Retrocochlear and CANS Pathologies Flashcards

Lecture 10-11 (91 cards)

1
Q

Several conditions that affect the _____ and its connections to the ______?

A

Auditory nerve and Central auditory nervous system (CANS)

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2
Q

Retrocochlear disorders are?

A

several conditions that affect the auditory nerve and its connections to the central auditory nervous system

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3
Q

What are some common retrocochlear diseases and disorders?

A
  • genetic/congenital conditions
  • auditory processing disorders
  • intracranial neoplasms
  • trauma to auditory nerve and temporal lobe
  • cerebrovascular accidents
  • central auditory nervous system dysfunction
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4
Q

Neoplasia means?

A

new growth

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5
Q

neoplasm is?

A

Abnormal mass tissue

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6
Q

Neoplasm is a disorder of ?

A

the cell cycle

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7
Q

growth of neoplasm is?

A

uncoordinated, competes with normal cells/tissue for energy and nutrition

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8
Q

neoplasms are:

A

purposeless, prey on host, and autonomous

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9
Q

Intracranial neoplasms

A

-benign or malignant tumors
- mass lesions of brain parenchyma (functional tissue)
- meninge tumors
- tumors of structures near brain tissue (affect function of brain tissue)

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10
Q

intracranial tumors arise from?

A

neuroglia cells

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11
Q

Neuroglia cells are the _______ of central nervous system

A

non excitable; supporting cells

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12
Q

neuroglial cells are ____ than neurons; lack ___; and do NOT ____ with other cells

A

smaller, axons, synapse

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13
Q

what are the four type of neuroglial cells?

A

1) Astrocytes
2) Oligodendrocytes
3) Microglia
4) Ependymal cells

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14
Q

Astrocytes

A
  • star shaped
  • support nerve cells
  • function as electrical insulators
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15
Q

Astrocytes provide a barrier

A

at synapses that contain neurotransmitters or hormones such as dopamine
- lack implicated in Parkinsons

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16
Q

Astrocytes play a role in formation of:

A

Blood brain barrier

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17
Q

Oligodendrocytes are active in ?

A

formation of myelin sheath for central nerve fibers

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18
Q

Microglia are:

A

small glial cells

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19
Q

Microglia are activated in presence of?

A

inflammation and degenerative processes within the CNS

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20
Q

what kind of function does Microglia have?

A

Phagocytic function

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21
Q

microglia ingest and removes?

A

neural residue

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22
Q

Ependymal cells are ?

A

epithelial cells that line the cerebrospinal fluid filled brain cavities and spinal cord

