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All auditory disorders can be classified into two main classifications:
EXOGENOUS and ENDOGENOUS
EXOGENOUS -
outside the system
ENDOGENOUS-
within the system
Exogenous-
caused by inflammatory disease, toxicity noise, accident, or injury that inflicts damage to any part of the auditory system;
Endogenous-
HEREDITARY- auditory deficits are inherited by the child from the parents

conditions originate in the genetic characteristics of an individual

-not all congenital (present at birth) hearing disorders are hereditary, nor are all hereditary disorders congenital
Genetic Transmission of Hearing Loss

(Prevalence, Estimated disorders, and alternative name)
genetic hearing loss makes up about 50% of auditory disorders

- It is estimated that there are more than 400 genetic syndromes in which hearing loss is a regular or occasional feature


ENDOGENEOUS- within the system or genetic
Mendelian Laws (genetic)-
Hearing Loss is usually governed by Mendelian Laws of inheritance (whether occurring as one manifestation of a particular syndrome or a group of signs that characterize a disorder or condition)

Mandelian Laws are genetic traits may be dominant, recessive, or sex-linked (x-linked)
Important Information to Remember about GENES and CHROMOSOMES
Genes are located on chromosomes and they come in pairs (except the male sex chromosome)

One member of each gene pair is inherited from each parent

Humans have 22 autosomes (non sex-determining chromosomes) and 1 pair of sex chromosomes (females have 2 X’s and males have XY)
Autosomal Dominant Inheritance
In Autosomal Dominant inheritance, the trait is carried from 1 generation to another

(Autosome- NOT sex chromosome)

Typically, one parent exhibits the trait; statistically 50% chance they pass on the trait to ANY child

The abnormal gene is one of the ordinary paired chromosomes and NOT a sex chromosome.

Only necessary for one parent to have the abnormal gene

Believed to account for 20% of genetically caused deafness

The risk of having a hearing impaired child is 50%.

The parent who carries the abnormal gene frequently exhibits the trait
Autosomal Dominant Inheritance: Statistics
- One parent exhibits the trait and statistically 50% chance they pass on the trait to ANY child

- Only necessary for one parent to have the abnormal gene

- Believed to account for 20% of GENETICALLY caused deafness

- The risk of having a hearing impaired child is 50%

- The parent with the abnormal gene frequently exhibits the trait because it is a dominant trait and you only need it from one parent in order to show symptoms of the trait
Autosomal Recessive Inheritance
Occurs when EACH parent carries one abnormal gene for a condition (both parents must carry gene)

Parents are heterozygous (only one abnormal gene) and are carriers for the trait and show no signs of the condition

- Recessive genes account for:
MOST cases of genetic HEARING LOSS and accounts for about 80% of genetic DEAFNESS

- 25% probability of having a child who is homozygous (2 abnormal or 2 normal genes) and expresses the trait

- 50% probability of having a child who is heterozygous and is a carrier

- Both parents are clinically normal/ often are often heterozygous carriers of a single abnormal recessive gene (one normal/ one abnormal)

- Appearance of the trait in the offspring requires that and individual possess two abnormal genes, one from each parent
Autosomal Recessive Inheritance: Statistics
Recessive genes account for most cases of genetic HEARING LOSS and accounts for about 80% of genetic DEAFNESS

- 25% probability of having a child who is homozygous (2 abnormal or 2 normal genes) and expresses the trait.

- 25% chance that the child will have the trait and a 25% chance that the child will NOT have the trait and will NOT be a carrier

- 50% probability of having a child who is HETEROzygous, carrying one normal and one abnormal gene

- 50% chance the child will not have the trait but still be a CARRIER
X-linked Inheritance (SEX LINKED)
– X-Linked inheritance is a much less frequent occurrence, 2-3% of deafness

– Gene responsible for determining this condition is on the X chromosome (sex chromosome)

– Males have 1 X and 1 Y chromosome

- Females have 2 X chromosomes, they are CARRIERS of the trait because they have XX genes

– Affected fathers produce daughters who are carriers

– If mother is a carrier there is a 50% probability that each son will be affected and a 50% probability that each daughter will be a carrier.
Advances in Hereditary Deafness-
Through gene mapping (identifying chromosomal location of a gene) and localization (isolating the gene responsible for the disorder) it is now possible to identify genes responsible for deafness

Chromosomal locations of approximately 70 genes for nonsyndromic deafness have been mapped
When does conductive HL occur?
What is conductive HL?
- Conductive HL occur when there is an complication somewhere between the outer and middle ear

- Person with a conductive HL exhibits normal threshold sensitivity by bone conduction measurements and decreased threshold values via air conduction (air bone gap)

- NORMAL BC and DECREASED AC
- OUTER and MIDDLE ear problems, but the inner ear hearing is still NORMAL
What does conductive Hearing Loss do to the middle and inner ear?
- A variety of disorders can lead to conductive HL

- All of these disorders RESULT in alternation of the mechanics of the external ear of the middle ear system

- Basically, the inner is is not affected while the mechanics in the outer and middle ear are harmed
Examples of Mechanical changes:
Blockage of external or middle ear

- Increase in stiffness in the Tympanic Membrane or middle ear system

- Increase in the mass of the middle ear

- These mechanical system alterations of the outer and middle ear produce varying degrees of conductive hearing loss

+ INCREASE in the STIFFNESS of a mechanical system= GREATEST EFFECT on LOW FREQUENCIES

+ A change in MASS effects HIGH FREQUENCIES
INCREASE in the STIFFNESS of a mechanical system has that kind of effect on frequency?
GREATEST EFFECT on LOW FREQUENCIES

