Hearing Loss Associated with Cardiac Defects

Question 1

Is JLNS common?

Answer 1

Yes
It is the 3rd more common AR syndromic hearing loss

Question 2

What is the transmission mode of JLNS?

Answer 2

AR

Question 3

Can carriers of JLNS also die suddenly due to cardiac problems?

Answer 3

Yes

Question 4

Are potassium channels insufficient in JLNS?

Answer 4

Yes
Causes hearing loss and heart problems

Question 5

What are the clinical features of JLNS?

Answer 5

Long-QT interval (>500 ms) on the EKG with functional heart disease, congenital deafness, and sudden death

Question 6

What does long QT mean?

Answer 6

Heart takes longer to recharge between beats

Question 7

Are syncopal episodes common for people with JLNS?

Answer 7

Yes
In most cases, syncopal attacks are precipitated by fear, excitement, physical exertion, and loud noises
These children should not be allowed to swim unsupervised due to higher incidence of fainting spells, increasing risk of drowning

Question 8

What are the audiological findings of JLNS?

Answer 8

Congenital deafness
Bilateral severe-to-profound SNHL
Complete degeneration of organ of corti
Loss of sensory hair cells
Generally no vestibular symptoms reported

Question 9

Are seizures reported in some cases of JLNS?

Answer 9

Yes

Question 10

Is SIDS linked to JLNS?

Answer 10

Possibly
Just a hypothesis

Question 11

Is genetic counseling imperative for people with JLNS?

Answer 11

Yes

Question 12

What are some ways to manage JLNS?

Answer 12

Avoid stress, exercise, and drugs
Have a cardiac consult
Beta blockers to treat irregular heartbeat
Pacemakers and defibrillators

Question 13

Is consanguinity common in JLNS?

Answer 13

Yes

Question 14

What is a differential diagnosis for JLNS?

Answer 14

Ward-Romano syndrome

Question 15

What is ward-romano syndrome?

Answer 15

Long QT syndrome but with normal hearing
AD inheritance