Internotionol Journol of Pediatric Otorhinolotyngology, 21 (1991) 79-83 0 1991 Elsevier Science Publishers B.V. 0165-5876/91/%03.50
PEDOT
19
00689
Case Report
Cerebrospinal fluid otorrhea at myringotomy. A meningocele through a defect in the tegmen Nicholas Deportment
of Otolotyngologv,
S. Jones
Guy’s and Lewishorn Hospitals,
London SEI 9RT (U.K.)
(Received 29 June 1990) (Accepted 22 October 1990)
Key words: Cerebrospinal
fluid otorrhea;
Temporal
bone;
Meningocele;
Myringotomy
Abstract
Spontaneous cerebrospinal fistulae can simulate secretory otitis media or be discovered at myringotomy, but the diagnosis is frequently made after one or more episodes of meningitis. Congenital perilabyrinthine fistulae are extremely rare, and unlike translabyrinthine fistulae there is usually no sensorineural hearing loss. This is the first reported case of a congenital meningocele through the tegmen in a child. An 11-year-old boy presented with signs and symptoms which mimicked serous otitis media. A cerebrospinal fistula was produced at myringotomy and this was closed surgically via an endaural approach. A CT scan defined a defect in the tegmen tympani anterior to the superior semicircular canal.
Introduction
Acquired cerebrospinal otorrhea is far more common than congenital otorrhea, and it usually results from trauma, tumor or surgery [6]. Congenital cerebrospinal fistulae are less common and can present as rhinorrhoea if the eardrum is intact, as otorrhea if there is a perforation, at myringotomy for a presumed serous otitis media [6,17,22,24], or by repeated attacks of meningitis [3,13,20]. Cerebrospinal otorrhea in children is usually associated with a defect in the otic capsule [6,9,21,22,24], a cochlear hearing loss, recurrent meningitis [3], and occasionally with vestibular problems. They often present before the age of 6 years and are found to have a sensorineural hearing loss associated with a congenital defect of the labyrinth. The
Correspondence:
N.S. Jones, 98, Red Lion Lane, Plumstead,
London
SE18 4LE, U.K.
80
labyrinthine deformity is usually more severe than that described by Mondini [21]. Congenital perilabyrinthine fistulae are extremely rare [21], and cochlear function is usually preserved. Although unusual, anatomical defects have been described at Hyrtl’s fissure, the facial canal, the petrous apex, the posterior fossae, the petromastoid suture, the internal auditory meatus and less commonly the cochlear aqueduct [6,7,9,21,22]. Reports differ as to the frequency of anatomical defects in the tegmen, from very few [ll] to 20-34s [2,12]. While defects in the tegmen leading to spontaneous CSF otorrhea have been described in adults [5,6,11,14,15, 17,181, none has been reported in children. Meningoceles and encephaloceles in the middle ear have been reported in adults [4,6,15,19] and are usually a result of trauma or mastoid surgery. Congenital meningoceles in children are extremely rare, although one has been described through Hyrtl’s fissure [7]. There is a single report of an encephalocele in the middle ear [8], without a history of previous trauma or mastoid surgery.