•Stiffness is proportional to frequency
Stiffness increases, frequency increases


If something because more stiff, it will be harder for the low frequencies to move them or cause vibration
ligaments
tendons
the tympanic membrane
the air enclosed in the ear canal and middle ear space
A change in MASS affects what?
HIGH FREQUENCIES

•Mass is inversely proportional to frequency
– Mass decreases, frequency increases
pars flacida of the tympanic membrane
ossicles
perilymph in the cochlea
mesenchyme clinging to the ossicles, in infants
External Otitis-
inflammation of inner and outer ear canal; originated by viruses or bacteria and can be caused by many diseases
Herpes simplex (NOT STD type)-
-Coldsore herpes
-Associated with the external ear disorders aquired after birth

-invades inner ear canal or pinna
Viral bullous myringitis-
involves outer layer of Tympanic Membrane and spreads laterally to the external ear canal
Anomalies of the External Ear-
- Most external ear abnormalities are the result of an inherited trait

- Abnormalities can occur in isolation or as part of other alterations to the skull and face (called craniofacial defects)
Pinna deformity:
range from very mild (difficult for the lay person to identify) to more severe (much as a total absence of the pinna or a complete closure of the ear canal)

- Severity of the pinna deformity sometimes offers and indication of the corresponding status of the middle ear space

- Generally, more severe the deformation the the outer ear, the GREATER the conductive HL (pg 167)
The more the outside of the ear is deformed, the less sound will travel through and reach the middle and inner ear
Microtia –
very small or deformed pinna; most severe type
Atresia or Aural Atresia –
Ear canal is partially or totally closed off or absent

absence (could be a total or partial absence) or closure of the external auditory meatus (ear canal)

often associated with MICROTIA
Stenosis –
Opening present (of the ear canal) but very narrow

Condition associated with External ear disorders
Microtia and Atresia
(Statistics and prevalence)
are bilateral in 15-20% of cases, are known to affect the right ear more than the left, and they are slightly more prevalent in males than females
Anomalies of External Ear and Face
Facial Anomalies
Skin tags
Pre-auricular pits
Pre-auricular pit with fistula
Pre-auricular Skin tags
Complex Craniofacial Anomalies (Syndromes)
External ear anomalies sometimes get quite complex and include several stigmata (physical or mental marks that aid in diagnosis).
There are hundreds of syndromes like this – most of these include conductive hearing loss (HL), but some have Sensorineural HL.
Acquired External Ear Disorders
Trauma
Otitis externa – viral or bacterial
Fungus Infections
Herpes Infection
Collapsed canal – may result in significant conductive hearing loss
Foreign bodies
Wax impaction
Bony growths (exotoses)
Trauma-
sharp objects can pierce or tear the TM; such trauma can also disrupt the ossicular chain (separation and displacement of the ossicles) which results in a mild to moderate conductive HL
Otitis externa (External Otitis) –
inflammation of the inner or outer ear canal; viral or bacterial can be caused my many diseases (swimmers ear- bacteria)
  Viral bullous meningitis-
Involves outer layer of Tympanic Membrane and spreads laterally to the external ear canal
Fungus Infections-
swimmer's ear
(In rare instances these diseases can produce severe swelling of the canal and subsequent Hearing Loss)
  Herpes Infection-
non std invades pinna or ear canal
Collapsed canal –
- External ear disorder

- Collapsed ear canal can produce a conductive HL on the order of 15 to 30 db HL, greatest loss at 2000Hz

- Occasionally find collapse of and ear canal brought about by pressure that results from earphone placement
(pressure from the ear phones against the side of the head moves pinna forward causing the soft cartilaginous portion of the ear canal to close)
- About 3% of a clinical caseload in both adults and children/ more frequent in adults and elderly but it has been reported in neonates

- Insert headphones can prevent this
Foreign bodies-
- One of the aquired external ear disorders

- Ear canal is large enough to fit some objects that can puncture or get stuck in the ear canal (this can produce a mild conductive HL)

- ear canal is warm and moist which creates an environment that some objects like and expand in (beans)
Wax impaction-
- Can cause abnormalties in the external ear

- In some individuals, some ear canals generate an excessive amount of cerumen (earwax)

- Earwax must be removed periodically or will build up and block sound transmission of the middle ear

- If it gets too deep (qtip) it gets forced into middle ear!!
Cysts and tumors
- Both cyst and tumors can be found in the external ear and ear canal

- CYST- closed cavity or sac that lies underneath the skin and often filled with a liquid or semisolid material

- IF SWOLLEN from infection, a cyst can cause HL

- Tumors can arise in pinna or ear canal
Benign tumors can have a vascular origin or can simply can be an outgrowth of bone

MALIGNANCIES- frequencies arise from pinna but can arise in ear canal; occasionally these lesions become quite large and close off ear canal
MIDDLE EAR DISORDERS
- Many outer ear anomalies may also have middle ear implications

- Middle ear anomalies may include malformation of the ossicles or fusion of the ossicles.

