Presentation
Bilateral conductive hearing loss since birth with recurrent right otitis media.
Patient Data
Right ear:
anotia: grade 4 (Marx classification 1); Absence of auricle.
external auditory canal atresia: Atresia bony plate at the medial part (type B Weerda classification). Expanded external auditory canal with soft tissue seen within.
middle ear/tympanic cavity is normal in size; Complete opacification of tympanic cavity. Soft tissue may represent cerumen/wax.
ossicles anomalies: moderate dysplastic malleus and incus (absence of manubrium of malleus and long process of incus adhered to the posterior wall; normal stapes is not visualized at the oval window
mastoid pneumatization: absent (underpneumatization).
facial nerve course: abnormal course with anterior displacement of mastoid segment. Tympanic and mastoid segments are small in caliber. Tympanic segment is eroded.
oval and round windows: oval window is atretic. Normal round window.
vascular anomalies: no aberrant internal carotid artery; no high riding jugular bulb
Inner ear: normal.
Left ear:
microtia: grade 2 (Marx classification 1); Some normal features are present but upper ear is severely deficient.
external auditory canal :Stenotic. Tympanic membrane is thickened.
middle ear/tympanic cavity is normal in size.
ossicles anomalies: Absence of stapes and long process of incus.
mastoid pneumatization: Reduced.
facial nerve course: abnormal course with anterior displacement of mastoid segment (same level as round window)
oval and round windows: Oval window is atretic. Round window is open
vascular anomalies: no aberrant internal carotid artery; no high riding jugular bulb
Inner ear: normal
Right auricle - anotia: grade 4 (Marx classification 1); Absence of auricle.
Left auricle - microtia: grade 2 (Marx classification 1); Some normal features are present but the upper ear is severely deficient.
Case Discussion
This patient has had right external auditory canal atresia (type B Weerda classification with atretic bony plate at medial part) and left external auditory canal stenosis. He has had conductive hearing loss since birth. This HRCT temporal bone performed is for pre-surgical planning.
The Yeakley and Jahrsdoerfer 2 grading system is useful for the pre-operative HRCT temporal bone to decide the operation's outcome. This grading system is based on nine high-resolution CT scan parameters:
normal stapes
open oval window
middle ear space
facial nerve position
malleus-incus complex
mastoid pneumatization
incus-stapes connection
open round window
external ear appearance (external auditory canal)
Each parameter is assigned 1 point (if normal or mildly dysplastic), except for the stapes which was allocated 2 points. Total 10 points.
Points (5 or below) - not a surgical candidate.
8 points and above - good surgical outcome.
7 points - fair outcome.
6 points - marginal improvement.
Our patient scored
- 2 points for the right ear (middle ear space and open round window)
- 3 points for the left ear (middle ear space, open round window, malleus-incus complex).
Obviously, the scores are not optimal for surgical treatment such as atresioplasty for bilateral ears. Patient underwent right tympanomastoidectomy for the recurrent ear infection.Soft tissue within the atretic right external auditory canal and middle ear cavity prove to be congenital cholesteatoma histopathologically from the surgery.
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