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Infantile congenital petrosal cholesteatoma: A case report and literature review
International Journal of Pediatric Otorhinolaryngology (2005) 69, 1703—1707
www.elsevier.com/locate/ijporl
CASE REPORT
Infantile congenital petrosal cholesteatoma: A case report and literature review Kazuharu Yamazaki a,*, Hiroaki Sato a, Kazuo Murai b, Kaoru Ogawa c a
Department of Otorhinolaryngology, Iwate Medical University, Uchimaru 19-1, Morioka, Iwate 020-8505, Japan b Department of Otorhinolaryngology, Iwate Rosai Hospital, Iwate, Japan c Department of Otorhinolaryngology, Keio Medical University, Tokyo, Japan Received 25 March 2005; accepted 8 April 2005
KEYWORDS Congenital petrosal cholesteatoma; Facial nerve paralysis; Infant
Summary Congenital cholesteatoma of the middle ear is frequently seen in younger children including infant. By contrast, that of the petrous bone is rarely seen in younger children. We report an exceptional case occurring in 23-month-old infant who presented with unilateral facial nerve paralysis at about 18-month-old. Computed tomography and magnetic resonance imaging revealed congenital cholesteatoma measuring approximately 2 cm in the area of the right geniculate ganglion. The mass was completely eradicated via the middle fossa approach, which allowed for preservation of hearing. The facial nerve maintained intact during surgery and paralysis showed partial recovery after the operation. To our knowledge, the present case seems to be the youngest case of congenital petrosal cholesteatoma reported, and also demonstrates congenital petrosal cholesteatoma could exhibit facial nerve paralysis in early childhood. # 2005 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Tumors occurring in the petrous part of the temporal bone are predominantly vestibular schwannoma, with cholesteatoma only comprising 4% [1]. Moreover, petrosal cholesteatoma accounts for only 1—3% of all cholesteatomas and is comparatively rare [2,3]. The petrous bone is considered a ‘‘silent area’’, and tumors in the region tend to be asymptomatic in the
* Corresponding author. Tel.: +81 19 651 5111; fax: +81 19 652 8642.
early stage. Many cases thus display extensive expansion of the tumor by the time symptoms appear. Compared with cholesteatomas in other regions, facial nerve paralysis is considered characteristic for petrosal cholesteatoma. Cawthorne [4] described facial nerve paralysis as progressing gently and quietly in petrosal cholesteatoma, representing a key point of difference to vestibular schwannoma. We encountered a case of petrosal cholesteatoma in a 23-month-old girl who developed facial nerve paralysis at 18-months-old. The tumor was removed via the middle cranial fossa approach. This case is thought to be very rare given the age of the patient at
0165-5876/$ — see front matter # 2005 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijporl.2005.04.027
1704
appearance of facial nerve paralysis, and we discuss the case with reference to the existing literature.
K. Yamazaki et al.
lagopthalmos was not seen and no leakage of food from the angle of the mouth was present. The facial nerve paralysis was Grade IV by the House—Brackmann classification.
2. Case report 3.2. CT The patient was a 23-month-old girl with no family or previous medical history of note, and no previous episodes of myringitis. Her mother had noticed that movements of the right face decreased at about 18months-old, and consulted a public health nurse. However, symptoms were attributed to a habit, and no further action was taken. The patient developed chicken pox at 21-month-old, and underwent a checkup at a nearby pediatric clinic. At this time, right facial nerve paralysis was noted, and the patient was referred to our clinic and hospitalized in early 2001 for surgical intervention.
3. Laboratory findings 3.1. Otorhinolaryngological findings No abnormalities were identified in the ear, nose or pharynx. Movement of the right face was poor, but
An expansive lesion was identified above the petrous bone on coronal section and forward of the petrous bone on axial CT (Fig. 1A and B). No relation to the middle ear was apparent. Development of the mastoid air cells was good. A round destruction image was seen above the acoustic meatus in the middle fossa of the petrous bone, on the superolateral aspect of the bone on three-dimensional CT (Fig. 1C).
