Journal of Postgraduate Medicine
 Open access journal indexed with Index Medicus & ISI's SCI  
Users online: 1503  
Home | Subscribe | Feedback | Login 
About Latest Articles Back-Issues Articlesmenu-bullet Search Instructions Online Submission Subscribe Etcetera Contact
 :: Next article
 :: Previous article 
 :: Table of Contents
 ::  Similar in PUBMED
 ::  Search Pubmed for
 ::  Search in Google Scholar for
 ::Related articles
 ::  [PDF Not available] *
 ::  Citation Manager
 ::  Access Statistics
 ::  Reader Comments
 ::  Email Alert *
 ::  Add to My List *
* Registration required (free) 

  IN THIS Article
 ::  Abstract
 ::  Introduction
 ::  Conclusion
 ::  References
 ::  Article Figures

 Article Access Statistics
    PDF Downloaded0    
    Comments [Add]    
    Cited by others 1    

Recommend this journal


Year : 1989  |  Volume : 35  |  Issue : 2  |  Page : 93-7

Profile of congenital cholesteatomas of the petrous apex.

Correspondence Address:
C E DeSouza

Login to access the Email id

Source of Support: None, Conflict of Interest: None

PMID: 0002695622

Rights and PermissionsRights and Permissions

 :: Abstract 

Congenital cholesteatomas of the petrous apex are now frequently being approached by otologists. Involvement of the cerebello pontine angle by this lesion produces a myriad of signs and symptoms. Otological as well as neurosurgical literature is reviewed to achieve an overall understanding of the nature and behaviour of this uncommon but interesting lesion.

Keywords: Bone Diseases, congenital,epidemiology,surgery,Cholesteatoma, congenital,epidemiology,surgery,Human, Temporal Bone,

How to cite this article:
DeSouza C E, Menezes C O, DeSouza R A, Ogale S B, Morris M M, Desai A P. Profile of congenital cholesteatomas of the petrous apex. J Postgrad Med 1989;35:93

How to cite this URL:
DeSouza C E, Menezes C O, DeSouza R A, Ogale S B, Morris M M, Desai A P. Profile of congenital cholesteatomas of the petrous apex. J Postgrad Med [serial online] 1989 [cited 2023 Sep 25];35:93. Available from:

 :: Introduction Top

Cholesteatomas are well known to cause otorrhea and deafness. However, congenital cholesteatomas of the petrous apex very rarely cause otorrhea. They often have to be distinguished from other lesions that might occur in the cerebello-pontine angle. They can present in an unusual fashion and can be missed. However, an alert otolaryngologist with an understanding of their behaviour will not be misled.


Congenital cholesteatomas may arise from the embryonic inclusions of the epidermal portion of the epiblasts during the closure of the neural tube.[30],[43] Trauma or differentiation from multipotential cell rests[47] may also be responsible. Nager[40] refers to cholesteatomas simply as aberrant epithelial remnants and therefore blastomatous malformations. Gacek[23] and Delozier et al[16] argued that cholesteatomas of the petrous apex originate from epithelial cells from Seesels pouch which get encased within the fibrocartilage of the foramen lacerum during cephalic flexure and may give rise to cholesteatomas in later life. Aimi[2] believes that petrous cholesteatomas are neuroectodermal in origin. They have also been postulated to arise from pial tissues in the plexus tufts.[4] Cholesteatomas develop in the posterior basal cistern. Their capsule consists of a thickening of arachnoid matter and develop within the arachnoid mater.[46]

A true congenital cholesteatoma is that which has its nidus of squamous epithelium present at birth and may be found anywhere in the temporal bone depending on where the cell rests may be situated. Such cholesteatomas are sterile but can expand and erode bone, implying that bacterial infection per se is not an essential aspect of cholesteatoma advancement 38 [Figure - 1].


It is well known that congenital cholesteatomas of the petrous apex may occur without otorrhea and in an otherwise normal temporal bone.[20],[21]

Facial nerve deficits are the first lesions to occur followed by a unilateral hearing loss.[37]

Peron and Schukneckt[44] and Kerr and Smyth[34] on the other hand found that the commonest presenting symptom was unilateral hearing loss, followed by palsies of the 7th nerve.

