Developmental abnormalities of the ear

In everyday practice, a doctor of any specialty no more often than not has to deal with congenital anomalies in the development of certain organs. Both functional and cosmetic aspects require attention. As a rule, anomalies are accompanied by significant impairments in the function of the auditory analyzer, which plays a major role in the formation of the child's speech and his psychosomatic development in general, and in case of bilateral pathology leads to disability. It is important to be well aware of the various variations of defects, since in many cases multi-stage interventions by several specialists may be required.

Externally, ear malformations are quite variable: from enlargement of the auricle or its individual elements (macrotia) to complete absence of the auricle (microtia, anotia); additional formations in the parotid region (ear appendages, parotid fistulas) or abnormal position of the auricle. An angle of 90 degrees between the auricle and the lateral surface of the head (lop-earedness) is also considered abnormal.

Developmental anomalies of the external auditory canal (atresia or stenosis), auditory ossicles, labyrinth - a more severe congenital defect with hearing impairment.

ICD-10 code

• Q16 Congenital anomalies (malformations) of the ear causing CSC hearing impairment.
• Q17 Other congenital anomalies (malformations) of the ear.
• A distinction is made between malformations of the outer, middle and inner ear: microtia (Q17.2), macrotia (Q17.1), protruding ear or lop-earedness (Q17.5), accessory auricle (Q17.0), parotid fistulas, stenosis and atresia of the external auditory canal (Q16. I), local malformations of the auditory ossicles (Q16.3), anomalies of the labyrinth and internal auditory canal (Q16.9).

Epidemiology of ear malformations

According to the combined data of domestic and foreign authors, congenital defects of the organ of hearing are observed in approximately 1 out of 7000-15000 newborns, more often on the right side. Boys suffer on average 2-2.5 times more often than girls.

Causes of ear developmental abnormalities

Most cases of ear malformations are sporadic, but about 15% are hereditary.

Symptoms of ear developmental abnormalities

The most common congenital malformations of the hearing organ are observed in Konigsmark, Goldenhar, Treacher-Collins, Mobius, and Nager syndromes.

In Konigsmark syndrome, microtia, atresia of the external auditory canal, and conductive hearing loss are observed. The outer ear is represented by a vertically located skin-cartilaginous ridge without an external auditory canal, the face is symmetrical, and there are no developmental defects of other organs.

Audiometry reveals conductive hearing loss of grades III-IV. Inheritance of Konigsmark syndrome occurs in an autosomal recessive manner.

Ear Development Anomalies - Symptoms

Diagnosis of ear developmental anomalies

According to most authors, the first thing an otolaryngologist should do when a child with an ear anomaly is born is to evaluate the hearing function. Objective hearing methods are used to examine young children - determining thresholds using short-latency SEP and OAE recording methods; conducting acoustic impedance analysis. In patients over 4 years of age, hearing acuity is determined by the intelligibility of spoken and whispered speech, as well as by tonal threshold audiometry. Even with a unilateral anomaly in an apparently healthy second ear, the absence of hearing impairment must be proven.

Microtia is usually accompanied by conductive hearing loss of grade III (60-70 dB). However, lesser or greater degrees of conductive and sensorineural hearing loss may be observed.

Ear Development Anomalies - Diagnostics

Treatment of ear developmental anomalies

In bilateral conductive hearing loss, normal speech development of the child is facilitated by wearing a hearing aid with a bone vibrator. Where there is an external auditory canal, a standard hearing aid can be used.

A child with microtia has the same chance of developing otitis media as a healthy child, since the mucous membrane from the nasopharynx continues into the auditory tube, middle ear, and mastoid process. There are known cases of mastoiditis in children with microtia and atresia of the external auditory canal (surgical treatment is necessary).

Ear Development Anomalies - Treatment

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