- Middle ear cavity may be absent or just have a slit-like opening.
The problem of Otitis Media
- One of the most common childhood diseases

- Inflammation of middle ear cavity

- Otitis Media is often classified on the basis of the temporal sequence of the disease

- Disease is categorized according to the duration of the disease process
Otitis Media
Subacute otitis media
Acute otitis media (AOM)
Chronic otitis media (COM)
Otitis Media –
inflammation of the middle ear without reference to cause or pathogenesis
Acute otitis media (AOM) –
rapid onset of signs and symptoms, persists for a week to ten days and then resolves rapidly; symptoms: budging reddened tympanic membrane, pain, and upper respiratory infection; e.g. otalgia and fever, of acute infection of middle ear
Chronic otitis media –
(COM)- if disease has a slow onset and persists for 3 months or more; symptoms- large central perforation in the eardrum and discharge of fluid through the perforation
Subacute otitis media-
persists past acute stage but it is not yet chronic
Otitis Media-Also can be classified according to the type of fluid in the middle ear cavity:
If the fluid is purulent or Suppurative (like fluid found in most AOM) it will contain:
white blood cells, some cellular debris, and many bacteria

- Otitis Media with Effusion (OME)
- Middle ear effusion (MEE) - serous, mucoid, and purulent

- Otorrhea
Otitis Media with Effusion (OME) –
Inflammation of the middle ear with a collection of liquid in middle ear space

One of the most prevalent childhood diseases: 76-95% of children have atleast one episode by age 6
- Most prevalent within the first 2 years of life and also see more cases in males than females
  Middle ear effusion (MEE) –
Liquid in middle ear resulting from otitis media:
-SEROUS– thin watery- a fluid that is free of cellular debris and many bacteria
-MUCOID– thick & viscous; mucus-like
+ sometimes the fluid is mucoid, having secreted from the mucosal lining of the middle ear
+ Mucoid fluid is thick and contains white blood cells, few bacteria, and some cellular dedris

-PURULENT– puss-like
Otorrhea –
discharge from the ear.
There are two ways a person might have a hearing loss:

Prevalence?
1) born with a congenital hearing loss
2) during course of the lifetime

1/1000 have a profound loss at birth
3/1000 some degree of hearing loss
Congenital Hearing Loss:
May be conductive, sensorineural, or mixed

Can also vary in degree: mild, moderate, severe, profound

Can be unilateral or bilateral

Hearing Loss you are born with
Nosyndromic Hearing Loss:
Genetic hearing loss is broken into 2 types,
- Person just has genetic hearing loss
Accounts for 2/3 of all generic hearing loss
Of these 2/3, CX26 accounts for about half of the 2/3, other third are due to other issues
Syndromic Hearing Loss:
- Category of genitic hearing loss

- In addition to hearing loss you may have other clinical disabilities
Most common genetic hearing loss:
(identified in the late 1990’s) Connexin 26 Cx26
Waardenburg Syndrome:
AUTOSOMAL DOMINANT Inherited Hearing Loss:
If you have this gene, it is a sensorineural hearing loss that may be unilateral and bilateral
Accounts for 3% of childhood hearing loss

Symptoms: white forelock, premature graying, eyes are different colors (one brown one blue), widely spaced eyes (called hypertelorism), fused eyebrows, partial albinism, high nasal bridge
Treacher Collins Syndrome:
AUTOSOMAL DOMINANT Inherited Hearing Loss:
Produces conductive hearing loss

Symptoms: craniofacial abnormalities (poorly developed cheekbones, Atresia-absence of ear canal, small pinna, Preauricular skin tags- could be a clue of conductive hearing loss, notching of eyelids, malformations of the external ear canal, micrognathia- an abnormally small mandible, cleft palate)
Coloboma
Little narrow slit openings in eyelid
Hole in eye
Associated with Treacher Collins and Charge Syndrome
Neurofibromatosis I and II:
AUTOSOMAL DOMINANT
NSF II: progressive sensorineural hearing loss

Symptoms: café olespots on arms caused by acoustic tumors on 8th CN or other CN
Usher’s Syndrome:
AUTOSOMAL RECESSIVE Inherited Hearing Loss:
The most common recessive syndrome; Accounts for 10% of childhood hearing loss
Three Types- All three types have progressive visual loss due to retinitis pigmentosa
Usher’s Syndrome Type 1:
Congenital, profound, SNHL; Absent vestibular function
Usher’s Syndrome Type 2:
Moderate, SNHL
Downward sloping- high-frequencies have more hearing loss
Normal vestibular function
Usher’s Syndrome Type 3:
Progressive SNHL
Variable vestibular problems
NIDCD:
“National Institute of Deafness and Other Common Disorders”
Jervell and Lange-Neilsen Syndrome:
AUTOSOMAL RECESSIVE Inherited Hearing Loss:
Produces a profound bilateral sensorineural hearing loss
Abnormal heart rhythm associated with this; leads to syncope

Syncopal episodes- (temporary suspension from consciousness caused by generalized cerebral ischemia for a short time- could die from this)
Pendred’s Syndrome:
AUTOSOMAL RECESSIVE Inherited Hearing Loss:
Mixed or sensorineural hearing loss; severe to profound SNHL; 15% may be progressive
Associated with variable inner ear malformations
Thyroid GOITER- an abnormally large thyroid and SNHL
Norrie Syndrome:
X LINKED Inherited Hearing Loss:

May produce a progressive, sensorineural hearing loss

Specific ocular symptoms-
congenital or rapidly progressive blindness
Males lose vision very rapidly (may even lose vision right at birth)
Developmental disorders
Fungus Infections-
(swimmer’s ear) Aquirred hearing loss
MIDDLE EAR DISORDERS- Overview Characteristics
Many outer ear anomalies may also have middle ear implications
Middle ear anomalies may include malformation of the ossicles or fusion of the ossicles.
Middle ear cavity may be absent or just have a slit-like opening.
Middle Ear Anomalies and Disorders:
Classifications of Otitis Media
Otitis Media without effusion
Acute Otitis Media (AOM)
Otitis Media with effusion (OME)
Otitis Media without effusion:
Characteristics of Middle ear
opaque or erythema tympanic membrane (TM) with no effusion

(No fluid escaping)
Acute Otitis Media (AOM):
Rapid, short onset of signs & symptoms- otalgia, otorrhea, fever, irritability, anorexia, vomiting, diarrhea
TM is bulging, opaque, and has limited or no mobility to pneumatic otoscopy.
Otitis Media with effusion (OME):
Patient relatively asymptomatic; lacks signs/symptoms of AOM.

