- Email: [email protected]
Adult onset spontaneous CSF otorrhea with oval window fistula and recurrent meningitis: MRI findings
Adult onset spontaneous CSF otorrhea with oval window fistula and recurrent meningitis: MRI findings V. RUPA, DLO, MS, (ENT), A. JOB, DLO, MS, (ENT), and V. RAJSHEKHAR, MCh, (NEURO),* Tamil Nadu, India
S
pontaneous cerebrospinal fluid (CSF) otorrhea as the result of a congenital dural fistula causing recurrent meningitis is a rare entity. Two distinct clinical presentations of this condition have been described, based on single case reports and a small series of patients published over the last century.1,2 The more common juvenile variety, reportedly seen in 72% of cases, is represented by an infant or young child with a history of recurrent episodes of meningitis, unilateral, or bilateral severe to profound sensorineural hearing loss and an inner ear anomaly, typically, Mondini’s dysplasia.3 In addition, a history of recurrent middle ear infections or frank CSF otorrhea after myringotomy for suspected middle ear effusion may be present.2 An oval window fistula and/or enlarged cochlear aqueduct are often associated findings.3 In contrast, adult onset CSF otorrhea, a considerably rarer clinical entity, is typically seen in an older patient, aged 40 years or more, with a congenital tegmen or posterior fossa defect through which dura and brain may herniate.1-3 Either middle ear effusion or CSF otorrhea (or otorhinorrhea) is the presenting feature. Occasionally, these patients may present with meningitis.4 Fewer than 5 cases of adult onset CSF otorrhea from a congenital labyrinthine anomaly presenting with recurrent meningitis have been reported in the literature to date.3,4 Because of the rarity of such a presentation, we report 1 more case of spontaneous CSF otorrhea through an oval window fistula involving an adult patient with recurrent meningitis. We also highlight the utility of magnetic resonance imaging (MRI) in demonstrating the communication between the subarachnoid space and the vestibule through a defect at the fundus of the internal auditory canal as well as an associated inner ear anomaly. CASE REPORT A previously healthy, 33-year-old male had a history of 3 From the Departments of ENT, Speech & Hearing (Drs Rupa and Job) and Neurological Sciences (Dr Rajshekhar) at Christian Medical College & Hospital, Vellore, India. Reprint requests: Dr V. Rupa, DLO, MS, (ENT), Department of ENT, Speech & Hearing, Christian Medical College & Hospital, Vellore632004, Tamil Nadu, India; e-mail, [email protected]. Otolaryngol Head Neck Surg 2001;124:344-6. Copyright © 2001 by the American Academy of Otolaryngology–Head and Neck Surgery Foundation, Inc. 0194-5998/2001 $35.00 + 0 23/78/113511 doi:10.1067mhn.2001.113511 344
episodes of meningitis over a period of 4 years. After the last episode of meningitis, which had occurred 3 weeks earlier, he complained of a clear, watery discharge from his right ear and right nostril. After the second episode of meningitis he noticed a right-sided severe hearing loss. CSF cultures during each episode of meningitis were negative. No detailed ENT examination had been performed during previous attacks of meningitis that had not been associated with noticeable otorrhea or rhinorrhea. Significant history included falling off a motorcycle and deep sea diving 8 and 12 years earlier. There was no history of ear infections. On examination, he was found to be afebrile and alert. There was a trickle of clear fluid from the right nostril. Rigid nasal endoscopy revealed that the fluid was issuing from the right eustachian tubal orifice. Otoscopy revealed an outpouching of the posterosuperior quadrant of the tympanic membrane of the fundus from which a clear fluid escaped intermittently. A right-sided profound sensorineural hearing loss was revealed by pure tone audiometry. CT scanning of the brain showed an irregular linear defect suggestive of a fracture (later proven to be artefactual) of the petrous temporal bone, near the arcuate eminence. Thin slice (1.5 mm thick) T2 weighted MRI scanning of the posterior fossa (Figs 1 and 2) showed the presence of CSF in a widened internal auditory canal communicating with a dilated vestibule on the right side. There was also a dysplastic cochlea that, with the dilated vestibule, was suggestive of a Mondini type of defect. In contrast, the cochlea and vestibule were of normal configuration on the left side with no evidence of any communication between the vestibule and the internal auditory canal. The presence of a Mondini type of dysplasia on the right side was, however, recognized only on detailed review of thin slice MR images postoperatively. In view of the history of injury, evidence in the literature of adult onset CSF otorrhea being predominantly associated with tegmen defects, and CT appearances of a possible temporal bone fracture, a middle fossa craniotomy was performed first and the tegmen tympani and antri completely exposed. However, detailed examination of the lateral aspect of the anterosuperior surface of the petrous temporal bone did not show any defect. At the same time and with the patient under the same anesthesia, a right tympanotomy was performed. CSF appeared to be gushing with force and filling the middle ear as rapidly as it was suctioned. A large aural suction tip placed in the region of the footplate appeared to completely stem the flow of CSF into the middle ear revealing a circular defect in the center of
