Evaluation of the transmastoid plugging approach for superior semicircular canal dehiscences: a retrospective series of 30 ears.

PURPOSE: The classical surgical approach for superior semicircular canal dehiscences (SSCD) is via the extradural middle cranial fossa. This pathway is used to resurface or to plug the SSC. In this paper, we present long-term data on an alternative route: the transmastoid pathway. The predictive factors for a successful surgery are equally presented in this paper. METHODS: Thirty reports of patients operated between September 2007 to January 2020 were analysed. SSCD was confirmed by the association of concordant complaints, audiometric data, cervical vestibular evoked myogenic potentials (cVEMP) responses and computerized tomography findings. Before and after surgery, the following factors were analysed: auditory and vestibular subjective symptoms, Tullio phenomenon, pure-tone audiometry thresholds for air and bone conduction, air-bone gap, cVEMP threshold, and computerized tomography data, for instance the size of the dehiscence. RESULTS: The follow-up is 21 months on average. The transmastoid approach significantly improves all symptoms (although there were less probing results for the vestibular symptoms). Objectively, we can observe, a closure of the audiometric air-bone gap on the low frequencies and an improvement in the cVEMP. The only correlation that was identified was between the preoperative cVEMP results and the postoperative air conduction. CONCLUSIONS: The originality of this study is the long postoperative follow-up. It allowed us to evaluate the symptoms in the long term and to determine a predictive factor of postoperative complication, which has not yet been described until today.The transmastoid plugging technique is safe and effective. Additional long-term data with a larger cohort are needed to confirm our results and correlation studies.

Intratemporal facial nerve schwannomas: multicenter experience of 80 cases.

PURPOSE: To provide more data on the clinical presentation and natural evolution of facial nerve schwannomas and to provide guidance for therapeutic decision making. METHODS: A retrospective case review of eighty patients diagnosed with a facial nerve schwannoma between 1990 and 2018 in ten tertiary referral centers in Europe was performed. Patients' demographics, symptomatology, audiometry, anatomical site (segments involved), size and whenever possible volume measurement were registered. RESULTS: At presentation, transient or persistent facial palsy was the most common symptom, followed by hearing loss. The schwannoma involved more than one segment in the majority of the patients with the geniculate ganglion being most commonly involved. Initial treatment consisted of a wait and scan approach in 67.5%, surgery in 30% and radiation therapy in 2.5% of the patients. Tympanic segment schwannomas caused mainly conductive hearing loss and were more prone to develop facial palsy at follow-up. Internal auditory canal or cerebellopontine angle schwannomas presented with significantly more sensorineural hearing loss. CONCLUSIONS: Although modern imaging has improved diagnosis of this tumor, choosing the best treatment modality remains a real challenge. Based on the literature review and current findings, more insights into the clinical course and the management of facial nerve schwannomas are provided.

The MO-meatocanalplasty: a modification of the M-meatoplasty to address the superior quadrants and the bony canal.

The meatoplasty of the external auditory canal is a frequently performed otologic procedure in recurrent otitis externa, eczema or frequent accumulation of cerumen due to a narrow meatus of the external ear canal. Numerous surgical techniques have been described. The M-meatoplasty described by Mirck for addressing the external meatus is widely used. However, this technique does not sufficiently enlarge the external ear canal in all cases. Specifically in patients where the ear canal narrowing is most prominent in the postero- and/or anterosuperior quadrants of the lateral meatus the technique needs some modifications. In these cases, an oblique conversion of the M-meatoplasty, the MO-meatocanalplasty, is useful. In cases where the bony canal is also narrow this modification allows for a bony canalplasty while avoiding a retro-auricular approach. The MO-meatocanalplasty can be used in combination with myringoplasty and tympanoplasty.

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.

Congenital Retrosigmoid Cholesteatoma: Case Series and Literature Review.

