|Year : 2013 | Volume
| Issue : 2 | Page : 75-78
Congenital cholesteatoma of petrous apex: Rare case report: Diagnostic and management challenge
Arun Dehadaray, Maitri Kaushik, Haris Qadri, Poonam Goyal
Department of ENT, Bharati Vidhyapeet Hospital, Pune, Maharashtra, India
|Date of Web Publication||15-Jun-2013|
Bharathi Hospital, Satara Road, Pune, Maharashtra
Source of Support: None, Conflict of Interest: None
A rare case of congenital cholesteatoma of petrous apex with facial nerve palsy and its successful management is reported. 49 year old female presented with progressive vertigo since 2 years. Patient developed tinnitus and hearing loss in the right ear since 7 months and right sided complete facial asymmetry since 6 months. She had normal right tympanic membrane and complete right lower motor neuron facial nerve palsy. She also had profound sensorineural hearing loss with positive Cerebellar signs. Magnetic resonance imaging and High resolution computed tomography with contrast temporal bone showed extensive bony destruction and petrous apex lesion. Facial nerve and vestibular cochlear nerve was compressed by abnormal soft-tissue present in the internal auditory meatus. Transmastoid translabyrinthine exploration was carried out for petrous apex lesion. Intra-operative extensive bony erosion was noted in the temporal bone. Erosion was extending upto Internal Acoustic Meatus compressing VII and VIII nerve bundle. Post-operatively patient was relieved of vertigo and tinnitus. Though hearing didn't improve, but there was an improvement in facial palsy. Congenital petrous apex cholesteatoma is very rare case. With no specific radiological signs congenital cholesteatoma is difficult to diagnose pre-operatively. It was a challenge to treat surgically such a rare case with extensive neurosurgical presentation without any neurological deficit. Patient showed improvement official nerve after the 1 year of surgery.
Keywords: Congenital cholesteatoma, Petrous apex, Facial nerve palsy
|How to cite this article:|
Dehadaray A, Kaushik M, Qadri H, Goyal P. Congenital cholesteatoma of petrous apex: Rare case report: Diagnostic and management challenge. Indian J Otol 2013;19:75-8
|How to cite this URL:|
Dehadaray A, Kaushik M, Qadri H, Goyal P. Congenital cholesteatoma of petrous apex: Rare case report: Diagnostic and management challenge. Indian J Otol [serial online] 2013 [cited 2019 Jul 17];19:75-8. Available from: http://www.indianjotol.org/text.asp?2013/19/2/75/113505
| Introduction|| |
A rare case of congenital cholesteatoma of petrous apex in 49-year-old female is presented here, who was surgically managed successfully with the post-operated facial nerve improvement.
Congenital cholesteatoma of the temporal bone is a rare disease and involvement of petrous apex is very rare. It has to be distinguished from other lesions of cerebello-pontine angle and petrous apex like cholesterol granuloma, archanoid cyst, mucocele, epidermis cyst and acoustic neuroma etc., Overall incidence of congenital cholesteatoma ranges from 2% to 5% of all cholesteatoma cases. Male predominance of 3:1 is seen.  Congenital cholesteatoma may arise from the embryonic inclusions of the epidermal portion of the epiblasts during the closure of neural tube. Cholesteatoma of the petrous apex originate from epithelial cells from sesells pouch which get encased with the fibrocartilage of the foramen lacerum during cephalic flexure and may give rise to cholesteatoma in later life thus neuroectodermal in origin.  It is well-known that congenital cholesteatoma of petrous apex may occur without otorrhoea and in otherwise normal temporal bone. Facial nerve deficit is the first presentation to occur followed by a unilateral sensorineural hearing loss.  Computed tomography (CT) scanning is an important investigation for various lesions involving the cerebello-pontine angle. Cholesteatoma doesn't have specific radiological sign on CT scan and therefore, very difficult to diagnose. But rarely any case of congenital cholesteatoma of petrous apex has been reported at an age of 49 year in a female patient.  There are several surgical approaches for petrous lesion. The middle cranial fossa approach is preferred by neurosurgeons, and the transmastoid translabyrinthine approach is preferred by otologists.
