Long-term outcomes of a transmastoid lateral semicircular canal approach to congenital CSF otorrhea in children associated with recurrent meningitis and severe inner ear malformation.

OBJECTIVE: To investigate the long-term effectiveness of transmastoid lateral semicircular canal approach (TMLSCCA) to repair cerebrospinal fluid (CSF) leakage in children associated with recurrent meningitis and severe congenital inner malformation. METHOD: A retrospective study was conducted in a university hospital, academic medical center. Fifteen children with recurrent meningitis, secondary to severe congenital inner ear malformation, were included in the study. All of them had CSF associated otorrhea and treated using TLSCCA to repair CSF otorrhea by packing the vestibule with muscle and fascia. Observation of the status of postoperative CSF leakage, recurrence of meningitis and complication were conducted. RESULTS: None of the cases had recurrent meningitis and CSF leakage after their TLSCCA procedure in the follow-up period of 1-8.5 years. One case presented with transient facial nerve paralysis and completely recovered 3 months later. CONCLUSION: TLSCCA for CSF otorrhea in children with recurrent meningitis secondary to congenital inner ear malformation is an alternative approach that offers some advantages.

Spontaneous resolution of cerebrospinal fluid otorrhoea in a patient with a Hyrtl's fissure.

OBJECTIVE: This paper reports a rare case of cerebrospinal fluid leak due to a Hyrtl's fissure and discusses the non-operative management of the case. Background and case report: Cerebrospinal fluid otorrhoea is a rare phenomenon arising from an abnormal communicating tract between the subarachnoid space and middle ear. Affected patients are at a higher risk of developing meningitis and other neuro-otological complications. There are four common congenital causes of cerebrospinal fluid otorrhoea in the region of a normal labyrinth. This paper describes a case of cerebrospinal fluid in the middle ear resulting from a Hyrtl's fissure, which resolved spontaneously. CONCLUSION: A literature search indicated this to be the first case with such a resolution without the need for any intervention.

Use of the translabyrinthine approach to repair congenital spontaneous cerebrospinal fluid leakage in five Chinese patients with Mondini dysplasia.

OBJECTIVES: To introduce a more effective surgical technique for the repair of spontaneous incurable cerebrospinal fluid (CSF) leakage (CSF otorrhea). METHODS: The cases of five Chinese patients with Mondini dysplasia and CSF leakage were reviewed. All patients presented with rhinorrhea or otorrhea from childhood and recurrent meningitis attacks. CSF leakage was repaired surgically using the translabyrinthine approach. RESULTS: Facial paralysis occurred in the first two patients, but was cured 2 months later. The other three patients recovered well and had no facial paralysis or complication. CSF leakage did not recur in any patient after follow-up periods of 0.5-3 years. CONCLUSION: Mondini dysplasia with CSF leakage is rare. Adequate diagnosis and treatment are required to avoid life-threatening complications, such as meningitis. We recommend the translabyrinthine approach as an alternative surgical technique that reduces recurrence.

Congenital tuberculosis with facial nerve palsy.

Congenital tuberculosis is unusual, and central nervous system involvement is even more rare. We report a case of congenital tuberculosis presenting with facial palsy and purulent otorrhea.

Cerebrospinal fluid otorrhea in a patient with congenital inner ear dysplasia.

A case of a 10-month-old boy with a cerebrospinal fluid (CSF) fistula in his right ear is reported. In the same ear, the patient also showed congenital inner ear dysplasia. The CSF fistula was obstructed surgically. Surgical exploration showed a fistula superior to the oval window and a normally shaped stapes. The stapes was removed and the fistula was closed by obliteration of the vestibulum with the temporal fascia and fat tissue. The location of the fistula was very rare; to our knowledge, this is the first reported case of CSF fistula superior to the oval window. The relationship between perilymphatic fistula and the microfissure revealed by temporal bone study is discussed.

Imaging of congenital anomalies of the temporal bone.

