Pendred Syndrome Causes Hearing Loss.

1
Pendred Syndrome
The Pendred Syndrome is a genetic disorder which
is associated with the Hearing Loss. It can also
affect the thyroid gland and this condition is
named as goiter. A thyroid gland is a
butterfly-shaped organ which is located at the
base of the neck. And, It produces the hormones.
Basically, this syndrome affects the bodys
ability to make a protein called pendrin, which
is important for normal functions of the inner
ear and thyroid.

• Symptoms of Pendred Syndrome
• Inner ear changes
• Enlargement of the thyroid gland
• Sensorineural Hearing loss present at the birth
  time.
• Balance problems due to altered function of the
• auditory system.

2
Pendred Syndrome

• Characteristics of Pendred Syndrome
• Cochlea with too few turns Cochlea is a
  (spiral-shaped)part of the inner ear which
  converts sounds into electrical signals which are
  sent to the brain. The cochlea of a person with
  Pendred syndrome may only have one-and-a-half
  turns whereas a normal and healthy cochlea has
  two-and-a-half turns. However, not everyone with
  Pendred syndrome has an abnormal cochlea.
• Enlarged Vestibular Aqueduct When the
  Vestibular Aqueduct is enlarged, the
  endolymphatic duct(balloon-shaped structure, end
  of the vestibular aqueduct) and sac grow large
  with excess fluid in comparison to their normal
  sizes.

3
Pendred Syndrome and Hearing Loss

• Pendred Syndrome can cause sensorineural
  deafness/hearing impairment. The syndrome causes
  hearing impairment, which is associated with
  inner ear malformations such as an enlarged
  vestibular aqueduct (EVA) and Cochlea with too
  few turns.
• The Vestibular Aqueduct is like a bony canal
  which connects the inner ear with the inside of
  the skull. There are some people who have the
  abnormality in the shaped of the cochlea. In this
  case, the cochlea has too few turns than a
  cochlea of normal hearing person.
• As you know that the loss of hearing often
  happens suddenly. Some people with this syndrome
  have the bilateral hearing loss. It means loss of
  hearing is in both ears.

4
Pendred Syndrome and Hearing Loss

• Acquired Hearing Loss
• Acquired Hearing Loss can show after birth and
  can happen at any time of illness or injury
  during childhood or adolescence. It can happen
  because of-
• Ear infections
• Head injury
• Medicines that are toxic to the ear
• Chickenpox
• Flu
• Loud noises like concerts and explosions
• Measles
• Meningitis