32 Hearing Loss & Ototoxicity Flashcards

1
Q

What is sensorineural hearing loss (SNHL)?

A

What is sensorineural hearing loss (SNHL)?

Sensorineural hearing loss is caused by inadequate sound processing by the end organ of hearing, the cochlea (sensory), or poor transmission by the eighth cranial nerve (neural   ) to the central nervous system. The function of the cochlea is to convert sound energy into electrical impulses that are then transmitted to the auditory centers in the brain. In sensorineural hearing loss, this pathway is disrupted.

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2
Q

What is conductive hearing loss (CHL)?

A

What is conductive hearing loss (CHL)?

Conductive hearing loss is caused by impaired sound transmission to the inner ear. Abnormalities in the external canal, tympanic membrane, ossicles, and middle ear account for this type of hearing loss. The maximal amount of CHL is 60 decibels,

CHL over 50 decibels is most likely caused by ossicular chain pathology.

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3
Q

What should your history include when evaluating hearing loss?

A

What should your history include when evaluating hearing loss?

When evaluating any type of hearing loss, it is important to ask about laterality, duration, progression*, *current severity*, associated factors (such as otalgia, otorrhea, tinnitus, vertigo, and aural fullness), ototoxic medication use, head trauma, family history (to assess for genetic factors), *autoimmune disease, and prior otologic surgery.

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4
Q

What should your physical exam include when evaluating a patient with hearing loss?

A

What should your physical exam include when evaluating a patient with hearing loss?

A complete head and neck exam should be performed, with a focus on the otologic and neurologic exams. The otologic exam should include examination of the pinna, external auditory canal, tympanic membrane, and middle ear. Tuning fork tests (Weber and Rinne) and pneumatic otoscopy should also be routinely performed.

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5
Q

How can you differentiate between SNHL and CHL on an audiogram?

A

How can you differentiate between SNHL and CHL on an audiogram?

The two main ways to differentiate between SNHL and CHL on an audiogram include the presence of an:

  1. air–bone gap
  2. abnormal tympanogram.

An air–bone gap is present during conductive or mixed (both SNHL and CHL) hearing losses, and is caused by differences in air-conducted and bone-conducted stimuli. During the vibratory portion of the audiogram, a patient with CHL will be better able to hear the stimulus because the transmission of sound through the mastoid bone is bypassing the site of blockage in the external or middle ear.

The tympanogram measures the compliance of the tympanic membrane. Increased compliance (type Ad) can indicate ossicular chain discontinuity, whereas decreased compliance (type As) can indicate otosclerosis, both of which can cause a conductive hearing loss. Poor compliance (type B) on a tympanogram can indicate a tympanic membrane perforation or middle ear effusion.

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6
Q

What are the common causes of SNHL?

A

What are the common causes of SNHL?

The most common causes are:

  • Presbycusis
  • Noise exposure (i.e., machinery, artillery, loud music)
  • Heredity

Less common causes are acoustic trauma, ototoxicity, sudden idiopathic hearing loss, autoimmune hearing loss, Meniere’s disease, tumors, and infections (i.e., meningitis, viral labyrinthitis).

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7
Q

What is presbycusis?

A

What is presbycusis?

Presbycusis is age-related hearing loss. This is the most common type of hearing loss in adults, encompassing the great majority of adult onset hearing loss. Presbycusis is bilateral, symmetric, slowly progressive high-frequency loss. Onset is in adults over 60 years, and the exact cause is unknown. Hearing aids are the most effective treatment.

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8
Q

What guidelines are in place for newborn hearing screening?

A

What guidelines are in place for newborn hearing screening?

The U.S. federal government mandates newborn hearing screening, but programs are state regulated. The most commonly used test is otoacoustic emissions (OAE), which tests the outer hair cell response to acoustic stimulation. The other common test is auditory brainstem response (ABR), in which the eighth nerve and central nervous system produce sounds in response to an acoustic stimulation. In either case, if the test is abnormal, the newborn is referred for further testing.

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9
Q

What is the incidence of congenital hearing loss?

A

What is the incidence of congenital hearing loss?

One to three infants per 1000.

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10
Q

What risk factors predispose children to hearing loss?

A

What risk factors predispose children to hearing loss?

Risk factors that have been correlated with congenital hearing loss are (per Joint Committee on Infant Hearing):

  • Family history of childhood onset permanent hearing loss
  • In-utero infections (ToRCH = toxoplasmosis, other [syphilis, parvovirus, varicella], rubella, cytomegalovirus, herpes)
  • Illness requiring NICU admission for more than 48 hours
  • Craniofacial anomalies, especially involving the pinna or external auditory canal
  • Characteristic signs of syndromes known to cause hearing loss

Other factors associated with increased rates of congenital hearing loss in the literature:

  • Low birth weight (less than 1500 grams)
  • Hypoxia
  • Hyperbilirubinemia
  • Low APGAR scores
  • Head trauma
  • Ototoxic medications
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11
Q

What are the developmental milestones for pediatric speech and hearing?

A

What are the developmental milestones for pediatric speech and hearing?