Otolaryngology– Head and Neck Surgery Volume 124 Number 3
RUPA et al
345
A Fig 2. Thin slice (1.5 mm thick) coronal T2-weighted MR images of the brain. Note large communication between the internal auditory canal and the dilated vestibule along with a dysplastic cochlea on the right side and the normal canal and the turns of the cochlea on the left side.
pack. A lumbar drain was inserted, and strict bed rest enforced postoperatively for 10 days. The patient was asymptomatic at discharge. Follow-up 2 years later revealed no recurrence of otorrhea, rhinorrhea, or meningitis. DISCUSSION
B Fig 1. Thin slice (1.5 mm thick) asymmetric axial T2-weighted MR images of the posterior fossa. A, Note communication between the distal end of a dilated internal auditory canal on the right side and a dilated vestibule. Cochlea on the right is also dysplastic. Note the normal internal auditory canal on the left. B, Axial slice at slightly different level reveals a normal cochlea and vestibule on the left side.
the stapes footplate. Stapedectomy was performed, and a temporalis fascia graft laid across the oval window. The graft was held in place by the repositioned stapes. After scarifying the promontory, the middle ear was also packed with bits of fascia, overlaid by pieces of compressed gelfoam, and the tympanomeatal flap replaced. There were some episodes of transient rhinorrhea within the first week after surgery. The patient was discharged after 3 weeks of bed rest and made an uneventful recovery. Two months later he returned with recurrent rhinorrhea. At tympanotomy, the graft was found to be considerably displaced and CSF flowed freely from the oval window defect. This time the vestibule was packed tightly with generous amounts of temporalis muscle, cortical bone chips, and fascia. The middle ear was also packed with fascia. The tympanomeatal flap was replaced, and the canal packed tightly with a medicated gauze
Spontaneous CSF otorrhea in an adult usually directs the surgeon to look for a tegmen or posterior fossa defect.1-3 Although our patient was an adult, the clinical picture of CSF otorrhea, recurrent meningitis, oval window fistula, and severe sensorineural hearing loss more closely resembled that seen in children. This is very unusual and suggests the need to exclude a congenital labyrinthine anomaly by high resolution CT scanning or thin slice MRI scanning of the temporal bone even in the older patient. Among the few cases of spontaneous adult onset otorrhea described in the literature,1,3,4 the case described by Gundersen and Haye4 comes the closest in presentation to our patient. These authors described a 61-year-old man who had 26 episodes of meningitis and an oval window fistula. In their patient, similar to our case, the site of communication between the vestibule and subarachnoid space was at the lateral end of the internal auditory canal. Packing of the internal auditory canal was required to finally stop the leak. Patients with perilymph fistula from a defect in the oval window usually present with hearing loss and vertigo.3 Several of these patients may have a classical Mondini’s dysplasia with hypoplastic cochlea, dilated vestibule, and enlarged semicircular canals. Fistulae that are characterized by hearing loss, vertigo, and tinnitus may occur at any age, whereas those that produce
346
Otolaryngology– Head and Neck Surgery March 2001
RUPA et al
otorrhea and meningitis are predominantly seen in children. Our patient was unusual in presenting with CSF otorrhea and meningitis at such a late age. It is possible that given the presence of a preexistent oval window dehiscence, his deep sea diving precipitated the otorrhea that would have remained silent for the 7 years preceding the first episode of meningitis. The role of MRI in accurate localization of the site of communication between the subarachnoid space and vestibule merits special mention. A CSF fistula is visualized as a dural-bone defect with hyperintense fluid signal continuous with that in the basal cisterns on T2-weighted images. Johnson et al5 studying 24 patients with suspected CSF fistula found MRI to have 100% sensitivity in localizing the presence and site of the leak. They also found that MRI could accurately identify dural fistulae even in patients without active leaks at the time of testing.