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23
Q

Some ependymal cells facilitate

A

CSF circulation and are involved in production of CSF

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24
Q

Benign tumors

A
  • slow growing
  • well defined borders, surgical removal effective
  • DO NOT METASTASIZE
  • not life threatening
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25
Malignant tumors
- fast growing - invasion and destruction of other structures - life threatening - Metastasize
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Intra-axial tumors
tumor resides in the brain tissue ex: astrocytoma, glioblastoma
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extra-axial tumors
resides outside brain, originates from brain tissue - neither neuronal/glial EX: meningiomas/ pineal & pituitary
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Where is the temporal bone positioned between?
middle and posterior cranial fossa
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4 segments of the temporal bone
1) Petrous 2) Squamous 3) Mastoid 4) Tympanic
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What segment of temporal bone houses inner ear?
Petrous (hardest part of temporal bone)
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What segment of the temporal bone forms the EAC?
Tympanic (matures by 3 years of age)
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Most vascular tumors of the temporal bone are ?
Benign
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Two categories of vascular tumors for classification?
1) Hemangiomas 2) Vascular malformations
34
Hemangiomas result in malformation of ?
angioblastic fetal tissue
35
Hemangiomas are made of
extra blood vessels
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Hemangiomas are characterized by
- rapid growth (proliferation phase) - slow period of involution (decrease in size)
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Vascular malformations are
abnormal development of blood vessels
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what is more common Hemangiomas or vascular malformations
Vascular malformations
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Vascular malformations are:
- present at birth - grow in proportion with body w/o regression - present any time during life
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vascular malformations present either
in the IAC or geniculate ganglion of VII N located in the fallopian canal
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why is there tumor preference for the region of CN VII
extensive blood supply surrounding the geniculate ganglion
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For Vascular malformations and Hemangiomas when do patients typically present with symptoms
3rd decade of life
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For vascular malformations/ hemangiomas: when the geniculate ganglion is site of origin, what is almost always present
VII N dysfunction (weakness or twitch)
44
Symptoms of vascular malformations and Hemangiomas
- hemifacial spasm - tinnitus - Conductive HL (geniculate ganglion eroding into ME) - Progressive SNHL ( IAC site) - Vertigo (lesion affecting CNVIII)
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Diagnosis of vascular malformations and hemangiomas
- case history and symptoms
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Diagnosis w/ Imaging for vascular malformations and hemangiomas ?
- high resolution CT Scan -MRI with contrast
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Imaging for vascular malformations and hemangiomas: T2 weighted images
lesions appear hyperintense on T2 fluid is bright on T2
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Imaging for vascular malformation/ hemangiomas. Geniculate lesions
- difficult to visualize on MRI, but can be detected on high resolution CT
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Differential diagnosis of vascular malformations/hemangiomas?
- meningiomas - VII N schwannoma - Cholesteatoma
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Why is meningioma a DD of vascular malformations /hemangiomas
irregular margins and may contain specks of calcium
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Why is CN VII schwannoma a DD of vascular malformations/ hemangiomas
hemangiomas prodcues facial nerve symptoms when smaller in size compared to CN VII schwannoma
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Why are cholesteatoma DD of vascular malformations/ hemangiomas
vascular lesion seen in ME cavity or invades other nearby structures
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treatment of vascular malformation and hemangiomas
surgical removal
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myelin sheath covering myelinated axons in CNS is formed by
oligodendrocytes
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in peripheral nervous system the myelin sheath is formed by ?
connective tissue cells, schwann cells
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What are myelin sheath of schwann cells of all peripheral nerves referred to?
neurilemmal sheath of schwann
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What is the most common benign tumor of the temporal bone and cerbellopontine angle?
Schwannoma - 6% intracranial tumors - 91% of all tumors in and around the temporal bone
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Within the temporal bone, the anatomic location of schwannoma is typically in three sites
1) internal auditory canal from CN VIII N 2) Jugular foramen for CN IX and X 3) fallopian canal of the VII CN
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vestibular schwannomas arise from ?
Schwann cells
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Vestibular schwannomas are
- benign extra axial tumors typically unilateral except for NF 2
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Incidence in vestibular schwannoma
- rare before 30 - 40 to 60 , 6th decade diagnosis higher incidence in females
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Vestibular schwannoma has two phases related to pressure:
1) otologic phase 2) neurologic phase
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The otologic phase of vestibular schwannoma
tumor compresses in the IAC HL: - compression of vascular supply of CnVIII - direct compression of CN VIII in IAC
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neurologic phase of vestibular schwannoma
tumor compresses on other intracranial structures
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Signs and Symptoms of Vestibular schwannoma
- hearing loss - headache - tinnitus - unsteady gait - imbalance/diziness - facial paralysis
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Audiometric findings of vestibular schwannoma: PURE TONE
- unilateral high frequency SNHL asymmetrical more profound at higher frequencies Brainstem lesions show flat unilateral SNHL
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Audiometric findings of vestibular schwannoma: TONE DECAY
positive tone decay
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Audiometric findings of of vestibular schwannoma: OAES
Normal OAES -decreased contralateral suppression
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Audiometric findings of vestibular schwannoma: SPEECH
WRS worse than pure tone thresholds, WRS worse in noise - positive rollover ratio
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Audiometric findings of vestibular schwannoma: IMMITTANCE
- tympanogram normal - ARTS = abnormal/absent (VIII lesion) -abnormal ipsilateral/contralateral for affected side (right ipsi right contra) brainstem lesion = cross over pathways affected normal ipsi, absent contra
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positive retrocochlear sign RD +++
reflex amplitude declines >50% in 5 sec at 500 and 1000 Hz
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questionable retrocochlear sign RD ++
reflex amplitude declines >50% in 5 sec at 1000 HZ but not 500 Hz
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not a significant retrocochlear sign RD +
reflex amplitude declines <50% in 5 sec at 500 and 1000 Hz
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ABR findings with vestibular schwannoma
increased wave V latency increased I-V inter wave latency - absent/abnormal wave V on affected size
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Treatment for Vestibular schwannoma
- observation -stereotactic radiosurgery (radiation) - surgery
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Meningioma
tumor that grows from membranes that surround the brain and spinal cord called the meninges
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Meningioma Incidence
later decades of life more common in females - associated with progesterone and breast cancer
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Meningioma etiology
- association with NF2 - radiation therapy to head - genetics * chromosomes 1,7,10, and 14 and telomerase activation * Di George syndrome is AD caused by deletion parts 22q
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Audiometric S/S of meningiomas
- progressive unilateral SNHL -vertigo - tinnitus - nausea/vomitting
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Audiometric findings of meningiomas
- VIII CN involvement * abnormal ARTS affected side * normal tymp * positive acoustic reflex decay * positive roll over and poorer scores of speech in noise * abnormal ABR NORMAL OAES
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Differential Diagnosis of Meningioma
- otitis media - Paraganglioma (glomus tumor) - Facial VII nerve involvement - Vestibular schwannoma
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Treatment of Meningioma
Surgical excision complete resection
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Benign Tumors of the temporal bone
- osteoma (tumor in the EAC) - paraganglioma (glomus tumor) - neurofibromatosis 2 - Facial nerve schwannoma
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Cortical tumors affecting the auditory cortex may show normal results for
peripheral auditory tests - Pure tone audiometry, ARTs, OAES WRS poorer in noise
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Cortical tumors with ABR
ABR will be normal if periphery is normal because ABR measures responses up to brainstem level
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Cortical tumors what signs are common
headaches and diziness
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Malignant tumors tumors of temporal bone examples:
adenocarcinoma and osteosarcoma
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Malignant tumors are often diagnosed at late stages of disease and present with dismal prognosis
symptoms similar to chronic suppurative otitis media
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Malignant tumors audiologic signs and symptoms are
- aural discharge with or without blood - otalgia - hearing loss - tinnitus
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Malignant tumors signs and symptoms of cranial neuropathies
- facial paralysis - headache - cochleovestibular deficits (SNHL and vestibular symptoms)
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Metastatic tumors of the temporal bone is metastasis from other sites to temporal bones, include:
breast cancer lung cancer renal carcinoma lymphoma and leukemia thyroid cancer osteoblastoma melanoma