Cochlear Implants in Alström Syndrome....................Alström syndrome is a rare condition that affects many body systems. ... Alström syndrome is characterized by a progressive loss of vision and hearing, a form of heart disease that enlarges and weakens the heart muscle (dilated cardiomyopathy), obesity, type 2 diabetes (the most common form of diabetes), and short stature. ghr.nlm.nih.gov › condition › alstrom-syndrome Alström syndrome - Genetics Home Reference - NIH

Cochlear Implants in Alström Syndrome.:

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Cochlear Implants in Alström Syndrome.

Ann Otol Rhinol Laryngol. 2020 Feb 05;:3489420903061

Authors: Flavia G, Samanta G, Patrizia T, Ezio C, Francesca D, Pietro M, Roberto B

Abstract

OBJECTIVES: Too little is known about hearing loss rehabilitation in patients with Alström syndrome (AS). Benefits of hearing aids (HA) have not been fully documented and only one case treated with a Cochlear Implant (CI) has been described in the proceedings of a conference. Furthermore, comorbidities and risk of complications following surgical intervention may contraindicate Cochlear Implant procedures in these patients. The present case report concerns the first AS patient with CI in the literature.

METHODS: After reporting a concise description of the audiological profile of patients with AS described in the literature, the case of a 22-year-old woman with genetically confirmed Alström syndrome who underwent a sequential bilateral CI (Bi-CI) rehabilitation is reported. Audiological results before and after cochlear implantation are described.

RESULTS: The patient showed an excellent functional outcome with CIs, which enabled her to achieve communicative, social and academic results comparable with her peers, and no complications occurred.

CONCLUSIONS: AS is not necessarily an absolute contraindication to CI. For many AS patients, a good cognitive function and adequate life expectancy represent a clear indication to prompt and adequate hearing rehabilitation with CIs. The description of this type of clinical cases could in the future also generate indications for a tailored audiological treatment of patients with very specific needs, such as patients with Alström Syndrome.

PMID: 32019320 [PubMed - as supplied by publisher]