Erwin Offeciers

Imaging of Intralabyrinthine Schwannomas: A Retrospective Study of 52 Cases with Emphasis on Lesion Growth

BACKGROUND AND PURPOSE: Only a few case reports and small series of intralabyrinthine schwannomas (ILSs) have been reported. The purpose of this study was to assess prevalence, MR characteristics, location, clinical management, and growth potential/patterns of ILSs in the largest series reported. MATERIALS AND METHODS: Lesion localization, MR characteristics, lesion growth, and clinical management were reviewed in 52 patients diagnosed with an ILS between February 1991 and August 2007 in 2 referral centers. The number of ILSs and vestibulocochlear schwannomas in the cerebellopontine angle/internal auditory canal was compared to assess the prevalence. RESULTS: ILSs most frequently originate intracochlearly, are hyperintense on unenhanced T1-weighted images, enhance strongly after gadolinium administration, and are sharply circumscribed and hypointense on thin heavily T2-weighted 3D images. The scala tympani is more frequently or more extensively involved than the scala vestibuli. Follow-up MR imaging, available in 27 patients, showed growth in 59% of subjects. Growth was seen from the scala tympani into the scala vestibuli and from the scala vestibuli to the saccule and vice versa. Twelve lesions were resected, and the diagnosis of ILS histopathologically confirmed. CONCLUSION: ILSs can account for up to 10% of all vestibulocochlear schwannomas in centers specializing in temporal bone imaging, grow in more than 50%, and are most frequently found intracochlearly, often anteriorly between the basal and second turn. Cochlear ILSs most often originate in the scala tympani and only later grow into the scala vestibuli. Growth can occur from the cochlea into the vestibule or vice versa through the anatomic open connection between the perilymphatic spaces in the scala vestibuli and around the saccule.

Auditory brainstem implantation in children and non-neurofibromatosis type 2 patients: a consensus statement.

On the 18th of September 2009, a group of health care professionals and scientists involved in implantation of the auditory system attended a meeting convened by the Hacettepe Cochlear Implant Group. The aim of the meeting was to have a detailed discussion on the pressing and pertinent issues around

Diffusion-weighted magnetic resonance imaging of the temporal bone.

This paper summarizes the value of diffusion-weighted magnetic resonance imaging in the evaluation of temporal bone pathology. It highlights the use of different types of diffusion-weighted magnetic resonance imaging in the different types of cholesteatoma, prior to first stage surgery and prior to second look surgery. The value of diffusion-weighted magnetic resonance imaging in the evaluation of pathology of the apex of the petrous bone and the cerebellopontine angle is also discussed.

Long-term follow up after bony mastoid and epitympanic obliteration: radiological findings.

OBJECTIVE The canal wall up bony obliteration technique lowers the incidence of recurrent cholesteatoma, but carries the potential risk of obliterating residual cholesteatoma. The objective of this study was to report long-term follow-up radiological findings after performing a canal wall up bony obliteration technique procedure, in order to detect residual and/or recurrent cholesteatoma. PATIENTS Fifty-one patients presenting with a cholesteatoma or a troublesome cavity were operated upon using the canal wall up bony obliteration technique, and were evaluated by follow-up imaging a mean of 76.4 months post-operatively (range, 53.8-113.6 months). INTERVENTION All patients were evaluated with high resolution computed tomography and magnetic resonance imaging (including delayed contrast, T1-weighted imaging and non-echo-planar, diffusion-weighted imaging). RESULTS Imaging revealed the presence of one residual, one recurrent and one congenital petrosal apex cholesteatoma. On high resolution computed tomography, completely obliterated mastoid filled with bone was observed in 74.5 per cent (38/51) of patients, and an aerated middle-ear cavity in 64.7 per cent (33/51). High resolution computed tomography clearly detected any associated soft tissue present in the middle-ear cavity (18/51) and in the obliterated mastoids (13/51), but could not characterise this tissue. Non-echo-planar, diffusion-weighted magnetic resonance imaging clearly identified all three cholesteatomas, and differentiated them from other associated soft tissues. No cholesteatoma was found within the obliterated mastoids. CONCLUSION Long-term follow up indicated that the canal wall up bony obliteration technique is a safe method with which to treat primary and recurrent cholesteatoma and to reconstruct unstable cavities. Soft tissue was found quite often in the middle ear and obliterated mastoids. High resolution computed tomography identified its presence but could not further characterise it. However, non-echo-planar, diffusion-weighted magnetic resonance imaging succeeded in differentiating soft tissues, enabling detection of residual or recurrent cholesteatoma after a canal wall up bony obliteration technique procedure.