BACKGROUND: This study aimed to discuss 3 cases of congenital cholesteatoma located posterior to the sigmoid sinus, with no/minimal involvement of mastoid, and compare them with cases presented in the literature to better define this rare entity. METHODS: Retrospective chart analysis of 3 congenital cholesteatomas located posterior to the sigmoid sinus treated surgically in 2 skull-base centers and literature review. Though congenital cholesteatoma can arise outside the middle ear, only a few cases presenting in the retrosigmoid occipital bone have been described earlier. RESULTS: In all 3 patients, there was a delay in the presentation, as symptoms were nonspecific or lacking, leading in 1 case to severe complications. Computed tomography and magnetic resonance imaging, especially diffusion-weighted imaging scans, allowed accurate diagnosis and surgical planning. Surgery happened to be challenging due to the tight adherence of the cholesteatoma to the thinned dural surface. Complete excision was achieved in all the cases. CONCLUSION: Congenital cholesteatoma located posterior to the sigmoid sinus is a rare entity and is even more exceptional after a critical review of the literature. Complete excision is quintessential to prevent intradural extension or infection. The most important surgical issue is the management of the posterior fossa dura and the sigmoid sinus. We recommend meticulous dissection with slow peeling of the epithelial lining from the dura. Bipolar coagulation of the dura may help in avoiding recidivism. Moreover, cerebrospinal fluid (CSF) leak during dissection has to be avoided as long as possible, because the loss of tension of the already thinned dura makes its peeling particularly difficult.

Ossicular reconstruction: hydroxyapatite bone cement versus incus remodelling: how to manage incudostapedial discontinuity.

The objective of this study was to compare the hearing outcome using hydroxyapatite (HA) bone cement to bridge the incudostapedial gap versus incus remodelling for ossiculoplasty in case of incudostapedial discontinuity. A non-randomized retrospective study was conducted at a tertiary referral otologic centre. The intervention in 24 primary cases of conductive hearing loss was subsequent middle ear inspection where incudostapedial discontinuity was observed. HA bone cement was used in 10 consecutive cases, and incus remodelling was performed in 14 consecutive cases. Air-bone gap (ABG), bone-conduction (BC) thresholds, and air-conduction (AC) thresholds were evaluated preoperatively and at 3, 6 and 12 months postoperatively. No patients were lost to follow-up. Pure-tone averages were calculated according to the guidelines of the Committee on Hearing and Equilibrium for the evaluation of conductive hearing loss. The Amsterdam Hearing Evaluation Plots are presented. The postoperative ABG closure to within 20 and 10 dB at 12 months was, respectively, 80 and 40% in the HA bone cement group and 57.1 and 28.6% in the standard ossiculoplasty group (no statistically significant difference). However, we observed a statistically significant difference in ABG gain at 6 and 12 months favoring the HA bone cement cases. No short-term or intermediate-term adverse reactions were observed. Hydroxyapatite bone cement bridging ossiculoplasty offers a better intermediate-term ABG gain than standard ossiculoplasty. This new technique is a valuable alternative to conventional ossiculoplasty and presents the practical advantage of being easier and faster.

A mutation in HOXA2 is responsible for autosomal-recessive microtia in an Iranian family.

Microtia, a congenital deformity manifesting as an abnormally shaped or absent external ear, occurs in one out of 8,000-10,000 births. We ascertained a consanguineous Iranian family segregating with autosomal-recessive bilateral microtia, mixed symmetrical severe to profound hearing impairment, and partial cleft palate. Genome-wide linkage analysis localized the responsible gene to chromosome 7p14.3-p15.3 with a maximum multi-point LOD score of 4.17. In this region, homeobox genes from the HOXA cluster were the most interesting candidates. Subsequent DNA sequence analysis of the HOXA1 and HOXA2 homeobox genes from the candidate region identified an interesting HOXA2 homeodomain variant: a change in a highly conserved amino acid (p.Q186K). The variant was not found in 231 Iranian and 109 Belgian control samples. The critical contribution of HoxA2 for auditory-system development has already been shown in mouse models. We built a homology model to predict the effect of this mutation on the structure and DNA-binding activity of the homeodomain by using the program Modeler 8v2. In the model of the mutant homeodomain, the position of the mutant lysine side chain is consistently farther away from a nearby phosphate group; this altered position results in the loss of a hydrogen bond and affects the DNA-binding activity.

Skin reactions following BAHA surgery using the skin flap dermatome technique.

The objectives of the study were to determine the incidence of skin reactions and complications associated with bone-anchored hearing aid (BAHA) implantation. The study is a retrospective case review done in a tertiary referral center. One hundred thirty-eight consecutive patients between 1998 and 2008 underwent implantation of a BAHA and were regularly seen for follow-up. Indications included conductive or mixed hearing loss where a hearing aid cannot be used and since 2000 also had contralateral single-sided perceptive hearing loss. BAHA implantation was done by creating a pedicled flap using the skin flap dermatome technique. Postoperative incidence of skin reactions and complications were measured. Significant postoperative complications requiring revision surgery occurred 37 times in 30 patients. Normal skin healing was seen in 52 patients (63.4%), while abnormal skin healing occurred in 30 patients (36.6%). This study showed that skin problems occur more often than expected. Because of the skin problems with the skin flap technique, the authors have switched to the linear incision technique, hoping to decrease the incidence of skin problems.

