Otosclerosis

1. Otosclerosis is genetically transmitted by

Autosomal dominance with complete penetrance

Autosomal dominance with incomplete penetrance

Autosomal recessive with complete penetrance

Autosomal recessive with incomplete penetrance

Otosclerosis is a condition that affects the bones in the middle ear, causing hearing loss. The correct answer is autosomal dominance with incomplete penetrance. This means that the condition is inherited in an autosomal dominant manner, where a single copy of the mutated gene is enough to cause the condition. However, the penetrance is incomplete, meaning that not everyone who inherits the mutated gene will develop the condition. This suggests that other factors, such as environmental or additional genetic factors, may influence the expression of the condition.

Explanation

Otosclerosis is a condition that affects the bones in the middle ear, causing hearing loss. The correct answer is autosomal dominance with incomplete penetrance. This means that the condition is inherited in an autosomal dominant manner, where a single copy of the mutated gene is enough to cause the condition. However, the penetrance is incomplete, meaning that not everyone who inherits the mutated gene will develop the condition. This suggests that other factors, such as environmental or additional genetic factors, may influence the expression of the condition.

2. A patient presents for their post-op visit following stapedotomy for otoslclerosis. They are complaining of severe SNHL. What diagnosis was most likely missed on initial evaluation prior to surgery?

Prior Hx of skullbase fracture

Acoustic Neuroma

Meniere disease

Semicircular Canal Dehiscence

(Meniere disease is an absolute contraindication for stapedectomy)

Explanation

(Meniere disease is an absolute contraindication for stapedectomy)

3. What is the minimum amount of time to wait before attempting a stapedectomy on the second ear?

2 months

4 months

6 months

12 months

The minimum amount of time to wait before attempting a stapedectomy on the second ear is 6 months. This is because stapedectomy is a surgical procedure to treat hearing loss caused by otosclerosis, a condition where the stapes bone in the middle ear becomes fixed and cannot vibrate properly. Performing the surgery on the second ear too soon after the first surgery may increase the risk of complications and affect the healing process. Waiting for 6 months allows sufficient time for the first ear to heal and ensures a better outcome for the second surgery.

Explanation

The minimum amount of time to wait before attempting a stapedectomy on the second ear is 6 months. This is because stapedectomy is a surgical procedure to treat hearing loss caused by otosclerosis, a condition where the stapes bone in the middle ear becomes fixed and cannot vibrate properly. Performing the surgery on the second ear too soon after the first surgery may increase the risk of complications and affect the healing process. Waiting for 6 months allows sufficient time for the first ear to heal and ensures a better outcome for the second surgery.

4. The artery that courses in the obturator foramen of the stapes is the embryologic remnant of the _____

First branchial arch

Second branchial arch

Third branchial arch

Fourth branchial arch

(the persistant stapedial artery must be divided or the procedure must be aborted)

Explanation

(the persistant stapedial artery must be divided or the procedure must be aborted)

5. A profuse perilymph gusher secondary to a patent cochlear aqueduct is seen more frequently which side?

Left

Right

A profuse perilymph gusher secondary to a patent cochlear aqueduct is seen more frequently on the left side.

Explanation

A profuse perilymph gusher secondary to a patent cochlear aqueduct is seen more frequently on the left side.

6. A patient with suspected otosclerosis presents to your office for testing. What may be a characteristic finding on audiogram?

Normal speech discrims, 5dB loss at 2000Hz and 20dB loss at 500 and 4000Hz

Abnormal speech discrims,20dB loss at 2000Hz and 5dB loss at 500 and 4000Hz

Abnormal speech discrims, 5dB loss at 2000Hz and 20dB loss at 500 and 4000Hz

Normal speech discrims, 20dB loss at 2000Hz and 5dB loss at 500 and 4000Hz

(otherwise known as Carhart notch)

Explanation

(otherwise known as Carhart notch)

7. What is the risk of postoperative deafness in stapedectomy?

2%

4%

6%

8%

The risk of postoperative deafness in stapedectomy is 2%. This means that out of every 100 stapedectomy procedures, 2 patients may experience deafness after the surgery. Stapedectomy is a surgical procedure performed to treat hearing loss caused by otosclerosis, a condition where the stapes bone in the middle ear becomes fixed and cannot vibrate properly. While the procedure is generally safe and effective, there is still a small risk of complications, including postoperative deafness.