TM is opaque, retracted or bulging

Fluid level present is middle ear space

Can be serious enough to erode bone (ossicles, tegmen typmpani – which may cause meningitis).
What is the most common complication from otitis media?
Hearing loss is the most common complication from otitis media. Hearing sensitivity may range from normal to moderate HL(50 dB).
What is the treatment for otitis media?
Antibiotics and placement of PE (pressure equalization) tubes
CAUSE OF OTITIS MEDIA
Believed that otitis media develops because of Eustachian tube obstruction

Eustachian tube provides for pressure equalization and fluid drainage of middle ear- if pressure equalization system is obstructed, negative pressure can develop in the middle ear cavity

Negative pressure can develop in the middle ear cavity; the negative pressure sucks the fluid from the membranous lining of the middle ear canal

The fluid has accumulated from the mucosal lining of the middle ear and had no place to escape because the Eustachian tube is blocked (blockage can be caused by a number of things- large adenoid tissue in the nasopharyngeal area and inflammation of the mucous lining tube
Eustachian tube in children
Muscular function of the Eustachian tube is poor in children with OM and children with a history of middle ear disease

Muscle responsible for opening and closing of tube(tensor veli palatine) is relatively inefficient among young infants and children

ALSO, Eustachian tube is at a 10° angle in kids vs. a 45° angle in adults/ short in infants; because of angle and shortness of the tube in children, fluid is able to reach the middle ear from the nasopharyngeal area with relative ease and has difficulty escaping from the middle ear cleft
OTOSCLEROSIS-
Middle ear disorder
Means hardening of the ear
Common of conductive hearing loss

Manifested by a buildup of spongifying bone on the osseous labyrinth (usually in the area of the oval window)

Buildup of bone around oval window immobilizes the footplate of the stapes and interferes with sound transmission to the inner ear (the growth may invade the walls of the cochlea- if this happens then the HL become sensorineural)

Reported to affect 5-10% of whites
2.5 times more common in women and may be exacerbated during pregnancy
Occurs less frequently in African Americans and Asians
Onset usually between 20s – 40s
Bilateral more common than unilateral.
Has genetic predisposition
Audiologic Manifestations of Otosclerosis
Progressive conductive HL (moderate hearing thresholds for air conduction with greater loss in the low-frequency region and relatively normal bone conduction thresholds)

Carhart Notch (max. @ 2000 Hz) page 181
Management of Otosclerosis
Most common approach is surgery to the stapes.

Stapedectomy – removal of the stapes and prosthesis is substituted for the stapes. (WANT TO GET A GOOD SURGEON)

Shallow tympanogram

Can have normal compliance in both ears, but one ear would be more compliant than the other one
Stapedectomy
Removal of the stapes and prosthesis is substituted for the stapes

- This is the most common approach for the management of Otosclerosis which is when there is a build up of bone around the oval window thus immobilizing the footplate of the stapes
Other Ossicular Disorders
Fixation may occur with other ossicles besides the stapes
The ossicles may be disarticulated, or pulled apart, producing a variety of different conductive hearing losses. (where incus and stapes connect called the linticular process - this is where they most often break)
Deep tympanogram
Sensorineural Hearing Loss"
Hearing losses that occur at or central to the inner ear are called sensorineural hearing losses (SN HL)
So, in SN HL both AC and BC responses are abnormal.

This type of hearing loss is usually permanent in nature.

The term sensorineural is derived from the fact that in cochlear pathology there is damage to the sensory structures within the cochlea. This destruction eventually leads to some neural degeneration of afferent nerve fibers, which are connected to the hair cells.
One of the most common characteristics of patients with SN HL is…
One of the most common characteristics of patients with SN HL is that they hear speech, but cannot understand it (except for profound HL) (LOUDNESS RECRUITMENT)
Cochlear Pathology (perceptual consequences) of a SNHL
- Can be born with SN HL or can acquire it

- SN HL that deals with the cochlea

- One of the most common characteristics of patients with SN HL is that they hear speech, but cannot understand it (except for profound HL).
(Loudness recruitment) – a more rapid growth in loudness in the ear with cochlear pathology than in normal ear

- presents problems when trying to fit patient with a hearing aid. (Small changes in intensity seem like sound changes in magnitude)

Persons with SN HL may have some speech abnormalities depending on severity and age of onset. May include: misarticulated speech, poor monitoring skills without hearing aid (i.e. may speak at levels that are inappropriately loud).
Loudness recruitment:
- Happens with Sensorineural Hearing loss / Cochlear pathology

- defined as a more rapid growth in loudness in the ear with cochlear pathology than for the normal hearing ear.

-This presents problems when trying to fit patient with a hearing aid.

- Another way to say this is: Small changes in intensity seem like sound changes in magnitude
(shouting is not the way to deal with HL)
SN HL
- Threshold by BC is within 5 dB of AC threshold
- Persons with SN HL may have some speech abnormalities depending on severity and age of onset. May include: misarticulated speech, poor monitoring skills without hearing aid (i.e. may speak at levels that are inappropriately loud).

- Can be born with SN HL or can acquire it

- One of the most common characteristics of patients with SN HL is that they hear speech, but cannot understand it (except for profound HL)
Causes of Cochlear Pathologies in Infants
High risk factors
JCIH- Joint Commission on Infant hearing (Factors associated with permanent congenital, or progressive HL in childhood: Caregiver concern, family history of childhood HL, Neonatal intensive care unit >5days or any of the diseases below, in-utero infections, and any physical findings (like ear tags, or white forelock, ect.)

