Case #205675

Sex: 
Female
Race: 
White, not Hispanic
Age in Years : 
0.02
Post Mortem Time: 
15.00
Location: 
Massachusetts Eye & Ear Infirmary, Boston, MA
Cause of Death: 
Cardiorespiratory failure in association with CHARGE association
Right Tray: 
1575
Left Tray: 
1576
Right Cut Angle: 
Horizontal
Left Cut Angle: 
Horizontal
Days in Fixative: 
89
Unstained Right: 
324
Unstained Left: 
324
Fixative: 
10% Formalin
Decalcification Agent: 
EDTA
Days of Decalcification: 
84
Surgical Specimen: 
No
Audiogram: 
No
Vestibular Testing: 
No
Cytocochleogram: 
No
Otologic Diagnosis: 
1. CHARGE association 2. Mondini dysplasia, cochlea, bilateral 3. Oval window, aplasia, bilateral 4. Round window, aplasia, bilateral 5. Utricular macula, aplasia, bilateral 6. Posterior and lateral semicircular canals, aplasia, bilateral 7. Superior semicircular canal, aplasia, left 8. Superior semicircular canal, cupula, dysplastic, right 9. Endolymphatic sac, hypoplastic, bilateral 10. Facial nerve, anomalous course, bilateral 11. Artifact, removal, amputation, external canal, middle ear and mastoid, bilateral
Histopathology: 
Right ear. This specimen is in a good state of postmortem preservation and histologic preparation. The external auditory canal, middle ear, eustachian tube, carotid canal and the mastoid are missing from the specimen due to artifact of removal. The entire inner ear is present. The cochlea is short and consists of 1.5 to 2 turns. The organ of Corti, stria vascularis, spiral ligament, and cochlear neurons are present and appear normal. There is no endolymphatic hydrops. There is a bony expansion of Rosenthal's canal in the lower basal turn which communicates with the scala tympani and which appears to be filled with perilymph. In the vestibular system, the saccule is somewhat dilated. Hair cells are present in the saccular macula. The utricle is hypoplastic and its macula has not developed. The cupula of the superior semicircular canal is dysplastic: it is represented by a basophilic-staining structure that is encapsulated. The lateral and posterior semicircular canals have not developed. The endolymphatic sac is hypoplastic. The footplate has not differentiated from the otic capsule. The fissula ante-fenestram and the fossula post-fenestram are both present. The round window membrane niche has not developed; its location is represented by an area of mesenchyme that remains separated from the middle ear air space by bone of the promontory. The internal auditory canal contains the facial, cochlear and vestibular nerves. The facial nerve trunk appears to be of normal size in the labyrinthine segment. The trunk then appears to split into two main divisions with the bulk of fibers leaving in the direction of the greater superficial petrosal nerve. The tympanic segment of the nerve courses over the promontory and its diameter is smaller than average. The nerve becomes progressively thinner as it courses through the temporal bone. The geniculate ganglion is not present in its usual location; instead, ganglion cells are interspersed amongst nerve fibers in the tympanic and mastoid segments. Left ear. This specimen is in a good state of postmortem preservation and histologic preparation. The external auditory canal, middle ear and mastoid, eustachian tube area and carotid canal are missing due to artifact of removal. The pathology in the inner ear is similar to the opposite side, but the changes are somewhat more severe. The cochlea is short and consists of 1.5 turns. Hair cells are present throughout. The number of cochlear neurons is significantly less than normal. There is no endolymphatic hydrops. The vestibular system shows the saccule with a macula. However, the utricle and semicircular canals have not developed. There is an inferior extension of the membranous labyrinth which is connected to the saccule, and this extension appears to be either a large endolymphatic duct or a hypoplastic inferior utricular recess. The endolymphatic sac is hypoplastic. The footplate has not differentiated. The fissula ante-fenestram and the fossula post-fenestram are both present. The area of the round window niche is represented by mesenchymal tissue that remains separated from the middle ear by bone. A small focus of cartilage and bone is embedded within this mesenchymal tissue. The internal auditory canal contains the cochlear, vestibular and facial nerves. Scarpa's ganglion cells are present. The labyrinthine segment of the facial nerve appears to be of average size. The nerve branches into two main divisions at the end of the labyrinthine segment with the bulk of the nerve following the course of the greater superficial petrosal nerve. The tympanic segment of the nerve consists of two or three small branches that are not covered by bone as they course over the promontory.

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