Hospital Based Practice – Deafness.

Childhood deafness.
• Can be hereditary or acquired.
• Hereditary defects may be present at birth, or appear later on.
• Autosomal dominant syndromes.
○ Wardenburg’s
 Sensorineural hearing loss
 Pigment abnormalities
 Telecanthus.
• Increased distance between medal canthi.
○ Klipper – Feil
 KFS gene on chromosome 8
 Congenital fusion of cervical vertebrae
 Nystagmus
 Deafness
 CNS signs.
 Classically, mirror movements occur.
• Voluntary movement in one limb are involuntarily mimicked in another.
○ Treacher – Collins’
 Lower lid notching.
 Oblique palpable fissures.
 Flattening of malar bones
 Hypoplastic zygoma.
 If associated features, known as Franceschetti’s syndrome.
• Mandibular defects
• Ear defects
• Deafness.
 May be due to mutations in TCOF1 gene.
○ Pierre Robin
 Neonatal difficulty in feeding and breathing.
 Micrognathia.
• Short chin.
 Cleft palate
 Eye abnormalities.
 Prevent tongue falling back by nursing in prone position.
○ Alport’s
 X – linked or autosomal recesive.
• X – link form caused by mutations in COL4A5 genes that encode the αS –
chain of type _ IV collagen.
 Sensorineural deafness.
 Glomerulonephritis with basket weaving of basemenet membranes.
• Pyelonephritis
• Haematuria
• Renal failure.
 Typical age at death is 20 – 30 years.
• Autosomal recessive syndromes.
○ Pendred.
 Sensorineural deafness.
 Goitre.
○ Usher’s
 Sensorineural deafness.
 Retinitis pigmentosa
○ Jewell – Lange – Neilson.
 Sensorineural deafness.
 Long QT interval.

• Acquired.
○ In utero.
 Maternal infections.
• Rubella
• CMV
• Influenza
• Syphilis
 Ototoxic drugs.
 Metabolic upset, eg. Maternal diabetes.
○ Perinatal.
 Anoxia
 Birth trauma
 Cerebral palsy
 Kernicterus
○ Post natal.
 Meningitis
• Main cause
 Ototoxic drugs
 Lead
 Skull fractures.

• Universal newborn hearing screening

○ Screening within hours of birth is the best way to ensure deafness is detected and managed.
 Management should be implemented before 6 months of age.
○ The main focus is to avoid language delay.
○ The best test is by detecting otoacoustic emissions (OAE)
 Microphone in external meatus detects tiny choclear sounds made by movement in
the basilar membrane.
 Abnormal or equivocal in 3 – 8% of neonates.
○ 84% of abnormal results on OEA are due to external ear canal obstruction.
 In these patients test audiological brainstem responses (ABR).

○ Prevalence.
 0.9 – 3.24/1000 have permanent, bilateral heading loss of > 35 dB
 Increases to 5.95/1000 have when unilateral and moderate hearing loss is included.
• Subjective hearing loss in the older child.
○ Distraction test.
 Can be used when patient 6 months or more.
 Tester in front of patient attracts their attention.
 Tester to the side tries to distract the patient with a rattle or conversational voice.
○ Conditioned response audiometry.
 Can be used from 2 years.
 Child trained to put pegs in a hole or give toys to a parent in response to a particular
auditory cue.
○ Speech discriminatation.
 Can be used from 2 years.
 Patient touches selected objects, cued by acoustically similar phrases, eg. Key/tree.
○ Pure tone audiogram.
 Can be used from 5 years.

• Treatment.
○ Once hearing loss is detected, aim to provide as good hearing as possible to help speaking and
education.
○ Teachers of the deaf make arrangements of fitting hearing aids and help monitor progress.
 Children tend to need higher gain on their hearing aids than adults.
 Ear moulds may need regular changing to maintain a good fit.
○ Encourage parents of deaf children to talk as much as possible to the child.
○ Children may be educated at
 Mainstream schools, with visits from teachers or the deaf.
 I n schools for the deaf or partially hearing.
 In special units of mainstream schools.
○ Cochlear implants may be suitable.
 The shorter the duration of deafness, the better the prognosis.
 Funding decisions should not be delayed.
 Give pneumococcal vaccine 2 weeks before insertion, or asap if implant already in
situ.

 Implants may be damaged by.