| Case Report|| |
49-year-old female patient presented with progressive vertigo since 2 years, which worsened over last 8 months. Patient developed tinnitus and hearing loss in the right ear since 7 months. She noticed right sided facial asymmetry 6 months back, which progressed to complete facial nerve palsy. At other center patient was initially diagnosed and treated as Bell's palsy. From there she was referred to neurosurgeon in Bharati hospital as CT scan showed cerebello-Pontine angle lesion. During neurosurgical exploration neurosurgeon detected cholesteatoma at very early stage involving mastoid, thus, case was with held and referred to Ear Nose Throat Department. Complete Ear Nose Throat evaluation revealed normal right tympanic membrane, complete right lower motor neuron facial nerve palsy and profound sensorineural hearing loss in the right ear on pure tone audiogram [Figure 1]. Cerebellar signs were positive. Magnetic resonance imaging (MRI) and high resolution CT with contrast temporal bone was advised to see the extent of bony involvement in the temporal bone and skull base. This showed 4.5 × 2.3 × 4.6 a bony destroying (lytic lesion) lesion involving mastoid, posterior external auditory canal, bony labyrinth, petrous apex, and internal auditory meatus [Figure 2]. Facial nerve and vestibular cochlear nerve was compressed by abnormal soft tissue present in the internal auditory meatus. Patient was explored transmastoid translabyrinthine for petrous apex lesion. Intra-operative extensive bony erosion by cholesteatoma was noted in the temporal bone as destruction of posterior external auditory canal, lateral cortex of mastoid, mastoid air cells, bony labyrinth and internal auditory meatus. Due to extensive bony destruction sigmoid sinus, posterior fossa dura, jugular bulb area and middle cranial fossa dura were exposed. There was erosion of Internal Acoustic Meatus compressing VII and VIII nerve bundle. Internal Acoustic Meatus was completely cleared of cholesteatoma, thus decompressing VII and VIII nerve bundle [Figure 3]. Cholesteatoma was extending beyond Internal Acoustic Meatus towards petrous apex which was carefully and pains takingly removed. Inferiorly cholesteatoma was extending towards jugular bulbar engulfing lower IX, X, XI nerves. All cholesteatoma was cleared carefully preserving continuity of nerve bundles. Cholesteatoma from dura over arcuate eminence was removed. All the efforts were taken towards complete surgical removal of cholesteatoma. Cavity obliteration was not done. Post-operatively patient was relieved of vertigo and tinnitus. Facial paralysis and hearing didn't improve and was same as pre-operative. Histopathology was suggestive of cholesteatoma.
|Figure 3: Petrous apex, posterior fossa dura, VII nerve bundle, lower cranial nerve bundle and jugular bulb intraoperatively|
Click here to view
Repeat check CT scan is planned after 6 months.
| Discussion|| |
Congenital cholesteatoma of petrous apex is rare. Cholesteatoma of petrous apex has two different origins, congenital, and acquired. Congenital cholesteatoma is believed to arise from squamous cell rest.  In fetal life there is a close relation of tympanic ring with internal auditory canal that makes migration of external canal ectoderm possible. 83-94% presents with sensorineural hearing loss and 20-25% presents with lower motor neuron facial paralysis. They are destructive in nature. It has been always a challenge to diagnose congenital cholesteatoma on radiological investigations also. Congenital cholesteatoma is generally not distinguish able from cholesterol granuloma and mucocele on CT as well as T1 and T2 weighted MRI images even with gadolinium. [Table 1] depicts imaging features of petrous apex lesions.  Thus to diagnose congenital cholesteatoma pre-operatively is a challenge, as in this case it was intra-operative diagnosis. 
Transmastoid Translabyrinthine approach with or without transcochlear approach is the basic surgery for removal of petrous apex cholesteatoma. However these approach are insufficient to remove cholesteatoma situated deep in petrous apex and adherent to middle cranial fossa dura so middle cranial fossa approach is advised for such cases.  In our case we could remove whole disease on middle cranial fossa dura by transmastoid translabyrinthine approach. This was achieved by removing superior semicircular canal which corresponds to arcuate eminence. Preservation of cochlear and vestibular function while eradicating extensive cholesteatoma in petrous apex is not considered. Vertigo in our case pre-operatively was because of compression over eight nerve which got relieved after surgery. Vertigo in congenital cholesteatoma is because of compression over eighth nerve in contrary to vertigo from acquired cholesteatoma which is because of involvement of labyrinth. When facial nerve is damaged in extensive cholesteatoma of petrous apex, literature suggests even sacrificing official nerve which has residual function for complete eradication.  Authors suggest preservation of continuity of nerve in extensive cholesteatoma even if facial nerve is completely paralysed as the literature suggests some improvement official nerve function post-operatively. Thus it was the challenge for the surgeon to eradicate such extensive disease extending to middle cranial fossa dura, posterior fossa dura, cerebello-pontine angle, petrous apex, internal auditory meatus, skull base while maintaining continuity of VII, VIII, IX, X, XI cranial nerves. Post-operatively all lower cranial nerve function was normal. Obliteration of the cavity is not recommended as there area chance of residual cholesteatoma and its possible complications like rupture of dura, internal carotid artery and jugular bulb. In recurrence it becomes difficult to assess cholesteatoma in obliteration cavity by CT and MRI. 
Regular follow-up for facial nerve improvement and 6 monthly MRI is planned for residual cholesteatoma.
| Conclusion|| |
Congenital petrous apex cholesteatoma is very rare case. With no specify radiological signs congenital cholesteatoma is difficult to diagnose pre-operative. It was a challenge to treat surgically such a rare case with extensive neurosurgical presentation without any neurological deficit. Patient showed improvement of facial nerve after 1½ year of surgery [Figure 4].
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]