Ear anomalies
Last reviewed: 23.04.2024
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In everyday practice, the doctor of any specialty is not more often confronted with congenital anomalies in the development of certain organs. Attention requires both functional and cosmetic aspects. As a rule, anomalies are accompanied by significant disturbances in the function of the auditory analyzer, which plays a big role in the formation of the child's speech and its psychosomatic development as a whole, with bilateral pathology leading to disability. It is important to know well the various variations of vices, since in many cases multi-step interventions of several specialists may be required.
Externally, the developmental defects of the ear are very variable: from an increase in the auricle or its individual elements (macro-cells) to the complete absence of an auricle (microtia, anotia); additional formations in the parotid region (ear pendants, parotid fistula) or an incorrect position of the auricle. Abnormal is also considered the angle of 90 degrees between the auricle and the lateral surface of the head (lop-ears).
Anomalies in the development of the external auditory canal (atresia or stenosis), auditory ossicles, labyrinth - a more severe congenital malformation with hearing impairment.
ICD-10 code
- Q16 Congenital malformations (malformations) of the ear, cause COC hearing loss.
- Q17 Other congenital malformations (malformations) of the ear.
- There are malformations of the outer, middle and inner ear: a microtia (Q17.2), a macro (Q17.1), protruding ear, or a lop-ears (Q17.5), an additional auricle (Q17.0). Parotid fistulas, stenosis and atresia of the external auditory canal (Q16. I), local defects of auditory ossicles (Q16.3), labyrinth anomalies and internal auditory meatus (Q16.9).
Epidemiology of ear anomalies
According to the combined data of domestic and foreign authors, congenital malformations of the hearing organ are observed in about 1 in 7000-15000 newborns, more often in right-sided localization. Boys suffer and average 2-2.5 times more often than girls.
Causes of ear anomalies
Most cases of malformations of the ear are sporadic, but about 15% is hereditary.
Symptoms of ear anomalies
The most common congenital malformations of the hearing organ are observed in the syndromes of Konigsmark, Goldenhar, Tricher-Collins, Moebius, Nager.
In the syndrome of Konigsmark note microtia, atresia of the external auditory canal, conductive hearing loss. The outer ear is represented by the vertically calculating skin-cartilaginous roller without an external auditory meatus, the face is symmetrical, there are no malformations of other organs.
With audiometry, conductive hearing loss of III-IV degree is revealed. The inheritance of the Konigsmark syndrome occurs in an autosomal recessive type.
Diagnosis of ear anomalies
According to most authors, the first thing that an otolaryngologist should do when a child is born with an ear anomaly is to assess the auditory function. Lengthy surveys of young children use objective methods of hearing research - the detection of thresholds by methods of registration of short-latency SVP and UAE; conducting acoustic impedance measurements. In patients older than 4 years, the severity of hearing is determined by the intelligibility of perception of spoken and whispered speech, as well as by tone threshold audiometry. Even with a one-sided anomaly, I externally healthy second ear, the absence of a violation of the auditory function should be proven.
Microtia is usually accompanied by conductive hearing loss of the third degree (60-70 dB). However, less or more degrees of conductive and sensorineural hearing loss can be observed.
Anomalies of ear development - Diagnosis
Treatment of anomalies of ear development
When bilateral kanduktivnoy hearing loss to normal speech development of the child helps to wear a hearing aid with a bone vibrator. Where there is an external auditory meatus, a standard hearing aid can be used.
A child with a microtia has the same probability of developing otitis media as in a healthy child, since the nasopharyngeal mucosa continues into the auditory tube, the middle ear and the mastoid process. There are cases of mastoiditis in children with a microtia and atresia of the external auditory canal (surgical treatment is necessary).
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