presented by coleen maritz audiologist

Presented by Presented by

Coleen MaritzColeen Maritz

AudiologistAudiologist

1. Anatomy of the ear1. Anatomy of the ear

2. Types of Hearing Loss2. Types of Hearing Loss

Conductive Hearing LossConductive Hearing Loss-- Resultant of outer or middle ear Resultant of outer or middle ear pathology. When the transmission of pathology. When the transmission of sound is interrupted in the outer ear orsound is interrupted in the outer ear ormore frequently in the middle ear.more frequently in the middle ear.

-- Children: most common cause is otits Children: most common cause is otits media. Adults: most common cause media. Adults: most common cause

is is otosclerosis.otosclerosis.

Types of Hearing Loss Cont.Types of Hearing Loss Cont.

Sensorineural Hearing LossSensorineural Hearing Loss-- Resultant of inner ear and/or auditory Resultant of inner ear and/or auditory

nerve damage.nerve damage.

-- When the hair cells of the cochlea or When the hair cells of the cochlea or the acoustic nerve are damaged.the acoustic nerve are damaged.

Types of Hearing Loss Cont.Types of Hearing Loss Cont.

Mixed Hearing LossMixed Hearing Loss-- Involves a combination of a Involves a combination of a conductive conductive and sensorineural hearing and sensorineural hearing loss.loss.

3. Causes of Hearing Loss3. Causes of Hearing Loss

Causes of Conductive Hearing Causes of Conductive Hearing LossLoss

o Outer and middle ear disorders Outer and middle ear disorders may may cause a conductive hearing loss.cause a conductive hearing loss.

Outer & Middle Ear DisordersOuter & Middle Ear Disorders

DISORDERSDISORDERS DEFINITIONDEFINITION

Microtia & AtresiaMicrotia & Atresia Congenital Congenital malformations of ear & malformations of ear & external auditory canal. external auditory canal. Microtia-abnormal Microtia-abnormal smallness of auricle. smallness of auricle. Atresia-Absence of Atresia-Absence of opening of external opening of external auditory meatus.auditory meatus.

Impacted Cerum & Impacted Cerum & Foreign BodiesForeign Bodies

Excessive ear wax Excessive ear wax obstructing ear canalobstructing ear canal

Outer & Middle Ear Disorders ContOuter & Middle Ear Disorders ContDISORDERSDISORDERS DEFINITIONDEFINITION

Tympanic membrane Tympanic membrane perforationperforation

Hole in the ear drumHole in the ear drum

Otitis mediaOtitis media Middle ear infection. Middle ear infection. Primary caused by Primary caused by Eustachian tube Eustachian tube dysfunction. Increased dysfunction. Increased risk: Craniofacial risk: Craniofacial anomalies, Down’s anomalies, Down’s syndrome, allergies-syndrome, allergies-cigarette smoke cigarette smoke exposure.exposure.

Outer & Middle Ear Disorders ContOuter & Middle Ear Disorders Cont

DISORDERSDISORDERS DEFINITIONDEFINITION

OtosclerosisOtosclerosis Disorder of bone growth Disorder of bone growth that affects the stapes that affects the stapes and the bony labyrinth of and the bony labyrinth of the cochlea. Affects the cochlea. Affects sound transduction sound transduction through middle ear.through middle ear.

Outer & Middle Ear Disorders ContOuter & Middle Ear Disorders ContDISORDERSDISORDERS DEFINITIONDEFINITION

CholesteatomaCholesteatoma Pocket that forms in the Pocket that forms in the ear usually due to Otitis ear usually due to Otitis media. Normal media. Normal shedding of epithelium shedding of epithelium results in growth of results in growth of cholesteatoma. Smells cholesteatoma. Smells bad if it should burst.bad if it should burst.

Other Middle ear Other Middle ear Disorders:Disorders:Physical traumaPhysical trauma

Gun shot, Blow to Gun shot, Blow to

head.head.

Outer & Middle Ear Disorders ContOuter & Middle Ear Disorders Cont

DISORDERSDISORDERS DEFINITIONDEFINITIONBaro-traumaBaro-trauma Sudden change in Sudden change in

atmospheric atmospheric

pressure (Diver, pressure (Diver, aeroplane).aeroplane).

