An unfortunate case of Pendred syndrome.

[pendred syndrome]

To report a patient with Pendred syndrome who developed life-threatening hypokalaemia as an unpredicted consequence of implant-induced imbalance and alcohol dependency , leading to multiple cardiac arrests .Addenbrooke 's Hospital , Cambridge , UK .C ase report and review of the English language literature concerning Pendred syndrome and cochlear implantation in Pendred syndrome patients .Pendred syndrome is an autosomal recessive disorder which mainly affects the inner ear , thyroid and kidneys . It accounts for 10 per cent of syndromic hearing loss cases . The majority of Pendred syndrome patients are referred to cochlear implant programmes for hearing assessment and therapy . They may also have an underlying metabolic abnormality which is not clinically apparent .Providing cochlear implants to patients with Pendred syndrome demands extensive knowledge of this condition , in order to avoid potential morbidity .

Diseases
Validation

Diseases presenting "hearing loss" symptom

22q11.2 deletion syndrome

achondroplasia

adrenomyeloneuropathy

alexander disease

benign recurrent intrahepatic cholestasis

canavan disease

cohen syndrome

congenital toxoplasmosis

dentinogenesis imperfecta

fabry disease

familial mediterranean fever

heparin-induced thrombocytopenia

hirschsprung disease

holt-oram syndrome

homocystinuria without methylmalonic aciduria

hydrocephalus with stenosis of the aqueduct of sylvius

kabuki syndrome

kallmann syndrome

neonatal adrenoleukodystrophy

pendred syndrome

von hippel-lindau disease

wolf-hirschhorn syndrome

This symptom has already been validated