ME Tumors

Question 1

most common benign soft tissue tumor of the ME (rarely malignant)

Answer 1

paragangliomas

Question 2

can happen anywhere in body, including under nail beds

Answer 2

paragangliomas

Question 3

Second most common benign tumor of the temporal bone

Answer 3

paragangliomas

Question 4

Arising along the course of the Jacobson nerve primarily in the tympanic cavity

Answer 4

glomus tympanicum

Question 5

Arise from the dome of the internal jugular vein bulb and involve jugular foramen and related structures

Answer 5

glomus jugulare

Question 6

slow progressive growth spreading through path of least resistance such as temporal bone air cells and ET
Diagnosis can occur ~ 4 to 10 years after initial occurrence
They can grow to large proportions (typically, glomus jugulare)

Answer 6

glomus tympanicum & jugulare

Question 7

describe paraganglioma (glomus tumor)

Answer 7

typically unilateral, reddish-purple, highly vascular, and lobulated
Glomus tumors arise from paraganglia cells
They were incorrectly called glomus tumors originally because it was believed that they originated from true glomus (arteriovenous) complexes

Question 8

small groups of flat, oval, vascular paraganglionic bodies connected with the ganglia of the sympathetic nervous system

Answer 8

paraganglia cells

Question 9

exist throughout the temporal bone including the dome of the internal jugular vein, promontory of the ME, Arnold and Jacobson’s nerves

Answer 9

paraganglia cells

Question 10

why were they incorrectly called glomus tumors originally

Answer 10

it was believed that they originated from true glomus (arteriovenous) complexes
But the name glomus tumor persists

Question 11

jacobsons nerve is one of the areas it can come from

Answer 11

glomus tympanicum

Question 12

heredity of glomus tumors

Answer 12

Sporadic or AD inheritance with 100% penetrance
Mutation of gene SDHD mapped to 11q23 may be involved in these tumors
Genomic imprinting may be involved

Question 13

Affected individuals inherit the disease from their father but expression of the phenotype may not be observed in off-springs of affected females until transmission through a male carrier

Answer 13

glomus tumors

Question 14

incidence of paraganglioma

Answer 14

Patients usually present after the 5th decade of life
More common in females (males may be carriers if genetic)
Even with modern genetic testing, the majority of paragangliomas appear to be sporadic
However, approximately one-third to one-half are associated with an inherited syndrome including some cases of NF1
So far 10 genes have been associated with paragangliomas with or without pheochromocytoma

Question 15

Typically arises from the promontory of the ME

Answer 15

glmous tympanicum

Question 16

signs of glomus tympanicum

Answer 16

Pulsatile tinnitus because of the tumor vascularity is often the first presenting symptom
Tympanic membrane may appear red due to increased vasculature of the ME cavity
Lateral growth through the TM can mimic a bleeding polyp
Growth of tumor can inhibit ossicular mobility resulting in a conductive hearing loss
Medial growth towards the inner ear can cause SNHL, facial nerve dysfunction, and/or vertigo

Question 17

treatment of glomus tympanicum

Answer 17

Surgery: Complete tumor removal is seen in > 90% of cases

Question 18

arise from the dome of the internal jugular vein or proximal portion of Arnold’s or Jacobson’s nerve
More common and extensive than glomus tympanicum because of the space available to grow

Answer 18

glomus jugulare

Question 19

symptoms of glomus jugulare

Answer 19

Hearing loss
Otalgia
Aural fullness
Vertigo, if involvement of vestibular division of VIII N
Hoarseness and dysphagia due to involvement of the CN IX, X, and XI because of proximity to site of origin of glomus jugulare
Involvement of XII (hypoglossal) nerve indicates more extensive disease

Question 20

otoscoppy of glomus jugulare

Answer 20

red mass filling the ME cavity or lower portion of the ME cavity may be visible

Question 21

pure tone results of glomus jugulare

Answer 21

conductive hearing loss may be present depending on the size of the tumor and involvement of ossicles
Sometimes a mixed hearing loss may occur if neural involvement has occurred

Question 22

immittance

Answer 22

Jerger type AS tymps

Jerger type B

Tympanogram also will show jagged edges which will correspond to the patient’s pulse

Pulsating tympanogram, suspect glomus tumor

Question 23

treatment of jugulare

Answer 23

surgery or radiation