Case report
An ll-year-old boy presented with a 7-year-history suggestive of recurrent secretory otitis media. At the age of 5 he had been referred to an otolaryngologist with a year’s history of snoring, mouth breathing, recurrent tonsillitis, and poor hearing. An audiogram showed a low frequency left-sided hearing loss of 45 dB with a right-sided loss of 30dB with bilateral flat tympanograms. After 4 months of conservative management he underwent adenotonsillectomy and insertion of ventilation tubes. At follow-up his parents reported an improvement in his hearing, but while audiometry showed normal right-sided hearing there was a residual left-sided loss of 45dB below 2 kHz. No further action was taken at that time except for 9 monthly reviews. Both ventilation tubes extruded after approximately one year. He continued to have a history of a fluctuating hearing loss after colds which resolved spontaneously. Further audiograms over the next 4 years showed a fluctuating loss in the right ear while the left remained unchanged with a low frequency conductive loss confirmed by bone conduction testing. He then developed a persistent bilateral conductive loss with an otoscopic appearance of secretory otitis media and was listed for insertion of ventilation tubes. There was no history of any head injury and the family history was clear. At myringotomy the right ear was found to have thin yellow fluid which was aspirated and a ventilation tube was inserted. The appearance of the left tympanic membrane was slightly opaque and consistent with secretory otitis media. At myringotomy there was a gush of clear fluid with approximately 10 ml of fluid dripping out of the ear in the first two min. An attempt was made to stop the drainage of fluid by packing the ear canal with gelatin sponge as well as elevating the head of the table to reduce fluid pressure. These attempts failed and clear fluid continued to ooze out of the ear. At exploratory tympanotomy the middle ear segment was found to contain a dehiscence of dura which draped itself over the ossicles and was readily peeled off these and the inner surface of the tympanic membrane. Some of the thin
Fig 1. CT scan. Coronal
section just anterior
to the left cochlea
showing
a defect in the tegmen
white sac was sent for histology and was later reported as being ‘fibrous tissue lined by plump mesothelial like cells in keeping with dura’. The sac had a broad neck based on the tegmen and cerebrospinal fluid drained freely from a breach in the dura. The dura was pushed upwards and a temporalis fascia graft was supported by muscle and fat in the mesotympanum; this stopped the fistulous leak. Prophylactic antibiotics were given to prevent meningitis and he made an uneventful recovery. A CT scan was done to define the anatomical bony defect. It showed a large defect of the tegmen tympani (Fig. 1). There was no evidence of any ventricular enlargement and the bony labyrinth was normal. At a two year follow-up he has a 30 dB conductive hearing loss in his left ear without any other symptoms or episodes of meningitis.
Discussion Recognising a cerebrospinal fistula is clinically significant primarily because of the potential of developing meningitis. Although unusual, cerebrospinal fistula can feign a serous otitis media. In children this is most likely to be associated with a sensoineural hearing loss as the majority have translabyrinthine fistulae t&9,21,22,24]. Perilabyrinthine anatomical defects are rare although several routes have been described [6,7,9,21,22]. Defects of the tegmen leading to spontaneous
82
CSF otorrhea have been described in adults [5,6,11,14,15,17,18,23]. None have previously been reported in children. Meningoceles and encephaloceles in the middle ear have been reported in adults [4,6,15,16,19] but meningoceles in children are extremely rare, although one has been described through Hyrtl’s fissure [7]. This case adds to the few previous reports describing CSF otorrhea secondary to perilabyrinthine fistulae. However, it is unique in that it occurred in a child, through a defect in the tegmen, and that it was a meningocele. Establishing that otorrhea is cerebrospinal fluid can be done by immunofixation when there is any doubt [lo], as the use of glucose oxidase paper is inaccurate [9]. Pinpointing the site of a fistula is best done by high resolution CT scan [6] combined with intrathecal metrizamide if necessary [18]. Intrathecal 5% fluorescein pre- or intraoperatively can help locate the site of a fistula [9,19] but this was unnecessary in this case. Defects in the tegmen have been repaired via the middle cranial fossa, the mastoid [7] (if the lesion is posterior to the superior semicircular canal), or via a minicraniotomy [1,6]. We have sought neurosurgical advice which recommended no further intervention. However, his family has been made aware of theoretical potential for infection to spread from the middle ear to the meninges. The patient remains asymptomatic two years after surgery except for an ipsilateral 30 dB conductive hearing loss.
Acknowledgement
Mr. T.M. Harris, Consultant case.