Decompression of the internal auditory canal via the retrosigmoid approach in a patient with Camurati-Engelmann disease: illustrative case.

BACKGROUND: Camurati-Engelmann disease (CED) is a rare condition characterized by hyperostosis of the long bones and skull base. Symptoms include contractures and pain in affected extremities but can also include manifestations of cranial hyperostosis such as intracranial hypertension, Chiari malformation, exophthalmia, frontal bossing, and several cranial neuropathies due to cranial foraminal stenosis. OBSERVATIONS: This report describes a 27-year-old patient with suspected CED who developed progressive intermittent facial nerve paresis, hemifacial spasms, and a decrease in hearing. There were no symptoms of increased intracranial pressure or vertigo. Radiological evaluation showed a significant thickening of the skull base with serious bilateral internal auditory canal stenosis. Because of the progressive nature of the aforementioned cranial neuropathies in combination with the correlating severe radiological compression, a surgical decompression of the facial nerve and vestibulocochlear nerve was performed via a retrosigmoid approach with intraoperative monitoring. Postoperative facial nerve function was intact. Hearing and vestibular function were unchanged. There were no more episodes of facial nerve palsy or spasm. LESSONS: To the authors' knowledge, this is the first report to describe decompression of the internal auditory canal via a retrosigmoid approach for symptomatic facial and cochlear nerve compression in a patient with CED.

Middle ear cholesteatoma: non-echo-planar diffusion-weighted MR imaging versus delayed gadolinium-enhanced T1-weighted MR imaging--value in detection.

PURPOSE: To retrospectively compare non-echo-planar (non-EP) diffusion-weighted (DW) imaging, delayed gadolinium-enhanced T1-weighted magnetic resonance (MR) imaging, and the combination of both techniques in the evaluation of patients with cholesteatoma. MATERIALS AND METHODS: This institutional review board-approved study, for which the need to obtain informed consent was waived, included 57 patients clinically suspected of having a middle ear cholesteatoma without a history of surgery and 63 patients imaged before "second-look" surgery. Four blinded radiologists evaluated three sets of MR images: a set of delayed gadolinium-enhanced T1-weighted images, a set of non-EP DW images, and a set of both kinds of images. Overall sensitivity, specificity, negative predictive value (NPV), and positive predictive value (PPV), as well as intra- and interobserver agreement, were assessed and compared among methods. To correct for the correlation between different readings, a generalized estimating equations logistic regression model was fitted. Results were compared with surgical results, which were regarded as the standard of reference. RESULTS: Sensitivity, specificity, NPV, and PPV were significantly different between the three methods (P < .005). Sensitivity and specificity, respectively, were 56.7% and 67.6% with the delayed gadolinium-enhanced T1-weighted images and 82.6% and 87.2% with the non-EP DW images. Sensitivity for the combination of both kinds of images was 84.2%, while specificity was 88.2%. The overall PPV was 88.0% for delayed gadolinium-enhanced T1-weighted images, 96.0% for non-EP DW images, and 96.3%for the combination of both kinds of images. The overall NPV was 27.0% for delayed gadolinium-enhanced T1-weighted images, 56.5% for non-EP DW images, and 59.6% for the combination of both kinds of images. CONCLUSION: MR imaging for detection of middle ear cholesteatoma can be performed by using non-EP DW imaging sequences alone. Use of the non-EP DW imaging sequence combined with a delayed gadolinium-enhanced T1-weighted sequence yielded no significant increases in sensitivity, specificity, NPV, or PPV over the use of the non-EP DW imaging sequence alone.

Subarcuate supralabyrinthine approach for supralabyrinthine petrosal cholesteatoma.