These factors may require that newborn be placed in the Neonatal Intensive Care Unit (NICU)
Viral or bacterial disease
Infectious diseases frequently transmitted to infant by mother in utero.
Condition is referred to as pre-natal (refers to something that occurs to the fetus before birth), congenital (implies before birth but usually before the 28th week of gestation), or perinatal (Occurring during birth or from 28th week of gestation to 7 days after delivery)
Disease can also occur later in life and this is referred to as postnatal.
JCIH:
Joint Commission on Infant hearing
Factors associated with permanent congenital, or progressive HL in childhood:
Caregiver concern
Family history of childhood HL
Neonatal intensive care unit >5days
Any of the diseases; Hyperbilirubinemia, Persistent pulmonary hypertension in newborn (PPHN) , Extra Corporeal Membrane Oxygenation (ECMO, exposure to ototoxic medications, or asphyxia
Any physical findings (like ear tags, or white forelock, ECT.)

These factors may require that newborn be placed in the Neonatal Intensive Care Unit (NICU)
Viral or bacterial disease in newborns
Infectious diseases frequently transmitted to infant by mother in utero.

Condition is referred to as pre-natal
- refers to something that occurs to the fetus before birth
- congenital- implies before birth but usually before the 28th week of gestation
- perinatal- Occurring during birth or from 28th week of gestation to 7 days after delivery

Disease can also occur later in life and this is referred to as postnatal.
Pre-natal:
Refers to something that occurs to the fetus before birth
Congenital:

(In respect to infant birth)
Implies before birth but usually before the 28th week of gestation
Perinatal:
Occurring during birth or from 28th week of gestation to 7 days after delivery; occurred or developed right before or right after birth
Postnatal:
Disease can also occur later in life
Hyperbilirubinemia-
bilirubin levels are excessively high; jaundice; cannot clear bilirubin like it should; if jaundice is so great that it requires a blood transfusion and puts them at high risk for HL
Persistent pulmonary hypertension in newborn (PPHN) –
Typically occur in fullterm or post term infants

Characteristic of hypoxemia (subnormal oxygen levels in blood)

Different way for getting oxygen when they are in the womb vs. when the baby comes out of the womb
Blood pressure decreases

If baby system does not function correctly when it is born, they often get assisted ventilation
Extra Corporeal Membrane Oxygenation (ECMO)-
tube in side of neck and the non oxygenated blood drains out and oxygen is added to the blood through a pump (a treatment of PPHN)
Exposure to ototoxic medications-
(gentamycin and tobramycin) or loop diuretics (furosemide/ lasix) which decreases sodium
Asphyxia-
blockage of infant’s airway; strangulation such as getting caught in the umbilical cord

-High risk factor of infant hearing loss
Prenatal Diseases often causing infant hearing loss include...
Many prenatal diseases comprise the (S)TORCH complex, an acronym for these diseases.
Any disease in TORCH complex is considered high risk for HL

Syphilis, Toxoplasmosis, Rubella, Cytomegalovirus (CMV), Bacterial Meningitis
Syphilis:
transmitted to the child by and intrauterine infection from the mother
-may have delayed onset (can manifest itself anywhere between the 1st to the 6th decade of life)
-severe to profound HL
-VDRL- can help determine if syphilis is present in the mother
-if the onset is early in life then the SNHL is profound and bilateral
-adult onset is fluctuating and asymmetric and it may be gradual or sudden onset
Toxoplasmosis:
Mothers can contract it through partially cooked meat, feces

Leads to progressive SNHL

Moderate in many cases; bilateral

Disease is transmitted via the placenta

LUES disease- sometimes called
Rubella:
Virus is carried through the bloodstream to the placenta to infect fetus; not many cases anymore

In the mid sixties there was a severe out break of rubella (25% of congenital hearing loss in that time were caused by this) mother often did not know she had the virus;

Rubella could cause vision problems, small heads, mental retardation, stunt growth;

Causes profound bilateral SNHL- cookie bite audiogram present

Hearing loss may be progressive
Cytomegalovirus (CMV):
Most common viral disease known to cause HL

-about 1% annually

CMV responsible for about 20-30% of childhood HL


-many people carry this virus

-can be passed through salvia, tears, mothers milk

-can lead to developmental delays, one of the most common causes of unilateral SNHL

- HL is usually sudden and can vary from a mild, high frequency impairment to profound loss

progressive HL, vision loss, neurological problems;

CMV is most common virus to cause HL

- can be contracted when baby travels through birth canal

- no known cure but antiviral drugs treat symptoms

(cyt- cell / megal- abnormally large or long)
Bacterial Meningitis:
20% of babies with this will develop HL

Babies born with this are at high risk for a hearing loss
Herpes Zoster Oticus:
One of the POSTNATAL sensorineural diseases