Facial paralysis occurs more often in congenital cholesteatomas than in an acoustic neuroma because the cholesteatoma tends to encircle the 7th nerve and interfere with its blood supply.[3]

In some reports, headaches were the commonest symptom[27] while others stress the long duration of complaints and note that the lesion was often found to predominate in age groups between 20-40 years.[11],[56]

There have been reports where trigeminal neuralgia was the common presenting symptom,[5],[31],[33] while others note that mental changes occurred more often.[49]

Incidence Congenital cholesteatomas are relatively uncommon lesions of the cerebello-pontine angle.[18] Granick et al[26] found that cerebello-pontine angle cholesteatomas formed only 2% of his cases. They have been found to account for 0.2% to 1.0% of neoplasms of the central nervous systems.[8] The commonest tumours found at the cerebello-pontine ankle are acoustic neuromas[24],[48]

Interestingly, Behnke and Schindler[6] found cholesteatomas to be commoner than acoustic neuromas in their series. Scott[51] found the cerebello-pontine angle to be the commonest site at which congenital cholesteatomas were found. Nager40 found the majority (30-40%) of congenital cholesteatomas were situated in the cerebellopontine angle. The remaining were distributed to the optic, chiasma, diploe of the skull and the rhomboid fossa.

Peculiar Features

Recent biomathematical analysis suggest that cholesteatomatous lesions grow linearly at rates approximating those reported for skin rather than exponentially as most tumours do.[35] It is found that the protein content in the cerebro-spinal fluid is usually not elevated.[24],[55]

There are many reports in literature of the lesion undergoing a malignant transformation.[22],[36],[41],[52],[53],[67]

On CT scanning hyperdense cholesteatoma have also been reported.[17],[29],[39] There is an isolated report of cholesteatoma with an associated intracranial haemorrhage.[1] If its contents leak into the subarachnoid space, it can also cause aseptic or chemical meningitis.[7],[10],[50],[54]

Neuro-radio-diagnostic investigations

Before the advent of CT scanning, pneumo-encephalography was responsible for outlining the entire tumour and its relationship to adjacent structures.[14] A typical filigree appearance was seen.

CT scanning is an important investigation for various lesions involving the cerebello-pontine angle.[19],[28] CT scanning will show its various extensions and its effects on neural, vascular and cranial structures. It may erode into the ear[45] or may fill the entire ipsilateral posterior and middle fossae.[25]

The attenuation numbers of cholesteatomas (approximately -2 to + 10 Hounsfield units) and cerebrospinal fluid density are similar, so it is the mass effect that is most important when diagnosing a cholesteatoma[13] [Figure - 2]

The patients must be thoroughly investigated to locate the extent and site of the lesion to decide the surgical approach, otherwise an unsuitable approach might prove disasterous.[9]

For despite the absence of localizing signs, such as 7th and 8th cranial nerve involvement, the lesion may be extensive.[12]


The treatment of cholesteatomas of the petrous apex or anywhere else for that matter is always surgery.

Paparella and Rybak[43] suggest fistulization of the cholesteatoma into the ear. A case has been reported where the cholesteatoma was draining into the middle ear. Despite this, hearing was only marginally affected. The cholesteatoma continued to grow but because of the fistula keratin debris did not accumulate.[32] However, this may lead to an ascending infection into the cranial cavity and may cause meningitis, encephalitis or abscess formation.

Radical removal of the disease is the solution.[15] The residual epithelium can continue growing if it is not totally excised and the cholesteatoma can reform.

 :: Conclusion Top

Congenital cholesteatomas behave exactly like the acquired cholesteatomas that occur in the mastoid with the exception that they are sterile. If a patient presents with lesion that is suggestive of a pathology in the cerebello-pontine angle lesion, congenital cholesteatomas should be thought of as a possible pathology.