3.3. MRI The tumor displayed intermediate intensity on axial sections from T1-imaging, with one region of high intensity. On coronal T2-imaging, the tumor displayed a region of slight hyperintensity above the cochlea and involving the dura mater. Invasion into the brain tissue was not seen (Fig. 2A and B). Cholesteatoma, cholesterin granuloma, mucocele,
Fig. 1 CT scan of the temporal bone. An expansive lesion was identified above the petrous bone on coronal section (A) and forward of the petrous bone on axial section (B). A round destruction image was seen above the acoustic meatus in the middle fossa of the petrous bone, on the superolateral aspect of the bone on three-dimensional CT (C).
Infantile congenital petrosal cholesteatoma
1705
Fig. 3 Perioperative photography. Middle fossa exposure of the cholesteatoma in the petrous apex.
for the orbicularis oculi, 0% for the nasalis, and 7.8% for the orbicularis oris.
3.5. Auditory brainstem response Auditory brainstem response (ABR) was measured three times: once preoperatively and twice postoperatively. Latency was not extended, and thresholds were normal, with 10 dB in the right ear and 5 dB in the left. Fig. 2 MRI of the inner ear. The tumor displayed intermediate intensity on axial sections from T1-imaging (A), with one region of high intensity. On coronal T2-imaging (B), the tumor displayed a region of slight hyperintensity above the cochlea and involving the dura mater.
and schwannoma were considered as differential diagnoses following CT and MRI.
3.4. Electroneuronography Electroneuronography (ENoG) was performed on bilateral orbicularis oculi, nasalis (both M. compressor naris and M. dilator naris), and orbicularis oris muscles. Amplitude (mV) was measured at three points (L1: standing, L2: top, L3: bottom) and differences (L1 L2, L2 L3, L1 L3) were calculated and compared for both raw values and calculated percentages. Mean preoperative ENoG values (Table 1) on the affected side were 5.9%
4. Treatment Surgical intervention via the middle cranial fossa on the affected (left) side was scheduled for 6 days after admission. After removal of a bone flap, ablation and elevation of the temporal lobe from the middle cranial fossa, and destruction of the upper part of the petrous bone, cholesteatoma involving the dura mater was confirmed (Fig. 3). The tegmen tympani was opened to secure operative view and extent of the entire cholesteatoma was confirmed. Cholesteatoma had extended into the labyrinth of the facial canal, impinging on the facial nerve from above and destroying surrounding bone. Cholesteatoma was removed along with perineurium of the facial nerve, which was adherent to the tumor. Neither adhesion to dura mater nor leakage of cerebrospinal fluid was observed, and the dead
Table 1 Electroneuronography (ENoG) Orbicularis oculi m. Nasalis m. Orbicularis oris m.
18 October 2000
22 December 2000
7 June 2001
5.9% 0.0 7.8
0.0% 0.0 8.7
7.3% 0.0 45.5
ENoG was performed on bilateral orbicularis oculi, orbicularis nasalis and orbicularis oris muscles. Amplitude was measured at three points and show the each calculated percentages.
1706
cavity did not fill after removal of cholesteatoma, return of the bone flap, and wound closure. CT at 3 years postoperatively shows that an airfilled cavity was apparent in the attic region on axial section. The wound cavity on coronal section displayed signal hyperintensity that was attributed to granulation tissue. The facial nerve paralysis showed partial recovery (Grade III by the House—Brackmann classification).
5. Discussion The current case was diagnosed as congenital cholesteatoma in the petrous apex, according to the criteria of Derlacki and Cleims [5]. Numerous theories have been proposed to explain the pathogenesis of congenital cholesteatoma. Paparella and Rybak [6] reported that congenital cholesteatoma develops from a remnant of ectodermal tissue at the junction between the first and second branchial arches, whereas Aimi [7] suggested the possibility that ectodermal tissue of the external ear canal migrates into the middle ear. Piza et al. [8] proposed that cells of amniotic origin undergo squamous metaplasia to form congenital cholesteatoma. Michaels presented another theory in 1986 [9] that congenital cholesteatoma arises from epidermoid cell rests formed before 33 weeks of gestation near the tympanic orifice of eustachian tube. However, the report by Tos in 2000 [10] raised doubts about these previous theories, and thus the pathogenesis of this disease remains controversial. Congenital cholesteatoma predominantly involves the anterosuperior quadrant of the middle ear. According to Parisier and Weiss [11], 65% of cases occur in the anterosuperior quadrant and 15% in the posterosuperior quadrant, whereas Friedberg [12] reported that 31% of these cases develop in the anterosuperior quadrant and 5% in the posterosuperior quadrant. It is difficult to determine the origin of petrous apex cholesteatoma as it usually manifests as a large mass when detected with clinical symptoms. The current case developed on the geniculate ganglion. Teed [13] reported that epithelial cells of ectodermal origin form stratified squamous epithelium adjacent to the ganglion of the facial nerve during the embryonic period, and Fish [14] suggested the possibility that proliferating basal cells of the flaccid part of the tympanic membrane migrate through the epitympanum toward the geniculate ganglion. As cholesteatoma is rarely identified during the early stage of growth, the current case may provide important insight into the pathogenesis of cholesteatoma in the petrous apex.