A multicentre clinical evaluation of paediatric cochlear implant users upgrading to the Nucleus(®) 6 system.

OBJECTIVES The aim of this study was to investigate whether experienced paediatric cochlear implant users could show benefits to speech perception outcomes from the introduction of noise reduction and automated scene classification technologies as implemented in the Nucleus(®) 6 sound processor. Previous research with adult cochlear implant users had shown significant improvements in speech intelligibility for listening in noisy conditions and good user acceptance for upgrading to the Nucleus 6 processor. In adults, these improvements for listening in noise were primarily attributed to the use of a range of new input processing technologies including noise reduction, as well as introduction of automatic scene classification technology. METHODS Experienced paediatric cochlear implant users (n=25) were recruited from four clinics located in three countries. Research participants were evaluated on three occasions, an initial session using their Nucleus 5 sound processor; a second session in which participants used the Nucleus 6 processor programmed with the same technologies as were used in their Nucleus 5 sound processor; and a final session in which participants used the Nucleus 6 processor programmed with the default technologies including automatic scene classification (SCAN) which automatically selects the microphone directionality, noise reduction (SNR-NR), and wind noise reduction (WNR) technologies. Prior to both the second and third evaluations, research participants had approximately two weeks take-home experience with the new system. Speech perception performances on monosyllabic word tests presented in quiet and in noise, and a sentence test presented in noise, were compared across the three processor conditions. Acceptance of the Nucleus 6 default settings was assessed in a final session. RESULTS No group mean difference in performance was found for monosyllabic words in quiet. A significant improvement in speech perception was found for both monosyllabic words and sentences in noise with the default Nucleus 6 program condition as compared with the Nucleus 5 condition. No acceptance issues were noted for any of the children. CONCLUSIONS Experienced paediatric cochlear implant users showed a significant improvement in speech perception in listening in noise when upgraded to the Nucleus 6 sound processor primarily due to the introduction of a noise reduction technology, and all children accepted the default program. These findings suggest that school-aged children may benefit from upgrading to the Nucleus 6 sound processor using the default program.

The Bony Obliteration Tympanoplasty in Pediatric Cholesteatoma: Long-term Safety and Hygienic Results

Objective: To present the safety and hygienic results of a 5-year longitudinal study in a pediatric population undergoing surgery for extensive cholesteatoma using a canal wall up approach with bony obliteration of the mastoid and epitympanic space. Study Design: Retrospective consecutive study. Patients: Thirty-three children (⩽18 yr) undergoing surgery for cholesteatoma (34 ears) between 1997 and 2009. Interventions: Therapeutic. Setting: Tertiary referral center. Main Outcome Measures: 1) Residual and recurrent cholesteatoma rates at 5-year postsurgery, 2) postoperative waterproofing and hygienic status of the ear, and 3) required operation rate to achieve the safety and hygienic goals. Results: At 5 years no patients were lost in follow-up. This consecutive series design is rare in chronical otitis media treatment reporting. The standard residual rate at 5 years was 5.8%, representing two residual cholesteatomas in the middle ear. The standard recurrence rate at 5 years was 2.9%, representing one recurrent cholesteatoma. At 5-year follow-up all ears were free of otorrhea and waterproof and all external ear canals were patent and self-cleaning. The operation rate to reach this safety and hygienic status was 1.5 operations per ear at 5-year follow-up. Conclusion: The use of a canal wall up approach with obliteration of the mastoid cavity and epitympanic space to surgically treat cholesteatoma in children results in low residual and recurrence rates and a high rate of trouble-free ears in the long term.