• Direct trauma.
• MRI
• Surgical diathermy
• Dental pulp testers
• Therapeutic diathermy, used in physiotherapy departments.
 Ethical issues.
• Expensive intervention.
○ Cost per QALY is about £11400
• Debate over what level of deafness should be the cut off for implantation.
○ In the UK, we implant patients with deafness of > 110 dB
○ In the USA, they implant when deafness is > 95 dB
• There is no good research looking at whether patients feel that they benefit
from the implant, only whether those around them feel that there is an
improvement.
Deafness in Adults.
• 10% of UK adults are hearing impaired.
• Can be a frustrating and isolating disability.
• Management.
○ Determine type of deafness.
 Conductive.
• Impaired sound transmission via external canal and middle ear ossicles to
foot of stapes.
• Has a variety of causes.
○ External canal obstruction.
 Impacted wax
 Dicharge from otitis externa
 Foreign body
 Developmental abnormalities.
○ Drum perforation.
 Trauma
 Barotrauma
 Infection
○ Problems with ossicular chain.
 Otosclerosis
 Infection
 Trauma
○ Inadequate Eustachian tube ventilation of middle ear with effusion
present.
 Eg. Secondary to nasopharyngeal cancer.
 Sensorineural.
• Results from defects to central area of oval window (sensory) or cochlear
nerve (neural).
○ Occasionally due to pathology of more central pathways.
• Causes include.
○ Ototoxic drugs.
 Streptomycin
 Aminoglycosides.
• Especially gentomycin.
○ Post – infective.
 Meningitis
 Measles
 Mumps
 ‘Flu
 Herpes
 Syphilis
○ Cochlear vascular disease.
○ Meniere’s disease
○ Trauma
○ Presbyacusis.
• Rare causes include.
○ Acoustic neuroma
○ B12 deficiency
○ MS
 ○ Secondary brain CA.

○ Exclude dangerous causes.

 Be especially concerned if deafness is unilateral or asymmetric.
 Acoustic neuroma
 Cholesteatoma
 Effusion from nasopharyngeal carcinoma
○ Definitive management may be required.
 Surgery for perforations or otosclerosis
 Fitting of suitable hearing aids
 Fitting of cochlear implants.
• For profound sensorineural deafness.
• Stimulates residual neural tissue.
• Takes about 2 hours to fit under GA.
• An external device processes sound and transits it across the skin to a
subcutaneous receiver coil.
○ an electrode in the cochlear via round window.
○ Directly stimulates auditory nerve.
• The received signal is not normally sound.
○ Rehabilitation is needed to understand the new signals.
• Benefits include.
○ Improved lip – reading
○ Recognition of environmental sounds.
○ Relieved isolation.

• Sudden hearing loss.

○ Sensorineural.
 Defined as loss of 30 dB in 3 contiguous pure tone frequencies over < 72 hours.
 Incidence of 5 – 20/100000/year.
 Partial or complete spontaneous recovery occurs in 30 – 65%.
 Detailed evaluation reveals underlying disease in 10%
• Noise exposure.
• Gentamicin toxicity.
• Mumps
• Acoustic neuroma
• MS
• Vasculopathy
• TB
 Investigations.
• INR
• TSH
• Blood glucose
• Cholesterol
• ESR
• FBC
• LFT
• Viral titres
• Audiology
 • Evoked response audiometry
• CXR/ MRI
• pANCA
• Lymph node & nasopharyngeal biopsy.
○ Malignancy & TB
 Management.
• Refer promptly to ENT.
• Idiopathic causes often respond well to high – dose steroids and hyperbaric
oxygen.
○ Have to give promptly.
• High dose steroids are no more effective when combined with antivirals
• Intratympanic dexamethasone may have salvage role.
 Prognosis better if.
• Early presentation
• Mild
• Unilateral
• No vertigo.
○ Conductive.
 Cause is almost always identified..
• Infective
• Trauma
• Occlusive
• Fracture.

• Otosclerosis.
○ Prevelence of:
 0.5 – 2% clinically
 10% subclinically.
○ Cause is mainly Autosomal dominant.
 Incomplete penetration
 50% have a family history
 85% are bilateral.
○ Female:Male ratio of 2:1
○ Pathology.
 Vascular spongy bone replaces normal lamellare bone of otic capsule.
• Particularly around oval window which fixes the stapes footplate.
 Symptoms usually appear late in adult life.
 Made worse by pregnancy, menstruation and the menopause.
 There is conductive deafness.
• Hearing often improved by presence of background noise.
○ Complications.
 75% have tinnitus
 Mild and transient vertigo is common.
 10% have Schwartz’s sign.
• Pink tinge to tympanic membrane
○ Audiometry with masked bone conduction shows dip at 2 kHz.
 Cahart’s notch.
 ○ Treatment.
 Fluoride.
• Contraversial
 Hearing aids.
 Surgery.
• Stapedectomy.
• Stapedotomy.
• Replacing adherent stapes with an implant helps in 90% of cases.
• Many prefer surgery to wearing a hearing aid.
• 1 – 4% risk of dead ear.

• Presbyacusis.
○ Age related hearing loss from accumulated environmental noise toxicity.
○ Loss of high – frequency sounds starts before 30 years.
○ Rate of loss is progressive thereafter.
○ Deafness due to loss of hair cells is gradual and often not noticed until it is difficult to hear
speech.
 Constanants at 3 – 4 Hz are needed for speech discrimination.
○ Hearing is most affected in presence of background noise.
○ Hearing aids are the usual treatment.