K. Yamazaki et al.
In 1963, Cawthorne [4] reported 13 cases of congenital petrous apex cholesteatoma and characterized the symptoms as: (1) slow progression of facial nerve paralysis; (2) hypoacusis on the affected side and (3) loss of caloric nystagmus, with a peak incidence at 35—55 years of age. In 36 cases of petrous apex cholesteatoma reported by Peron and Schuknecht in 1975 [15], the initial symptom was hypoacusis in 28 cases (78%) and facial nerve paralysis in seven cases (20%). In 1989, Glasscock et al. further reported [16] that petrous apex cholesteatoma first involves the internal auditory canal followed by an extension into the labyrinth, and thus retrocochlear hearing loss appears before the development of facial nerve paralysis. Hence, facial nerve paralysis tends to develop slowly, typically after middle age. The incidence of facial nerve paralysis has been reported to be 42.5% by Sanna et al. [17] and 62.5% by Magliulo et al. [18] The current case represents a rare instance in which facial nerve paralysis developed as the initial symptom, despite the relatively small size of the tumor and young age of the patient. The translabyrinthine approach and middle cranial fossa approach are the most common surgical procedures for petrous apex cholesteatoma. Other approaches, such as the posterior cranial fossa, transethmoidal—transsphenoidal, and transpalatal—transclival approaches, are no longer in common use, due to their numerous disadvantages including limited visualization of lesions and a high risk of postoperative infection. The translabyrinthine approach provides good visualization through antrotomy, allowing access to middle ear lesions. However, access to a tumor involving the superior petrous apex requires destruction of the labyrinth, which results in the impairment of labyrinthine function and makes it difficult to preserve hearing in patients with intact inner ear function. As an open wound cavity is difficult to manage postoperatively, the cavity is usually obliterated with fat or muscle. The middle cranial fossa approach, that provides good visualization of the petrous part, is suitable for lesions confined to the superior petrous apex and this technique preserves inner ear function while total removal of the matrix is required. Cholesteatoma in the present case was confined to the superior petrous apex, for which the middle cranial fossa approach was well indicated.
6. Conclusions A 1-year-old infant with congenital petrous apex cholesteatoma manifesting facial nerve paralysis was presented. Although such cases are extremely
Infantile congenital petrosal cholesteatoma
rare, physicians should be aware of such cases for the accurate diagnosis and treatment of this disease.
References [1] A.J. Maniglia, Petrous apex cholesteatoma with posterior or middle cranial fossa extension, in: Y. Nakano (Ed.), Cholesteatoma and Mastoid Surgery, Kugler Publ, Amsterdam, New York, 1993, pp. 475—481. [2] J.L. Sheehy, D.E. Brackmann, Cholesteatoma surgery: management of the labyrinthine fistula–—a report 97 cases, Laryngoscope 89 (1979) 78—87. [3] M. Sanna, R. Gamoletti, G. Frau, et al. Facial nerve palsy and petrous bone cholesteatoma, in: Y. Nakano (Ed.), Cholesteatoma and Mastoid Surgery, Kugler Publ, Amsterdam, New York, 1993, pp. 493—497. [4] T. Cawthorne, Congenital cholesteatoma, Arch. Otolaryng. 78 (1963) 248—252. [5] E.L. Derlacki, J.D. Clemis, Congenital cholesteatoma of the middle ear and Mastoid, Ann. Otol. Rhino. Laryngol. 74 (1965) 706—727. [6] M.M. Paparella, L. Rybak, Congenital cholesteatoma, Otolaryngol. Clin. North Am. 11 (1978) 113—120. [7] K. Aimi, Role of the tympanic ring in the pathogenesis of congenital cholesteatoma, Laryngoscope 93 (1983) 1140— 1146.