Isolated squamous cell carcinoma of the tympanic membrane.

Squamous cell carcinomas (SCC) of the external auditory canal are rare tumors (1). An isolated localization on the tympanic membrane has never been previously reported. A 65-year-old man presented with hearing loss, tinnitus, and otorrhea secondary to a left-side chronic otitis externa. He had been previously treated with antibiotic eardrops for 6 months without improvement. Otoscopy revealed a squamous lesion on the posterior aspect of the eardrum. With the exception of some thickening of the tympanic membrane, a computed tomographic scan of the temporal bone was normal. A preliminary superficial biopsy revealed the presence of chronic inflammation, hyperplasia, and mild dysplasia of the epithelium. A deeper second biopsy was performed, revealing an in situ SCC with a moderate to well-differentiated infiltrating carcinoma, characterized by a hyperkeratotic, squamous epithelium showing marked variations in cell size, atypical mitoses, nuclear polymorphism, and hyperchromatosis. A total resection of the tympanic membrane was performed and followed by a myringoplasty. The operative specimen revealed a SCC with a maximum diameter of 4 mm. The depth of infiltration was 1.5 mm without extension through the tympanic membrane (Fig.1 and 2). The minimum distance of the lesion from the surgical margins was 1 mm. There has been no evidence of recurrence over a period of 1 year. Pseudoepitheliomatous hyperplasia is a benign to chronic reaction and can mimic an SCC (2). Such lesions resolve with conservative treatment. Because this was not the case in our patient, the biopsy was repeated. Early diagnosis of an SCC and correct evaluation of its extension and adequate surgery are necessary to obtain a high cure rate. Therefore, any lesion not improving with conservative treatment should be the object of repeated biopsies to rule out the presence of an SCC.

Handling an Isolated Malleus Handle Fracture: Current Diagnostic Work-up and Treatment Options.

Background: An isolated malleus handle fracture is rare and presents with vague otological symptoms. Diagnosis depends on careful history taking and otoscopic examination. Different treatment options are available. Reconstruction with hydroxyapatite bone cement is a relatively new technique. Objective: The aim of this article is to review the current diagnostic work-up and treatment options and to demonstrate our method of treatment with hydroxyapatite bone cement. Methods: Three cases, repaired with hydroxyapatite bone cement in a tertiary referral otologic center, were retrospectively analyzed. A review of the literature on diagnosis and treatment procedures was performed. Results: One fracture occurred by withdrawing a finger from the external auditory canal and 2 occurred without clear prior trauma. Our 3 cases were successfully repaired with hydroxyapatite bone cement. Conclusion: Diagnosis of an isolated malleus handle fracture still depends on careful clinical examination. In addition to pure tone audiometry, both low and higher frequency tympanometry may be useful. High resolution computed tomography with reformatting through the malleus handle is the most accurate imaging modality. Reconstruction with hydroxyapatite bone cement is reliable by restoring the original situation with only minimal manipulation and without additional ossicular interruption.

Jugular bulb diverticulum dehiscence towards the vestibular aqueduct in a patient with otosclerosis.

OBJECTIVES To demonstrate the need for computed tomography imaging of the temporal bone before considering revision stapes surgery in patients with recurrent or residual conductive hearing loss. CASE REPORT We report the case of a high-riding jugular bulb with an associated jugular bulb diverticulum, which was dehiscent towards the vestibular aqueduct, in a patient with confirmed otosclerosis who did not experience hearing improvement after stapedotomy. CONCLUSION This case demonstrates the usefulness of temporal bone computed tomography in the evaluation of patients with otosclerosis in whom stapedotomy has not improved hearing. In such patients, revision surgery to address residual hearing loss would eventually prove unnecessary and avoidable.

Consensus statement: Long-term results of ABI in children with complex inner ear malformations and decision making between CI and ABI

Consensus statement : Long-term results of ABI in children with complex inner ear malformations and decision making between CI and ABI