, myelopathy), and show characteristic brain hemosiderin deposit and cerebellar atrophy on brain MRI, and is diagnosed as Superficial Siderosis. Cessation of bleeding into the cerebrospinal fluid has been confirmed by treatment, such as surgical operation or oral administration. Patients whose symptom progression is objectively confirmed. The duration of disease does not matter (regardless of gender). 2) Patients who aged 20 years or older at the time of acquiring consent, and obtained document consent from the patient. 3) Patients who do not have abnormal findings such as leukopenia and liver dysfunction by blood test. 4) Clinical data for follow-up among patients who do not agree with the oral administration of a chelator, and agree to use their data will be target patient group.

Exclusion criteria

Exclusion criteria: 1) Patients with a history of hypersensitivity to deferiprone or other an iron chelator. 2) Patients taking internal medication or drip medication with side effects of leukopenia. 3) Pregnant women or patients who may be pregnant, lactating patients or patients wishing for pregnancy during the study period. 4) Patients who have difficult MRI imaging such as claustrophobia. 5) In addition, patients who are judged inappropriate to be included in the study by an investigator or a test sharing doctor.

Design outcomes

Primary

Measure	Time frame
Hearing ability, ataxia, iron deposition amount	—

Secondary

Measure	Time frame
Rate of treatment continuation, Blood biochemistry, Cerebrospinal fluid, adverse effects	—

Contacts

Public ContactSanjo Nobuo

Institute of Science Tokyo Hospital

n-sanjo.nuro@tmd.ac.jp+81-3-5803-5234

Outcome results

None listed

Source: JPRN (via WHO ICTRP) · Data processed: Jul 3, 2026