This article briefly presents the embryology of the ear and discusses the external auditory canal and middle ear, including first branchial cleft anomalies, external auditory canal atresia and stenoses, congenital cholesteatoma, and congenital teratoma. Topics related to the labyrinths include aplasia, the common-chamber anomaly, the pseudo-Mondini and Mondini malformations, semicircular canal dysplasia, and the large vestibular aqueduct. Vascular malformations and variations also are presented, including the absent and aberrant internal carotid artery, the persistent stapedial artery, and high jugular bulb; cerebrospinal fluid and perilymph fistulas are the subjects of the final section.

[Pneumococcal meningitis revealing dysplasia of the bony labyrinth in an infant]. / Méningite à pneumocoque révélatrice d'une dysplasie du labyrinthe osseux chez un nourrisson.

BACKGROUND: Dysplasias of the bony labyrinth are frequently associated with cerebrospinal fluid fistula and are usually discovered because of recurrent meningitis. CASE REPORT: A 1 year-old infant was admitted for a pneumococcal meningitis which appeared 2 days after the occurrence of a clear otorrhea from the right ear. The same organism was isolated from the otorrhea fluid, which also contained cerebrospinal fluid as confirmed cytochemically. The meningitis rapidly resolved with antibiotic treatment. Auditory brain stem responses were abolished from the right ear. CT of the temporal bones showed a pseudo-Mondini type labyrinth dysplasia at the right ear and Mondini type dysplasia at the left one. A translabyrinthine cerebrospinal fluid fistula was discovered by surgical exploration of the right ear, occurring through a perforation in the stapedial foot plate. The leak was cured by packing the vestibule and obturating both oval and round windows. Three years after the operation, the child did not experience any further episode of otorrhea or meningitis. CONCLUSIONS: Features suggesting a translabyrinthine fistula, especially otorrhea and deafness, should be systematically searched in any child with bacterial meningitis. Closure of these fistulas can prevent severe infectious recurrences.

[Mondini's dysplasia: a rare cause of recurrent meningitis in childhood]. / La displasia di Mondini: una causa rara di meningiti ricorrenti in età pediatrica.

Recurrent meningitis in children may be due to immune deficiency or abnormal communication of CSF spaces with mucosal spaces (basal skull fractures, encephalocele, etc.). We report a 6-year-old boy, suffering from recurrent meningitis and sensorineural hearing loss. CT scans of the temporal bones demonstrated a bilateral Mondini dysplasia. In patients with this congenital inner ear anomaly, recurrent meningitis may be due to a CSF-middle ear fistula. We think that it is necessary to investigate all patients with recurrent meningitis and sensorineural hearing loss with CT scans of the temporal bones.

[Congenital CSF otitic leakage presenting as rhinorrhea: report of a case].

A 10-year-old girl was brought to our ear, nose and throat clinic for consultation on her articulation problem in addition to an attack of bacterial meningitis and cerebrospinal fluid (CSF) rhinorrhea. These problems had bothered her and her family for about 5 years. In August 1988, she had received a bifrontal osteoplastic craniotomy for her CSF leakage, but the procedure was ineffective. Local examination showed bilaterally intact ear drums. The left ear drum looked cloudy and thick with poor mobility under pneumatoscopy. A tympanogram showed that B type. Pure tone audiometry revealed a profound sensorineural hearing loss in the left ear. A CT scan showed widening of the internal auditory canal and dysplasia of the left cochlea and vestibule in addition to fluid accumulation in the mastoid and mesotympanum. Under the impression of CSF otorrhea, an exploratory tympanotomy was performed, revealing an area of leakage in the tympanic cavity. It was sealed with abdominal fat, temporal connective tissue and tissue glue. All of the bothersome symptoms disappeared postoperatively during an 18-month follow-up.

[Diagnosis in recurrent bacterial meningitis; a literature study]. / Diagnostiek bij recidiverende bacteriële meningitis; een literatuurstudie.

Recurrent bacterial meningitis can be caused by different mechanisms. In order to make the diagnostics more efficient, we studied literature to find these underlying mechanisms. Two groups could be identified: group I, patients with a congenital or posttraumatic defect in the bones of the skull and group II, patients with a deficiency of a component of complement. The patients in group I were much younger at the time of their first meningitis and suffered more often from otitis, rhinorrhoea and deafness diagnosed before the first meningitis. Streptococcus pneumoniae was most frequently cultured in the cerebrospinal fluid, the group with complement deficiency showed Neisseria meningitidis almost exclusively. Family history revealed more members with infections caused by neisseriae spp. Although it was difficult to diagnose the bony defects, physical examination, radiography and high resolution CT scanning of the skull were helpful. Age, history, physical examination and cerebrospinal fluid culture were the most helpful in diagnostic factors.