Explanation

The risk of postoperative deafness in stapedectomy is 2%. This means that out of every 100 stapedectomy procedures, 2 patients may experience deafness after the surgery. Stapedectomy is a surgical procedure performed to treat hearing loss caused by otosclerosis, a condition where the stapes bone in the middle ear becomes fixed and cannot vibrate properly. While the procedure is generally safe and effective, there is still a small risk of complications, including postoperative deafness.

8. What is the percentage of patients in which there is increased vascularity of the cochlear promontory in the early phase of OS?

10%

20%

30%

40%

(The phase is otospongiosis and the sign is Schwartze sign – reddish hue behind an intact TM)

Explanation

(The phase is otospongiosis and the sign is Schwartze sign – reddish hue behind an intact TM)

9. Your patient is currently being treated medically with sodium fluoride for otosclerosis and presents with complaints of nausea, what is your next step in management?

Discontinue medication

Lower dose of medication

Recommend taking medication with food

Recommend taking medication at night

If a patient presents with complaints of nausea while being treated with sodium fluoride for otosclerosis, the next step in management would be to lower the dose of the medication. Nausea is a common side effect of sodium fluoride, and reducing the dosage may help alleviate this symptom while still providing the necessary treatment for otosclerosis. Discontinuing the medication completely may not be necessary if the patient is benefiting from it, and recommending taking the medication with food or at night may not address the underlying issue of nausea caused by the medication.

Explanation

If a patient presents with complaints of nausea while being treated with sodium fluoride for otosclerosis, the next step in management would be to lower the dose of the medication. Nausea is a common side effect of sodium fluoride, and reducing the dosage may help alleviate this symptom while still providing the necessary treatment for otosclerosis. Discontinuing the medication completely may not be necessary if the patient is benefiting from it, and recommending taking the medication with food or at night may not address the underlying issue of nausea caused by the medication.

10. A patient post-op stapedectomy is in your office a week after surgery. They complain that their hearing was better and now is worse. Audiogram confirms MHL severe in the high frequencies. What complication is suspected?

Acute otitis media

OW fistula

TM perforation

Reparative granuloma

Reparative granuloma is suspected as a complication in this case. A stapedectomy is a surgical procedure performed to improve hearing in patients with otosclerosis. However, in some cases, a reparative granuloma can form at the surgical site, leading to complications such as worsening of hearing. The presence of severe mixed hearing loss (MHL) in the high frequencies on the audiogram further supports this suspicion. Acute otitis media, OW fistula, and TM perforation are not likely to cause these specific symptoms.

Explanation

Reparative granuloma is suspected as a complication in this case. A stapedectomy is a surgical procedure performed to improve hearing in patients with otosclerosis. However, in some cases, a reparative granuloma can form at the surgical site, leading to complications such as worsening of hearing. The presence of severe mixed hearing loss (MHL) in the high frequencies on the audiogram further supports this suspicion. Acute otitis media, OW fistula, and TM perforation are not likely to cause these specific symptoms.

11. What patient has the least amount of risk in complications from stapedectomy?

75 y/o female with HTN

25 y/o female scuba diver

6 y/o female with congenital footplate fixation

40 y/o female chef

(scuba diver – risk of fistula, pediatric – higher risk of OW reclosure, chef – risk of chorda tympani injury)

Explanation

(scuba diver – risk of fistula, pediatric – higher risk of OW reclosure, chef – risk of chorda tympani injury)