Cochlear Implants in Alström Syndrome

Ann Otol Rhinol Laryngol. 2020 Aug;129(8):833-837. doi: 10.1177/0003489420903061. Epub 2020 Feb 5.


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.

Keywords: Alström syndrome; cochlear implant; deafness.

Publication types

  • Case Reports

MeSH terms

  • Alstrom Syndrome / complications*
  • Audiometry
  • Cochlear Implants*
  • Deafness / etiology
  • Deafness / physiopathology
  • Deafness / surgery*
  • Female
  • Humans
  • Speech Perception / physiology*
  • Young Adult