Ear developmental abnormalities: diagnosis

Instrumental research

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.

Children with a rudimentary auditory canal should be examined for cholesteatoma. Although visualization is difficult, otorrhoea, polyps or pain may be the first signs of the cholesteatoma of the external auditory canal. In all cases of detection of the cholesteatoma of the external auditory canal, the patient is shown surgical treatment.

Currently, to address the issue of surgical reconstruction of the external auditory canal and the implementation of ossiculoplasty, it is recommended to focus on the data of hearing research and CT of the temporal bone.

Detailed data of CT of the temporal bone in assessing the structures of the external, middle and inner ear in children with congenital atresia of the external auditory canal are necessary to assess the technical feasibility of forming the external auditory canal, the prospects of improving the hearing, and the risk assessment of the forthcoming operation. Below are some typical anomalies.

Congenital anomalies of the inner ear can only be confirmed by CT of the temporal bones. The most famous of them is the anomaly of Mondini, the stenosis of the windows of the labyrinth, the stenosis of the internal auditory canal, the anomaly of the semicircular canals until their absence.

Indications for consultation of other specialists

With congenital ear defects, medical genetic research and consultation of the maxillofacial surgeon are shown.

The main task of medical genetic counseling of any hereditary diseases is the diagnosis of syndromes and the establishment of empirical risk. The genetic counselor collects a family history, makes up the medical pedigree of the family of counselors, conducts surveys of probands, siblings, parents and other relatives. Specific genetic studies should include dermatoglyphics, karyotyping, determination of sexual cromatin.

In patients with the syndrome of Tricer-Collins and Goldenenhar, in addition to microtia and atresia of the external auditory canal, the development of the facial skeleton is noted due to hypoplasia of the mandibular branch and temporomandibular joint. Such a patient shows the consultation of the maxillofacial surgeon and orthodontist to decide the need for retraction of the mandibular branch. Correction of congenital underdevelopment of the lower jaw greatly improves the appearance of patients. Thus, in cases when a microtia is detected as a hymn of a volume in the presence of a congenital hereditary pathology of the lin zone in the rehabilitation complex of patients with a microtia, consultations of maxillofacial surgeons should be included.

Differential diagnosis of ear anomalies

Differential diagnosis of congenital anomalies development is difficult only in the case of local malformations of auditory ossicles. Differentiate should be with exudative middle otitis media, posttraumatic rupture of the auditory ossicles, tumors of the middle ear.

[1], [2], [3], [4], [5], [6]