Cochlear Implantation Outcomes in Paget's Disease of the Bone: A Systematic Review

The cause of sensorineural hearing loss in Paget's disease is unknown, but it may occur due to impingement on the auditory nerve of effects of bony remodeling on the cochlea. Our goal was to review disease-specific prognostic factors of Paget's disease of the bone on cochlear implant (CI) outcomes.

PubMed, Embase, and Web of Science were queried from inception to January 2024 for case reports and series available in the English language.

Following PRISMA guidelines, articles describing CI outcomes in patients with Paget's disease were reviewed. Data were collected on demographics, Paget's disease activity and history, hearing, and surgical outcomes. Study validity was assessed using the Joanna Briggs Institute's critical appraisal tools and AMSTAR 2 criteria.

Of the 125 abstracts screened, 8 articles met the inclusion criteria, comprising 9 cases. The cohort included 4 males and 5 females, aged 3 to 86 years. Hyper-osteoblastic changes in the petrous part of the temporal bone and/or the otic capsule were observed on CT in 8 patients. Electrode insertion favored cochleostomy (n=5) over the round window technique (n=1). All patients had initial improvement in hearing and speech recognition scores. Long-term hearing outcomes at 36 to 72 months were reported in 8 cases, with 7 maintaining improved hearing outcomes and 1 reporting gradual worsening by 36 months. No complications were reported.

Cochlear implantation can effectively treat hearing loss in patients with Paget's disease of the bone and may be more technically challenging due to underlying anatomic changes. Imaging may help guide surgical planning and insertion approach. Larger cohort studies are needed to quantify the risks associated with cochlear implantation in patients with Paget's disease of the bone.