Neurology International 2018; volume 10:7625 A giant cholesteatoma of the mastoid extending into Introduction Correspondence: Yuekang Zhang, Department of Neurosurgery, Post Graduate Training the foramen magnum: Cholesteatomas are benign, slowly pro- Centre, West China Hospital, Sichuan gressive lesions that are usually found in the University; 37 Guo Xue Xiang Road, A case report and review 1-5 temporal bone. They are epidermoid Chengdu, Sichuan 610041, P.R. China. of literature cysts of embryologic origin that result in Tel.: +86.18980601975 - Fax: +86.02885422490. progressive desquamation and trapping of E-mail: [email protected] Seidu A. Richard,1-3 Li Qiang,1 squamous epithelium behind an intact tym- panic membrane.6 They can be congenital Key words: Cholesteatoma, Case report, Zhi Gang Lan,1 Yuekang Zhang,1 or acquired.1,4,5 They can be classified into Dizziness, Epidermoid Cyst, Hearing loss, Chao You1 supralabyrinthine, infralabyrinthine, mas- Tinnitus. 1Department of Neurosurgery, West sive labyrinthine, infralabyrinthine-apical, Contributions: SAR conceived the project and and apical.1,4 They have the penchant for China Hospital, Sichuan University, designed the study; SAR, LQ and ZQL col- 2 China; Department of Immunology, the petrous apex, skull base, and internal lected patient’s data; YZ and CY provided Jiangsu University, China; 3Department auditory canal. Also, these lesions may technical assistance in the study; SAR pre- of Surgery, Volta Regional Hospital, Ho, infiltrate other vital soft tissue structures pared the illustration, analyzed data and wrote such as the sigmoid sinus, jugular vein and Ghana the paper. artery, and the cerebellopontine angle.1,4,5 Symptoms associated with cholesteatomas Conflict of interest: the authors declare no includes vertigo, tinnitus, otorrhea, and potential conflict of interest. otalgia.1,7 To the best of our understanding Abstract no data has reported cholesteatoma that Funding: none. extends from the temporal bone into the Cholesteatomas are very rare benign, Received for publication: 31 January 2018. foramen magnum. We therefore present a progressive lesions that have embryologic Accepted for publication: 4 February 2018. case of cholesteatoma extending down into only derivation and usually result in progressive the foramen magnum and review of litera- This work is licensed under a Creative exfoliation and confinement of squamous ture on the pathogenesis of cholesteatoma. Commons Attribution NonCommercial 4.0 epithelium behind an intact or preciously License (CC BY-NC 4.0). infected tympanic membrane. To the best of use our understanding no reports demonstrates ©Copyright S.A. Richard et al., 2018 the extension of cholesteatoma from the Case Report Licensee PAGEPress, Italy Neurology International 2018; 10:7625 temporal bone into the foramen magnum. We present a case of 67-year-old man doi:10.4081/ni.2018.7625 We therefore present a case of with recurrent tinnitus in the left ear and cholesteatoma extending down into the dizziness for over one-year duration. He foramen magnum. We report a case of 67- had mild unilateral hearing loss, no otor- year-old man with a giant cholesteatoma rhea, vertigo, pain, cognitive impairment, with mild obstructive hydrocephalus. Left extending into the foramen magnum with- vomiting, facial numbness, hypoesthesia, frontal white matter showed a spotty lesion out substantial destruction of the mastoid blurred vision, choking and movement dis- that is hypo-intense on T1 and Hyper- and petrous temporal bones. The patient’s orders. Past history of ear infection was intense on T2 which could be the beginning major symptoms were recurrent tinnitus in unremarkable. All other systems were unre- of mild degenerative process. There were the left ear and dizziness with unilateral markable. There was no family history of no abnormalities on the temporal bone and conductive hearing loss. A working diagno- such disease. He did not have any ear sur- for that matter the whole skull. A working sis of cholesteatomas was made combining geries prior to the presentation. On exami- diagnosis epidermoid or arachnoid cyst was the symptoms and magnetic resonance nation, there were no obvious deformities made combining the symptoms and MRI imaging findings. He was then successfullyNon-commercialaround the ears. Otoscopy revealed an intact findings (Figure 1). operated on with very minimal postopera- tympanic membrane with no visible pathol- After general anesthesia, the patient tive complications. Cholesteatomas origi- ogy in the mesotympanum. Examination of was put in the park-bench position. Routing use of inferior cranial nerves monitoring nating from the mastoid bone often linger cranial nerves was unremarkable. such as electromyographic (EMG) and