  • 0 to 3 months: startled by loud sounds, calmed by familiar voice
  • 6 months: localize sounds
  • 9 months: respond to name and can mimic sounds
  • 12 months: say first words
  • 18 months: can follow simple commands
  • 2 years: say 20 or more words and put together 2-word sentences
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12
Q

When should SNHL be evaluated with imaging?

A

When should SNHL be evaluated with imaging?

  • In children, MRIs or CT scans of the temporal bones are usually performed for all SNHL, although the majority of these scans will be normal. The most common finding in pediatric SNHL is enlarged vestibular aqueduct.
  • In adults, MRI of the IACs with contrast is performed for asymmetric* SNHL or *sudden SNHL to evaluate for acoustic neuroma (Figure 32-2) and other cerebellopontine angle tumors.
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13
Q

What medications can cause ototoxicity?

A

What medications can cause ototoxicity?

  • Antibiotics (specifically aminoglycosides and vancomycin)
  • Chemotherapy drugs (cisplatin)
  • Loop diuretics (furosemide, ethacrynic acid)
  • NSAIDs and salicylates (aspirin)
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14
Q

How do ototoxic medications cause hearing loss?

A

How do ototoxic medications cause hearing loss?

Aminoglycosides damage both vestibular and cochlear hair cells.

  • Streptomycin & Gentamycin: vestibular > hearing
  • Neomycin & Tobramycin: hearing > vestibular
  • Vancomycin potentiates the ototoxic effect of aminoglycosides, but does not appear to be ototoxic alone.
  • Cisplatin damages outer hair cells.
  • Loop diuretics damage the stria vascularis (which maintains the endocochlear potential).
  • NSAIDs & Salicylates cause damage to the cochlea, only occurring with high doses, which is almost always completely reversible with discontinuation of the drug.

Since most ototoxic medications are excreted by the renal system, renal impairment can lead to higher rates of ototoxicity.

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15
Q

How does ototoxicity present?

A

How does ototoxicity present?

The first symptom is usually (1) tinnitus, followed by (2) perception of hearing loss. Hearing loss is usually symmetric and affects the high frequencies first. With continued exposure, the lower frequencies are affected.

The American Academy of Audiology recommends ototoxicity audiology protocols that include pure tone and DPOAE (a type of otoacoustic emission) testing at ultra-high frequencies (8 kHz to 20 kHz) because the earliest changes occur at frequencies greater than 8 kHz.

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16
Q

Which cause of SNHL is preventable?

A

Which cause of SNHL is preventable?

Noise exposure associated hearing loss is the only preventable cause of hearing loss. Common situations causing noise exposure hearing loss are exposure to industrial machinery, military or other exposure to gunfire, repeated loud music exposure, and acoustic trauma. Acoustic trauma is a single event of very loud noise that causes a permanent change in hearing (such as an explosion). Prevention includes both avoidance and use of hearing protection.

17
Q

What treatments are available for SNHL?

A

What treatments are available for SNHL?

Bilateral mild to severe SNHL is usually treated with hearing aids. Profound bilateral SNHL can be treated with a cochlear implant, which converts sound energy into electrical energy, directly stimulating the hearing nerve. Unilateral SNHL can be treated with a regular hearing aid, a contralateral routing of signals (CROS) hearing aid or a bone conduction implant. CROS hearing aids use the hearing aid on the poorer hearing ear as a microphone, transmitting the sound to the better hearing ear. A bone conduction implant is a hearing aid that turns sound energy into vibrational energy. An abutment is surgically implanted in the skull, to which the patient attaches a hearing aid. The vibration transmits through the skull to the better hearing ear.

In cases of acoustic neuroma, surgery or radiation can sometimes improve hearing. For sudden idiopathic and immune-mediated SNHL, steroid therapy can be effective in improving the amount of hearing loss.

18
Q

What are the causes of sudden SNHL?

A

What are the causes of sudden SNHL?

Most cases of sudden SNHL are considered idiopathic because a cause is never identified. Sudden SNHL can rarely be caused by an acoustic neuroma, which is why an MRI of the internal auditory canals is recommended during workup. Other rare causes are syphilis, Lyme disease, vascular disease, or autoimmune disease.

19
Q

How should sudden SNHL be treated?

A

How should sudden SNHL be treated?

Mayo Protocol:

  • Prednisone taper, 60 mg x10d, 40 mg x2d, 20 mg x2d
  • Intratympanic steriod injection

The mainstay of therapy for sudden SNHL is steroids. This is usually given as an oral burst and taper of prednisone for 10 to 14 days, with a maximal dose of 60 milligrams daily. For patients who cannot tolerate oral steroids (for example, brittle diabetics or those in whom steroids exacerbate psychiatric disease), an alternative is transtympanic steroid injection. This procedure involves filling the middle ear space with a concentrated steroid solution. Antivirals are not recommended.

20
Q

Describe immune-mediated SNHL and its initial treatment.

A

Describe immune-mediated SNHL and its initial treatment.

