Cochlear implantation in patients with Jervell and Lange-Nielsen syndrome, and a review of literature

Cochlear implantation in common forms of genetic deafness

“…The authors noted gradual improvement on the categories of auditory performance (CAP) and speech intelligibility rating (SIR). The CAP and SIR were sustained at 7 and 5, respectively, at 36 months follow-up 73. Daneshi et al implanted three children less than 3 years of age.…”

“…Beta blockers are recommended for control of the arrhythmia. Implantable cardioverter-defibrillator can reduce mortality 70-73…”

“…It may be prudent to perform an EKG on those with congenital deafness before operation to identify at risk individuals and prevent possible cardiac complications 73. Beta blockers should be considered perioperatively.…”

Cochlear implants in genetic deafness

“…On ECG, QT interval is often >0.44 s in male patients and >0.46 s in female patients, which can lead to ventricular tachycardia/fibrillation or torsive ventricular tachycardia or even sudden death, often triggered by excitement, strenuous exercise or noise that leads to high levels of catecholamine release (as seen when swimming in about 16% of such cases) (Chorbachi et al, 2002;Daneshi et al, 2008). b blockers are often used to control arrhythmia, while pace-maker and defribrillator implants are used to reduce sudden death (Chorbachi et al, 2002;Yanmei et al, 2008).…”

“…The two younger brothers enjoyed good hearing results from cochlear implant. Yanmei et al (2008) reported a 3 years old child who achieved a CAP score of 7 and a SIR score of 5 at 36 months after cochlear implant. In a follow up study by Daneshi et al (2008) with three children younger than 3 years, the CAP and SIR scores were 6 and 4, respectively, at 48 months after cochlear implant, and all three children were able to attend regular education.…”