Congenital cholesteatomas of the petrosal apex account for 1% to 3% of all cholesteatomas and often present an important surgical challenge. This report describes an exceptional case of a "nondestructive" translabyrinthine surgical approach to a large congenital petrosal cholesteatoma that threatened the vestibulum, superior semicircular canal, facial nerve, and internal auditory canal. We applied a nonconventional transmastoid subarcuate supralabyrinthine approach in a 20-year-old patient by accessing the lesion through the center of the superior semicircular arch without damaging the integrity of the canal. This led to a complete removal of the petrosal cholesteatoma with preservation of hearing and vestibular function. Follow-up imaging performed 1 and 2 years after operation by means of non-echo-planar diffusion-weighted imaging did not show residual cholesteatoma. This report describes the first successful use of a subarcuate supralabyrinthine approach through the arches of the superior semicircular canal in a case of petrosal cholesteatoma.

Cochlear Patency after Translabyrinthine and Retrosigmoid Vestibular Schwannoma Surgery.

OBJECTIVES: To assess the incidence and onset of cochlear obliteration after translabyrinthine and retrosigmoid vestibular schwannoma surgery. MATERIALS AND METHODS: We retrospectively identified a consecutive series of eighty ears in eighty vestibular schwannoma patients who were treated via a translabyrinthine or retrosigmoid approach by a single neuro-otological surgical team in a tertiary referral center from May 2011 to January 2018. Postoperative, high- resolution T2-weighted turbo spin echo three-dimensional magnetic resonance (MR) images of the posterior fossa were evaluated at the level of the membranous labyrinth and internal auditory canal. Perilymphatic patency of the vestibule, basal, and apical cochlear turns were scored and classified as patent, hypointense, partially obliterated, or completely obliterated. RESULTS: Twenty-five vestibular schwannomas were treated with surgery via a translabyrinthine approach, and fifty-five were treated using a retrosigmoid approach; of these, 8% and 65%, respectively, showed no signs of perilymphatic alterations in the basal or apical turns, while 84% and 20%, respectively, showed partial or complete obliteration in the basal or apical turns with a mean postoperative interval of 127 and 140 days, respectively. All the patients who underwent multiple MR scans and had a completely patent perilymphatic system on the first postoperative scan remained patent during subsequent scans; 16% of the patients showed worsened perilymphatic appearance. The onset of cochlear obliteration occurred within 2-7 months in most translabyrinthine patients. CONCLUSION: These findings may support the need for simultaneous cochlear electrode or dummy implantation in translabyrinthine surgery. Second-stage implantation could be feasible in cases where a retrosigmoid approach is used; however, the implantation should be considered within the initial months to avoid cochlear obliteration. Findings on the first postoperative MR could indicate the need for intensified MR follow-up and may even predict the occurrence of cochlear obliteration.

The Value of Diffusion-Weighted MRI in the Long-term follow-up After Subtotal Petrosectomy for Extensive Cholesteatoma and Chronic Suppurative Otitis Media.

OBJECTIVE: To report the long-term follow-up with diffusion-weighted magnetic resonance imaging (DW MRI) after subtotal petrosectomy (SP) with blind sac closure of the external auditory canal for extensive cholesteatoma and chronic suppurative otitis media. STUDY DESIGN: Retrospective clinical record study. SETTING: Tertiary referral center. PATIENTS: Thirty-one patients (31 ears) with extensive cholesteatoma and 17 patients (19 ears) with chronic suppurative otitis media without cholesteatoma who underwent SP between July 1995 and December 2015. INTERVENTIONS: All 48 patients were followed clinically and with DW MRI to rule out residual cholesteatoma. MAIN OUTCOME MEASURE: Residual cholesteatoma, indicated by a marked hyperintensity on non-echoplanar (non-EP) DW MRI. RESULTS: In the cholesteatoma group the mean interval between surgery and the latest DW MRI was 3.9 years. Seven patients presented with a residual cholesteatoma pearl. The mean interval between surgery and detection of residual disease was 3.7 years. In the chronic suppurative otitis media group the mean interval between surgery and the latest DW MRI was 5.3 years. Residual cholesteatoma was found in three patients. Mean interval between surgery and the detection of disease was 4.5 years. CONCLUSIONS: This study shows the importance of DW MRI in the follow-up after SP for cholesteatoma and chronic suppurative otitis media. A little higher residual cholesteatoma rate was found compared with earlier studies, where patients were followed only clinically. One may wonder whether reintervention is always needed or whether in selected cases with small pearls, one may still observe these by a watchful waiting policy with DW MRI.

Cochlear Erosion due to a Facial Nerve Schwannoma.

