Case 20 Microtia

Kristopher M. Day and Raymond J. Harshbarger

Case 20 Microtia

Case 20 (a, b) A 32 year-old female enters to discuss interest in correcting the deformity of her right ear.

20.1 Description

  • Adult female with grade 3 “lobular type” microtia of the right ear

  • Absence of external auditory meatus suggesting right-sided hearing loss

  • Examination shows small, malformed, hypoplastic residual cartilage with abnormal lobule orientation and aural atresia

20.2 Work-Up

20.2.1 History

  • Hearing loss and previous hearing aid placement

    • Possible use of bone anchored hearing aid (BAHA)

  • Family history of ear abnormalities, facial clefts, congenital hand conditions, or other craniofacial syndromes

    • Visual impairment (e.g., oculoauricovertebral syndrome)

    • Renal disease (e.g., branchio-oto-renal syndrome)

    • Cardiac disease (e.g., CHARGE syndrome)

20.2.2 Physical Examination

  • Classify anomaly

    • Unilateral or bilateral

    • Severity by grade

    • Presence or absence of external ear canal and/or hearing loss

  • Grades of ear hypoplasia (see Fig. 20-1)

    1. Mild hypoplasia: Smaller ear, all elements present

    2. Moderate hypoplasia: Some missing elements; “concha-type,” possibly canal stenosis

    3. Microtia: Small peanut-shaped remnant; “lobule-type,” usually with aural atresia

    4. Anotia: Complete absence of external ear

  • Characteristic associated conditions

    • Hemifacial microsomia: Facial asymmetry

      • Goldenhar: Preauricular skin tags and epibulbar dermoids

    • Treacher Collins syndrome: Malar hypoplasia and micrognathia

    • Nager syndrome: High nasal bridge and thumb hypoplasia

      Fig. 20.1 Grades of ear hypoplasia. (Source: Reinisch J, Tahiri Y, eds. Modern Microtia Reconstruction. In: Derderian CA, Microtia. Switzerland: Springer; 2019:23–41.)

20.2.3 Pertinent Imaging and Diagnostic Studies

  • Audiometric testing

  • Temporal bone and craniomaxillofacial computerized tomography protocols

20.3 Consultations

  • Audiologist

    • Hearing evaluation

    • Commonly have conductive hearing loss > sensorineural hearing loss

      • Inner ear usually intact

      • Middle ear atresia common

  • Otolaryngologist

    • Evaluate for BAHA candidacy:

      • Initial temporary hearing aid worn with head strap prior to ear reconstruction

      • Critical to initiate early for speech development in bilateral microtia

      • Permanent BAHA placed postoperatively to avoid injury to skin envelope

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Jul 17, 2021 | Posted by in General Surgery | Comments Off on Case 20 Microtia
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