First symptom is a burning pain close to the ear
Recurrence of herpes virus (often the chicken pox virus or shingles)
Can cause painful burning blisters on hear hearing canal, and invade 7th CN and lead to facial paralysis
Can also cause vertigo
Mumps:
at one time it was the leading cause for unilateral SN hearing loss
Measles:
Another cause of SNHL; Hl affect 6-10% of measles patients
Atypical audiometric configuration is a sever to profound bilateral high-frequency SN impairment
Also show conductive HL
Postnatal Diseases associated with sensorineural hearing loss-
Mumps
Measles
Chicken Pox
Influenza
Meningitis
Herpes Zoster Oticus
Meniere’s Disease
Affects membranous inner ear; cause my an over abundance of endolymph in the ear and may result in a SNHL
Has a rising low frequency component in audiogram
Fairly common
Classic symptoms include: progressive fluctuant HL; episodic vertigo ; tinnitus ; fullness (do not have to have all four to have disease, but they are the four symptoms that characterize the disease)
Low frequency SNHL- this is something that sets Miniere’s disease apart; usually SN HL is over all frequencies or high freq
fluctuant- one of the VERY FEW hearing losses that can reverse itself; repeated episodes can eventually cause a permanent HL if episodes occur frequently enough
Vertigo- very dizzy; episodic; comes and goes (few minutes to several days)
Tinnitus- often a roaring tinnitus (seashell)
Fullness- feels like there is a lot of pressure in your ear; pressure is in inner ear; membrane gets distended
May be unilateral or bilateral
Treatments- low salt diet so keeps you from retaining fluid (many time this is all they need); if that diet doest work- try endolymphatic shunt surgery- puts shunt in the inner ear and drains the fluid (doesn’t always work); remove the labyrinth- no dizziness but you are now deaf
Classic symptoms of Meniere’s Disease include:
progressive fluctuant HL; episodic vertigo ; tinnitus ; fullness (do not have to have all four to have disease, but they are the four symptoms that characterize the disease)
Something that sets Miniere’s disease apart-
Low frequency SNHL; usually SN HL is over all frequencies or high freq
Meniere’s Disease is fluctuant meaning that it is-
one of the VERY FEW hearing losses that can reverse itself; repeated episodes can eventually cause a permanent HL if episodes occur frequently enough
Vertigo-
very dizzy; episodic; comes and goes (few minutes to several days) ( associated with Meniere’s Disease)
Tinnitus-
often a roaring tinnitus (seashell) (Meniere’s Disease)
Fullness-
feels like there is a lot of pressure in your ear; pressure is in inner ear; membrane gets distended
Meniere’s Disease Treatments-
- A low salt diet so keeps you from retaining fluid (many times this is all they need)

- If that diet doesn't work- try endolymphatic shunt surgery- puts shunt in the inner ear and drains the fluid (doesn’t really work)

Lastly, remove the labyrinth- no more dizziness but you are now deaf
Ototoxic Drugs
A negative side effect of some antibiotic drugs result in the production of severe high-frequency SNHL

A variety of factors can determine whether HL is produced in a specific patient

Aminoglycosides – family of antibiotics that usually includes the mycin drugs (kanamyosin, streoptomysin, gentamyosin, neomyosin, tobramyosin)

Gtotoxic drugs reach the inner ear through the bloodstream; the resulting damage is greater in the base of the cochlea and the outer hair cells are typically the primary targets, with only limited damage appearing in other cochlear structures

Results in an audiometric pattern of moderate to sever high-frequency SNHL in both ears

Some ototoxic drugs can cause a temporary or reversible effect (aspirin)
Factors to consider with Ototoxic Drugs
-Dosage
-Patient susceptibility- people react in different ways
-Toxicity of agent- some are more mild than others (gentamyosin is more mild)
-Normalcy of kidney function- how well the kidneys are doing plays a factor
Presbycusis:
Hearing loss due to the aging process

Remember: Presby- old age
Factors of Presbycusis
Begins around 40-50 years
Cannot say it is STRICTLY an aging process; other factors play into this
Greater for men than women- because men usually expose themselves to more loud noises
Sloping HF SNHL- most common types of presbycusis- sensory and neural- associated with the Sloping HF audiograms
With a notch around (3000-6000) in audiogram is a characteristic of noise exposure (AIR CONDUCTION NOTCH)
In the aging population, what are the only two diseases that precede hearing loss?
Arthritis and hypertension
Retrocochlear pathology:
Refers to pathologies central to cochlea; it refers to damage to nerve fibers along the ascending auditory pathways from the internal auditory meatus to the cortex
Retrocochlear pathology usually involves what?
Usually involves some type of tumor, called acoustic neuromas, but may include other CNS disease (e.g. multiple sclerosis MS- non tumor)
Acoustic neuromas:
type of tumor; more often called acoustic (vestibular) schwanomas, often they are on the vestibular portion of the auditory portion rather than the auditory portion
Retrocochlear Pathology Characteristics:
-Often, auditory manifestations of the retrocochlear pathology are subtle (such as no HL for pure tones is measured)

-Audiogram – varies in degree; by pure tones have very normal hearing, and sometimes you have severe hearing loss

-When HL present – usually HF SN but HL is usually unilateral

-Asymmetry is a reason to question what is causing it in one ear

-acoustic reflex decay- look at change over static acoustic admittance over ten seconds; if amplitude of response decreases by 50% or more we call it positive reflex decay (positive sign for retrocochlear pathologies)
Retrocochlear Pathology: Common Complaints
-Tinnitus affected side
-dysequilibrium or gait problems
-WR varies from normal to poor
-Acoustic reflexes usually absent or elevated when affected ear stimulated
-Acoustic reflex decay – positive
-ABR abnormal
Non Organic Hearing loss-
Not a true hearing loss, feign hearing loss, generally looking for some kind of compensation, looking for attention or looking for an excuse
May not be in agreement between pure tone thresholds and speech thresholds; speech are often higher because they have a harder time establishing a reference point
(psychogenic HL, psudeohypocusis)
Prevalence of a profound hearing loss in infants is…
1 in 1000
About 2 or 3 of every 1000 infants have…
A mild or moderate hearing loss
The most common birth defect in the US is…
Hearing Loss
Deafness costs our society…
Deafness is very costly to our society (entitlements- 2 billion; education- 121.8 billion; lost productivity- 2.5 billion)
Why do we need to test ALL babies for hearing loss?
Only half of those babies who have hearing loss have visible “risk” factors

We want to diagnose ASAP
Why do we need to identify hearing loss in infants?
By identifying children early, they are able to develop better speech and language, better in school in general, and better reading comprehension
Value of Screening for a Disorder / Factors that Determine Whether to Screen"
Importance of Disorder
Prevalence of the disease
Acceptable criteria for diagnosis-
Acceptable diagnostic and treatment facilities
Cost-effectiveness
An Acceptable Screening Test should be....