In conclusion, we present a rare case of spontaneous CSF otorrhea and recurrent meningitis as a result of an oval window fistula in an adult patient. The site of communication between CSF in the subarachnoid space and the middle ear via the internal acoustic meatus and vestibule as well as the presence of an inner ear anomaly was best demonstrated on T2-weighted images of MRI scans. REFERENCES 1. Weider DJ, Guerkink NA, Saunders RL. Spontaneous cerebrospinal otorhinorrhoea. Am J Otol 1985;6:416-21. 2. Wetmore SJ, Hermann P, Fisch U. Spontaneous cerebrospinal fluid otorrhea. Am J Otol 1987;8:96-102. 3. Althaus SR. Perilymph fistulas. Laryngoscope 1981;91: 538-62. 4. Gundersen T, Haye R. Cerebrospinal otorrhea. Arch Otolaryngol 1970;91:19-23. 5. Johnson DB, Brennan P, Toland J, et al. Magnetic resonance imaging in the evaluation of cerebrospinal fluid fistulae. Clin Radiol 1996;51:837-41.
S
pontaneous cerebrospinal fluid (CSF) otorrhea as the result of a congenital dural fistula causing recurrent meningitis is a rare entity. Two distinct clinical presentations of this condition have been described, based on single case reports and a small series of patients published over the last century.1,2 The more common juvenile variety, reportedly seen in 72% of cases, is represented by an infant or young child with a history of recurrent episodes of meningitis, unilateral, or bilateral severe to profound sensorineural hearing loss and an inner ear anomaly, typically, Mondini’s dysplasia.3 In addition, a history of recurrent middle ear infections or frank CSF otorrhea after myringotomy for suspected middle ear effusion may be present.2 An oval window fistula and/or enlarged cochlear aqueduct are often associated findings.3 In contrast, adult onset CSF otorrhea, a considerably rarer clinical entity, is typically seen in an older patient, aged 40 years or more, with a congenital tegmen or posterior fossa defect through which dura and brain may herniate.1-3 Either middle ear effusion or CSF otorrhea (or otorhinorrhea) is the presenting feature. Occasionally, these patients may present with meningitis.4 Fewer than 5 cases of adult onset CSF otorrhea from a congenital labyrinthine anomaly presenting with recurrent meningitis have been reported in the literature to date.3,4 Because of the rarity of such a presentation, we report 1 more case of spontaneous CSF otorrhea through an oval window fistula involving an adult patient with recurrent meningitis. We also highlight the utility of magnetic resonance imaging (MRI) in demonstrating the communication between the subarachnoid space and the vestibule through a defect at the fundus of the internal auditory canal as well as an associated inner ear anomaly. CASE REPORT A previously healthy, 33-year-old male had a history of 3 From the Departments of ENT, Speech & Hearing (Drs Rupa and Job) and Neurological Sciences (Dr Rajshekhar) at Christian Medical College & Hospital, Vellore, India. Reprint requests: Dr V. Rupa, DLO, MS, (ENT), Department of ENT, Speech & Hearing, Christian Medical College & Hospital, Vellore632004, Tamil Nadu, India; e-mail, [email protected]. Otolaryngol Head Neck Surg 2001;124:344-6. Copyright © 2001 by the American Academy of Otolaryngology–Head and Neck Surgery Foundation, Inc. 0194-5998/2001 $35.00 + 0 23/78/113511 doi:10.1067mhn.2001.113511 344