1707
[8] J. Piza, M. Gonzales, C.C. Northorp, R.D. Eavey, Meconium contamination of the neonatal ear, J. Pedatr. 115 (1989) 910—914. [9] L. Michaels, An epidermoid formation in the developing middle ear: possible source of cholesteatoma, J. Otolaryngol. 15 (1986) 169—174. [10] M. Tos, A new pathogenesis of mesotympanic (congenital) cholesteatoma, Laryngoscope 110 (2000) 1890— 1897. [11] S.C. Parisier, M.H. Weiss, Recidivism cholesteatoma surgery, Ear Nose Throat J. 70 (1991) 362—364. [12] J. Friedberg, Congenital cholesteatoma, Laryngoscope 104 (1994) 1—25. [13] W. Teed, Cholesteatoma verum tympani, Arch. Otolaryngol. 24 (1936) 455—474. [14] U. Fish, ‘Congenital’cholesteatomas of the supralabyrinthine region, Clin. Otolaryngol. 3 (1978) 369—376. [15] D.L. Peron, H.F. Schuknecht, Congenital cholesteatoma with other anomalies, Arch. Otolaryngol. 101 (1975) 498— 505. [16] M.E. Glasscock, C.I. Woods, D.S. Poe, et al. Petrous apex cholesteatoma, Otolaryngol. Clin. North Am. 22 (1989) 981— 1002. [17] M. Sanna, Z. Carlo, R. Gamoletti, et al. Petrous bone cholesteatoma, Skull. Base Surg. 3 (1993) 201—213. [18] G. Magliulo, G. Terranova, C. Sepe, et al. Petrous cholesteatoma and facial paralysis, Clin. Otolaryngol. 23 (1998) 253—258.
www.elsevier.com/locate/ijporl
CASE REPORT
Infantile congenital petrosal cholesteatoma: A case report and literature review Kazuharu Yamazaki a,*, Hiroaki Sato a, Kazuo Murai b, Kaoru Ogawa c a
Department of Otorhinolaryngology, Iwate Medical University, Uchimaru 19-1, Morioka, Iwate 020-8505, Japan b Department of Otorhinolaryngology, Iwate Rosai Hospital, Iwate, Japan c Department of Otorhinolaryngology, Keio Medical University, Tokyo, Japan Received 25 March 2005; accepted 8 April 2005
KEYWORDS Congenital petrosal cholesteatoma; Facial nerve paralysis; Infant
Summary Congenital cholesteatoma of the middle ear is frequently seen in younger children including infant. By contrast, that of the petrous bone is rarely seen in younger children. We report an exceptional case occurring in 23-month-old infant who presented with unilateral facial nerve paralysis at about 18-month-old. Computed tomography and magnetic resonance imaging revealed congenital cholesteatoma measuring approximately 2 cm in the area of the right geniculate ganglion. The mass was completely eradicated via the middle fossa approach, which allowed for preservation of hearing. The facial nerve maintained intact during surgery and paralysis showed partial recovery after the operation. To our knowledge, the present case seems to be the youngest case of congenital petrosal cholesteatoma reported, and also demonstrates congenital petrosal cholesteatoma could exhibit facial nerve paralysis in early childhood. # 2005 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Tumors occurring in the petrous part of the temporal bone are predominantly vestibular schwannoma, with cholesteatoma only comprising 4% [1]. Moreover, petrosal cholesteatoma accounts for only 1—3% of all cholesteatomas and is comparatively rare [2,3]. The petrous bone is considered a ‘‘silent area’’, and tumors in the region tend to be asymptomatic in the
* Corresponding author. Tel.: +81 19 651 5111; fax: +81 19 652 8642.