Otolaryngologist,
for allowing me to publish this
References 1 Adkins, W.Y. Osguthorpe, J.D., Mini-craniotomy management of CSF otorrhea from tegmen defects, Laryngoscope 93 (1983) 1038-1040. 2 Ahren, C. and Thuhn, C.A., Lethal intracranial complications following innation in treatment of serous otitis media and due to defects in the petrous bone, Acta Otolaryngol., 60 (1965) 407-421. 3 Bottema, T., Spontaneous cerebrospinal fluid otorrhea, Arch. Otolaryngol., 101 (1975) 693-694. 4 Dedo, H.H. and Sooy, F.A., Endaural brain hernia (encephalocele) diagnosis and treatment, Laryngoscope, 80 (1970) 1090-1099. 5 Dysart, B.R., Spontaneous cerebrospinal otorrhea, Laryngoscope, 69 (1959) 9355939. 6 Ferguson, B.J., Wilkins, R.H., Hudson, W. and Farmer, J., Spontaneous CSF otorrhea from tegmen and posterior fossa defects, Laryngoscope, 96 (1986) 635-644. 7 Gacek, R.R. and Leipzig B., Congenital cerebrospinal otorrhea, Ann. Otol., 88 (1979) 358-365. 8 Gavilan, J., Trujillo, M. and Gavilan, C., Spontaneous encephalocele of the middle ear, Arch. Otolaryngol., 110 (1984) 206-207. 9 Hicks, G.W., Wright Jr., J.W. and Wright III, J.W., Cerebrospinal fluid otorrhea, Laryngoscope, 90, Suppl 25, No 11, pt 2, (1980) l-25. 10 IrjaIa, K., Suonpaa, J. and Laurent, B., Identification of CSF leakage by immunofixation, Arch. Otolaryngol., 105 (1979) 447-448.
83 11 Jahrsdoerfer, R.A., Richtsmeier, W.1. and Cantrell, R-W., Spontaneous CSF rhinorrhea, Arch. Otolaryngol., 107 (1981) 257-260. 12 Kapur, T.R. and Bangash, W., Tegmental and petromastoid defects in the temporal bone. J. Laryngol. Otol., 100 (1986) 1129-1132. 13 Kaseff, L.G., Nieberding, P.H., Shorago G.W. and Huertas, G., Fistula between the middle ear and subarachnoid space as a cause of recurrent meningitis: detection by means of thin-section, complexmotion tomography, Radiology, 135 (1980) 105-108. 14 Kaufman, B., Nulsen F.E., Weiss, M.H., Brodkey IS., White, R.J. and Sykora, G.F., Acquired spontaneous, nontraumatic normal-pressure cerebrospinal fluid fistulas originating from the middle fossa, Radiology, 122 (1977) 379-387. 15 Kline, O.R., Spontaneous cerebrospinal otorrhea, Arch. Otolaryngol, 18 (1933) 34-39. 16 Koch, H., Meningocele of the temporal bone, Acta. Otolaryngol, 38 (1950) 59-61. 17 Kramer, S.A., Yanagisawa, E. and Smith, H.W., Spontaneous cerebrospinal fluid otorrhea simulating serous otitis media, Laryngoscope, 81 (1971) 1083-1089. 18 Myer, C.M., Miller, G.W. and Ball, J.B., X-Ray study of the month-Spontaneous cerebrospinal fluid otorrhea, Ann. Otol. Rhinol. Laryngol., 94 (1985) 96-97. 19 Neely, J.G., Neblett, C.R. and Rose, J.E., Diagnosis and treatment of spontaneous cerebrospinal fluid otorrhea, Laryngoscope, 92 (1982) 609-642. 20 Nenzelius, C., On spontaneous cerebrospinal otorrhea due to congenital malformations. Acta Otolaryngol, 39 (1951) 314-328. 21 Phelps, P., Congenital cerebrospinal fluid fistulae of the petrous temporal bone, Clin. Otolaryngol.. 11 (1986) 79-92. 22 Ryczko, B., Brodsky, L., Stanievich, J.F. and Pordell, R., Spontaneous cerebrospinal fluid otorrhea in a deaf infant, Int. J. Pediatr. Otorhinolaryngol., 16 (1988) 245-251. 23 Wetmore, S.J., Herrmann, P. and Fisch, U., Spontaneous cerebrospinal fluid otorrhea. Am. J. Otol.. 8 (1987) 96- 102. 24 Wolfowitz. B.. Spontaneous CSF otorrhea simulating serous otitis. Arch. Otolaryngol., 105 (1979) 496-499.
