AIP0STASDS AUIRBCULD
® Deformity of shape, cosmetic defect ® Therapy: plastic correction - otoplasty ® Children from the age of 6 years
A OF EAC
® Congenital defect of auricle development (microtia) or missing auricle (anotia) is often combined with the congenital defect of the EAC (stenosis, atresia)
® Auditory canal stenosis means that it is narrower than 4 mm
® Dg: CT, objective hearing examination (exclusion of congenital defect of the middle and inner ear or the auditory track)
® Conductive hearing loss
MICROTIA (
A»TIA
Grade 1
Smaller than normal, but the ear has mostly normal anatomy
Grade 2
Part of the ear looks normal, usually the lower half
The canaJ may be normal, small or completely closed
Grade 3
Just a small remnant of "peanut-shaped" skin and cartilage
There is no canal, which is called aural atresia
Grade 4
Complete absence of both the external ear and the ear canal, also called "anotia"
ATRESIA OF EAC
® It depends on examination results and on hearing affliction extent (unilateral or bilateral affliction)
®Aim: provide communication
® Hearingaid devices (BAHA)
® Surgery: tympanoplasty, plastic of external auditory canal or auricle
FDSTyiA AiMIS C0MSEIHIQTAL
® External opening is placed near the tragus and the inner opening between cartilaginous and bone part of the EAC
©Complication - inflammation (secretion, swelling or erythema)
®Th: Exstirpation,
ATB (inflammation)
STOUCTy^Al ANOMALIES ©F MMLE EA[R
® Usually connected with atresia of EAC and anomalies of auricle
® Isolated / part of syndroms (more common, e.g. Treacher Collins)
® Usually unilateral
® Hearing-impairment
® Dg: CT, objective hearing examination
®Th: hearing-aid devices, tympanoplasty
STOUCTy^Al ANOMALIES ©F mme^ emi
®20% of children with SNHL (sensorineural hearing loss) have CT anomalies of inner ear
® Cochlear anomalies
® Enlarged vestibular aqueduct (EVA)
® Semicircular canal dysplasia
COCHLEAR ANOMALIES
©Michel deformity or complete labyrinthine aplasia (cochlea + vestibulum)
® Cochlear aplasia
® Common cavity malformation to the
cochlea and vestibule
® Cochlear hypoplasia
©Cochlear incomplete partition type I
(including cystic cochleovestibular anomaly)
©Cochlear incomplete partition type II (Mondini dysplasia)
COCHLEAR APLASIA
>
DEFECTS ©F MEMM
> 60% congenital
• Damage of auditory organ during development (1. trimester, prenatal period)
• Genetic hearing defects
Non-syndromic 70%
80% autosomally recessive (Connexin 26 - protein)
19% autosomally dominant
1 -2% X chromozom Syndromic 30%
• Infection: Rubeola, CMV, toxoplasmosis...
>40% gained
• Perinatal period - premature, asphyxia...
• Postnatal period - meningitis, injury...
sy»gi©ms e®«ecteo WTU
amqmaues ®r the ea^l
Anomaly of external + middle ® Treacher-collins syndrome
Anomaly of inner ear
® Pendred' s syndrome: eva + Mondini dysplasia,
affiction of thyroid gland, 2. most common
® Usher' S Syndrome: partial or total hearing loss and vision loss that worsens over time
® Waardenburg's syndrome: unilateral or bilateral SNHL, pigmentation changes
® Alport's syndrome: progressive SNHL, affliction of
kidneys
©Q)N(3EGiTAl PEFECTS ©F
AUPOTO^Y MEm
® Auditory neuropathy
® Aplasia or hypoplasia of auditory
® Demyelinization disease
OMtPAOGIMEMT ((MEWiSMS])
® Newborn hearing screening ® Otoacoustic emissions (OAE)
® Otoscopy, tympanometry
® Examination of evoked potentials (BERA Brainstem Evoked Response Audiometry)
®CT / MRI, genetic examination