episodes of meningitis over a period of 4 years. After the last episode of meningitis, which had occurred 3 weeks earlier, he complained of a clear, watery discharge from his right ear and right nostril. After the second episode of meningitis he noticed a right-sided severe hearing loss. CSF cultures during each episode of meningitis were negative. No detailed ENT examination had been performed during previous attacks of meningitis that had not been associated with noticeable otorrhea or rhinorrhea. Significant history included falling off a motorcycle and deep sea diving 8 and 12 years earlier. There was no history of ear infections. On examination, he was found to be afebrile and alert. There was a trickle of clear fluid from the right nostril. Rigid nasal endoscopy revealed that the fluid was issuing from the right eustachian tubal orifice. Otoscopy revealed an outpouching of the posterosuperior quadrant of the tympanic membrane of the fundus from which a clear fluid escaped intermittently. A right-sided profound sensorineural hearing loss was revealed by pure tone audiometry. CT scanning of the brain showed an irregular linear defect suggestive of a fracture (later proven to be artefactual) of the petrous temporal bone, near the arcuate eminence. Thin slice (1.5 mm thick) T2 weighted MRI scanning of the posterior fossa (Figs 1 and 2) showed the presence of CSF in a widened internal auditory canal communicating with a dilated vestibule on the right side. There was also a dysplastic cochlea that, with the dilated vestibule, was suggestive of a Mondini type of defect. In contrast, the cochlea and vestibule were of normal configuration on the left side with no evidence of any communication between the vestibule and the internal auditory canal. The presence of a Mondini type of dysplasia on the right side was, however, recognized only on detailed review of thin slice MR images postoperatively. In view of the history of injury, evidence in the literature of adult onset CSF otorrhea being predominantly associated with tegmen defects, and CT appearances of a possible temporal bone fracture, a middle fossa craniotomy was performed first and the tegmen tympani and antri completely exposed. However, detailed examination of the lateral aspect of the anterosuperior surface of the petrous temporal bone did not show any defect. At the same time and with the patient under the same anesthesia, a right tympanotomy was performed. CSF appeared to be gushing with force and filling the middle ear as rapidly as it was suctioned. A large aural suction tip placed in the region of the footplate appeared to completely stem the flow of CSF into the middle ear revealing a circular defect in the center of
Otolaryngology– Head and Neck Surgery Volume 124 Number 3
RUPA et al
345
A Fig 2. Thin slice (1.5 mm thick) coronal T2-weighted MR images of the brain. Note large communication between the internal auditory canal and the dilated vestibule along with a dysplastic cochlea on the right side and the normal canal and the turns of the cochlea on the left side.
pack. A lumbar drain was inserted, and strict bed rest enforced postoperatively for 10 days. The patient was asymptomatic at discharge. Follow-up 2 years later revealed no recurrence of otorrhea, rhinorrhea, or meningitis. DISCUSSION
B Fig 1. Thin slice (1.5 mm thick) asymmetric axial T2-weighted MR images of the posterior fossa. A, Note communication between the distal end of a dilated internal auditory canal on the right side and a dilated vestibule. Cochlea on the right is also dysplastic. Note the normal internal auditory canal on the left. B, Axial slice at slightly different level reveals a normal cochlea and vestibule on the left side.
