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OBJECTIVES: Endolymphatic sac tumors (ELSTs) are rare adenomatous tumors arising in the petrous bone, either spontaneously (isolated) or in association with von Hippel-Lindau (VHL) disease. For 9 patients, this study describes the typical features and management of ELST and the clinical outcome of treatment. Similarities and differences are identified for ELST in isolation and in association with VHL disease. STUDY DESIGN: Case series. SETTING: Tertiary referral center. PATIENTS: The patient database was searched for patients diagnosed with ELST. All other tertiary referral centers in The Netherlands were invited to do likewise. INTERVENTIONS: The patient records were screened for presenting symptoms, hearing, imaging, treatment, and treatment outcome. Histopathologic finding was reviewed by a senior pathologist. RESULTS: Six patients with an isolated ELST and 3 patients with an ELST in association with VHL disease were identified. In the isolated ELST group, 5 patients were treated surgically and 1 patient was administered primary radiotherapy. In the VHL group, 1 patient underwent surgery and 2 patients were treated conservatively after a wait-and-magnetic resonance imaging-scan protocol. All individual treatment outcomes are scrupulously presented in this article. CONCLUSION: This study compiles and describes 9 cases of ELST. In 8 of 9 patients, the ELST could be controlled by surgery, radiotherapy, or a wait-and-scan protocol. The large interpatient differences make it difficult to standardize the management of this disease. The management is strongly influenced by comorbidity and tumor stage.

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Although in the past cochlear implantation was considered contraindicated in patients with acute (AOM) or chronic suppurative otitis media (CSOM) with or without middle ear cholesteatoma, recent developments now make it possible to perform cochlear implantation in these patients. Various procedures are available to make the ears of patients with either acute or CSOM suitable for cochlear implantation and to minimize the risk of recurrence of the disease, device extrusion, or intracranial complications. This review discusses these different approaches for optimizing implant survival and preventing complications related to otitis media. We performed a comprehensive literature search of the MEDLINE database. Cochlear implantation can be safely performed in patients with otitis media. However, the infection should be adequately controlled well before implantation, and all measures should be taken to prevent recurrent disease. Therefore, the procedure used should be tailored to individual clinical findings. This article provides a guideline to optimize the course of action in patients suffering from AOM, CSOM or their sequelae in preparation for cochlear implantation.

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CONCLUSIONS: Open-set speech perception in children with an inner ear malformation is equal to that of other congenitally deaf children after an average of 2 years follow-up. OBJECTIVE: To analyze audiological performance after cochlear implantation in a sample of children with radiographically detectable malformations of the inner ear compared to performance in prelingually deafened children at large. MATERIALS AND METHODS: Nine children with osseous inner ear malformations were compared to 22 congenitally deaf children, all of whom underwent cochlear implantation. All subjects were tested on their electrical evoked compound action potential. Speech perception tests were performed using the monosyllabic trochee polysyllabic test without visual support and the open-set monosyllabic wordlist. RESULTS: In all, 20% of the congenitally deaf children in our center study have inner ear abnormalities. Inner ear malformations were limited to incomplete partition of the cochlea; none of the subjects had common cavity malformations. Electrical compound action potentials were successfully recorded in both groups intraoperatively. Speech perception tests on open-set speech yielded an average of 48.8% (SD 21.2%) in the group of children with inner ear malformations vs 54.5% (SD 21.1%) in congenitally deaf children. In four of nine cases with an inner ear malformation we encountered a minor CSF leak.

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Vascular malformations of the cerebello-pontine angle (CPA) arising from the capillary plexus surrounding Scarpa's ganglion are rare tumors. We report a case of so-called "hemangioma" of the CPA which was operated on via a trans-labyrinthine approach based on a preoperative diagnosis of vestibular schwannoma. Although there are some differences between these tumors in terms of the clinical, biological and especially MRI findings, surgery is usually performed based on an assumption of vestibular schwannoma. Surgery is the treatment of choice for these lesions as early intervention ensures better postoperative facial function. It is important to make the differential diagnosis if conservative management of vestibular schwannoma is adopted.

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Endolymphatic hydrops continues to be considered as a pathological factor in the etiology of Meniere's disease. We have developed the two-phase endolymphatic hydrops model, which seems to represent a functional model combining multiple etiologies, and which may resemble the fluctuating characteristics of Meniere's disease. A transmission electron microscopic study was performed on the endolymphatic sacs of four groups of guinea-pig cochleas: (1) controls, (2) non-operated, aldosterone-treated cochleas, (3) operated (dissection of the endolymphatic sac) cochleas, (4) operated and aldosterone-treated cochleas. Light and electron microscopy showed a normal morphology in the controls. Aldosterone as a single treatment resulted in an increased activity of the marginal cells in the stria vascularis. Dissection induced a gradient of degenerative effects and cell loss in the intracellular and extracellular structures of the sensory cells, the stria vascularis and Reissner's membrane, which may be reversible. Subsequent administration of aldosterone induced severe damage and increased cell loss, which may be irreversible. Our findings demonstrate changes that may be reversible due to the compromising effect of a single treatment, and irreversible changes due to interaction of both compromising factors. These findings support our two-phase concept.

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