Facial nerve schwannomas are rare benign neoplasms. We report a case of a 60-year-old woman who initially presented with vestibular complaints. Magnetic resonance imaging (MRI) revealed a facial nerve schwannoma centered on the right geniculate ganglion extending in the labyrinthine segment. The patient consulted again after 2 months because she developed a sudden and severe right-sided sensorineural hearing loss. MRI showed no progression or pathological enhancement in the membranous labyrinth. A cone beam computed tomography (CT) of the temporal bone was performed and revealed a large erosion at the region of the geniculate ganglion in open communication with the middle turn of the cochlea. This case report demonstrates the importance of CT in facial nerve schwannomas for evaluating the impact on the surrounding structures.

Detection of postoperative residual cholesteatoma with non-echo-planar diffusion-weighted magnetic resonance imaging.

OBJECTIVE: The aim of this study was to analyze the role of non-echo-planar imaging (non-EPI)-based diffusion-weighted (DW) magnetic resonance imaging (MRI) for the detection of residual cholesteatoma after canal wall-up mastoidectomy before eventual second-look surgery. STUDY DESIGN: Prospective and blinded study. SETTING: Tertiary referral center. PATIENTS: The study group included the surgical, clinical, and imaging follow-up of 32 consecutive patients after primary cholesteatoma surgery. INTERVENTIONS: All patients were investigated with MRI, including late postgadolinium T1-weighted sequence and non-EPI-DW sequence, 10 to 18 months after first-stage cholesteatoma surgery by experienced surgeons using a canal wall-up mastoidectomy. The non-EPI-DW images were evaluated for the presence of a high-signal intensity lesion consistent with residual cholesteatoma. Imaging findings were correlated with findings from second-stage surgery in 19 patients, clinical follow-up examination in 11 patients, and, in 2 patients, clinical and MRI follow-up examination. RESULTS: Non-EPI-DW sequences depicted 9 of 10 residual cholesteatomas. The only lesion missed was a 2-mm cholesteatoma in an examination degraded by motion artifacts in a child. All other diagnosed cholesteatomas measured between 2 and 6 mm. Sensitivity, specificity, positive predictive value, and negative predictive value were 90, 100, 100, and 96%, respectively. CONCLUSION: Except for motion artifact-degraded examinations, non-EPI-DW MRI is able to detect even very small residual cholesteatoma after first-stage surgery by showing a high-signal intensity lesion. It has the capability of selecting patients for second-look surgery, avoiding unnecessary second-look surgery.

Audiological outcomes after cochlear implantation in a patient with Melnick-Needles syndrome.

This is the first report on a successful cochlear implantation in a patient suffering from the Melnick-Needles syndrome (MNS). This case study presents an MNS patient with peri-lingual profound hearing loss, implanted at the age of 38. MNS is a rare X-linked genetic bony dysplasia characterized by skeletal and facial abnormalities. Various soft tissue defects and hearing loss have also been described in MNS; however, this is the first report on bilateral inner ear malformation as a phenotypic feature of MNS. At 15 months after cochlear implantation there was 91% speech identification in open-set monosyllabic CVC test. In noise, the results obtained with the CVC lists were the following: 67% for SNR +15 dB, 52% for SNR +10 dB and 36% for SNR +5 dB. The patient is able to discriminate the differences in all 22 phoneme pairs in the APE phoneme discrimination test. This allows the patient to obtain significant communication ability through the telephone. Conclusions are as follows. 1) Bilateral inner ear malformations and bilateral profound perceptive hearing loss possibly belong to the phenotypic features of MNS. 2) Cochlear implant is potentially a good functional solution for patients suffering from MNS and profound hearing loss. 3) Exceptionally good results on auditory performance have been obtained in this MNS patient with peri-lingual profound hearing loss and almost 36 years duration of deafness.

What is the Required Frequency of MRI Scanning in the Wait and Scan Management?

The wait and scan policy is being increasingly used as the first measure after the diagnosis of a vestibular schwannoma (VS) using magnetic resonance imaging (MRI). As part of the European Academy of Otology and Neuro-Otology (EAONO) position statement on VS, the frequency of imaging has been studied in the literature. Among 163 studies, 29 fulfilled the inclusion criteria and were scored using the Grading of Recommendations, Assessment, Development, and Evaluation system. Because tumor growth rate during the first 5 years of follow-up is predictive of further growth during the upcoming years, a protocol for wait and scan is useful for centers dealing with this condition. The EAONO proposal is that after the initial diagnosis by MRI, a first new MRI would take place after 6 months, annually for 5 years, and then every other year for 4 years, followed by a lifelong MRI follow-up every 5 years. The first early MRI is to screen for fast-growing tumors, and the lifelong follow-up with tapered intervals is to detect late repeated growth.

