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Cochlear Implantation Surgery in Jervell and Lange-Nielsen Syndrome Patients: Our Experience

  • By Rajashree D Godbole, Joyshankar Jana, Prithviraj Gauri, Amol B Mhetre
  • Case Reports
  • October-December 2019

Long QT syndrome (LQTS) is a genetic abnormality of ventricular repolarization. Congenital LQTS results from mutation in the genes encoding cardiac ion channels. Acquired LQTS is caused by drugs or metabolic abnormalities. The age of presentation in congenital LQTS may vary from in utero to adulthood.1 Majority of the events occurring in patients of LQTS are related to physical activity, excitement, and emotional stress.1 All patients of LQTS are not symptomatic. Many times LQTS patients are misdiagnosed and treated for epilepsy. Family history of sudden death may be present, but family history of sensorineural deafness is extremely uncommon. The morbidity and mortality are very high in patients with LQTS because of abnormal cardiac repolarization and prolonged QT interval. The anesthesiologists should be aware of necessary interventions and treatment modalities for LQTS patients.


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