Ear developmental abnormalities: symptoms

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.

In Goldenhar syndrome (oculoauriculovertebral dysplasia, hemifascial microtemia), microtia, macrostomy, development of the mandibular branch, vertebral anomaly in the cervical and thoracic spine (occipitation, wedge vertebrae, cervical synostosis, super-vertebral vertebrae) are observed. Of the other anomalies, there are spinal bulb dermoid, lipodermoid, parotid glands, congenital heart disease (45% of cases), congenital cleft lip and palate (7%), most cases are considered sporadic, however autosomal dominant, autosomal recessive and multifactorial inheritance types occur. Manifestations of congenital malformations of the ear vary from complete aplasia to moderate deformation of the auricle, shifted forward and downward. Ear pendants are located from the tragus to the corner of the mouth. With audiometry, conductive hearing loss of III-IV degree is revealed. In 40-50% of cases, hypoplasia of the canal of the facial nerve is observed.

With the syndrome of Tricer-Collins (mandibularfacial dysostosis), an antimonogoloid cut of the eye gaps, micrognathia, macroglossia, and microtia are observed. Other manifestations of vice are hypoplasia of the thumbs and wrists, the coloboma of the lower eyelid. Dystopia of the canal of the facial nerve is detected more often than with other syndromes. Type of inheritance is autosomal recessive. Audiologically, with pronounced manifestations of the syndrome, IV degree of conductive hearing loss is more often diagnosed.

Classification of ear anomalies

The existing classifications of congenital malformations of the hearing organ are constructed according to clinical, etiological and pathogenetic features.

S.N. Lapchenko identified local vices, hypogenesis of the hearing organ (light, medium, severe severity), dysgenesis of the hearing organ (light, medium, severe), and mixed forms.

The classification of R. Tanser includes five degrees of vices.

• I degree - anotia.
• II degree - complete hypoplasia (microtia):
  • A - with an atresia of the external auditory canal;
  • B - without atresia of the external auditory canal.
• III degree - hypoplasia of the middle part of the auricle.
• IV degree - hypoplasia of the upper part of the auricle;
  • A - a collapsed ear;
  • В - ingrown ear;
  • C - complete hypoplasia of the upper third of the auricle.
• V degree - lop-ears.

N.R. Schuknecht in his classification of auditory atresia divides into types.

• Type A - atresia in the cartilaginous part of the ear canal (shown meatoplasty to prevent the development of cholesteatoma).
• Type B - atresia, both in the cartilaginous and in the bone part of the ear canal, register a decrease in hearing of the II-III degree (surgical rehabilitation is not always successful).
• Type C - all cases of complete atresia.
• Type D - complete atresia of the ear canal with weak pneumatization of the temporal bone, accompanied by an abnormal arrangement of the canal of the facial nerve and the capsule of the labyrinth (the performance of auditory improvement is contraindicated).

Despite the variety of classifications presented, most otorhinolaryngologists prefer the classification of Marx, according to which four degrees of deformation of the external and middle ear are distinguished.

• I degree - a hypoplasia of an auricle (separate elements of an auricle are unrecognizable).
• II degree - deformations of the auricle of various degrees (some of the elements of the auricle do not differentiate).
• III degree - ears in the form of a small rudiment.
• IV degree - absence of auricle.

Starting with the second degree, microtia is accompanied by an anomaly of development of the external auditory canal.

[1], [2], [3], [4]