Pendred syndrome
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Pendred syndrome is a genetic disorder leading to congenital bilateral (both sides) sensorineural hearing loss and goitre with euthyroid or mild hypothyroidism (decreased thyroid gland function). There is no specific treatment, other than supportive measures for the hearing loss and thyroid hormone supplementation in case of hypothyroidism. It is named after Vaughan Pendred (1869–1946), the British doctor who first described the condition in an Irish family living in Durham in 1896.[2][3] It accounts for 7.5% to 15% of all cases of congenital deafness.[4]
Quick Facts Other names, Specialty ...
Pendred syndrome | |
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Other names | Goiter-deafness syndrome[1] |
The normal cochlea has 2 & a half turns, but, in Pendred Syndrome, there is abnormal partitioning (the central bony core is reduced in size and complexity)and a reduced number of turns leading to a Mondini cochlea which has a basal turn and a dilated apical turn (1 & a half turns). There is also a dilated endolymphatic duct and sac with a widened vestibular aqueduct | |
Specialty | Endocrinology |
Named after | Vaughan Pendred |
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