5A00.02

ICD-11 MMS 2026-01

Pendred syndrome

Description

Pendred syndrome is characterised by the association of congenital bilateral neurosensory deafness, thyroid goitre, cochleovestibular malformation and potential vestibular dysfunction.

Index terms

  • Pendred syndrome
  • Goitre deafness
  • goitre-deafness syndrome

Exclusions from above levels

  • 18 – Pregnancy, childbirth or the puerperium
  • http://id.who.int/icd/entity/1004987305 – Transitory endocrine or metabolic disorders specific to fetus or newborn

Sibling codes

  • 5A00.00 – Permanent congenital hypothyroidism with diffuse goitre
  • 5A00.01 – Permanent congenital hypothyroidism without goitre
  • 5A00.03 – Transient congenital hypothyroidism
  • 5A00.04 – Congenital hypothyroidism due to iodine deficiency
  • 5A00.0Y – Other specified congenital hypothyroidism
  • 5A00.0Z – Congenital hypothyroidism, unspecified

Foundation entity

Pendred syndrome – http://id.who.int/icd/entity/1156056623

Part of: 5A00.0: Congenital hypothyroidism › 5A00: Hypothyroidism › 05: Endocrine, nutritional or metabolic diseases