 :: References Top

1.Abou-Samra, M., Marlin, A.E., Story, J. L. and Brown, W. E.: Cranial epidermoid tumour associated with subacute extradural hematoma. Case report. J. Neurosurg., 53: 574-575, 1980.  Back to cited text no. 1    
2.Aimi, K.: Role of the tympanic ring in the pathogenesis of congenital cholesteatoma. Laryngoscope, 93: 1140-1146, 1983  Back to cited text no. 2    
3.Antoli-Candela, F. and Stewart, T. J.: The pathophysiology of otologic facial paralysis. Otolaryngol. Clin. North Amer. 7: 309-330, 1974.  Back to cited text no. 3    
4.Bailey, P.: Cruveilhier's "Tumeurs per lees". Surg. Gynaecol. Obstet., 31: 390-401, 1920.  Back to cited text no. 4    
5.Baumann, C. H. H. and Bucy, P. C.: Paratrigeminal epidermoid tumours. J. Neurosurg., 13: 455-468, 1956.  Back to cited text no. 5    
6.Behnke, E. E. and Schindler, R. A.: Dermoid of the petrous apex. Laryngoscope 94: 779-783, 1984.  Back to cited text no. 6    
7.Berger, M. S. and Wilson, C. B.: Epidermoid cysts of the posterior fossa. J. Neurosurg., 62: 214-219, 1985.  Back to cited text no. 7    
8.Boggan, J. E., Davis, R. L., Zorman, G. and Wilson, C. B.: Intrasellar epidermoid cyst. A case report. J. Neurosurg., 58: 411-415, 1983.  Back to cited text no. 8    
9.Brookler, K. H., Pulec, J. L. and Hall berg, O. E.: Congenital cholesteatoma of the temporal bone-Negative suboccipital exploration. Arch. Otolaryngol., 90: 449-452, 1969.  Back to cited text no. 9    
10.Cantu, R. C. and Ojemann, R. G.: Glucosteroid treatment of keratin meningitis following removal of a fourth ventricle epidermoid tumour. J. Neurol. Neurosurg Psychiat., 31: 73-75, 1968.  Back to cited text no. 10    
11.Clark, J. B. and Six, E. G.: Epidermoid tumour presenting as a tension pneumocephalus. Case report, J. Neurosurg., 60: 1312-1314, 1984.  Back to cited text no. 11    
12.Cole, T. B. and McCoy, G.: Congenital cholesteatoma of the temporal bone and sphenoid sinus. Report of a case. Arch. Otolaryngol., 87: 576-579, 1968.  Back to cited text no. 12    
13.Curtin, H. D., CT of acoustic neuroma and other tumors of the ear. Radiol. Clin. North Amer., 22: 77-105, 1984.  Back to cited text no. 13    
14.Dee, R. H., Kishore, P. R. and Young, 1-l. F.: Radiological evaluation of cerebello-pontine angle epidermoid tumor. Surg. Neurol., 13: 293-296, 1980.  Back to cited text no. 14    
15.DelaCruz, A.: The transcochler approach to meningiomas and cholesteatomas of the cerebello pontine angle. In, "Neurological Surgery of the Ear and Skull Base." Editor: D. E. Brackmann, Raven Press. New York, 1982, pp. 353-360.  Back to cited text no. 15    
16.DeLozier, H. L., Perkins, C. W. ands Gacek, R. R.: Mucocoele of the petrous apex. J. Laryngol. Otol., 93: 177-180, 1979.  Back to cited text no. 16    
17.Dunn, R. C., Archer, C. A., Rapport, R L. and Looi, L. M.: Unusual CT-dense posterior fossa epidermoid cyst. Case report. J. Neurosurg., 55: 654-656, 1981.   Back to cited text no. 17    
18.Eekhof, J. L., Thomeer, R. T. and Bots G. Th. A. M.: Epidermoid tumour in the lateral ventricle. Surg. Neurol., 23: 189-192, 1985.  Back to cited text no. 18    
19.Fawcitt, R. A. and Isherwood, I.: Radio diagnosis of intracranial pearly tumours with particular reference to the value of computer tomography. Neuroradiology. 11: 235-242, 1976.  Back to cited text no. 19    
20.Flood, L. M. and Kemink, J. L.: Surgery in lesions of the petrous apex. Otolaryngol. Clin. North Amer., 17: 565-575 1984.  Back to cited text no. 20    
21.Flood, L. M., Kemink, J. L. and Graham, M. D.: The investigation and management of petrous apex erosion. J. Laryngol. Otol., 93: 439-450, 1985.  Back to cited text no. 21    