early stage. Many cases thus display extensive expansion of the tumor by the time symptoms appear. Compared with cholesteatomas in other regions, facial nerve paralysis is considered characteristic for petrosal cholesteatoma. Cawthorne [4] described facial nerve paralysis as progressing gently and quietly in petrosal cholesteatoma, representing a key point of difference to vestibular schwannoma. We encountered a case of petrosal cholesteatoma in a 23-month-old girl who developed facial nerve paralysis at 18-months-old. The tumor was removed via the middle cranial fossa approach. This case is thought to be very rare given the age of the patient at
0165-5876/$ — see front matter # 2005 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijporl.2005.04.027
1704
appearance of facial nerve paralysis, and we discuss the case with reference to the existing literature.
K. Yamazaki et al.
lagopthalmos was not seen and no leakage of food from the angle of the mouth was present. The facial nerve paralysis was Grade IV by the House—Brackmann classification.
2. Case report 3.2. CT The patient was a 23-month-old girl with no family or previous medical history of note, and no previous episodes of myringitis. Her mother had noticed that movements of the right face decreased at about 18months-old, and consulted a public health nurse. However, symptoms were attributed to a habit, and no further action was taken. The patient developed chicken pox at 21-month-old, and underwent a checkup at a nearby pediatric clinic. At this time, right facial nerve paralysis was noted, and the patient was referred to our clinic and hospitalized in early 2001 for surgical intervention.
3. Laboratory findings 3.1. Otorhinolaryngological findings No abnormalities were identified in the ear, nose or pharynx. Movement of the right face was poor, but
An expansive lesion was identified above the petrous bone on coronal section and forward of the petrous bone on axial CT (Fig. 1A and B). No relation to the middle ear was apparent. Development of the mastoid air cells was good. A round destruction image was seen above the acoustic meatus in the middle fossa of the petrous bone, on the superolateral aspect of the bone on three-dimensional CT (Fig. 1C).
3.3. MRI The tumor displayed intermediate intensity on axial sections from T1-imaging, with one region of high intensity. On coronal T2-imaging, the tumor displayed a region of slight hyperintensity above the cochlea and involving the dura mater. Invasion into the brain tissue was not seen (Fig. 2A and B). Cholesteatoma, cholesterin granuloma, mucocele,
Fig. 1 CT scan of the temporal bone. An expansive lesion was identified above the petrous bone on coronal section (A) and forward of the petrous bone on axial section (B). A round destruction image was seen above the acoustic meatus in the middle fossa of the petrous bone, on the superolateral aspect of the bone on three-dimensional CT (C).
Infantile congenital petrosal cholesteatoma
1705
Fig. 3 Perioperative photography. Middle fossa exposure of the cholesteatoma in the petrous apex.
for the orbicularis oculi, 0% for the nasalis, and 7.8% for the orbicularis oris.
3.5. Auditory brainstem response Auditory brainstem response (ABR) was measured three times: once preoperatively and twice postoperatively. Latency was not extended, and thresholds were normal, with 10 dB in the right ear and 5 dB in the left. Fig. 2 MRI of the inner ear. The tumor displayed intermediate intensity on axial sections from T1-imaging (A), with one region of high intensity. On coronal T2-imaging (B), the tumor displayed a region of slight hyperintensity above the cochlea and involving the dura mater.
and schwannoma were considered as differential diagnoses following CT and MRI.
3.4. Electroneuronography Electroneuronography (ENoG) was performed on bilateral orbicularis oculi, nasalis (both M. compressor naris and M. dilator naris), and orbicularis oris muscles. Amplitude (mV) was measured at three points (L1: standing, L2: top, L3: bottom) and differences (L1 L2, L2 L3, L1 L3) were calculated and compared for both raw values and calculated percentages. Mean preoperative ENoG values (Table 1) on the affected side were 5.9%
4. Treatment Surgical intervention via the middle cranial fossa on the affected (left) side was scheduled for 6 days after admission. After removal of a bone flap, ablation and elevation of the temporal lobe from the middle cranial fossa, and destruction of the upper part of the petrous bone, cholesteatoma involving the dura mater was confirmed (Fig. 3). The tegmen tympani was opened to secure operative view and extent of the entire cholesteatoma was confirmed. Cholesteatoma had extended into the labyrinth of the facial canal, impinging on the facial nerve from above and destroying surrounding bone. Cholesteatoma was removed along with perineurium of the facial nerve, which was adherent to the tumor. Neither adhesion to dura mater nor leakage of cerebrospinal fluid was observed, and the dead
Table 1 Electroneuronography (ENoG) Orbicularis oculi m. Nasalis m. Orbicularis oris m.