the stapes footplate. Stapedectomy was performed, and a temporalis fascia graft laid across the oval window. The graft was held in place by the repositioned stapes. After scarifying the promontory, the middle ear was also packed with bits of fascia, overlaid by pieces of compressed gelfoam, and the tympanomeatal flap replaced. There were some episodes of transient rhinorrhea within the first week after surgery. The patient was discharged after 3 weeks of bed rest and made an uneventful recovery. Two months later he returned with recurrent rhinorrhea. At tympanotomy, the graft was found to be considerably displaced and CSF flowed freely from the oval window defect. This time the vestibule was packed tightly with generous amounts of temporalis muscle, cortical bone chips, and fascia. The middle ear was also packed with fascia. The tympanomeatal flap was replaced, and the canal packed tightly with a medicated gauze
Spontaneous CSF otorrhea in an adult usually directs the surgeon to look for a tegmen or posterior fossa defect.1-3 Although our patient was an adult, the clinical picture of CSF otorrhea, recurrent meningitis, oval window fistula, and severe sensorineural hearing loss more closely resembled that seen in children. This is very unusual and suggests the need to exclude a congenital labyrinthine anomaly by high resolution CT scanning or thin slice MRI scanning of the temporal bone even in the older patient. Among the few cases of spontaneous adult onset otorrhea described in the literature,1,3,4 the case described by Gundersen and Haye4 comes the closest in presentation to our patient. These authors described a 61-year-old man who had 26 episodes of meningitis and an oval window fistula. In their patient, similar to our case, the site of communication between the vestibule and subarachnoid space was at the lateral end of the internal auditory canal. Packing of the internal auditory canal was required to finally stop the leak. Patients with perilymph fistula from a defect in the oval window usually present with hearing loss and vertigo.3 Several of these patients may have a classical Mondini’s dysplasia with hypoplastic cochlea, dilated vestibule, and enlarged semicircular canals. Fistulae that are characterized by hearing loss, vertigo, and tinnitus may occur at any age, whereas those that produce
346
Otolaryngology– Head and Neck Surgery March 2001
RUPA et al
otorrhea and meningitis are predominantly seen in children. Our patient was unusual in presenting with CSF otorrhea and meningitis at such a late age. It is possible that given the presence of a preexistent oval window dehiscence, his deep sea diving precipitated the otorrhea that would have remained silent for the 7 years preceding the first episode of meningitis. The role of MRI in accurate localization of the site of communication between the subarachnoid space and vestibule merits special mention. A CSF fistula is visualized as a dural-bone defect with hyperintense fluid signal continuous with that in the basal cisterns on T2-weighted images. Johnson et al5 studying 24 patients with suspected CSF fistula found MRI to have 100% sensitivity in localizing the presence and site of the leak. They also found that MRI could accurately identify dural fistulae even in patients without active leaks at the time of testing.
In conclusion, we present a rare case of spontaneous CSF otorrhea and recurrent meningitis as a result of an oval window fistula in an adult patient. The site of communication between CSF in the subarachnoid space and the middle ear via the internal acoustic meatus and vestibule as well as the presence of an inner ear anomaly was best demonstrated on T2-weighted images of MRI scans. REFERENCES 1. Weider DJ, Guerkink NA, Saunders RL. Spontaneous cerebrospinal otorhinorrhoea. Am J Otol 1985;6:416-21. 2. Wetmore SJ, Hermann P, Fisch U. Spontaneous cerebrospinal fluid otorrhea. Am J Otol 1987;8:96-102. 3. Althaus SR. Perilymph fistulas. Laryngoscope 1981;91: 538-62. 4. Gundersen T, Haye R. Cerebrospinal otorrhea. Arch Otolaryngol 1970;91:19-23. 5. Johnson DB, Brennan P, Toland J, et al. Magnetic resonance imaging in the evaluation of cerebrospinal fluid fistulae. Clin Radiol 1996;51:837-41.