ENT Pathology 6 - Osteosclerosis, Presbycusis, Vestibular schwannoma, H.P.V, E.B.V,Paraganglionoma Flashcards
What are some causes of congenital hearing loss in children?
Congenital cholesteatoma Rubella infection Tuner’s syndrome
What are the presenting features of tuner’s syndrome?
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
In children with congenital hearing loss, what is a treatment option?
Treatment option is cochlear implant to regain some hearing
What is an example of ear pathology that can cause both conductive or sensorineural hearing loss? (more commoonly conductive)
Osteosclerosis
What happens with osteosclerosis? What bone is usually involved?
It involves the abnormal hardening of bone - usually the stapes bone is involved causing stapes fixation resulting in a conductive hearing loss
What can normally be given for management of osteosclerosis?
A hearing aid
Age related hearing loss What is this and what is it associated with?
Presbycusis, associated with accumulated environmental noise exposure
In presbycusis, what frequencies are commonly lost first? What is the mainstay treatment?
Commonly high frequencies are lost first with mainstay of treatment being hearing aids
Tumour of the myelin cells of CN VIII What is this?
Vestibular schwannoma (acoustic neuroma)
What bone is the vestibular schwannoma usually within and what is its most common location?
Arises within the temporal bone Most commonly located at the cerebellopontine angle
What produces myelin in the PNS and in the CNS?
CNS _ oligodendrocytes PNS - Schwann cells
What imaging technique is required in acoutic neuroma? How can it present?
Use MRI to detect Can cause vertigo, hearing loss and tinnitus If it impinges on the facial nerve it can cause facial paralysis
What can acoustic neuroma often be confused with due to its presentations?
Meniere’s disease
If bilateral and young, what do you think for acoutisc neuromas?
NF2 - neurofibromatosis type 2 (NF2 gene mutation)
What autoimmune disorder is characteried by small vessel vasculittis to resp tract and kindeys?
Wegener’s granulomatosis - granulomatosis with polyangiitis
Which antibodies are attacked in Wegeners?
cANCA antibodies attacking PR3 (proteinase 3)
EBV is linked with an increased chance of nasopharyngeal carcinoma and burkitt’s lymphoma What does it do to cause the survival of Bcells?
It hijacks Thelper cells leading to proliferation and survival of Bcells
What does EBV produce activating cyclin D and promoting transition from G0 to G1? (this can cause the development of cancer cells)
Produces EBNA-2 (Epsteinn Barr virus Nuclear Antigen 2 - latent antibody in B lymphocytes)
Strains 6 and 11 of human papilloma virus (HPV) can cause genital warts What else do they have an association with?
Association with squamous papillomas
What is the most common cause of cancer in young people who have never smoked or drank?Term
Human Papilloma Virus (HPV)
What are the two types of tumours that arise from chromaffin cells?
Phaeochromocytoma and paraganglionoma Paraganglionoma affects extra-adrenal structures as they arise from the neuroendocrine chromaffin cells outside of the adrenal gland
How do both phaeochromocytomas and paraganglionomas present?
Both with sweating, palpitaions and headaches
What is the most common benign tumour of the salivary glands? Which is most commonly affected?
Pleomorphic adenoma Paratid gland is most commonly affected - usually affects females above the age of 60
What is the second most commonn benign salivary tumour (almost always with the parotid) and has a strong association with smoking?
Warthin’s tumour - both this and pleomoprhic adenoma usually have surgery
A rare malignant tumour of the salivary glands (minor and major) What is this?
Adenoid cystic carcinoma
A 70 year old man presents with hearing loss, blood stained discharge from the ear and facial paralysis What is this likely to be?
Squamous cell carcinoma of the ear
What are the features of squamous cell carcinoma of the ear?
Hearing loss Otorrhea which may be blood stained Progressive otalgia (ear pain) Sometimes can have facial paralysis