TumoursTumours Neoplasms-Glomus Neoplasms-Glomus tumor, often causes tumor, often causes tinnitus as well.tinnitus as well.

TympanosclerosisTympanosclerosis Whitish plaques on the Whitish plaques on the tympanic membrane.tympanic membrane.

Causes of Hearing Loss Cont.Causes of Hearing Loss Cont.

Causes of Sensorineural Hearing LossCauses of Sensorineural Hearing Loss11. . Prenatal Congenital infectionsPrenatal Congenital infections

* CMV – viral infection; type of herpes * CMV – viral infection; type of herpes virus transmitted in utero; can cause virus transmitted in utero; can cause hearing loss, blindness and seizures hearing loss, blindness and seizures* HIV – affinity for CNS; opportunistic * HIV – affinity for CNS; opportunistic infections cause hearing loss eg. infections cause hearing loss eg. Meningitis Meningitis* Rubella – viral infection * Rubella – viral infection


* Syphilis – often causes progressive * Syphilis – often causes progressive hearing loss; onset after 2 years of agehearing loss; onset after 2 years of age

* Toxoplasmosis – caused by parasite * Toxoplasmosis – caused by parasite infection transmitted through contaminated infection transmitted through contaminated food; causes hydrocephalus, mental food; causes hydrocephalus, mental retardation and hearing lossretardation and hearing loss


2.2. Perinatal CausesPerinatal Causes HypoxiaHypoxia Birth traumaBirth trauma HyperbilirubenemiaHyperbilirubenemia Ototoxic medicationOtotoxic medication Premature infants = increased risk for Premature infants = increased risk for

hearing losshearing loss


3. Acquired hearing loss3. Acquired hearing loss Herpes zoster oticus (Ramsay Hunt Herpes zoster oticus (Ramsay Hunt

Syndrome) – same virus that causes Syndrome) – same virus that causes chickenpox; can cause hearing losschickenpox; can cause hearing loss

Mumps – acute systemic viral disease; Mumps – acute systemic viral disease; common in childhood over the age of 2 years; common in childhood over the age of 2 years; often causes unilateral hearing lossoften causes unilateral hearing loss

MeaslesMeasles MeningitisMeningitis Serous labyrinthitis (inflammation of labyrinth)Serous labyrinthitis (inflammation of labyrinth)


Ototoxic medication:Ototoxic medication:

[[Oto=ear. Ototoxic=Toxic to the earOto=ear. Ototoxic=Toxic to the ear]]Ototoxic medications are drugs that have the potential to cause Ototoxic medications are drugs that have the potential to cause damage to the inner ear structures which may result in temporary damage to the inner ear structures which may result in temporary or permanent hearing loss or an aggravation of an existing or permanent hearing loss or an aggravation of an existing sensorineural hearing loss. Consuming more than one ototoxic sensorineural hearing loss. Consuming more than one ototoxic medication at a time increases your risk to develop a medication at a time increases your risk to develop a sensorineural hearing loss. sensorineural hearing loss.


1. Aminoglycosides (antibiotics):1. Aminoglycosides (antibiotics):• AmikacinAmikacin• GentamycinGentamycin• GaramycinGaramycin• KanamycinKanamycin• NetilmycinNetilmycin• TobramycinTobramycin• StreptomycinStreptomycin• ViomycinViomycin• NeomycinNeomycin


Aminoglycosides (antibiotics) cont:Aminoglycosides (antibiotics) cont:

These medications are toxic when used intravenously in These medications are toxic when used intravenously in serious life threathening situations. The blood levels of these serious life threathening situations. The blood levels of these medications are usually monitored to prevent ototoxicity. medications are usually monitored to prevent ototoxicity. Topical preparations and ear drops containing these antibiotics, Topical preparations and ear drops containing these antibiotics, Neomycin, Gentamycin, have not been demonstrated to be Neomycin, Gentamycin, have not been demonstrated to be

toxic in humans. toxic in humans.