22.Fox, H. and South, E. A.: Squamous cell carcinoma developing in an intracranial epidermoid cyst (cholesteatoma). J. Neurol. Neurosurg. Psychiat., 28: 276-281, 1965.  Back to cited text no. 22    
23.Gacek, R. R.: Diagnosis and management of primary tumors of the petrous apex. Ann. Otol. Rhinol. Laryngol., 84 (Supplement 18): 1-20, 1975.  Back to cited text no. 23    
24.Gardner, G., Robertson, J. H. and Clark W. C.: 105 patients operated upon for cerebello-pontine angle tumours. Experience using combined approach and CO2 laser. Laryngoscope, 93: 1049-1055, 1983.   Back to cited text no. 24    
25.Graham, M. D. and Kemink, J. L.: Neoplasms. In, "Otolaryngology-Head and Neck Surgery." Editor: C. W. Comming7, J. M. Fredrickson, L. A. Harker, C. J. Krause and D. E. Schuller, Vol. 4, 1st edition, C. V. Mosby Company, St. Louis Toronto, 1986, pp. 3047-3060.  Back to cited text no. 25    
26.Granick, M. S., Martuza, R. L., Ojemann, R. G., Parker, S. W. and Montgomery, W. W.: Cerebello-pontine angle meningiomas: Clinical manifestations and diagnosis. Ann. Otol. Rhinol. Laryngol.. 94: 34-38, 1985.  Back to cited text no. 26    
27.Grant, F. C. and Austin, G. M.: Epidermoids: clinical evaluation and surgical results. J. Neurosurg., 7: 190-198, 1950.   Back to cited text no. 27    
28.Harnsberger, H. R., Davis, K. R., Parkin, J. L., Osborn, A. G. and Smoker, W.: The tailored CT evaluation of persistent facial nerve paralysis. Laryngoscope, 96: 347-352, 1986.  Back to cited text no. 28    
29.Hasegawa, H., Bitoh, S., Nakata, M. Fujiwara, M. and Yasuda, H.: intracranial epidermoid mimicking meningioma. Surg. Neurol. 15: 372-374, 1981.  Back to cited text no. 29    
30.Higashi, K. and Wakuta, Y.: Epidermoid tumour of the lateral ventricle. Surg. Neurol., 5: 363-365, 1976.  Back to cited text no. 30    
31.Hori, T., Numata, H., Hokama, Y., Mumoka, K., Takami, M. and Saito, Y.: Trigeminal pain caused by a parapontine, epidermal cyst. Surg. Neurol., 19: 517-519, 1983.  Back to cited text no. 31    
32.Horn, K. L., Shea, J. J. and Brackmann D. E.: Congenital cholesteatoma of the petrous pyramid. Arch. Otolaryngol, 111: 621-622, 1985.  Back to cited text no. 32    
33.House, W. F. and Doyle, J. B.: Early diagnosis and removal of primary cholesteatoma causing pressure to the VIIIth nerve Laryngoscope, 72: 1053-1063, 1962.  Back to cited text no. 33    
34.Kerr, A. G. and Smyth, G. D. L.: Congenital cholesteatoma of the temporal bone A case report. Laryngoscope, 77: 86-88, 1967.  Back to cited text no. 34    
35.Kline, L. B. and Galbraith, J. G.: Para seller epidermoid tumor presenting as painful ophthalmoplegia. Case report J. Neurourg., 54: 113-117, 1981.  Back to cited text no. 35    
36.Lewis, A. J., Cooper, P. W., Kassel, E. E. and Schwartz, M. L.: Squamous cell carcinoma in a suprasellar epidermoid cyst. Case report. J. Neurosurg., 59: 538-541 1983.  Back to cited text no. 36    
37.Mathog, R. H. and Viscomi, G.: Otologic manifestations of retrocochlear disease. In, "Otolaryngology", Editors: M. M. paparella and D. A. Schumrick, Vol. 2, 2nd Edition, W.B. Saunders Company, Philadelphia, London, Toronto, 1980, pp. 1898-1919.  Back to cited text no. 37    
38.Meyerhoff, W. L. and Truelson, J.: Cholesteatoma staging. Laryngoscope, 96: 935-939, 1986.  Back to cited text no. 38    
39.Nagashima, C., Takahama, M. and Saka guchi, A.: Dense cerebellopontine epidermoid cyst. Surg. Neurol., 17: 172-177 1982.  Back to cited text no. 39    
