Search

We report a case of unilateral inner ear malformation that presenting with recurrent meningitis and bilateral deafness. A 8-months-old boy with congenital left-side hearing impairment, diagnosed in newborn screening, suffered bacterial meningitis twice within 7 months of birth, becoming bilateral totally deaf there after. Temporal bone computed tomography (CT) indicated left-side inner ear malformation, Mondini dysplasia, and a cerebrospinal fluid (CSF) fistula. CSF from a stapes footplate defect was repaired surgically. Cochlear implantation was successful in the right ear 3 months after the first operation. In the 14 months since the second operation, he has had no bacterial meningitis and has begun speaking two-word sentences. Early diagnosis and prompt surgical intervention are thus crucial in avoiding meningitis sequelae.

We report on a 58-year-old female with inner ear anomalies associated with recurrent meningitis. During surgical treatment, severe cerebrospinal fluid leakage through a bony defect in the stapedial footplate was observed after the stapes was removed. Intralabyrinthine obliteration with the temporal muscle fascia was performed and the oval window was sealed with an auricular cartilaginous chip. No sign of meningitis has occurred after the operation.

Many cases of traumatic cerebral spinal fluid (CSF) rhinorrheas arise from head injury or surgery. Cases of nontraumatic CSF rhinorrhea arise rarely due to tumors or inherent bone defects. We report a case of CSF rhinorrhea associated with meningoencephalocele at two bone defect loci requiring surgery. A 65-year-old man experienced watery rhinorrhea of the right nasal cavity in March 1997, followed a year later by meningitis yielding the diagnosis of CSF rhinorrhea. Computed tomography (CT) showed two bone defects and the associated meningoencephalocele in the right ethmoidal cell and left sphenoidal sinus. Initial skull base reconstruction surgery was conducted only for the right side where the symptom occurred. Ten years later, recurrent meningitis necessitated the left side skull base reconstruction surgery for a large infected defect for which could not obtain endoscopic sinus surgery closure. Combined transcranial surgery eventually attained sufficient closure.

A 5-year-old girl with unilateral total deafness suffered from recurring meningitis. High-resolution computed tomography of the temporal bone revealed dysplasia of the bony labyrinth and enlarged fundus of the internal auditory canal (Mondini dysplasia). In the operation, anomaly of the stapes and leakage of cerebrospinal fluid were found. Intralabyrinthine obliteration with the temporal muscle fascia and auricular cartilaginous chip was performed after removal of the stapes. No sign of meningitis has occurred after the operation.

A case of inner ear anomalies (Mondini dysplasia) associated with recurrent meningitis was found in a 5-year-old boy. The patient had 3 episodes of meningitis since he was 3 years old. During surgical treatment, we found cerebrospinal fluid (CSF) leakage through a bony defect in the stapedial footplate. The inner ear was obliterated with small pieces of connective tissue and the oval window was sealed with auricular cartilaginous chips. He has remained free from recurrent meningitis during 7 months follow up.

Cerebrospinal fluid (CSF) rhinorrhea is potentially serious because it may lead to recurrent meningitis. Therefore, early diagnosis and appropriate treatment of CSF rhinorrhea are required.We treated 13 patients with CSF rhinorrhea during the past 15 years. Four of them had meningitis. All 13 were successfully treated surgically with a rhinological approach in 12 and an intracranial approach in one. On the basis of our experience, RI counts of intranasal pledgets and detection of tau-transf errin in the nasal discharge were helpful in the diagnosis of CSF rhinorrhea. A coronal CT scan was useful in the determination of the precise location of the fistula.For a surgical treatment, a rhinological approach with minimal morbidity was indicated in most patients. Key points of the operation are: 1) careful observation of the fistula under an operating microscope or a rigid nasal endoscope, 2) water-tight closure of the fistula with fibrin glue and two or three layers of autogenous fascia, 3) intra-and postoperative CSF drainage to prevent an increase of intracranial pressure.

Mondini 型内耳奇形を伴う反復性髄膜炎の2例を報告した. 1例は卵円窓前上部より, 他はアブミ骨底板中央の行欠損上り髄液漏を認めた. アブミ骨を除去して前庭内へ筋肉, 筋膜, 人工硬膜などを充填し, 外側を同様に閉鎖する方法が確実であることを述べた.

A case of inner ear anomalies (Mondini dysplasia) associated with recurrent meningitis found in a 10-year-old boy is reported.The patient had cerebrospinal fluid (CSF) rhinorrhea since he was three years old and had five episodes of meningitis. The left ear of the patient was totally deaf. Exploratory tympanotomy revealed CSF leakage through the bony defect of the stapedial footplate. As the leakage recurred after an attempt to obstruct the vestibular window, the translabyrinthine approach to the internal auditory meatus was performed. Packing the internal auditory meatus with several pieces of muscle and gelatine sponge stopped the CSF leakage and the recurrence of meningitis.