with the patients for many years in a sub- Audiometry demonstrated total hearing loss auditory brainstem responses (ABRs) was clinical state and progress into a massive with reduced middle ear compliance in the left ear. All laboratory investigations as well carried out to maximize the preservation of size before causing symptoms. Patients with as other ancillary investigations (CXR, the cranial nerves. We used the suboccipital unilateral conductive hearing loss who are ECG etc) were normal. paramedian approach. A linear suboccipital otherwise asymptomatic and have a normal Pre-operative MRI done showed a paramedian incision was made and extend- tympanic membrane should be suspected lesion that is hypo-intense on T1, hyper- ed down to the occipital hairline. A high- with a progressive cholesteatoma. intense on T2 and hypointensive on FLAIR speed miller was used to perform a suboc- Cholesteatoma should be one of the work- and showed no enhancement with gadolini- cipital paramedian craniotomy. ing diagnosis when an elderly patient pres- um administration. The lesion was about Additionally, posterior rim of foramen mag- ent with unilateral conductive hearing loss 3.0×2.5×2.5 cm in diameter. The lesion dis- num and the lamina of C1 were also that is associated with tinnitus and dizzi- placed the medulla oblongata to the right removed to gain wider exposure of the ness. side and compressed the bilateral cerebel- lesion. After placing several tack-up stitches lum hemispheres. The third, fourth and to the dura, the lesion was observed lying bilateral ventricles are slightly enlarged between jugular foramen and the foramen [page 28] [Neurology International 2018; 10:7625] Case Report magnum. The lesion was about 3.0×2.5×2.5 pathogenesis which includes: i) the exis- acquired cholesteatoma still remains to be cm in diameter. The microscope was used tence of an ectopic epidermis rest, ii) in- distinctly clarified, it is believed to be mul- throughout the operation. The lesion was growth of meatal epidermis, iii) metaplasia tifactorial, as many theories have been rec- completely suctioned out without any cra- following infection/inflammation, and fas- ommended and researched on.13 It has been nial nerves dysfunction. Post-operative CT- cinatingly, iv) reflux of amniotic fluid con- demonstrated that iatrogenic or non-iatro- scan done confirmed total resection of the taining squamous epithelium in utero into genic tympanic membrane trauma such as lesions with very slight edema around the the middle ear. Nonetheless, the epithelial perforation, displacement, retraction or brain stem (Figure 2). Samples of the lesion rest theory is generally accepted.13 invagination, tympanic membrane disease, are as shown in (Figure 3). Pathology report Congenital cholesteatomas in the middle tympanic cavity mucosa disease, ear infec- confirmed cholesteatoma or epidermoid ear frequently present with cunning hearing tion, and Eustachian tube dysfunction are cyst (Figure 4). The patient had a good loss during childhood years and are usually likely to initiate acquired cholesteatoma recovery and discharge home on the seventh found incidentally behind the tympanic pathogenesis.13,15 The pathogenesis of day after operation. Hearing improved membrane during routine otoscopy.2,14 acquired aural cholesteatoma has also been markedly. A scheduled review was arranged Nevertheless, congenital cholesteatomas explained by four basic theories: i) invagi- on the third month after operation. One and may be absolutely asymptomatic, particu- nation of the tympanic membrane (retrac- half year follow-up show no recurrence of larly when their origin is from the mastoid tion pocket cholesteatoma), ii) basal cell the lesion. where the diagnosis occurs only as an inci- hyperplasia, iii) epithelial in-growth dental finding on imaging hence the diagno- through perforation (the migration theory), sis is often belated until mid to late adult- and iv) squamous metaplasia of middle ear hood because of the asymptomatic nature of epithelium.13 The most widely accepted Discussion the disease.2,14 In such cases, the most com- pathogenesis of acquired cholesteatoma is mon symptoms are dizziness, pain, or the theory that negative pressure causes a Cholesteatomas or epidermoid cysts swelling that may be localized to the tempo- deepening retraction pocket that, when originate from progressive desquamation of ral area of the head or to the neck.2,14 obstructed, desquamated keratin cannot be the epithelium.1,2,4,5,7 They are classified Even though the pathophysiology of the cleared from the recess, and cholesteatoma into congenital or acquired base on their only pathogenesis.1,4,5 In our case, it was very difficult to arrive at a definitive conclusion on whether the lesion is congenital