It should have what characteristics?
• Selected test should be the most sensitive or effective to detect condition set out to identify.
• A good screening test should be – acceptable, reliable, valid, and cost-effective.
Importance of Disorder-
Value of Screening for a Disorder Characteristic

- We want to know the functional implication the disorder has on person’s life and make sure our diagnosis is important, worthwhile, and as helpful as possible
Prevalence of the disease: in regards to the Value of Screening for a Disorder
If the overall prevalence of a diseases id too low or too high, not a huge need for assessment until there are specific characteristics to support the test
"Acceptable" criteria for diagnosis of a screening test: Question we have to ask
Do the symptoms for this disease occur regularly enough to make a diagnosis and feel confident about it? How confident?

We don't want criteria to be too wide, nor do we want it to be too narrow...
Acceptable diagnostic and treatment facilities-
- Part of the Criteria for the Value of a Screening test

- Need a way to get help once diagnosed; people do not just a name for a disease, they want treatment
Cost-effectiveness-
the best treatment for the littlest amount of money

Hearing screening that is the most cost effective in relation to the results and its accuracy

- determined to tests how good a hearing screening is
What should and acceptable Screening Test be able to do?
Selected test should be the most sensitive or effective to detect condition set out to identify

- Measure whatever it is testing with the highest amount to sensitivity and specificity
Valid Screening Test-
Is the method used to screen really testing what it should be testing? What it is supposed to be testing?

Validity is a property of good screening

Validity consists of sensitivity and specificity, the higher these numbers are, the more valid the experiment
Cost-effective Screening Test-
Find the most effective screening for the littlest amount

-A property of good screening
Purpose of Screening Tests-
- Used to examine a population of individuals and identify those with a disorder versus those without a disorder

- Need populations of both groups the create and evaluate screening tests
When evaluating a screening test, what does every population need?
Every population includes both groups of disordered individuals and non-disordered individuals
What is the purpose of the Audiologist in hearing screening evaluations?
Audiologists role is to place each group in their proper categories

- Both the disordered and the non disordered need to be classified correctly and diagnosed
Every hearing screening should be compared to…

Why?
- A gold standard

- In order to find out what the sensitivity and specificity is

- thus telling us how truly effective the screening is
The success of a screening test depends on what two factors:
Specificity and Sensitivity
Sensitivity-
Tells you the proportion of the population that has the disease and test positive

A/ A+C (100)

People who REALLY DO have the disorder/ All who test POSITIVE for that disorder

(Trick to remember- Usually people are more sensitive when they ACTUALLY HAVE A DISORDER)
Specificity-
- Tells you the proportion of the population without the disease who has a negative test finding

D/ B+D (100)

People who DO NOT have the disease/ TOTAL who tested NEGATIVE
What would an ideal screening test consist of?
100% specificity and sensitivity
How do specificity and sensitivity impact hearing screening evaluations?
For a screening test, we want the highest levels of these two factors. In an Ideal screening test we would have 100% specificity and sensitivity.

We change the criteria of what we want to include in out “disabled” population to try to get the best levels of these.

CHANGING ONE AFFECTS THE OTHER
When looking at results in a hearing evaluation, if a person were to FAIL the screening, how would he or she test in accordance to the disease?
They would test POSITIVE for the disease
Prevalence =
proportion of individuals in the population who have the disorder or how often the disorder occurs. The equation correlating with the cart is: (A+C/ A+B+C+D)
Positive Predictive Value-
- Probability of disease in a patient when the test is abnormal

PROBABILITY that the patient that actually HAS THE DISEASE when the test is ABNORMAL

A/ A+B
Negative Predictive Value-
the probability of a patient NOT having the disease when the test is NEGATIVE

D/ C+D
Positive and Negative Predictive values are affected by…
Prevalence of the Disease
When the prevalence of a disease in the population is low, than predictive value…
Is closer to zero
When prevalence DECREASES…
Positive predictive value also DECREASES
What is important to remember when creating and evaluating hearing screening tests?
Cannot develop your test on one population and then apply it to another population because you will get misleading information
FALSE POSITIVE-
When a person is NORMAL for the condition and fails the screening test meaning they DO NOT have the disease

"B"
FALSE NEGATIVE-
Occurs when person what HAS THE DISEASE and passes the test

- Gives them the FALSE diagnosis that they are negative to the disease

" C "
A good screening should be: (Factors)
Acceptable
Reliable
Valid
Cost-effective
Give an example of a chart used in a hearing screening
Disorder Non-disorder
Fail/ Positive for Disorder 27 7
Pass/ Negative for Disorder 3 63

If you have the disorder then you fail the screening and test POSITIVE for the disease
Sensitivity= A/ A+C (100) 27/30= 90%
Specificity= D/ B+D (100) 63/70= 90%


PPV= 28/ 34 = 80%
Prevalence is 30% in this sample
Give an example of a chart used in a hearing screening

(relate to previous example)
Fail/ Positive for Disorder 9 9
Pass/ Negative for Disorder 1 81

Prevalence of disease is lower now; 10%
Positive Predictive Value = (9/18) = 50%
You always want to compare your screening test results to what?
A gold standard
Universal Newborn Hearing Screening Program regulations for infants with disorders-
suggest that hearing disorder be identified by 3 months of age and treatment be implemented by 6 months
When should you use ABR tests for babies?
When the babies that are in the NICU for more than 5 days they are in greater danger for hearing loss and should be tested using ABR.