Immune-mediated SNHL is usually bilateral and rapidly progressive. Many patients have roaring tinnitus. The most common treatment is high-dose steroid therapy, usually with a maximum dose of 60 milligrams for 4 weeks, followed by a taper. If the hearing loss returns after the steroids are weaned, other long-term immunosuppressant medications (such as methotrexate) may be used.

21
Q

What types of trauma can cause hearing loss?

A

What types of trauma can cause hearing loss?

Acoustic trauma occurs due to exposure to a very loud noise. This can cause either a temporary threshold shift, which will resolve over time, or a permanent threshold shift, which does not resolve with time.

Barometric trauma most commonly manifests as pain due to increased pressure in the middle ear which can lead, in severe cases, to tympanic membrane rupture. More rarely, pressure changes in the inner ear can lead to SNHL. Finally,

Head trauma can lead to either CHL or SNHL. Temporal bone fractures through the cochlea lead to SNHL, whereas fractures of the ossicles or tympanic membrane injury lead to CHL. Concussive injuries can also cause hearing loss, but this is less common.

22
Q

What are the common causes of CHL?

A

What are the common causes of CHL?

The most common causes are:

  1. Cerumen impaction
  2. Foreign body
  3. Tympanic membrane perforation
  4. Middle ear effusion
  5. Ossicular chain abnormalities (such as otosclerosis)
23
Q

What is otosclerosis?

A

What is otosclerosis?

Otosclerosis is fixation of the stapes footplate in the oval window caused by abnormal remodeling of bone in this area. Otosclerosis causes either unilateral or bilateral conductive hearing loss that can have a Carhart notch pattern (artificial 10 to 15 decibel decrease in bone conduction at 2000 Hz). Many cases of otosclerosis are hereditary. Treatment includes either stapedectomy* or a *hearing aid.

24
Q

How much conductive hearing loss does ossicular chain disruption produce?

A

How much conductive hearing loss does ossicular chain disruption produce?

While the amount of loss for other causes of CHL is variable, ossicular chain disruption usually causes a greater than 50 decibel hearing loss. “Maximal” CHL is 60 decibels.

25
Q

What is cholesteatoma?

A

What is cholesteatoma?

Cholesteatoma is accumulation of keratin debris in the middle ear or external auditory canal. If left untreated, the debris continues to accumulate and can cause erosion into surrounding tissues, leading to tympanic membrane perforation, damage to the ossicular chain, and even perilymphatic fistulas or tegmen defects. Cholesteatomas can become infected and can lead to recurrent otorrhea.

26
Q

When should nasopharyngoscopy be performed in the evaluation of CHL?

A

When should nasopharyngoscopy be performed in the evaluation of CHL?

Unilateral serous effusion lasting more than 3 months or without a preceding history of acute otitis media should be evaluated with a nasopharyngoscopy to assess for obstruction of the eustachian tube opening. Also, any serous effusion associated with

  • recurrent epistaxis
  • headache
  • vision changes
  • painless neck mass
27
Q

When should CHL be evaluated with imaging?

A

When should CHL be evaluated with imaging?

  1. After head trauma should be evaluated with temporal bone CT scan.
  2. Suspicion for cholesteatoma or middle ear mass (such as glomus tumor) should also be evaluated with a temporal bone CT.
  3. Prior to surgical correction of CHL in children, imaging is often performed to check for abnormalities of the inner ear such as enlarged vestibular aqueduct.
28
Q

What surgical therapies are used to address CHL?

A

What surgical therapies are used to address CHL?

  • Perforated tympanic membrane can be repaired with tympanoplasty.
  • Middle ear effusion can be treated by myringotomy with or without an ear tube.
  • Stapedectomy can be done for otosclerosis, in which the stapes is replaced with a prosthesis.
  • Cholesteatoma is treated with mastoidectomy to remove all of the squamous and keratinous debris.
  • If the ossicular chain is disrupted, either a partial or total reconstruction prosthesis can be used to reconstruct the chain.
29
Q

What are the indications for placement of pressure equalization tubes in children?

A

What are the indications for placement of pressure equalization tubes in children?

Per the 2013 clinical practice guidelines on tympanostomy tubes in children, tubes should be placed in children who have both:

  1. persistent middle ear effusion for more than 3 months and
  2. documented evidence of hearing difficulty, vestibular problems, ear discomfort, or are at high risk for speech delay.

Tubes should also be placed in children with recurrent acute otitis media if persistent effusion is present when the child is evaluated in the office.

30
Q

What is semicircular canal dehiscence and how does it cause hearing loss?

A

What is semicircular canal dehiscence and how does it cause hearing loss?

This condition, also called labyrinthine fistula, can cause CHL by creating a third window into the inner ear (the first two are the oval and round windows). This is most commonly caused by a dehiscence of bone along the superior semicircular canal next to the dura. CHL occurs because sound energy is lost through the fistula. Tullio sign (dizziness or nystagmus after a loud sound) and Hennebert sign (dizziness or nystagmus after Valsalva maneuver or pneumatic otoscopy) are usually positive if a fistula is present.