An unfortunate case of Pendred syndrome.

OBJECTIVE

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.

SETTING

Addenbrooke's Hospital, Cambridge, UK.

METHOD: Case report and review of the English language literature concerning Pendred syndrome and cochlear implantation in Pendred syndrome patients.

RESULT: 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.

CONCLUSION

Providing cochlear implants to patients with Pendred syndrome demands extensive knowledge of this condition, in order to avoid potential morbidity.