PEDOT
19
00689
Case Report
Cerebrospinal fluid otorrhea at myringotomy. A meningocele through a defect in the tegmen Nicholas Deportment
of Otolotyngologv,
S. Jones
Guy’s and Lewishorn Hospitals,
London SEI 9RT (U.K.)
(Received 29 June 1990) (Accepted 22 October 1990)
Key words: Cerebrospinal
fluid otorrhea;
Temporal
bone;
Meningocele;
Myringotomy
Abstract
Spontaneous cerebrospinal fistulae can simulate secretory otitis media or be discovered at myringotomy, but the diagnosis is frequently made after one or more episodes of meningitis. Congenital perilabyrinthine fistulae are extremely rare, and unlike translabyrinthine fistulae there is usually no sensorineural hearing loss. This is the first reported case of a congenital meningocele through the tegmen in a child. An 11-year-old boy presented with signs and symptoms which mimicked serous otitis media. A cerebrospinal fistula was produced at myringotomy and this was closed surgically via an endaural approach. A CT scan defined a defect in the tegmen tympani anterior to the superior semicircular canal.
Introduction
Acquired cerebrospinal otorrhea is far more common than congenital otorrhea, and it usually results from trauma, tumor or surgery [6]. Congenital cerebrospinal fistulae are less common and can present as rhinorrhoea if the eardrum is intact, as otorrhea if there is a perforation, at myringotomy for a presumed serous otitis media [6,17,22,24], or by repeated attacks of meningitis [3,13,20]. Cerebrospinal otorrhea in children is usually associated with a defect in the otic capsule [6,9,21,22,24], a cochlear hearing loss, recurrent meningitis [3], and occasionally with vestibular problems. They often present before the age of 6 years and are found to have a sensorineural hearing loss associated with a congenital defect of the labyrinth. The
Correspondence:
N.S. Jones, 98, Red Lion Lane, Plumstead,
London
SE18 4LE, U.K.
80
labyrinthine deformity is usually more severe than that described by Mondini [21]. Congenital perilabyrinthine fistulae are extremely rare [21], and cochlear function is usually preserved. Although unusual, anatomical defects have been described at Hyrtl’s fissure, the facial canal, the petrous apex, the posterior fossae, the petromastoid suture, the internal auditory meatus and less commonly the cochlear aqueduct [6,7,9,21,22]. Reports differ as to the frequency of anatomical defects in the tegmen, from very few [ll] to 20-34s [2,12]. While defects in the tegmen leading to spontaneous CSF otorrhea have been described in adults [5,6,11,14,15, 17,181, none has been reported in children. Meningoceles and encephaloceles in the middle ear have been reported in adults [4,6,15,19] and are usually a result of trauma or mastoid surgery. Congenital meningoceles in children are extremely rare, although one has been described through Hyrtl’s fissure [7]. There is a single report of an encephalocele in the middle ear [8], without a history of previous trauma or mastoid surgery.
