An unfortunate case of Pendred syndrome

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A Sanei-Moghaddam1, T Wilson, S Kumar

  • 1Department of Otolaryngology, Addenbrooke's Hospital, Cambridge, UK. al.sane@doctors.net.uk

The Journal of Laryngology and Otology
|July 13, 2011
PubMed

Insights

A patient with Pendred syndrome experienced severe hypokalemia and cardiac arrest due to cochlear implant complications and alcohol use. This case highlights the need for careful management in Pendred syndrome patients undergoing cochlear implantation.

Area of Science:

  • Genetics and Audiology
  • Endocrinology

Background:

  • Pendred syndrome is a common genetic cause of sensorineural hearing loss, affecting the inner ear, thyroid, and kidneys.
  • Patients with Pendred syndrome often require cochlear implantation for hearing rehabilitation.
  • An underlying metabolic abnormality may be present but not clinically apparent.

Observation:

  • A patient with Pendred syndrome developed life-threatening hypokalemia.
  • This complication arose from an implant-induced imbalance and alcohol dependency.
  • The patient suffered multiple cardiac arrests as a result.

Findings:

  • Pendred syndrome, an autosomal recessive disorder, accounts for 10% of syndromic hearing loss.
  • Cochlear implantation is frequently pursued by Pendred syndrome patients.
  • Unforeseen metabolic issues can arise in these patients.

Implications:

  • Cochlear implantation in Pendred syndrome requires comprehensive understanding of the condition.
  • Proactive management is crucial to prevent severe morbidity.
  • This case underscores the importance of considering metabolic factors and substance use in managing Pendred syndrome patients with implants.
Abstract

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