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.

Long-term Results of Troublesome CWD Cavity Reconstruction by Mastoid and Epitympanic Bony Obliteration (CWR-BOT) in Adults.

OBJECTIVE: To present the long-term surgical outcome of the bony mastoid and epitympanic obliteration technique with canal wall reconstruction (CWR-BOT) in adults with an unstable cavity after previous canal wall-down surgery for extensive cholesteatoma. STUDY DESIGN: Retrospective study. INTERVENTIONS: Therapeutic. SETTING: Tertiary referral center. PATIENTS: Fifty consecutive adult patients undergoing a CWR-BOT between 1998 and 2009. MAIN OUTCOME MEASURE(S): (A) Recurrence and residual rates of cholesteatoma, (B) postoperative hygienic status of the ear, including postoperative aspect of the tympanic membrane and external ear canal integrity (EAC), (C) functional outcome, and (D) long-term safety issues. RESULTS: (A) The percentage of ears remaining safe without recurrent or residual disease after CWR-BOT was 96% after a mean follow-up time of 101.8 months. Recurrent cholesteatoma occurred in 2% (n = 1) and a residual cholesteatoma was detected in 2% (n = 1) of the patients. (B) A safe dry, and trouble-free graft and selfcleaning EAC was achieved in 94%. (C) The postoperative hearing results showed a gain of 1.7 dB on pure-tone average air-conduction. (D) Nonecho planar diffusion-weighted imaging (non-EP DW magnetic resonance imaging) documented the residual (n = 1) and recurrent cholesteatoma (n = 1). The 1- and 5-year imaging follow-up revealed no other recurrent or residual disease. CONCLUSION: The CWR-BOT is a safe and very effective option for treatment of problematic unstable canal wall-down mastoid cavities, resulting in dry trouble-free ears.

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.

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.

Pathology of the vestibulocochlear nerve.

There is a large scala of pathology affecting the vestibulocochlear nerve. Magnetic resonance imaging is the method of choice for the investigation of pathology of the vestibulocochlear nerve. Congenital pathology mainly consists of agenesis or hypoplasia of the vestibulocochlear nerve. Tumoral pathology affecting the vestibulocochlear nerve is most frequently located in the internal auditory canal or cerebellopontine angle. Schwannoma of the vestibulocochlear nerve is the most frequently found tumoral lesion followed by meningeoma, arachnoid cyst and epidermoid cyst. The most frequently encountered pathologies as well as some more rare entities are discussed in this chapter.

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.

Imaging of temporal bone tumors.

Tumoral lesions of the temporal bone are relatively rare. Cross-sectional imaging plays an important role in the description of extension of these lesions. In certain lesions, imaging characteristics are rather specific, giving a clue to diagnosis. The most common tumoral lesions of the external, middle, and inner ear are discussed. Some rare lesions are also highlighted.

Mastoid and epitympanic bony obliteration in pediatric cholesteatoma.

OBJECTIVE: The primary goal of cholesteatoma surgery is complete eradication of the disease. To lower the recurrence rate in the pediatric population in canal wall up techniques and to avoid the disadvantages of canal wall down techniques, the bony obliteration technique with epitympanic and mastoid obliteration has been developed. The objective of this study was to evaluate the long-term surgical outcome and recurrence rate of this technique in children. STUDY DESIGN: Retrospective case review. SETTING: : Tertiary referral center. PATIENTS: Fifty-two children (<16 yr) were operated on in 90.4% (n = 47) for a primary or recurrent cholesteatoma and in 9.6% (n = 5) for an unstable cavity. INTERVENTION: In all cases, we closed the tympanoattical barrier and the posterior tympanotomy with sculpted cortical bone and then completed obliteration of the epitympanum and mastoid with bone pâté. A reconstruction of the middle ear was performed by means of an allograft tympanic membrane including the malleus handle and a sculpted allograft malleus or incus for columellar reconstruction. MAIN OUTCOME MEASURES: Recurrent rate; residual rate; functional outcome; hygienic status of the ear; long-term safety issues. RESULTS: The mean follow-up time was 49.5 months (range, 12-101.3 mo). Recurrent cholesteatoma occurred in 1.9% (n = 1). Residual cholesteatoma was detected in 15.4% (n = 8) of the cases. Postoperative hearing results revealed a median gain on pure-tone averages of 14.3 dB and a median postoperative air-bone gap of 25.6 dB. CONCLUSION: The mastoid and epitympanic BOT is an effective technique to lower the recurrence rate of cholesteatoma in the pediatric population. Follow-up by magnetic resonance imaging provides a safe, noninvasive method for postoperative detection of residual cholesteatoma.