Case report
An ll-year-old boy presented with a 7-year-history suggestive of recurrent secretory otitis media. At the age of 5 he had been referred to an otolaryngologist with a year’s history of snoring, mouth breathing, recurrent tonsillitis, and poor hearing. An audiogram showed a low frequency left-sided hearing loss of 45 dB with a right-sided loss of 30dB with bilateral flat tympanograms. After 4 months of conservative management he underwent adenotonsillectomy and insertion of ventilation tubes. At follow-up his parents reported an improvement in his hearing, but while audiometry showed normal right-sided hearing there was a residual left-sided loss of 45dB below 2 kHz. No further action was taken at that time except for 9 monthly reviews. Both ventilation tubes extruded after approximately one year. He continued to have a history of a fluctuating hearing loss after colds which resolved spontaneously. Further audiograms over the next 4 years showed a fluctuating loss in the right ear while the left remained unchanged with a low frequency conductive loss confirmed by bone conduction testing. He then developed a persistent bilateral conductive loss with an otoscopic appearance of secretory otitis media and was listed for insertion of ventilation tubes. There was no history of any head injury and the family history was clear. At myringotomy the right ear was found to have thin yellow fluid which was aspirated and a ventilation tube was inserted. The appearance of the left tympanic membrane was slightly opaque and consistent with secretory otitis media. At myringotomy there was a gush of clear fluid with approximately 10 ml of fluid dripping out of the ear in the first two min. An attempt was made to stop the drainage of fluid by packing the ear canal with gelatin sponge as well as elevating the head of the table to reduce fluid pressure. These attempts failed and clear fluid continued to ooze out of the ear. At exploratory tympanotomy the middle ear segment was found to contain a dehiscence of dura which draped itself over the ossicles and was readily peeled off these and the inner surface of the tympanic membrane. Some of the thin
Fig 1. CT scan. Coronal
section just anterior
to the left cochlea
showing
a defect in the tegmen
white sac was sent for histology and was later reported as being ‘fibrous tissue lined by plump mesothelial like cells in keeping with dura’. The sac had a broad neck based on the tegmen and cerebrospinal fluid drained freely from a breach in the dura. The dura was pushed upwards and a temporalis fascia graft was supported by muscle and fat in the mesotympanum; this stopped the fistulous leak. Prophylactic antibiotics were given to prevent meningitis and he made an uneventful recovery. A CT scan was done to define the anatomical bony defect. It showed a large defect of the tegmen tympani (Fig. 1). There was no evidence of any ventricular enlargement and the bony labyrinth was normal. At a two year follow-up he has a 30 dB conductive hearing loss in his left ear without any other symptoms or episodes of meningitis.
Discussion Recognising a cerebrospinal fistula is clinically significant primarily because of the potential of developing meningitis. Although unusual, cerebrospinal fistula can feign a serous otitis media. In children this is most likely to be associated with a sensoineural hearing loss as the majority have translabyrinthine fistulae t&9,21,22,24]. Perilabyrinthine anatomical defects are rare although several routes have been described [6,7,9,21,22]. Defects of the tegmen leading to spontaneous
82
CSF otorrhea have been described in adults [5,6,11,14,15,17,18,23]. None have previously been reported in children. Meningoceles and encephaloceles in the middle ear have been reported in adults [4,6,15,16,19] but meningoceles in children are extremely rare, although one has been described through Hyrtl’s fissure [7]. This case adds to the few previous reports describing CSF otorrhea secondary to perilabyrinthine fistulae. However, it is unique in that it occurred in a child, through a defect in the tegmen, and that it was a meningocele. Establishing that otorrhea is cerebrospinal fluid can be done by immunofixation when there is any doubt [lo], as the use of glucose oxidase paper is inaccurate [9]. Pinpointing the site of a fistula is best done by high resolution CT scan [6] combined with intrathecal metrizamide if necessary [18]. Intrathecal 5% fluorescein pre- or intraoperatively can help locate the site of a fistula [9,19] but this was unnecessary in this case. Defects in the tegmen have been repaired via the middle cranial fossa, the mastoid [7] (if the lesion is posterior to the superior semicircular canal), or via a minicraniotomy [1,6]. We have sought neurosurgical advice which recommended no further intervention. However, his family has been made aware of theoretical potential for infection to spread from the middle ear to the meninges. The patient remains asymptomatic two years after surgery except for an ipsilateral 30 dB conductive hearing loss.
Acknowledgement
Mr. T.M. Harris, Consultant case.