EAONO position statement on Vestibular Schwannoma: Imaging Assessment Question: How should growth of Vestibular Schwannoma be defined?

The relevance of defining the growth of vestibular schwannoma (VS) is that any significant VS growth may impact treatment strategy. A conservative treatment strategy is often proposed as a primary treatment option in the management of VS. Several authors have demonstrated that a significant proportion of VSs do not grow, and those that do, usually grow slowly. Surgical and/or radiosurgical treatment options may be offered to the patient according to the VS growth. Therefore, defining the VS growth is a determinant in managing treatment strategies. A comprehensive literature search was performed to examine the definition of tumor growth for VS. The literature review was conducted using PubMed and Embase databases dated back to 20 years (1995-2015) and was updated until February 2015. VS growth should be measured on contrast-enhanced T1-weighted images. Although there the overall quality of the present studies is low, all highlight a significant VS growth of > 2 mm, and/or 1.2 cm3, and/or 20% change in volume, and/or the square of the product of the 2 orthogonal diameters. We suggest that VS growth should instead change management strategies when a 3-mm increase in diameter on two consecutive MRI scans are performed 1 year apart.

The Bony Obliteration Tympanoplasty in Pediatric Cholesteatoma: Long-term Hearing Results.

OBJECTIVE: To present the hearing results of a 5-year longitudinal study in a pediatric population undergoing surgery for extensive cholesteatoma using a canal wall up (CWU) approach with bony obliteration of the mastoid and epitympanic space, with a standard residual rate of 5.8%, a recurrence rate of 2.9%, and all ears waterproof, free of otorrhea and all external ear canals patent and self-cleaning. 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: Hearing and gain in hearing at 1- and 5-year postsurgery: (1) pure-tone average (PTA), (2) pure-tone average high frequency, (3) pure-tone average including 3 kHz, (4) bone conduction at corresponding frequency averages, (5) gain at corresponding air conduction and bone conduction (gain at corresponding air conduction) frequency averages. (6) The Amsterdam Hearing Evaluation Plots were used to study the individual cases. RESULTS: The Amsterdam Hearing Evaluation Plots at 5-year showed in 58.8% of patients a positive gain air conduction. In 23.5% a successful functional result was achieved, defined as an air-bone gap closure to 20 dBHL or less. In 6 patients (17.6%) a limited bone conduction deterioration was shown all limited to maximum 20 dBHL. CONCLUSION: The CWU bony obliteration tympanoplasty in a consecutive series of pediatric extensive cholesteatoma shows a similar to slightly improved hearing outcome as compared with CWU surgery without BOT. Although the series shows a clearly reduced reoperation rate and a significantly improved safety and hygienic outcome as compared with CWU without BOT, better hearing outcomes remain desirable in this group of children.

The value of single-shot turbo spin-echo diffusion-weighted MR imaging in the detection of middle ear cholesteatoma.

INTRODUCTION: Single-shot (SS) turbo spin-echo (TSE) diffusion-weighted (DW) magnetic resonance imaging (MRI) is a non echo-planar imaging (EPI) technique recently reported for the evaluation of middle ear cholesteatoma. We prospectively evaluated a SS TSE DW sequence in detecting congenital or acquired middle ear cholesteatoma and evaluated the size of middle ear cholesteatoma detectable with this sequence. The aim of this study was not to differentiate between inflammatory tissue and cholesteatoma using SS TSE DW imaging. METHODS: A group of 21 patients strongly suspected clinically and/or otoscopically of having a middle ear cholesteatoma without any history of prior surgery were evaluated with late post-gadolinium MRI including this SS TSE DW sequence. RESULTS: A total of 21 middle ear cholesteatomas (5 congenital and 16 acquired) were found at surgery with a size varying between 2 and 19 mm. Hyperintense signal on SS TSE DW imaging compatible with cholesteatoma was found in 19 patients. One patient showed no hyperintensity due to autoevacuation of the cholesteatoma sac into the external auditory canal. Another patient showed no hyperintensity because of motion artifacts. CONCLUSION: This study shows the high sensitivity of this SS TSE DW sequence in detecting small middle ear cholesteatomas, with a size limit as small as 2 mm.