|Year : 1998 | Volume
| Issue : 1 | Page : 55-60
Congenital cholesteatoma: Ohod hospital expewrience
Mohammed Abdel-Rauf Massoud, Mutwakel Faleh Hajjaj
Department of ENT, ohod Hospital, Al Madinah, Saudi Arabia
|Date of Web Publication||16-Jun-2020|
Mutwakel Faleh Hajjaj
P.O. Box 1257, Al Madinah Al-Munwarah
Source of Support: None, Conflict of Interest: None
Congenital cholesteatoma of the middle ear and mastoid is an otologic challenge receiving increasing attention. Its site of origin and pathogenesis are still a matter of controversy. However, its power of eroding bone makes it a potentially dangerous condition that needs surgical intervention. We present two cases of congenital cholesteatoma which were involving the middle ear in one and localized in the mastoid in the other. Our pitfalls in the diagnosis were discussed after a literature review on the pathogenesis, diagnosis and treatment.
Keywords: congenital cholesteatoma, pathogenesis, diagnosis, treatment, temporal bone
|How to cite this article:|
Massoud MA, Hajjaj MF. Congenital cholesteatoma: Ohod hospital expewrience. Saudi J Otorhinolaryngol Head Neck Surg 1998;1:55-60
|How to cite this URL:|
Massoud MA, Hajjaj MF. Congenital cholesteatoma: Ohod hospital expewrience. Saudi J Otorhinolaryngol Head Neck Surg [serial online] 1998 [cited 2022 Nov 30];1:55-60. Available from: https://www.sjohns.org/text.asp?1998/1/1/55/286859
| Introduction|| |
Congenital cholesteatoma of the temporal bone is not a rare disease. , McDonald and associates  attributed to Jefferson and Smalley the first report about a case of congenital cholesteatoma of the petrous portion of the temporal bone in 1933. It was not until 1953, that House  described a middle ear cholesteatoma behind an intact drum membrane. However, interest in the possible congenital origin of some cholesteatomas was re-awakened by Cawthorneand Griffith  report of a series of nine deep temporal bone epidermoids, followed by Cawthorne’s  description of a small localised cholesteatoma behind an intact tympanic membrane. He proposed that this congenital cholesteatoma arose from a congenital cell rest. Cawthorne’s reports were shortly followed by Derlacki and Clemis’s  report of ten cases of congenital cholesteatoma of the middle ear and mastoid. They established clinical criteria for the diagnosis of congenital cholesteatoma that have been modified by Levenson and associates .
Congenital cholesteatoma within the temporal bone can occur in the middle ear, mastoid, petrous bone and the squama. However, intradural (cisternal) congenital cholesteatoma often involves the cerebellopontine angle region causing varying degrees of cochlear vestibular dysfunction .
Y.H.F.- an eleven year old male presented with 6 months history of right sided hearing loss. Physical examination revealed no abnormalities in the nose and throat and both tympanic membranes appeared normal. Tuning fork tests were suggestive of right conductive hearing loss. Pure tone audiometry showed a right ear to have a 40 db airbone gap with the bone conduction level at 5dB [Figure 1]A. Tympanometry showed type A tympanogram with absent stapedial reflex on the right side. A provisional diagnosis of right sided congenital stapedial fixation was made. Accordingly, the patient was scheduled for right tympanotomy.
Under general anaesthesia, microscopic examination showed a whitish mass behind the anterior attic with intact tympanic membrane. Tympanotomy revealed the presence of a well encapsulated epidermoid sac extending from the anterosuperior mesotympanum behind the handle of malleus into the attic and posterosuperior mesotympanum eroding the long process of incus. Otherwise, the middle ear mucosa was completely normal. Atticotomy was performed and the upper limit of the cholesteatoma was identified. The malleus head and body of incus were removed and the sac was dissected completely. The remaining part of incus was then transpositioned between the handle of malleus and head of stapes. The lateral epitympanic wall was reconstructed with a piece of tragal cartilage.
Two months post-operatively, the patient had a 25 dB drop of bone conduction level at 2 and 4 kHz. A year later, the patient had no evidence of recurrence of the disease and the same postoperative hearing level.[Figure 1]B&[Figure 1]C.
R.J.M., a 24 year old male presented with right sided otorrhea for 3 months. An aural polyp was found arising from the posterior meatal wall at the bony-cartilaginous junction. The tympanic membrane behind the polyp was normal and audiological evaluation showed bilateral normal peripheral hearing [Figure 2].
Under topical anaesthesia, the polyp was cut at its narrow pedicle; histopatholgical examination pointed to a cholesteatomatous origin of this polyp.
Radiological studies by plain films showed a large cavity in the lower part of right mastoid with preserved pneumatisation of the upper part.
CT scanning revealed a soft tissue lesion in the right mastoid eroding the posterior meatal wall and the bony plate covering the posterior cranial fossa dura,whereas,the middle ear and the ossicles were free.
