Hearing Loss: Dr. Timothy Gacani

Hearing loss
Dr. Timothy Gacani

• A person not able to hear as well as someone with normal hearing – hearing
thresholds of 25 dB or better in both ears – said to have hearing loss.
HL may be mild, moderate, severe, or profound.
Can affect one ear or both ears -> difficulty in hearing conversational speech or loud
sounds.
• 'Hard of hearing' : HL ranging from mild to severe. communicate through spoken

language and can benefit from hearing aids, cochlear implants, and other
assistive devices as well as captioning. more significant hearing losses -> cochlear
implants.
• 'Deaf' -> profound hearing loss: very little or no hearing. often use sign language
for communication.
• extremely common medical condition.
• The affected population vast, between neonates to elderly.
• multi-disciplinary team: surgeon, speech therapists, and social
workers.
Etiology
• Normal hearing function: sound waves • Conductive hearing loss: disruption of
arriving at auricle, causing vibration of transmission of sound waves to cochlea.
tympanic membrane.
• Most common: abnormal formation of

• Vibration transmitted via ossicles (malleus,
incus, stapes) to cochlea.
auricle or helix, cerumen impaction, ear
canal FBs, otitis externa or dysfunction
or fixation of ossicular chain.
• Subsequently, hair cells inside cochlea
stimulate VIIIth cranial nerve that transfers
stimuli to brain. • cholesteatoma, benign aggressive
growth into tympanic membrane, as well
• Hearing loss can be conductive or
as other benign or malignant tumors,
sensorineural. can result in conductive hearing loss.
• Sensorineural hearing loss (SNHL) from problematic transmission of
stimuli AFTER cochlea.
• could be related to hair cells dysfunction or 8th CN itself.
• main difference between the two kinds of hearing loss apart from
pathophysiological features is with conductive hearing loss perceive
sounds diminished, while SNHL patients perceive sounds diminished
and distorted.
• Hearing loss with problematic transmission before and after cochlea is mixed.
• In children population, genetic causes most common, accounting for > 50% of
hearing loss cases.
Genetic causes involve various syndromes that have hearing loss as one of their features.
• Prenatal causes also responsible for hearing loss in infants.
include exposure to various bacterial or viral infections as well as different teratogens.

• causes of hearing loss and deafness can be congenital or acquired.
Congenital causes
• HL present at or acquired soon after birth.
• can be by hereditary and non-hereditary genetic factors or by certain

complications during pregnancy and childbirth, including:
maternal rubella, syphilis or certain other infections during pregnancy;
low birth weight;
birth asphyxia (a lack of oxygen at the time of birth);
inappropriate use of particular drugs during pregnancy, such as aminoglycosides,
cytotoxic drugs, antimalarial drugs, and diuretics;
severe jaundice in the neonatal period, which can damage the hearing nerve in a
newborn infant.
Acquired causes
Acquired causes may lead to hearing loss at any age, such as:
• infectious diseases including meningitis, measles and mumps;
• chronic ear infections (CSOM);
• collection of fluid in the ear (OME);
• use of certain medicines, such as those used in treatment of neonatal infections, malaria,
drug-resistant tuberculosis, and cancers;
• injury to the head or ear;

• excessive noise, including occupational noise such as that from machinery and explosions;
• recreational exposure to loud sounds e.g. personal audio devices at high volumes and for
prolonged periods of time and regular attendance at concerts, nightclubs, bars and sporting
events;
• ageing, in particular due to degeneration of sensory cells; and
• wax or foreign bodies blocking the ear canal.
• Among children, chronic otitis media is a common cause of hearing loss.

• Otosclerosis and cholesteatomas; leading causes of conductive HL.
• Another entity in adult population: sudden sensorineural hearing loss.

common in audiology cases, and mostly idiopathic as majority of
patients present on an emergency basis without identifiable cause.
Epidemiology
• Over 5% of world’s population – 466 million people – has disabling hearing loss (432 million
adults and 34 million children).
• Disabling HL -> HL greater than 40 decibels (dB) in the better hearing ear in adults and a HL
greater than 30 dB in the better hearing ear in children.
• majority of disabling hearing loss live in low- and middle-income countries.
• 60% of childhood hearing loss is due to preventable causes.
• Approximately one third of people over 65 years of age affected by disabling hearing loss.
Prevalence in this age group greatest in South Asia, Asia Pacific and sub-Saharan Africa.
Pathophysiology
• HL occurs when sound stimuli transmission from outer part of ear to
the brain suffers disruption.
• The disruption can happen at any stage, either before or after

cochlea, and the HL conductive or sensorineural, respectively.
• If both sites, pre and post the cochlea affected: mixed.

History and Physical
• History in pediatric cases.
• questions regarding prenatal history of child, their delivery, and first days of
life as well as post-natal history up until moment of symptom presentation.
• A child with HL > non-reaction to sounds, behavioral problems, speech

issues, language delay, or even school failure, as well as mispronouncing
words.
• Family history, especially a member with early HL.