18 October 2000
22 December 2000
7 June 2001
5.9% 0.0 7.8
0.0% 0.0 8.7
7.3% 0.0 45.5
ENoG was performed on bilateral orbicularis oculi, orbicularis nasalis and orbicularis oris muscles. Amplitude was measured at three points and show the each calculated percentages.
1706
cavity did not fill after removal of cholesteatoma, return of the bone flap, and wound closure. CT at 3 years postoperatively shows that an airfilled cavity was apparent in the attic region on axial section. The wound cavity on coronal section displayed signal hyperintensity that was attributed to granulation tissue. The facial nerve paralysis showed partial recovery (Grade III by the House—Brackmann classification).
5. Discussion The current case was diagnosed as congenital cholesteatoma in the petrous apex, according to the criteria of Derlacki and Cleims [5]. Numerous theories have been proposed to explain the pathogenesis of congenital cholesteatoma. Paparella and Rybak [6] reported that congenital cholesteatoma develops from a remnant of ectodermal tissue at the junction between the first and second branchial arches, whereas Aimi [7] suggested the possibility that ectodermal tissue of the external ear canal migrates into the middle ear. Piza et al. [8] proposed that cells of amniotic origin undergo squamous metaplasia to form congenital cholesteatoma. Michaels presented another theory in 1986 [9] that congenital cholesteatoma arises from epidermoid cell rests formed before 33 weeks of gestation near the tympanic orifice of eustachian tube. However, the report by Tos in 2000 [10] raised doubts about these previous theories, and thus the pathogenesis of this disease remains controversial. Congenital cholesteatoma predominantly involves the anterosuperior quadrant of the middle ear. According to Parisier and Weiss [11], 65% of cases occur in the anterosuperior quadrant and 15% in the posterosuperior quadrant, whereas Friedberg [12] reported that 31% of these cases develop in the anterosuperior quadrant and 5% in the posterosuperior quadrant. It is difficult to determine the origin of petrous apex cholesteatoma as it usually manifests as a large mass when detected with clinical symptoms. The current case developed on the geniculate ganglion. Teed [13] reported that epithelial cells of ectodermal origin form stratified squamous epithelium adjacent to the ganglion of the facial nerve during the embryonic period, and Fish [14] suggested the possibility that proliferating basal cells of the flaccid part of the tympanic membrane migrate through the epitympanum toward the geniculate ganglion. As cholesteatoma is rarely identified during the early stage of growth, the current case may provide important insight into the pathogenesis of cholesteatoma in the petrous apex.
K. Yamazaki et al.
In 1963, Cawthorne [4] reported 13 cases of congenital petrous apex cholesteatoma and characterized the symptoms as: (1) slow progression of facial nerve paralysis; (2) hypoacusis on the affected side and (3) loss of caloric nystagmus, with a peak incidence at 35—55 years of age. In 36 cases of petrous apex cholesteatoma reported by Peron and Schuknecht in 1975 [15], the initial symptom was hypoacusis in 28 cases (78%) and facial nerve paralysis in seven cases (20%). In 1989, Glasscock et al. further reported [16] that petrous apex cholesteatoma first involves the internal auditory canal followed by an extension into the labyrinth, and thus retrocochlear hearing loss appears before the development of facial nerve paralysis. Hence, facial nerve paralysis tends to develop slowly, typically after middle age. The incidence of facial nerve paralysis has been reported to be 42.5% by Sanna et al. [17] and 62.5% by Magliulo et al. [18] The current case represents a rare instance in which facial nerve paralysis developed as the initial symptom, despite the relatively small size of the tumor and young age of the patient. The translabyrinthine approach and middle cranial fossa approach are the most common surgical procedures for petrous apex cholesteatoma. Other approaches, such as the posterior cranial fossa, transethmoidal—transsphenoidal, and transpalatal—transclival approaches, are no longer in common use, due to their numerous disadvantages including limited visualization of lesions and a high risk of postoperative infection. The translabyrinthine approach provides good visualization through antrotomy, allowing access to middle ear lesions. However, access to a tumor involving the superior petrous apex requires destruction of the labyrinth, which results in the impairment of labyrinthine function and makes it difficult to preserve hearing in patients with intact inner ear function. As an open wound cavity is difficult to manage postoperatively, the cavity is usually obliterated with fat or muscle. The middle cranial fossa approach, that provides good visualization of the petrous part, is suitable for lesions confined to the superior petrous apex and this technique preserves inner ear function while total removal of the matrix is required. Cholesteatoma in the present case was confined to the superior petrous apex, for which the middle cranial fossa approach was well indicated.