A corticol mastoidectomy revealed a localisation of the cholesteatoma sac to the mastoid tip area with erosion of the posterior meatal wall and exposure of the lateral sinus, posterior fossa dura and the mastoid segment of the facial nerve.The mastoid antrum and peri- antral cells were completely free and so was the middle ear. The sac was easily pealed off its surroundings and the cavity was obliterated by a pedicled muscle flap after applying a piece of tragal cartilage to close the posterior meatal wall fistula.
Post-operatively, the patient had normal hearing and he was free of recurrence for a 10 month follow up period.
As cited by Derlacki and Clemis , it was as early as 1854 when von-Remak postulated that cholesteatomas, in general, are due to the development of congenital epithelial cell rests.
It needed more than a century for this theory to be revived by Cawthorne  and Derlacki and Clemis  who suggested that these growths arise from embryonal inclusions of squamous epithelium or undifferentiated epithelium in the developing temporal bone.
Aimi  speculated that as most of the congenital cholesteatomas occurred near he tympanic isthmus, and because this is the junction of the first and second bronchial arches, migration of external canal ectoderm into the middle ear at an early stage of development may occur. This migration may be due, he thought, tofailure of the inhibitory function of the tympanic ring.
Another speculation was published by Michaels  who noted a squamous cell rest identifiable from 10 to 33 weeks of gestation in the anterosuperior lateral wall of the tympanic cavity. According to Michaels , if this structure which he has termed “epidermoid formation”, failed to involute,its expansion could result in the formation of a congenital cholesteatoma. This is the commonest site of congenital cholesteatoma of the middle ear .
Although the last two theories explain the occurrence of most of the middle ear congenital cholesteatomas, yet, they do not give an explanation for congenital cholesteatoma of the mastoid, petrous bone or multicentric cholesteatomas. These are thought to have a different pathogenesis than those commonly found in the anterosuperior tympanum of very young children .
The incidence of congenital cholesteatoma of the temporal bone was reported by Leong and Portmann  to be about 5% of all cases of cholesteatomas. However, a lower incidence, 2%, was reported by McDonald et al . Children are more frequently affected by the diseses However, adults were also reported to have congenita! cholesteatomas ,. Many authors ,, have noted a male preponderance, though no explanation has been given for this observation.
Associated congenital anomalies were common. These anomalies were reported to involve the external or middle ear , or other organs as cleft lip and palate .
In the early stage, a middle ear cholesteatoma is symptomless ,. When it progresses, it can destroy the conductive system of the middle ear [1-],,,, or it can obstruct the Eustachain tube orifice  leading to conductive hearing loss. Furthermore, extension of the cholesteatoma into the petrous apex may cause nerve affection and/or cochlear and vestibular dysfunction ,.
Congenital cholesteatoma when confined to the mastoid process may present as a mastoid swelling ,, but erosions of the posterior meatal wall with fistula formation may lead to the rare presentation of a draining ear with an intact tympanic membrane .
Physical examination may reveal a white mass behind an intact tympanic membrane. The mass is often limited to the anterior half of the tympanic cavity, however, the entire middle ear cavity may be involved , or the mass may be located in another site.
Radiologicaily, an isolated lytic lesion of the temporal bone with an intact drum is suggestive of congenital cholesteatoma.  High resolution C.T. scanning is particularly valuable in evaluation of congenital cholesteatoma.  It is the study of choice for cholesteatoma of the middle ear and mastoid. ,, MRI, however, is superior to C.T for evaluation of infected cholesteatoma, petrous apex and cerebellopontine cholesteatomas as well as for the evaluation of cholesteatomatous involvement of the facial nerve, membranous labryinth and intracranial structures.
Once diagnosed, a congenital cholesteatoma must be completely removed,, The surgical approach is decided according to the site and extension of the cholesteatoma. For lesions localised to the middle ear , a tympanotomy allows complete removal.,,, Atticotomy may be needed to expose upward extension of the disease. However, if the cholesteatoma extends posteriorly into the sinus tympani or into the mastoid antrum, it may be removed via an intact wall technique. , For more extensive lesions a canal wall down mastoid exploration may be necessary., Extensions into the petrous apex can be removed via radical mastoidectomy  or middle cranial fossa approach when there is serviceable hearing. Cortical mastoidectomy is enough to expose and remove a chosteatoma limited to the mastoid process. 
| Discussion|| |
Congenital cholesteatoma of the middle ear and mastoid is not a rare disease.[l], During the period (1992 - 1993), 93 cases of acquired cholesteatoma were operated upon in the E.N.T, department, Ohud Hospital, Madinah, Kingdum of Saudi Arabia. This makes the incidence of such a condition to be 2.15%; this is similar to that reported by McDonald et al , but less than the report of Leong and Portmann.
