673

08-177

Imaging Of Congenital Cholesteatoma With Atretic Ear- A Rare Case Report

A GHOSH, S SAHA, A SADHU, PV SAHA Abstract Congenital cholesteatoma is a rare entity in routine clinical practice. Secondary otitis media makes it difficult to diagnose. Chances of congenital cholesteatoma increase when it is associated with atretic ear. Appropriate imaging technique helps to diagnose and delineate the extent congenital cholesteatoma. Ind J Radiol Imag 2006 16:4:673-675 Key words : Congenital cholesteatoma, Atresia of ear , CT Scan INTRODUCTION Congenital malformation of external ear is often associated with malformation of middle and inner ear. External ear atresia is mostly bony rather than membranous; bony atresia of external ear is regularly associated with malformations of middle ear cavity and structures of middle ear1. Congenital aural atresia occurs once in every 10,000 births. It is associated with recognizable syndrome in 10% of cases. Congenital cholesteatoma are thought to be developed from epidermal cell rests or through basal cell proliferation and may occur anywhere within the middle ear cleft. CT Scan, especially High Resolution CT Scan is of great importance to delineate the extent of disease process and also to know the ossicular- chain status, condition of inner ear, and condition of seventh and eighth nerves. CT Scan in case of congenital aural atresia also helps prognostically to predict the outcome of surgical correction. CASE REPORT A 19 yr old male patient from eastern part of India presented with absence of external ear since birth and discharge from right infra-auricular fistula and decreased hearing since last 7 years. The discharge was scanty, foul smelling, intermittent in nature and reduced in medication. He also complained of inability to close his eyelids and dribbling of saliva from angle of mouth since last 6 years. On clinical examination, grade III microtia was found, as external ear remnant is little more than a nubbin of skin on the lateral aspect of face. There were also right-sided infra-auricular fistula, bilateral facial nerve paralysis and submucosal cleft palate. Pure tone audiometry showed moderate to severe conductive hearing loss in both ears. EMG study revealed absence of muscle fasciculation. HRCT of temporal bone in the axial and coronal planes showed cartilaginous part of external auditory canal and pinna of both sides are poorly developed (Fig 1). A calcified soft tissue mass in the right mastoid region with bony expansion, which is extending into the middle ear is noticed. Bony labyrinth and cochlear turns appear to be normal in both sides(Fig 2).

Fig 1- Ct Scan Of The Temporal Bone Showing Cholesteatoma In Right Temporal Bone

From the Dept of ENT, Medical College, 88, College Street, Kolkata 700073 Request for Reprints: Dr. Somnath Saha, Sundaram Apartments, Barat, Lake Town, Calcutta-700089 Received 7 August 2006; Accepted 10 October 2006

674

674 A Ghosh et al

IJRI, 16:4, November 2006 and Clemis's first report of ten cases of congenital cholesteatoma of the middle ear cavity soon followed Cawthorne's report. They had also proposed the criteria for defining congenital cholesteatoma. Cases of congenital aural atresia and intramembranous intratympanic cholesteatoma were excluded and were considered in another group. Congenital cholesteatoma may arise from cerebellopontine angle, petrous pyramid, jugular foss, middle ear cavity and mastoid antrum5. Acquired and congenital cholesteatoma are identical in their histopathological nature. They are keratin filled, epithelium-lined cysts that demonstrate linear growth pattern like skin6. Presence of Otitis media is the only differentiating feature between acquired and congenital cholesteatoma. The diagnosis requires high index of suspicion, knowledge of the sign and symptoms produced by an expanding mass in the mastoid region and imaging study of this region. Clinical presentation depends on the site of lesion. If it is present in the middle ear cavity, it will manifest as deafness in early childhood; alternatively if it is in petrous apex, patient will present with facial spasm in adulthood. Imaging plays a major role in diagnosing, delineating the extent of the disease and planning for the surgery. CT scan, both non contrast and contrast enhanced, is a reliable way to detect cholesteaoma since their keratin content gives them lower density than brain. Smooth or scalloped borders of the mass is typical for epidermoid tumour of the mastoid region5. In MRI, cholesteatomas have negligible or low intensity signals in T1W1 and high intensity signals in T2W16. They do not enhance with Gadolinium-DTPA. CT scan and MRI can be used to differentiate various pathologies of this region. Differential diagnosis includes schwannoma, meningioma, glomus tuomur, petrositis, histiocytosis, cholesterol granuloma, mucocoele and arachnoid cyst. Smooth or scalloped borders with kack of enhancement are two most important features that differentiate cholesteatomas with other pathologies. Cerebellopontine angle cholesteatomas can be differentiated from arachnoid cyst by utilizing FLAIR or diffusion weighted MRI. Petrous apex cholesteatoma can be distinguished from cholesterol granuloma by using MRI, cholesterol granuloma has increased signal intensity in T1W1 and T2W1 due to old haemorrhage7. MR angiography can be carried out if ine suspects carotid artery compression in a case of large apical lesion. CT scan also plays pivotal role in the planning the surgery and grading the outcome of the surgery in congenital aural atresia. A grading system based on preoperative HRCT of the temporal bones was developed in an effort to select those individuals with best chance of successful atresia surgery8. Pneumatization of mastoid, presence of stapes

