To analyze the clinical presentation, management, and outcome of two sisters affected by hereditary transthyretin related amyloidosis (ATTRv) and bilateral severe-to-profound hearing loss treated with cochlear implant.

A description of two patients underwent three cochlear implant (CI) surgery was performed. Age at time of implantation ranged from 48 to 53 years.

Both patients were initially equipped with a bilateral hearing aid and subsequently underwent cochlear implant. A bimodal stimulation was maintained thereafter. One case underwent bilateral surgery. Hearing outcomes were assessed by comparing pre- and post-operatively tonal and vocal hearing thresholds and speech perception tests (Common Evaluation Protocol Results in Rehabilitation Audiology).

In both patients, a significant and stable improvement of hearing threshold and speech perception was observed up to the last follow-up. No post-operative complication were observed during the follow-up.

ATTRv amyloidosis may be the cause of cochlear function impairment. Restoring the hearing in these group of patients is of paramount importance since the vision is often compromised too. Cochlear implant could be a viable strategy of rehabilitation for selected patients affected by severe-to-profound sensorineural hearing loss caused by ATTRv amyloidosis.