Otolaryngologist,
for allowing me to publish this
References 1 Adkins, W.Y. Osguthorpe, J.D., Mini-craniotomy management of CSF otorrhea from tegmen defects, Laryngoscope 93 (1983) 1038-1040. 2 Ahren, C. and Thuhn, C.A., Lethal intracranial complications following innation in treatment of serous otitis media and due to defects in the petrous bone, Acta Otolaryngol., 60 (1965) 407-421. 3 Bottema, T., Spontaneous cerebrospinal fluid otorrhea, Arch. Otolaryngol., 101 (1975) 693-694. 4 Dedo, H.H. and Sooy, F.A., Endaural brain hernia (encephalocele) diagnosis and treatment, Laryngoscope, 80 (1970) 1090-1099. 5 Dysart, B.R., Spontaneous cerebrospinal otorrhea, Laryngoscope, 69 (1959) 9355939. 6 Ferguson, B.J., Wilkins, R.H., Hudson, W. and Farmer, J., Spontaneous CSF otorrhea from tegmen and posterior fossa defects, Laryngoscope, 96 (1986) 635-644. 7 Gacek, R.R. and Leipzig B., Congenital cerebrospinal otorrhea, Ann. Otol., 88 (1979) 358-365. 8 Gavilan, J., Trujillo, M. and Gavilan, C., Spontaneous encephalocele of the middle ear, Arch. Otolaryngol., 110 (1984) 206-207. 9 Hicks, G.W., Wright Jr., J.W. and Wright III, J.W., Cerebrospinal fluid otorrhea, Laryngoscope, 90, Suppl 25, No 11, pt 2, (1980) l-25. 10 IrjaIa, K., Suonpaa, J. and Laurent, B., Identification of CSF leakage by immunofixation, Arch. Otolaryngol., 105 (1979) 447-448.
83 11 Jahrsdoerfer, R.A., Richtsmeier, W.1. and Cantrell, R-W., Spontaneous CSF rhinorrhea, Arch. Otolaryngol., 107 (1981) 257-260. 12 Kapur, T.R. and Bangash, W., Tegmental and petromastoid defects in the temporal bone. J. Laryngol. Otol., 100 (1986) 1129-1132. 13 Kaseff, L.G., Nieberding, P.H., Shorago G.W. and Huertas, G., Fistula between the middle ear and subarachnoid space as a cause of recurrent meningitis: detection by means of thin-section, complexmotion tomography, Radiology, 135 (1980) 105-108. 14 Kaufman, B., Nulsen F.E., Weiss, M.H., Brodkey IS., White, R.J. and Sykora, G.F., Acquired spontaneous, nontraumatic normal-pressure cerebrospinal fluid fistulas originating from the middle fossa, Radiology, 122 (1977) 379-387. 15 Kline, O.R., Spontaneous cerebrospinal otorrhea, Arch. Otolaryngol, 18 (1933) 34-39. 16 Koch, H., Meningocele of the temporal bone, Acta. Otolaryngol, 38 (1950) 59-61. 17 Kramer, S.A., Yanagisawa, E. and Smith, H.W., Spontaneous cerebrospinal fluid otorrhea simulating serous otitis media, Laryngoscope, 81 (1971) 1083-1089. 18 Myer, C.M., Miller, G.W. and Ball, J.B., X-Ray study of the month-Spontaneous cerebrospinal fluid otorrhea, Ann. Otol. Rhinol. Laryngol., 94 (1985) 96-97. 19 Neely, J.G., Neblett, C.R. and Rose, J.E., Diagnosis and treatment of spontaneous cerebrospinal fluid otorrhea, Laryngoscope, 92 (1982) 609-642. 20 Nenzelius, C., On spontaneous cerebrospinal otorrhea due to congenital malformations. Acta Otolaryngol, 39 (1951) 314-328. 21 Phelps, P., Congenital cerebrospinal fluid fistulae of the petrous temporal bone, Clin. Otolaryngol.. 11 (1986) 79-92. 22 Ryczko, B., Brodsky, L., Stanievich, J.F. and Pordell, R., Spontaneous cerebrospinal fluid otorrhea in a deaf infant, Int. J. Pediatr. Otorhinolaryngol., 16 (1988) 245-251. 23 Wetmore, S.J., Herrmann, P. and Fisch, U., Spontaneous cerebrospinal fluid otorrhea. Am. J. Otol.. 8 (1987) 96- 102. 24 Wolfowitz. B.. Spontaneous CSF otorrhea simulating serous otitis. Arch. Otolaryngol., 105 (1979) 496-499.