• Adult history acquisition more straightforward regarding onset of
symptoms, severity, presence of vertigo, neurological symptoms,
infections, and other conditions related to hearing loss.
• Past medical history, as well as family history, along with work and
noise exposure.
• P/E: full otolaryngology examination, with otoscopy bilaterally to rule out any reasons for
conductive HL.
• Foreign bodies, cerumen, infections, tympanic membrane perforations, as well as middle

ear effusion.
• IDn of dysmorphic and other physical findings essential, especially in young children and
infants.
facial abnormalities or asymmetry, ear, neck and skin anomalies, other organ dysfunction, or even
balance irregularities.
• Weber and Rinne (TFTs) useful to differentiate between SNHL and conductive hearing loss.
Evaluation
• Ideally, all infants should undergo a hearing evaluation to rule out any hearing
impairment by age of one month.
• Hearing loss evaluation should take place with various tests that differ according to the
age of the child.
• BAER (brainstem audio-evoked response) for early diagnosis of HL in newborns and
infants.
• Otoacoustic emissions also in newborns, easy, inexpensive technique, but less reliable
than BAER tests.
• audiometry with older children, aged 4 to 5 and older, who can respond to sound stimuli
according to instructions as well as Tympanograms.
Treatment / Management
Management of conductive HL focuses on treatment of underlying disease.
• Conservative methods e.g. removal of FB, micro-suction of cerumen or discharge
in EAC are necessary if ear canal is blocked.
• With OME, myringotomy to release middle ear fluid will allow sound wave to reach
cochlea, while ventilation/myringostomy tubes if OME is persistent, causing HL.
• HL can also present as a post-operative complication due to tympanosclerosis.
• If HL is due to cholesteatoma, -> surgical removal.

• Conservative treatment of sensorineural HL -> use of assistive
listening devices.
Hearing aids are devices designed to improve audition up until 40 to 60 dB.
• Surgical treatment for infants with SNHL, -> cochlear implantation

under age of 6 months.
Prevention
Overall, preventable causes of childhood hearing loss include:
• Infections e.g. mumps, measles, rubella, meningitis, cytomegalovirus
infections, and chronic otitis media (31%).
• Complications at time of birth, e.g. birth asphyxia, low birth weight,

prematurity, and jaundice (17%).
• Use of ototoxic medicines in expecting mothers and babies (4%).
• Others (8%)
Some simple strategies for prevention of hearing loss include:
• immunizing children against childhood diseases, including measles, meningitis, rubella and
mumps;
• immunizing adolescent girls and women of reproductive age against rubella before
pregnancy;
• preventing cytomegalovirus infections in expectant mothers through good hygiene; screening

for and treating syphilis and other infections in pregnant women;
• strengthening maternal and child health programmes, including promotion of safe childbirth;
• following healthy ear care practices;

• reducing exposure (both occupational and recreational) to loud sounds by raising awareness
about the risks; developing and enforcing relevant legislation; and encouraging individuals to use
personal protective devices such as earplugs and noise-cancelling earphones and headphones.
• screening of children for otitis media, followed by appropriate medical or surgical interventions;
• avoiding the use of particular drugs which may be harmful to hearing, unless prescribed and
monitored by a qualified physician;
• referring infants at high risk, such as those with a family history of deafness or those born with
low birth weight, birth asphyxia, jaundice or meningitis, for early assessment of hearing, to
ensure prompt diagnosis and appropriate management, as required;
• educating young people and population in general on hearing loss, its causes, prevention and
identification.

Hearing Loss: Dr. Timothy Gacani

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Hearing loss

Dr. Timothy Gacani

• 'Hard of hearing' : HL ranging from mild to severe. communicate through spoken

• Most common: abnormal formation of

• could be related to hair cells dysfunction or 8th CN itself.

• Prenatal causes also responsible for hearing loss in infants.

include exposure to various bacterial or viral infections as well as different teratogens.

• can be by hereditary and non-hereditary genetic factors or by certain

• chronic ear infections (CSOM);

• collection of fluid in the ear (OME);

• injury to the head or ear;

• ageing, in particular due to degeneration of sensory cells; and

• wax or foreign bodies blocking the ear canal.

• Among children, chronic otitis media is a common cause of hearing loss.

• Another entity in adult population: sudden sensorineural hearing loss.

• majority of disabling hearing loss live in low- and middle-income countries.

• 60% of childhood hearing loss is due to preventable causes.

• The disruption can happen at any stage, either before or after

• If both sites, pre and post the cochlea affected: mixed.

• A child with HL > non-reaction to sounds, behavioral problems, speech

• Family history, especially a member with early HL.

• Foreign bodies, cerumen, infections, tympanic membrane perforations, as well as middle

• HL can also present as a post-operative complication due to tympanosclerosis.

• If HL is due to cholesteatoma, -> surgical removal.

• Surgical treatment for infants with SNHL, -> cochlear implantation

• Complications at time of birth, e.g. birth asphyxia, low birth weight,

• Use of ototoxic medicines in expecting mothers and babies (4%).

• preventing cytomegalovirus infections in expectant mothers through good hygiene; screening

• following healthy ear care practices;

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