2. Erythromycin (antibiotics):2. Erythromycin (antibiotics):

• EESEES• ErycEryc• E-mycinE-mycin• IlosoneIlosone• PediazolePediazole

• BiaxinBiaxin• ZithromaxZithromax New derivatives of

Erythromycin


Erythromycin (antibiotics) cont:Erythromycin (antibiotics) cont:

These are usually ototoxic when given intravenously in dosages These are usually ototoxic when given intravenously in dosages of 2-4 grams per 24hours, especially if there is underlying kidney of 2-4 grams per 24hours, especially if there is underlying kidney insufficiency. The usual oral dosage of one gram per 24hours is insufficiency. The usual oral dosage of one gram per 24hours is not toxic. There are no significant reports of ototoxicity with new not toxic. There are no significant reports of ototoxicity with new Erythromycin derivatives, since they are given orally at lower Erythromycin derivatives, since they are given orally at lower dosages.dosages.


3. Vancomycin – Vancocin (antibiotics)3. Vancomycin – Vancocin (antibiotics)

This antibiotic is used in a similar manner as the aminoglycosides; This antibiotic is used in a similar manner as the aminoglycosides; when given intravenously in serious life-threatening infections. It is when given intravenously in serious life-threatening infections. It is potentially ototoxic. It is usually used together with the potentially ototoxic. It is usually used together with the aminoglycosides which enhances the possibility of ototoxicity.aminoglycosides which enhances the possibility of ototoxicity.


4. Salicylates (used in treatment of arthritis):4. Salicylates (used in treatment of arthritis):

Toxic effects usually appear after consuming an average of 6-8 Toxic effects usually appear after consuming an average of 6-8 pills per day. Toxic effects are reversible once medication is pills per day. Toxic effects are reversible once medication is discontinued. discontinued.

• Acetylsalicylic acidAcetylsalicylic acid• AspirinAspirin


5. Antimalarial medication-Quinine 5. Antimalarial medication-Quinine

Derivitaves:Derivitaves:

Quinine ingestion can cause a syndrome including tinnitus, Quinine ingestion can cause a syndrome including tinnitus, sensorineural hearing loss and vertigo. Recent studies suggest sensorineural hearing loss and vertigo. Recent studies suggest that quinine impairs outer hair cell mobility. Ototoxic effects are that quinine impairs outer hair cell mobility. Ototoxic effects are similar to asprin- reversible once medication is discontinued).similar to asprin- reversible once medication is discontinued).


Antimalarial medication-Quinine Derivitaves Antimalarial medication-Quinine Derivitaves (cont.):(cont.):

• Quinidex (causes tinnitus)Quinidex (causes tinnitus)• AtrabrineAtrabrine• PlaquenilPlaquenil• Quinine SulfateQuinine Sulfate• MefloquineMefloquine• ChloroquineChloroquine


6. Loop diuretics:6. Loop diuretics:

These medications are usually ototoxic when given These medications are usually ototoxic when given intravenously for acute kidney failure or acute hypertension. intravenously for acute kidney failure or acute hypertension. Rare cases have been reported when these medications are Rare cases have been reported when these medications are taken orally in high doses in people with chronic kidney taken orally in high doses in people with chronic kidney disease.disease.

• Edecrin (Ethacrynic acid)Edecrin (Ethacrynic acid)• Lasix (Furosemide)Lasix (Furosemide)• Bumex (Bumetanide)Bumex (Bumetanide)


7. Chemotherapeutic Agents:7. Chemotherapeutic Agents:

• CiaplatinCiaplatin• Nitrogen MustardNitrogen Mustard• VincristineVincristine

These medications are ototoxic when given for treatment of These medications are ototoxic when given for treatment of cancer. The ototoxic effects of these medications are enhanced cancer. The ototoxic effects of these medications are enhanced in patients who are already taking other ototoxic medications. in patients who are already taking other ototoxic medications.


8. 8. Nonsteroidal Anti-Inflammatory DrugsNonsteroidal Anti-Inflammatory Drugs Toxic effects usually appear after consuming an average of Toxic effects usually appear after consuming an average of

6-8 6-8 pills per day. Toxic effects are usually reversible once pills per day. Toxic effects are usually reversible once medications are discontinued.medications are discontinued.

• AdvilAdvil MotrinMotrin• AleveAleve NalfonNalfon• AnaproxAnaprox NaprosynNaprosyn• ClinorilClinoril NuprinNuprin• FeldeneFeldene PoradolPoradol• LodineLodine VoltarinVoltarin


Acoustic trauma – temporary or Acoustic trauma – temporary or permanentpermanent

MMénière’s disease – causes vertigo, énière’s disease – causes vertigo, hearing loss, tinnitus, pressure in ear; can hearing loss, tinnitus, pressure in ear; can be unilateral, fluctuating or progressivebe unilateral, fluctuating or progressive

Presbyacusis – age related sensorineural Presbyacusis – age related sensorineural hearing losshearing loss

Noise exposure- most common cause of Noise exposure- most common cause of HL.HL.