Fig 2- Ct Scan Showing Cholesteatoma With Atretic Ear Of Right Side.

During operation, zygomatic root of temporal bone and glenoid fossa were used for identifying the middle ear cleft. Pearly white cholesteatoma was removed from middle ear cavity, mastoid antrum and facial recess region. No ossicles were found except foot plate of stapes. Facial nerve was exposed but in normal course. Histopathological report of the mass revealed cholesteatoma. The patient was kept in regular follow up. DISCUSSION Disorder of pinna and external auditory canal are common entity in clinical otology. Both of the components of external ear- pinna and external auditory canal augment and transmit sound wave to tympanic membrane. During sixth week of gestation, auricle develops from six hillocks of His, which are mesenchymal proliferation of first and second pharyngeal arches. The first arch contributes in the formation of tragus and superior portion of helix, whereas second arch gives rise to antihelix, antitragus and lobule. It is only at 20th week of gestation when the development of auricle is completed. Invagination of the ectodermal element of first pharyngeal pouch forms external auditory canal. It grows medially to meet the endoderm of middle ear. The mesodermal component gets entrapped between these ventral and dorsal sites giving rise to meatal plug or plate. This plate resorbs by the end of 28th week of gestation and the external auditory canal recanalise2. Failure of recanalization leads to cangenital aural atresia. Almost 14% of atretic ears have congenital cholesteatoma3. Cawthorne reawakened the concept of possible congenital origin of cholesteatomas4 . Delarcki

675 IJRI, 16:4, November 2006 have been the most important criteria for atresia surgery.
5.

Imaging of Congenital Cholesteatoma 675

Otolaryngol 1963; 78: 248-252. Latack J T, Kartush J M, Kernick J L, et al- Epidermoids of the cerebello-pontine angle and temporal bone, CT and MRI aspects. Radiology 1985; 157: 361-366. griffin C, Delapaz R, Euzmann E- MR and CT correlation of cholesterol cysts of the petrous bone. A J N R 1987; 8: 825-829. Gomori J M, Gorman R I, Goldberg H I, et al- Intracranial hematomas imaging by high field MR. Radiology 1985; 157: 87-93. Jahrsdoerfer RA, Yeakley JW, Aguilar EA, et al. Grading system for the selection of patients with congenital aural atresia. Am J Otol 1992; 13: 6-12.

The cholesteatoma can be removed in totality and is preferred over the prior reconstruction of hearing or canal reconstruction in atretic ear. REFERENCES
1. 2. 3. 4. Jahrsdoerfer RA- Congenital atresia of the ear. Laryngoscope 1987; 88 Sadler TW. Langman's medical embryology. 7th ed. Baltimore: Williams and Wilkins; 1995. De La Cruz, Linthicum F H Jr., Luxford W M- Congenital atresia of external ear. Laryngoscope 1987. Cawthorne T- Congenital cholesteatoma. Arch

6.

7.

8.

676