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RESEARCH PRODUCT
The Jervell and Lange-Nielsen syndrome.
Francesco CusimanoEnrico MartinesCorrado Rizzosubject
Malemedicine.medical_specialtyPediatricsHeart diseaseElectrodiagnosisDeafnessSudden deathAutosomal recessive traitElectrocardiographyAudiometryLange-nielsen syndromemedicineCongenital sensorineural hearing lossHumansmedicine.diagnostic_testbusiness.industryInfantGeneral Medicinemedicine.diseaseSurgeryJervell and Lange-Nielsen syndromeLong QT SyndromeOtorhinolaryngologyPediatrics Perinatology and Child HealthFemalebusinessElectrocardiographydescription
Deafness and electrocardiographic changes (prolongation of the Q-T interval and inversion of the T wave) with a clinical picture of syncopal attacks and sudden death, were described as a distinct syndrome by Jervell and Lange-Nielsen in 1957. The syndrome is inherited as an autosomal recessive trait. In this study, all the cases reported since 1957 and their proposed prevalence are reviewed. The authors describe the 4 cases they have studied, all of which presented congenital sensorineural hearing loss and electrocardiographic changes characteristic of the syndrome. The relatively high number of cases they have encountered casts doubt on literature that states that the syndrome occurs more frequently in Northern-European populations. Consequently, it is advisable to perform an electrocardiogram in all children affected by congenital deafness.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 1991-07-01 | International journal of pediatric otorhinolaryngology |