40.Nager, G. T.: Epidermoids involving the temporal bone: clinical, radiological and pathological aspects. Laryngoscope, 85 (Suppl): 1-2, 1975.  Back to cited text no. 40    
41.Nosaka, Y., Nagao, S., Tabuchi, K. and Nishimoto, A.: Primary intracranial epidermoid carcinoma. Case report. J. Neurosurg., 50: 830-833, 1979.  Back to cited text no. 41    
42.Obrador, S. and Lopez-Zafra, J. J.: Clinical features of the epidermoids of the basal cisterns of the brain. J. Neurol. Neurosurg., Psychiat., 32: 450-454, 1969.  Back to cited text no. 42    
43.Paparella, M. M. and Rybak, L.: Congenital cholesteatoma. Otolaryngol. Clin North Amer., 1: 113-120, 1978.  Back to cited text no. 43    
44.Peron, D. L. and Schuknecht, H. F.: Congenital cholesteatoma with other anomalies. Arch. Otolaryngol., 101: 498-505, 1975.  Back to cited text no. 44    
45.Phelps, P. D., Toland, J. A. and Sheldon, P. W. E.: Erosions of the petrous temporal bone.; Laryngol. Otol., 84: 1205-1230, 1970.  Back to cited text no. 45    
46.Portmann, M., Sterkers, J. M. and Characon, C. H.: Tumours of the internal auditory meatus and surrounding structures, In, "Internal Auditory Meatus". Churchill Livingstone, London, New York, 1975, pp. 193-246.  Back to cited text no. 46    
47.Reeves, D. L.: Epidermoid (mixed) tumours of the central nervous system. J. Neurosurg., 26: 21-24, 1967.  Back to cited text no. 47    
48.Rosenbloom, S. B., Carson, B. S., Wang, H. and Rosenbaum, A. E., Udvarhely, G. B.: Cerebellopontine angle lipoma. Surg. Neurol., 23: 134-138, 1985.  Back to cited text no. 48    
49.Rosenbluth, P. R. and Lichtenstein, B. W.: Pearly tumour (epidermoid choler teatoma) of the brain. Clinico-pathologic study of two cases. J. Neurosurg., 17: 35-42, 1960.  Back to cited text no. 49    
50.Sansregret, A. and Morrissette, Y.: The radiological investigations of the petrous bone in meningitis of unknown origin. Neuroradiology, 2: 94-99, 1971.  Back to cited text no. 50    
51.Scott, M.: Epidermoid tumour (cholesteatoma) of the lateral cerebral ventricle. Case report. J. Neurosurg., 14: 110-113, 1957.  Back to cited text no. 51    
52.Smaltino, F. and Cucciniello, B.: Epidermoid tumour of the epiphyseal region. Case report. J. Neurosurg., 28: 63-66, 1968.   Back to cited text no. 52    
53.Toglia, J. U., Netsky, M. G. and Alexander, E. Jr.: Epithelial (epidermoid) tumours of the cranium. Their common nature and pathogenesis. J. Neurosurg., 23: 384-393, 1965.  Back to cited text no. 53    
54.Tomlinson, B. E. and Walton, J. N.: Granulomatous meningitis and diffuse parenchymatous degeneration of the nervous system due to an intracranial epidermoid cyst. J. Neurol. Neurosurg. Psychiat., 30: 341-348, 1967.  Back to cited text no. 54    
55.Tytus, J. S. and Pennybacker, J.: Pearly tumors in relation to the central nervous system. J. Neurol. Neurosurg. Psychiat., 19: 241-259, 1956.  Back to cited text no. 55    
56.Ulrich, J.: Intracranial epidermoids. A study on their distribution and spread. J. Neurosurg., 21: 1051-1058, 1964.  Back to cited text no. 56    
57.Wilson, E. S. and Sheft, D. J.: Epidermoid tumour of the skull with intracranial pneumatocoele. Case report. J. Neurosurg., 28: 600-602, 1968.  Back to cited text no. 57    


[Figure - 1], [Figure - 2]

This article has been cited by
1 Petrous apex cholesteatoma
Profant M, Steno J
ACTA OTO-LARYNGOLOGICA. 2000; 120 (2): 164-167


Print this article  Email this article
Previous article Next article
Online since 12th February '04
2004 - Journal of Postgraduate Medicine
Official Publication of the Staff Society of the Seth GS Medical College and KEM Hospital, Mumbai, India
Published by Wolters Kluwer - Medknow