Evidence map›Paper›PMID 39210073›Full record

ArticleEuropean archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery2025

Cochlear implants for hereditary ATTR amyloidosis: a case report of two cases.

Francesco Maccarrone, Maurizio Negri, Paola Benincasa, Maurizio Guida, Maurizio Falcioni, Leonardo Roncadi

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Article in European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

6 authors.

Francesco MaccarroneOtolaryngology-Head and Neck Surgery Department, "Ramazzini" Hospital of Carpi, Via Guido Molinari 2, Carpi, Italy.
Maurizio NegriOtolaryngology-Head and Neck Surgery Department, "Ramazzini" Hospital of Carpi, Via Guido Molinari 2, Carpi, Italy.
Paola BenincasaOtolaryngology-Head and Neck Surgery Department, "Ramazzini" Hospital of Carpi, Via Guido Molinari 2, Carpi, Italy.
Maurizio GuidaOtolaryngology and Otoneurosurgery Department, University Hospital of Parma, Viale Antonio Gramsci 14, Parma, Italy.
Maurizio FalcioniOtolaryngology and Otoneurosurgery Department, University Hospital of Parma, Viale Antonio Gramsci 14, Parma, Italy.
Leonardo RoncadiOtolaryngology-Head and Neck Surgery Department, "Ramazzini" Hospital of Carpi, Via Guido Molinari 2, Carpi, Italy. leonardo.roncadi.med@gmail.com.ORCID http://orcid.org/0009-0004-3452-327X

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

purposeTo 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.

methodsA 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).

resultsIn 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.

conclusionsATTRv 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.

Indexed as

Amyloid Neuropathies, FamilialCochlear ImplantationCochlear ImplantsHearing Loss, BilateralHearing Loss, SensorineuralFemaleHumansMiddle AgedSpeech PerceptionAmyloidosisCase seriesCochlear implantHearing loss

Identifiers

PMID39210073

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