Noise Exposure- Noise Exposure- Damage risk criteria expressed Damage risk criteria expressed

as the maximum permissible as the maximum permissible noise exposure for a given noise exposure for a given duration during a work day.duration during a work day.

Duration per Duration per day (in hours)day (in hours)

Sound level Sound level

(in dB)(in dB)

8.08.0

6.06.0

4.04.0

3.03.0

2.02.0

1.51.5

1.01.0

0.50.5

0.250.25

9090

9292

9595

9797

100100

102102

105105

110110

115 (club)115 (club)


Syndromes associated with Syndromes associated with Auditory DysfunctionAuditory Dysfunction

See handout See handout

4. 4. Prevalence of hearing lossPrevalence of hearing loss 3/1000 infants are born with congenital severe 3/1000 infants are born with congenital severe

bilateral hearing lossbilateral hearing loss Additional 3 children acquire hearing loss early Additional 3 children acquire hearing loss early

in childhoodin childhood Hearing loss occurs twice as frequently as other Hearing loss occurs twice as frequently as other

congenital abnormalities screened for in congenital abnormalities screened for in newborns combined: congenital hypothyroidism; newborns combined: congenital hypothyroidism; sickle cell anemia; phenylketonuria; sickle cell anemia; phenylketonuria; galactosemiagalactosemia

Prevalence = 10-20 times higher in NICU Prevalence = 10-20 times higher in NICU

Prevalence of hearing loss cont.Prevalence of hearing loss cont.

Prevalence in general populationPrevalence in general population:: 2/1000 = severe-profound HL2/1000 = severe-profound HL 5/1000 = moderate-severe HL5/1000 = moderate-severe HL 8/1000 = mild HL8/1000 = mild HL 150/1000 = middle ear infections150/1000 = middle ear infections

Prevalence expected to be even higher Prevalence expected to be even higher with increase in HIV/AIDS infectionwith increase in HIV/AIDS infection

5. Tinnitus5. Tinnitus

1. 1. Definition of tinnitusDefinition of tinnitus:: “…“…hearing a sound that is not related to any hearing a sound that is not related to any

external sound in the environment”external sound in the environment” Ringing sound in patient’s ears is generated Ringing sound in patient’s ears is generated

by patient’s own body, and is not present in by patient’s own body, and is not present in external environmentexternal environment

Most common types of sounds reported by Most common types of sounds reported by patients: ringing, hissing, buzzing, cricket patients: ringing, hissing, buzzing, cricket soundssounds

Tinnitus cont.Tinnitus cont.

2. 2. Neurophysiological model of Neurophysiological model of

TinnitusTinnitus Stages of tinnitus emergence:Stages of tinnitus emergence:

Generation- typically in the peripheryGeneration- typically in the periphery Detection – subcortical centersDetection – subcortical centers Perception and evaluation – cotical areasPerception and evaluation – cotical areas Sustained activation of emotional (Limbic) Sustained activation of emotional (Limbic)

and autonomic nervous systems and autonomic nervous systems


3. 3. Prevalence of tinnitusPrevalence of tinnitus• 17% of general population (44million)17% of general population (44million)• Clinically significant problems for Clinically significant problems for

approximately 4-5% of the populationapproximately 4-5% of the population

4. 4. Impact on patients’ livesImpact on patients’ lives• Ranges from mild irritation to total disability, Ranges from mild irritation to total disability,

and in some cases suicideand in some cases suicide


5. 5. CausesCauses• May be associated with sensorineural May be associated with sensorineural

hearing loss (damage to OHC and IHC)hearing loss (damage to OHC and IHC)• Ionic imbalance in the cochleaIonic imbalance in the cochlea• Dysfunction of cochlear neurotransmitter Dysfunction of cochlear neurotransmitter

systemssystems• CentralCentral• Certain medication may cause tinnitus: eg. Certain medication may cause tinnitus: eg.

antidepressants, benzodiazeptinesantidepressants, benzodiazeptines• Unknown causes in a lot of casesUnknown causes in a lot of cases