Both cases were found to fulfil the criteria of Derlacki and Clemis  and their modification by Levenson et al.  (Table I) Both patients had a cholesteatoma behind an intact normal tympanic membrane. Both had negative history of previous otorrhoea or ear surgery. Both showed a well encapsulated cholesteatoma sac with normal surrounding mucosa of the middle ear (case 1) and mastoid air cells (case 2).
The former case illustrates the peculiar presentation of a congenital cholesteatoma of the middle ear in a young boy with unilateral conductive hearing loss and a white mass behind an intact drum. Our pitfall in the diagnosis points to the utmost importance of using the microscope for ear examination; a point that has been emphasised by McDonald et al . This faulty preoperative diagnosis has been reported by several authors ,,.
However congenital cholesteatoma is an tympanic membrane important differential diagnosis to keep in mind When evaluating children with unilateral hearing loss especially when it is out of proportion to the loss normally seen with otitis media with effusion.
Congenital cholesteatomas of the mastoid are much less common than those of the middle ear, The case was reported by Derlacki and Clemis.  However, our latter case had a unique presentation of discharging ear with an intact tympanic membrane, which has, to our knowledge, only been reported once by Huang and Lee. 
The hypothesis of epidermoid formation  or migration of ectoderm from the external canal  can explain the pathogenesis of the former cholesteatoma, although its origin cannot be precisely determined. However, none of these hypotheses can give an explanation of the pathogenesis of mastoid cholesteatoma in case No.2. The observation agrees with the suggestion of Huang and Lee  that the site of origin and pathogenesis of such cholesteatoma is different from those commonly found in the anterosuperior tympanum in children. We agree with Cawthorne’s statement “Congenital cholesteatoma auris arise in the petrous or tympanic portions of the temporal bone. Becaues of its power of eroding bone, it is potentially dangerous. Once diagnosed, it must be removed completely and is unlikely to return. 
| Conclusion|| |
In conclusion, congenital cholesteatoma is not as unusual as it was thought before. The disease is found more frequently in children, but can affect adults. The patient usually presents with unilateral conductive hearing loss with a white mass behind a normal drum. Otorrhoea secondary to erosion of posterior meatal wall is a rare presentation. CT Scanning is the first oice diagnostic tool. Surgery is the one and only choice for managing such a condition in which silent danger lurks behindan intact tympanic membrane.
| References|| |
Shigemi H., Kaneda N., Hori F.,Watnabe N. and Mogi G. Congenital middle ear cholesteatoma.Report of 3 cases. Auris Nasus Larynx 1991;18:291-296 (English Abstract).
Arraign, M.A. Cholesteatoma in children. Otolaryngol Clin North Am 1994; 27: 573- 591.
McDonald T.J.,Cody D.T.R.and Ryan R.E. Congenital cholesteatoma of the ear. Ann Otol Rhinol Laryngol 1984:93:637-640.
House, H.P. An apparent primary cholesteatoma case report.Laryngoscope 1953;63:712-713.
Cawthorne, T. and Griffith A. Primary Cholesteatoma of the temporal bone. Arch Otolaryngol 1961;73: 252-261.
Cawthorne, T. Congenital Cholesteatoma. Arch Otolaryngol 1963;78:248-252.
Derlacki, E.L. and Clemis, J.D. Congenital Cholesteatoma of the middle ear and mastoid. Ann Otol Rhinol Laryngol 1965;74:706-727.
Levenson, M.J., Michaels, L. and Parisier, S.C. Congenital cholesteatomas of the middle ear in children: origin and management. Otolaryngol Clin North Am 1989;22:941-954.
Mafee, M.F. : MRI and CT in the evaluation of acquired and congenital cholesteatomas of the temporal bone. J Otolaryngol 1993;22:239-248.
Aimi, K. Role of the tympanic ring in the pathogenesis of congenital cholesteatoma. Laryngoscope 1983;93:1140-1146.
Michaels L. An epideroid formation in the developing middle ear: possible soutce of cholesteatoma. J Otol Laryngol 1986;15:169-174.
Leong,H.K. and Portmann M. Congenital cholesteatoma of the middle ear and mastoid. A proposes of 5 cases. Rev Laryngol Otol Rhinol Board 1992; 113:41- 45 (English Translation).
Schwartz R.H.,Grundfast K.M. and Feldman B. Cholesteatoma medial to an intact tympanic membrane in 34 young children. Paediatrics 1984:74:236-240.
Puranik K.R., MurthyP.S.N. Gobalakrishna, R. and Nayak D.R. Unuaually large mastoid antrum “mega antrum”. J Laryngol Otol 1992;106:164-165.
Huang, T.S. and Lee, F.P. Congenital cholesteatoma: review of twelve cases. Am J Otol 1994; 15: 276-281.
Sneryers W., Debrune F and Morlin J. Cholesteaoma of temporal bone: comparison of CT and operative findings, Acta Otorhinolaryngol Belg 1991; 45- 369-373
[Figure 1], [Figure 2]