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Congenital Cholesteatoma of the Mastoid Region

Emilio Mevio, Edoardo Gorini, Michele Sbrocca, Leonardo Artesi, Alberto Lenzi, Stefano Lecce and Mauro Mullace Otolaryngology -- Head and Neck Surgery 2002 127: 346 DOI: 10.1067/mhn.2002.128604 The online version of this article can be found at: http://oto.sagepub.com/content/127/4/346

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Congenital cholesteatoma of the mastoid region

EMILIO MEVIO, EDOARDO GORINI, MICHELE SBROCCA, LEONARDO ARTESI, ALBERTO LENZI, STEFANO LECCE, and MAURO MULLACE, Magenta, Italy

C ongenital cholesteatoma is an epidermoid cyst

arising from congenital remnants of keratinizing squamous epithelium in the temporal bone. The process is benign but slowly progressive, expansive, and destructive. The typical patient does not have a history of middle ear disease or middle ear surgery or evidence of current or past perforation of the tympanic membrane. Congenital cholesteatoma has been reported to occur in 5 sites in the temporal bone: petrous apex, cerebellopontine angle, mastoid, middle ear, and external auditory canal. The most frequent site of origin is the middle ear, whereas the rarest is the mastoid.1 The presenting features of this disease differ according to the site of origin. If it arises within the middle ear, it presents with hearing loss in children due to erosion of the ossicular chain. If the origin is within the petrous apex, it manifests in adults with the symptoms of facial nerve weakness caused by compression of the facial nerve and a space-occupying lesion of the brainstem. The rare localization in the mastoid affects the adult (30 to 50 years old) and is manifested as disturbed balance, not associated with involvement of the labyrinth but rather cerebellar compression, pain in the mastoid or neck, and swelling of the mastoid. We report the case of a patient with congenital cholesteatoma arising in the mastoid expanding into the middle and posterior cranial fossa with compression of the cerebellum and occlusion of the sigmoid sinus. CASE REPORT A 36-year old man presented with a 1-year history of recurrent episodes of positional dizFrom the Department of Otorhinolaryngology, Hospital Fornaroli, Magenta (Mi), Italy. Reprint requests: Mevio Emilio, Via Gravellone 37, 27100 Pavia, Italy; e-mail, emevio@libero.it. Otolaryngol Head Neck Surg 2002;127:346-8 Copyright 2002 by the American Academy of OtolaryngologyHead and Neck Surgery Foundation, Inc. 0194-5998/2002/$35.00 0 23/78/128604 doi:10.1067/mhn.2002.128604
346

ziness lasting only a few seconds. These episodes were triggered by exion-extension movements and rotation of the head to the right. There was no history of ear infection, trauma, hearing loss, or tinnitus. Physical examination revealed apparently normal tympanic membranes on both sides. Audiometric examination demonstrated a slight perceptive loss at high frequencies. The results of impedance testing and stapedius reexes were normal. There was no spontaneous nystagmus and no positional or positioning nystagmus. The head shaking test was negative. Caloric tests demonstrated that reex activity of the vestibular system was slightly reduced on the left. Auditory evoked potentials were tested but found to be normal. Magnetic resonance imaging of the brain showed a mass in the left mastoid cavity that did not involve the aditus ad antrum, the mastoid antrum, or epitympanic recess. The mass was, however, eroding the posterior and superior walls of the mastoid, invading the middle and posterior cranial fossa. The mass occluded the left sigmoid sinus and compressed the ipsilateral cerebellar hemisphere. The mass was hypointense in T1-weighted and hyperintense in T2-weighted images (Fig 1). Subsequent computed tomography scans conrmed these ndings and demonstrated the integrity of the cochlear labyrinth and the facial nerve (Fig 1). A modied radical mastoidectomy (retrolabyrinthine approach) was performed. At surgery the mastoid was found to be lled by cholesteatoma that had completely destroyed the tegmen mastoideum and the posterior fossa bony plate. The sigmoid sinus was closed by atelectasia, and the superior petrosal sinus had been exposed. The bony labyrinth was intact, and the endolymphatic sac was exposed and partially incorporated in the mass. The cholesteatoma was completely removed, and the large mastoid cavity was packed with abdominal adipose tissue to prevent cerebral and cerebellar ptosis. The day after the operation the patient reported the disappearance of his vestibular symptoms.