6. 6. TreatmentTreatment Unable to suppress tinnitus source – therefore Unable to suppress tinnitus source – therefore

do not currently have a curedo not currently have a cure Tinnitus Retraining TherapyTinnitus Retraining Therapy – helps patients – helps patients

to adapt to noise, and to change their brain’s to adapt to noise, and to change their brain’s perception of the tinnitus; makes use of noise perception of the tinnitus; makes use of noise generators to mask tinnitusgenerators to mask tinnitus

Success rate of 80%Success rate of 80%

6. Hearing Tests6. Hearing Tests 1. 1. AudiometryAudiometry

Puretone Puretone SpeechSpeech

2. 2. Immittance TestingImmittance Testing TympanometryTympanometry Acoustic ReflexesAcoustic Reflexes

3. 3. Electrophysiological TestingElectrophysiological Testing Otoacoustic Emissions (OAE)Otoacoustic Emissions (OAE) Auditory Brainstem Response (ABR)Auditory Brainstem Response (ABR)

Hearing Tests cont.Hearing Tests cont.

1. Audiometry1. Audiometry 1.1 What is audiometry & how does it work?1.1 What is audiometry & how does it work?

2 measures: 2 measures: intensity & frequencyintensity & frequency Intensity:Intensity:

• Loudness, measured in dBLoudness, measured in dB• Human ear: 0-120 dBHuman ear: 0-120 dB

Frequency:Frequency:• Pitch/ tone of sound, measured in HzPitch/ tone of sound, measured in Hz• Human ear: 20-20 000Hz; 125-8000 HzHuman ear: 20-20 000Hz; 125-8000 Hz


Air conduction audiometry:Air conduction audiometry: Earphones, outer ear, middle ear, inner ear, Earphones, outer ear, middle ear, inner ear,

n. VIIIn. VIII Bone conduction audiometry:Bone conduction audiometry:

Bone conductor on mastoidBone conductor on mastoid


1.2 Aims of Audiometry:1.2 Aims of Audiometry: Measure Measure degree degree of HL: minimal, mild, of HL: minimal, mild,

moderate, severe, profoundmoderate, severe, profound Measure Measure site of lesionsite of lesion in hearing mechanism in hearing mechanism Determine Determine causecause of HL of HL Determine Determine degree of handicapdegree of handicap (how HL (how HL

affects patient’s activities of daily life)affects patient’s activities of daily life)


1.2 Aims of Audiometry cont:1.2 Aims of Audiometry cont:

Obtain Obtain indications for appropriate indications for appropriate therapeutic and educational assistancetherapeutic and educational assistance – – hearing aids; assistive listening devices; hearing aids; assistive listening devices; speech-language therapy; school placementspeech-language therapy; school placement

Degree of Hearing LossDegree of Hearing Loss

Environmental sounds measured Environmental sounds measured on audiogramon audiogram

What is an Audiogram?What is an Audiogram?

An AUDIOGRAM is a graphic An AUDIOGRAM is a graphic representation of a hearing test.representation of a hearing test.

Normal HearingNormal Hearing

7. Types of Hearing Loss-7. Types of Hearing Loss-AudiogramAudiogram

1. Conductive hearing loss1. Conductive hearing loss Resultant of external or middle ear pathologyResultant of external or middle ear pathology Air conduction abnormalAir conduction abnormal Bone conduction normalBone conduction normal

Conductive Hearing LossConductive Hearing Loss

Types of Hearing Loss-Types of Hearing Loss-Audiogram cont.Audiogram cont.

2. Sensorineural hearing loss2. Sensorineural hearing loss Resultant of inner ear and/or auditory nerve Resultant of inner ear and/or auditory nerve

damagedamage Air conduction and bone conduction equally Air conduction and bone conduction equally

abnormalabnormal

Sensori-neural Hearing LossSensori-neural Hearing Loss

Types of Hearing Loss-Types of Hearing Loss-Audiogram cont.Audiogram cont.