6. Conclusions A 1-year-old infant with congenital petrous apex cholesteatoma manifesting facial nerve paralysis was presented. Although such cases are extremely
Infantile congenital petrosal cholesteatoma
rare, physicians should be aware of such cases for the accurate diagnosis and treatment of this disease.
References [1] A.J. Maniglia, Petrous apex cholesteatoma with posterior or middle cranial fossa extension, in: Y. Nakano (Ed.), Cholesteatoma and Mastoid Surgery, Kugler Publ, Amsterdam, New York, 1993, pp. 475—481. [2] J.L. Sheehy, D.E. Brackmann, Cholesteatoma surgery: management of the labyrinthine fistula–—a report 97 cases, Laryngoscope 89 (1979) 78—87. [3] M. Sanna, R. Gamoletti, G. Frau, et al. Facial nerve palsy and petrous bone cholesteatoma, in: Y. Nakano (Ed.), Cholesteatoma and Mastoid Surgery, Kugler Publ, Amsterdam, New York, 1993, pp. 493—497. [4] T. Cawthorne, Congenital cholesteatoma, Arch. Otolaryng. 78 (1963) 248—252. [5] E.L. Derlacki, J.D. Clemis, Congenital cholesteatoma of the middle ear and Mastoid, Ann. Otol. Rhino. Laryngol. 74 (1965) 706—727. [6] M.M. Paparella, L. Rybak, Congenital cholesteatoma, Otolaryngol. Clin. North Am. 11 (1978) 113—120. [7] K. Aimi, Role of the tympanic ring in the pathogenesis of congenital cholesteatoma, Laryngoscope 93 (1983) 1140— 1146.
1707
[8] J. Piza, M. Gonzales, C.C. Northorp, R.D. Eavey, Meconium contamination of the neonatal ear, J. Pedatr. 115 (1989) 910—914. [9] L. Michaels, An epidermoid formation in the developing middle ear: possible source of cholesteatoma, J. Otolaryngol. 15 (1986) 169—174. [10] M. Tos, A new pathogenesis of mesotympanic (congenital) cholesteatoma, Laryngoscope 110 (2000) 1890— 1897. [11] S.C. Parisier, M.H. Weiss, Recidivism cholesteatoma surgery, Ear Nose Throat J. 70 (1991) 362—364. [12] J. Friedberg, Congenital cholesteatoma, Laryngoscope 104 (1994) 1—25. [13] W. Teed, Cholesteatoma verum tympani, Arch. Otolaryngol. 24 (1936) 455—474. [14] U. Fish, ‘Congenital’cholesteatomas of the supralabyrinthine region, Clin. Otolaryngol. 3 (1978) 369—376. [15] D.L. Peron, H.F. Schuknecht, Congenital cholesteatoma with other anomalies, Arch. Otolaryngol. 101 (1975) 498— 505. [16] M.E. Glasscock, C.I. Woods, D.S. Poe, et al. Petrous apex cholesteatoma, Otolaryngol. Clin. North Am. 22 (1989) 981— 1002. [17] M. Sanna, Z. Carlo, R. Gamoletti, et al. Petrous bone cholesteatoma, Skull. Base Surg. 3 (1993) 201—213. [18] G. Magliulo, G. Terranova, C. Sepe, et al. Petrous cholesteatoma and facial paralysis, Clin. Otolaryngol. 23 (1998) 253—258.