Spontaneous atraumatic CSF otorrhea.

The clinical course of a five-month-old male with CSF otorrhea in the absence of head trauma is presented. The anatomy, workup, and management of this condition are described. The need to consider communication between the subarachnoid space and middle ear in any child with ear drainage, sensorineural hearing loss, or recurrent meningitis is emphasized.

Congenital perilymphatic fistula: a prospective study in infants and children.

A 3-year prospective study of 244 children (aged 5 months to 17 years) with sensorineural hearing loss of unknown cause revealed that 57 children (23%) had radiographic evidence of abnormalities of the temporal bone, detected by computed tomography scan, and/or progression of sensorineural hearing loss. Of these 57 children, 42 (74%) underwent surgical exploration, with 15 children (26%) of this subgroup demonstrating active congenital perilymphatic fistula. The prevalence of congenital perilymphatic fistula in a large, closely monitored population of children with unexplained sensorineural hearing loss appears to be at least 6% (15 of 244 children). The incidence may be higher because some congenital perilymphatic fistulae may leak intermittently and not be observed during middle ear surgery. Therefore, in children with suspected sensorineural hearing loss, early and frequent audiological evaluations should be made to rule out progressive/fluctuating sensorineural hearing loss. These children should also have radiographic imaging of their temporal bone by computed tomography scan. The use of both methods is the best indicator for congenital perilymphatic fistula.

Spontaneous cerebrospinal fluid otorrhea in a deaf infant.

Spontaneous (congenital) cerebrospinal fluid (CSF) otorrhea is a rare occurrence with less than 100 cases being reported in the literature. In infants and children, it may present as otitis media with effusion. A 4-month-old infant, who was diagnosed with severe to profound sensorineural hearing impairment and spontaneous CSF otorrhea, initially presented with otitis media with effusion. The patient presentation, diagnostic dilemmas and pathophysiology of this rare but interesting problem are discussed.

Congenital cerebrospinal fluid fistulae of the petrous temporal bone.

Spontaneous cerebrospinal fluid fistulae from the subarachnoid space into the middle ear cavity may be classified as perilabyrinthine or translabyrinthine. The very rare perilabyrinthine group, through bony defects close to but not involving the labyrinth, usually have normal hearing initially. The commoner translabyrinthine group are nearly always associated with anacusis, severe labyrinthine dysplasia and a route via the internal auditory meatus. The labyrinthine deformity is more severe than the type classically described by Mondini, and evidence of a dilated cochlear aqueduct in these cases is also unconvincing.

Meningitis and congenital fistula of the internal ear.

Congenital CSF otorrhea is seldom the cause of meningitis but has to be looked for especially if associated with a dead ear or with recurrent meningitis. Polytomograms of the mastoids are essential to rule out any abnormality of the internal ear. Three patients with a congenital CSF otorrhea and associated meningitis are reviewed. They all presented a Mondini type of dysplasia and had an oval window fistula. They were treated successfully with fascia and muscle obliteration of the vestibule.

The temporal bone as route of infection in recurrent meningitis.

Three successive episodes of recurrent meningitis in connection with an infection of the upper respiratory tract appeared to be related to a congenital idiopathic oval window CSF leak. An inner ear dysplasia was the underlying factor and accounted for the deafness and for the absence of signs of acute labyrinthis during these infectious episodes.

Congenital cerebrospinal otorrhea.

Four locations for congenital cerebrospinal fluid fistula in the region of a normal labyrinth are reviewed. A congenital leak may occur through the petromastoid canal, a wide cochlear aqueduct, Hyrtl's fissure, or the facial canal. A fistula through the initial segment of the fallopian canal was successfully repaired in a two-year-old boy who had three episodes of meningitis following otitis media. Knowledge of these four sites of congenital defects provides a guideline for the surgeon in the identification and repair of cerebrospinal fluid leaks in the region of the labyrinth.