3. Mixed hearing loss3. Mixed hearing loss Combination of conductive and sensorineural Combination of conductive and sensorineural

hearing losshearing loss Air conduction and bone conduction abnormalAir conduction and bone conduction abnormal Air conduction worse than bone conduction Air conduction worse than bone conduction

(air-bone gap)(air-bone gap)

Mixed Hearing LossMixed Hearing Loss


2. 2. Immittance TestingImmittance Testing

2.1 Tympanometry2.1 Tympanometry Test of middle ear functioningTest of middle ear functioning Measures pressure, compliance and volume Measures pressure, compliance and volume

of middle earof middle ear Able to determine if middle ear pathology is Able to determine if middle ear pathology is

present eg. Otitis Mediapresent eg. Otitis Media


2.2 2.2 Acoustic Reflex TestingAcoustic Reflex Testing Measures stapedial muscle reflex (middle ear) Measures stapedial muscle reflex (middle ear)

in response to loud stimulusin response to loud stimulus Able to estimate hearing thresholds in difficult Able to estimate hearing thresholds in difficult

to test populations, although not always to test populations, although not always accuratelyaccurately


3. Electrophysiological Testing3. Electrophysiological Testing 3.1 Otoacoustic Emissions (OAE)3.1 Otoacoustic Emissions (OAE)

measure the integrity of the outer hair cells of measure the integrity of the outer hair cells of the cochleathe cochlea

Auditory stimulus is presented to external ear Auditory stimulus is presented to external ear via a probevia a probe

Cochlea produces “echo” in response to Cochlea produces “echo” in response to stimulus – measured by probestimulus – measured by probe


Advantages of OAEsAdvantages of OAEs Non-invasiveNon-invasive Simple - quick and easy to administer (also Simple - quick and easy to administer (also

used in infant hearing screening)used in infant hearing screening) Frequency specificFrequency specific ObjectiveObjective Does not require behavioural cooperationDoes not require behavioural cooperation Useful in infants, young children, Useful in infants, young children,

neurologically compromised neurologically compromised


3.2 Auditory Brainstem Response (ABR)3.2 Auditory Brainstem Response (ABR) Electrophysiological procedureElectrophysiological procedure Based on the brainstem’s response to soundBased on the brainstem’s response to sound Soft click stimuli are presented to the ear, Soft click stimuli are presented to the ear,

whilst electrodes placed strategically on the whilst electrodes placed strategically on the head record the brain’s response to the head record the brain’s response to the stimulistimuli


Advantages of ABR:Advantages of ABR: Frequency specificFrequency specific Predicts hearing thresholdsPredicts hearing thresholds No behavioural response requiredNo behavioural response required Objective Objective Infants, young childrenInfants, young children

8.8. Intervention for Hearing LossIntervention for Hearing Loss

MedicalMedical

- surgical and/or medicinal treatment- surgical and/or medicinal treatment Hearing aids (HA)Hearing aids (HA) Implantable DevicesImplantable Devices

- Cochlear Implant (CI)- Cochlear Implant (CI)

- BAHA (Bone anchored hearing aid)- BAHA (Bone anchored hearing aid)

- Vibrant Sound Bridge Device- Vibrant Sound Bridge Device

Cochlear ImplantCochlear Implant- Electronic device for adults and children- Electronic device for adults and children with a severe to profound hearing loss.with a severe to profound hearing loss.- Unlike HA’s, which merely amplify sound, - Unlike HA’s, which merely amplify sound,

a CI bypasses the damaged hair cells a CI bypasses the damaged hair cells and stimulates the hearing nerve directly.and stimulates the hearing nerve directly.

- Implanted directly in the inner ear or cochlea- Implanted directly in the inner ear or cochlea and electronically stimulates the nerves via and electronically stimulates the nerves via electrodes.electrodes.

BAHABAHA

- Direct bone conduction- Direct bone conduction

- Bypasses conductive element- Bypasses conductive element

- Creates direct cochlea stimulation- Creates direct cochlea stimulation

- Conductive or mixed HL’s- Conductive or mixed HL’s

- To close the air-bone gap- To close the air-bone gap

Vibrant Sound Bridge DeviceVibrant Sound Bridge Device

- Implanted in the middle ear- Implanted in the middle ear

- Device mechanically vibrates the bones - Device mechanically vibrates the bones

in the middle ear.in the middle ear.

- Moderate to severe SNHL - Moderate to severe SNHL

presented by coleen maritz audiologist

Documents

mixed hearing loss

permanent hearing loss

types of hearing loss

progressive hearing

middle ear pathology

outer ear ormore

inner ear structures

common cause