ENT

Question 1

Describe the types of Ushers syndrome?

Answer 1

Type 1 – profound loss, congenital, absent vestibular response
Type 2- sloping audiogram, congenital and normal vestibular system
Type 3- Progressive variable vestibular system and onset

Question 2

What are some common findings in Wardenbergs syndrome?

Answer 2

Eyes often different colour
Eyes look wider apart but this is due to the slant of the eyelids
Congenital White forelock
Congenital SN

Question 3

What is the inheritance pattern of Treacher Collins?

Answer 3

Autosomal dominant

Question 4

What are some clinical signs seen in Treacher Collins?

Answer 4

Under development of cheek bones
Abnormal outer and middle ear
Conductive loss

Question 5

Name other syndromes related to hearing loss

Answer 5

Pendred syndrome
Dilated vestibular aqueducts
SN loss

Alport syndrome
Hearing loss can deteriorate

Question 6

What Congenital infections can cause hearing loss?

Answer 6

CMV causes 30%
Rubella
Syphilis
Herpes

Question 7

What program at birth screens for hearing losses?

Answer 7

Newborn Hearing Screening Programme
aims at birth to identify moderate, severe and profound deafness and hearing impairment in newborn babies.

**Misses mild losses or sloping losses

Question 8

What high risk groups have hearing monitored?

Answer 8

Cystic Fibrosis/ Chemotherapy before and after treatments due medication
CMV (Cytomegalovirus)
Syndromes as previously noted
Head Trauma
Cleft lip/palate
Downs Syndrome

Question 9

Damage to what areas of the ear can lead to hearing loss?

Answer 9

Hearing loss can occur from a problem with the outer ear, middle ear or the cochlea or nerve of hearing.

Question 10

What are the types of hearing loss?

Answer 10

Conductive Hearing Loss

Sensori-neural Hearing Loss

Mixed Hearing Loss

Question 11

What area does conductive loss affect and name some causes?

Answer 11

Conductive loss- a problem with the middle ear

Some Possible Causes
 Glue ear
 Ear wax
 Middle ear infection
 perforated eardrum
 abnormality of the outer ear
 eustachian tube dysfunction

Question 12

What are the managements for Conductive loss?

Answer 12

Most conductive losses will resolve themselves over time or are operable.
Hearing Aids may be offered for persistent losses (as an alternative to grommet insertion)
In the case of permanent conductive losses, Bone Anchored Hearing Aid (BAHA) may be fitted

Question 13

What are the managements for sensorineural loss?

Answer 13

Sensori-neural losses are usually permanent
These losses are usually managed by hearing aids
The aim is to raise the level of hearing so that as much speech is as audible as possible
In the case of profound hearing losses, who cannot receive sufficient benefit from hearing aids, cochlear implants may be recommended.