OAE tests are cheaper and done more often but ABR more effective

ABR is a stimulus of waves for an onset of ten seconds. Cochlear micro-phonic are electrical potentials generated by the inner ear. ABR can be plotted with the wave peaks up, or down.(depending on which way you set up the electrodes on your head.)
If babies fail their initial hearing screening they should be RE-screened when?
By the time they leave the hospital, or within ONE MONTH.
If an infant fails a hearing screening in ONE ear…
they should RE-screen both ears, not just the one that fails the test.
If a baby has been diagnosed with a condition that is associated with a potential hearing loss…
All babies who are readmitted to the hospital are tested, regardless of if they spent time in the NICU or not.
Screening test should yield maximum accuracy, in other words it should have…
high sensitivity and specificity
If you are born with a hearing loss, does it mean it is genetic?
Not all congenital (present at birth) hearing disorders are hereditary, nor are all hereditary disorders congenital
What are the statistics of being born with a profound hearing loss?

Birth rate of having some degree of hearing loss?
1/1000 have a profound loss at birth
3/1000 some degree of hearing loss
Congenital vs. Conductive
Congenital- born with it
(WITH GENES)

Conductive- Air bone gap- ossicles cannot "conduct" sound
What causes Meniere’s Disease, and what part of the ear does it affect?
Affects membranous inner ear

Fairly common disease
May be unilateral or bilateral

- Caused by an over abundance of endolymph in the ear and may result in a SNHL
What are the four classic symptoms of Meniere’s Disease?
Classic symptoms include:
1) progressive fluctuant HL
2) episodic vertigo
3) tinnitus
$) fullness

(do not have to have all four to have disease, but they are the four symptoms that characterize the disease)
Acceptable Screening Test
- Easy to administer and Interpret

- Generally well received by the public
What are two characteristics that set Meniere’s Disease apart from others?
1) Low frequency SNHL-
Usually SN HL is over all frequencies or high frequencies
- The audiogram have a rising low frequency component


2) Fluctuant- one of the VERY FEW hearing losses that can reverse itself
- repeated episodes can eventually cause a permanent HL if episodes occur frequently enough
Reliable Screening Test
- Should be CONSISTENT, providing results that do not differ significantly from one test to the next on the same individual
Gold Standard-
- The results of a gold standard test are universally accepted as proof that the disease or disorder is either present or absent

- All screening tests must be evaluated against an independent standard, known as the gold standard
What are the box of A represent in the hearing screening characteristics test?
A- the disease is both POSITIVE AND PRESENT

- They test positive for the disease and they DO actually have it (TRUE positives)
What are the box of B represent in the hearing screening characteristics test?
B- people who DO NOT have the disorder but test POSITIVE

FALSE POSITIVES
What are the box of C represent in the hearing screening characteristics test?
C- Those with the disorder who test NEGATIVE

FALSE NEGATIVES
What are the box of D represent in the hearing screening characteristics test?
- D- Represents thoses people who DO NOT have the disorder and who DO NOT test positive

- Test negative (bottom right corner)
Matrix of the operating characteristics of a screening test

How is it set up?
Positive and Present (A) / Positive Absent (B)

Negative and Present (C) / Negative Absent (D)
How is a matrix of the operating characteristics for a screening test set up?
Horizontal - IS the disease TRULY present or Absent? Do the participants actually have the disorder?

Vertical- Did they test positive for the disease, meaning they FAILED the test? Did they test negative?

TEST NEGATIVE = DOES NOT indicate a hearing loss, means that they PASSED the screening
What does A+ C represent in the matrix of the operating characteristics for a screening test?

B+ D
A+ C = Total of people who fail the test or test POSITIVE and indicate that they do have this hearing loss
(Deals with Sensitivity)


B+ D = Number of people who DO NOT have a disorder meaning that they PASS the test

(ADD columns up VERTICALLY)
Characteristics/ prevalence/ statistics of Otosclerosis
Reported to affect 5-10% of whites

2.5 times more common in women and may be exacerbated during pregnancy

Occurs less frequently in African Americans and Asians

Onset usually between 20s – 40s

Bilateral more common than unilateral.

Has genetic predisposition
Severity of hearing loss
Review: dB level associated with severity of hearing loss
• Mild (26-40 dB)
• Moderate (41-55 dB)
• Moderately severe (56-70 dB)
• Severe (71-90 dB)
• Profound (90 dB)
What are the three types of middle ear liquids associated with MEE? (Middle Ear effusion)
-serous – thin watery liquid
-mucoid – thick & viscous; mucus-like; secreted from the mucosal lining of the middle ear
-purulent – puss-like
What is cochlear pathology?
Diseases that are associated with the cochlea
High risk factors of hearing loss in infants include:
- Any condition that requires the newborn to stay in the NICU for more than 5 days
- Family history for hering loss
- Infections in utero ( TORCH)
- Physical findings associated with any type of (SN and conductive) hearing loss (ear tags, or white forelock)

These factors may require that newborn be placed in the Neonatal Intensive Care Unit (NICU)
JCIH- Joint Commission on Infant hearing
Developed high risk check list
-Factors associated with permanent congenital or progressive HL in childhood:
Caregiver concern, family history of childhood HL, Neonatal intensive care unit >5days or any of the diseases below, in-utero infections, and any physical findings associated with hearing loss of any kind
Viral or bacterial diseases in infants: Classifications of when they Occur
Infectious diseases frequently transmitted to infant by mother in utero

Condition is referred to as pre-natal, referring to something that occurs to the fetus before birth

Condition can be congenital implying before birth but usually before the 28th week of gestation

Or it can be perinatal which typically refers to the disease that occurs during birth or from 28th week of gestation to 7 days after delivery)

Disease can also occur later in life and this is referred to as postnatal