Pendred syndrome causing hearing loss.

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 body’s ability to make a protein called pendrin, which is important for normal functions of the inner ear and thyroid.

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 body’s 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