ENT Pathology 6 - Osteosclerosis, Presbycusis, Vestibular schwannoma, H.P.V, E.B.V,Paraganglionoma

Question 1

What are some causes of congenital hearing loss in children?

Answer 1

Congenital cholesteatoma Rubella infection Tuner’s syndrome

Question 2

What are the presenting features of tuner’s syndrome?

Answer 2

Short tature Amenorrhea Can have hearing impairment from birth Wide nipples Elbow deformity causing a wide angle Some heart defects such as coarctation of the aorta

Question 3

In children with congenital hearing loss, what is a treatment option?

Answer 3

Treatment option is cochlear implant to regain some hearing

Question 4

What is an example of ear pathology that can cause both conductive or sensorineural hearing loss? (more commoonly conductive)

Answer 4

Osteosclerosis

Question 5

What happens with osteosclerosis? What bone is usually involved?

Answer 5

It involves the abnormal hardening of bone - usually the stapes bone is involved causing stapes fixation resulting in a conductive hearing loss

Question 6

What can normally be given for management of osteosclerosis?

Answer 6

A hearing aid

Question 7

Age related hearing loss What is this and what is it associated with?

Answer 7

Presbycusis, associated with accumulated environmental noise exposure

Question 8

In presbycusis, what frequencies are commonly lost first? What is the mainstay treatment?

Answer 8

Commonly high frequencies are lost first with mainstay of treatment being hearing aids

Question 9

Tumour of the myelin cells of CN VIII What is this?

Answer 9

Vestibular schwannoma (acoustic neuroma)

Question 10

What bone is the vestibular schwannoma usually within and what is its most common location?

Answer 10

Arises within the temporal bone Most commonly located at the cerebellopontine angle

Question 11

What produces myelin in the PNS and in the CNS?

Answer 11

CNS _ oligodendrocytes PNS - Schwann cells

Question 12

What imaging technique is required in acoutic neuroma? How can it present?

Answer 12

Use MRI to detect Can cause vertigo, hearing loss and tinnitus If it impinges on the facial nerve it can cause facial paralysis

Question 13

What can acoustic neuroma often be confused with due to its presentations?

Answer 13

Meniere’s disease

Question 14

If bilateral and young, what do you think for acoutisc neuromas?

Answer 14

NF2 - neurofibromatosis type 2 (NF2 gene mutation)

Question 15

What autoimmune disorder is characteried by small vessel vasculittis to resp tract and kindeys?

Answer 15

Wegener’s granulomatosis - granulomatosis with polyangiitis

Question 16

Which antibodies are attacked in Wegeners?

Answer 16

cANCA antibodies attacking PR3 (proteinase 3)

Question 17

EBV is linked with an increased chance of nasopharyngeal carcinoma and burkitt’s lymphoma What does it do to cause the survival of Bcells?

Answer 17

It hijacks Thelper cells leading to proliferation and survival of Bcells

Question 18

What does EBV produce activating cyclin D and promoting transition from G0 to G1? (this can cause the development of cancer cells)

Answer 18

Produces EBNA-2 (Epsteinn Barr virus Nuclear Antigen 2 - latent antibody in B lymphocytes)

Question 19

Strains 6 and 11 of human papilloma virus (HPV) can cause genital warts What else do they have an association with?

Answer 19

Association with squamous papillomas

Question 20

What is the most common cause of cancer in young people who have never smoked or drank?Term

Answer 20

Human Papilloma Virus (HPV)

Question 21

What are the two types of tumours that arise from chromaffin cells?

Answer 21

Phaeochromocytoma and paraganglionoma Paraganglionoma affects extra-adrenal structures as they arise from the neuroendocrine chromaffin cells outside of the adrenal gland

Question 22

How do both phaeochromocytomas and paraganglionomas present?

Answer 22

Both with sweating, palpitaions and headaches

Question 23

What is the most common benign tumour of the salivary glands? Which is most commonly affected?

Answer 23

Pleomorphic adenoma Paratid gland is most commonly affected - usually affects females above the age of 60

Question 24

What is the second most commonn benign salivary tumour (almost always with the parotid) and has a strong association with smoking?

Answer 24

Warthin’s tumour - both this and pleomoprhic adenoma usually have surgery

Question 25

A rare malignant tumour of the salivary glands (minor and major) What is this?

Answer 25

Adenoid cystic carcinoma

Question 26

A 70 year old man presents with hearing loss, blood stained discharge from the ear and facial paralysis What is this likely to be?

Answer 26

Squamous cell carcinoma of the ear

Question 27

What are the features of squamous cell carcinoma of the ear?

Answer 27

Hearing loss Otorrhea which may be blood stained Progressive otalgia (ear pain) Sometimes can have facial paralysis