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Otolaryngology Head and Neck Surgery Volume 127 Number 4

MEVIO et al

347

Fig 1.A, Heterogeneous, hyperintense mass of the left mastoid shown by T2-weighted, magnetic resonance image. B, Coronal computed tomography scan of temporal bone showing the extent of cholesteatoma, the mastoid bone erosion, and the left sigmoid sinus and cerebellar hemisphere compression (arrows).

DISCUSSION We have found only 5 other reports in the literature of cases of congenital cholesteatoma arising in the mastoid.1-5 The mastoid is undoubtedly the rarest site for the onset of congenital cholesteatoma. The symptoms and imaging features of our case correspond to those described by Luntz et al3 and subsequently conrmed by other authors as pathognomonic of the disease: 1. The symptoms were neck pain, retroauricular swelling, and persistent disequilibrium. Physical ndings were unremarkable, and hearing tests showed a symmetric high tone sensorineural hearing loss. 2. Computed tomography showed an expanding lytic mass in the posterior part of the mastoid process. The middle ear, inner ear, and aditus ad antrum were normal.

3. Magnetic resonance imaging revealed an expanding lesion that was hyperintense in T2and hypointense in T1-weighted images. The rst point that should be highlighted is that the disease occurred in our patient, as in the other reported patients, in adulthood, unlike the age of onset of the development of congenital cholesteatomas in the other sites that are more frequently involved. Our patient had neck pain, probably because of inammation of the insertion of the muscles into the mastoid process. He did not, however, have the retroauricular swelling usually reported in these cases. This swelling is the result of outgrowth of the mass or reactive inammation evident only in more advanced cases. The vestibular signs in all the reported cases of mastoid cholesteatoma were caused by compression of the ipsilateral cerebellar hemisphere. Only Derlacki and Clemis2 presented the case of a patient with erosion of the bony labyrinth. In our case the only vestibular structure involved was the endolymphatic sac, which was spared during the surgery, and the patient had no postoperative labyrinthine symptoms. Another peculiar feature of our case was the complete atelectasia of the sigmoid sinus, indubitably a consequence of compression by the mass. At the most, extensive erosion of the bony plate covering the sigmoid sinus was reported in the other cases.2,3,5 The particular site and rarity of the lesion make careful differential diagnosis from all other possible tumors in the site essential: tympanojugular paragangliomas, tumors of the endolymphatic sac, and meningiomas. Although paragangliomas do cause osteolysis and invade the sigmoid sinus, they more frequently involve the infralabyrinthine region than the retrolabyrinthine one and are easily identied by contrast-enhanced imaging. The rare tumors of the endolymphatic sac are revealed as a heterogeneous gadolinium-enhanced mass in T1-weighted magnetic resonance images because of the presence of considerable vascularization between the foci of tumor tissue. Meningiomas do not usually present with osteolysis but rather show increased bone density.
REFERENCES

1. Nager GT. Pathology of the ear and temporal bone. Baltimore: Williams & Wilkins; 1993. p. 710-42.

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348 MEVIO et al

Otolaryngology Head and Neck Surgery October 2002

2. Derlacki EL, Clemis JD. Congenital cholesteatoma of the middle ear and mastoid. Ann Otol Rhinol Laryngol 1965; 74:706-27. 3. Luntz M, Telischi F, Bowen B, et al. Congenital cholesteatoma isolated to the mastoid. Ann Otol Rhinol Laryngol 1997;106:608-10.

4. Rashad U, Hawthorne M, Kumar U, et al. Unusual cases of congenital cholesteatoma of the ear. J Laryngol Otol 1999; 113:52-4. 5. Cureoglu S, Osma U, Oktay MF, et al. Congenital cholesteatoma of the mastoid region. J Laryngol Otol 2000;114:779-80.

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