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Diagnosis and Classification of Congenital Anomalies of the Internal Auditory Canal: A Literature Review and Clinical Experience in Patient Management

Authors:

Kozhantayeva S.K., Toropchina L.V., Zelikovich E.I., Kurilenkov G.V.

Place of work:

  • Marat Ospanov West Kazakhstan Medical University, Aktobe, Kazakhstan
  • Russian Medical Academy of Continuing Professional Education, Russia
  • Sverzhevsky Clinical Research Institute of Otorhinolaryngology, Russia

    • Reference link :

    Kozhantayeva S.K., Toropchina L.V., Zelikovich E.I., Kurilenkov G.V.. Diagnosis and Classification of Congenital Anomalies of the Internal Auditory Canal: A Literature Review and Clinical Experience in Patient Management.

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    Abstract:This study aims to analyze the results of a comprehensive examination of children with congenital anomalies of the internal auditory canal (IAC) and propose an X-ray-based classification of these pathologies, drawn from our clinical experience.
    Keywords: computed tomography of the temporal bone, classification of congenital malformations, internal auditory canal, cochlear nerve, hearing loss.
    Methods. We analyzed a group of 250 children (500 ears) examined between 2012 and 2024. These children presented with varying degrees of bilateral or unilateral sensorineural, conductive, or mixed hearing loss of unknown etiology. Following audiological examinations, high-resolution computed tomography (CT) of the temporal bones was performed. IAC assessment followed a structured algorithm, evaluating the presence, diameter, shape, state of the IAC floor, and cochlear field, as well as its position relative to surrounding structures. Patients with identified IAC congenital anomalies were referred for 3T magnetic resonance imaging (MRI) of the brain, specifically targeting the auditory nerves.
    Results. Congenital anomalies of the IAC were identified in 63 patients (25.2%), including stenosis, duplication, bulbous expansion, and partial or complete obliteration of the cochlear field. Audiological assessments in these patients indicated that a bone-air-bone gap may be present in some cases of congenital IAC anomalies, and otoacoustic emissions were recorded in others. Based on these findings, a classification system for IAC anomalies was proposed.
    Conclusion. Congenital anomalies of the IAC represent a significant cause of hearing loss, necessitating specific patient management strategies. Audiological characteristics alone are insufficient to localize these anomalies, emphasizing the importance of high-resolution temporal bone CT and, where appropriate, 3T MRI in diagnosing and managing these cases. Early detection of IAC anomalies allows for more targeted rehabilitation and treatment plans for children with congenital hearing loss.

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    Kozhantayeva S.K. - https://orcid.org 0000-0002-0184-2440

    Toropchina L.V. - https://orcid.org 0000-0003-4272-2311

    Zelikovich E.I. - https://orcid.org 0000-0003-1859-0179

    Kurilenkov G.V. - https://orcid.org 0000-0001-7756-2341

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