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.

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.

Value of high-resolution computed tomography and magnetic resonance imaging in the detection of residual cholesteatomas in primary bony obliterated mastoids.

PURPOSE: The objective of this study was to assess the value of high-resolution computed tomography (HRCT) and that of magnetic resonance imaging (MRI), including postcontrast T(1)-weighted images and echo-planar diffusion-weighted (EP-DW) images, in the detection of residual cholesteatomas after primary bony obliteration of the mastoid. PATIENTS AND METHODS: Twenty-three patients underwent a second-look surgery 8 to 18 months after they underwent a primary bony obliteration technique. All patients were evaluated by HRCT and MRI before their second-look surgery. A retrospective analysis was performed. RESULTS: A residual cholesteatoma was found in 2 of the 23 patients; both cases of cholesteatoma had a diameter less than 4 mm. In these 2 patients, residual cholesteatoma was found in the middle ear cavity and not in the obliterated mastoid. In all cases, HRCT showed a homogeneous obliteration of the mastoid cavity. On MRI, only one cholesteatoma pearl was detected using contrast-enhanced T(1)-weighted imaging. Findings from the EP-DW imaging were negative for all cases. CONCLUSION: This study demonstrates that HRCT is still the imaging technique of choice for the evaluation of bony obliterated mastoids. It shows the low sensitivity and specificity of HRCT for the characterization of an associated opacified middle ear and those of contrast-enhanced T(1)-weighted imaging and EP-DW imaging for the detection of small residual cholesteatomas after primary bony obliteration.

Congenital nasal pyriform aperture stenosis: a rare cause of neonatal nasal obstruction.

Congenital nasal pyriform aperture stenosis has been described as an unusual cause of neonatal nasal obstruction. Clinical suspicion is based on respiratory distress, cyclic cyanosis, apneas, and feeding difficulties. A bony overgrowth of the maxillary nasal processes is thought to be responsible for this deformity. This anomaly has been reported as an isolated feature or can be associated with craniofacial or central nervous system anomalies. Surgery is indicated in cases of severe respiratory distress, feeding difficulties, and when conservative methods fail.

The value of diffusion-weighted MR imaging in the diagnosis of primary acquired and residual cholesteatoma: a surgical verified study of 100 patients.

Our goal was to determine the value of echo-planar diffusion-weighted MR imaging in detecting the presence of primary acquired and residual cholesteatoma. One hundred patients were evaluated by preoperative magnetic resonance (MR) imaging with diffusion-weighted MR imaging. The patient population consisted of a first group of 55 patients evaluated in order to detect the presence of a primary acquired cholesteatoma. In the second group, 45 patients were evaluated for the presence of a residual cholesteatoma 8-18 months after cholesteatoma surgery, prior to second-look surgery. Surgical findings were compared with preoperative findings on diffusion-weighted imaging (DWI). The sensitivity, specificity, positive and negative predictive values of both groups was assessed. In the group of primary surgery patients, hyperintense signal compatible with cholesteatoma was found in 89% of cases with a sensitivity, specificity, positive and negative predictive value for DWI of 81, 100, 100 and 40%, respectively. In the group of second-look surgery patients, only one of seven surgically verified residual cases was correctly diagnosed using DWI, with a sensitivity, specificity, positive and negative predictive values of 12.5, 100, 100 and 72%, respectively. These results confirm the value of DWI in detecting primary cholesteatoma, but show the poor capability of DWI in detecting small residual cholesteatoma.

Stapedotomy with microdrill or carbon dioxide laser: influence on inner ear function.

OBJECTIVES: A prospective randomized audiological analysis of 336 otosclerosis operations was conducted to compare the evolution of bone conduction thresholds after primary stapedotomy with two different techniques to open the footplate: microdrill and carbon dioxide laser stapedotomy. METHODS: To monitor the inner ear function, we compared the preoperative bone conduction thresholds with the postoperative levels at day 2, week 2, week 6, and month 6. Evolution of the bone conduction was compared for the two studied groups (laser versus microdrill). RESULTS: An average bone conduction loss of 1.8 dB was measured at day 2 for the middle frequencies (0.5, 1, and 2 kHz). At 4 kHz, a bone conduction loss of 7 dB was found. The bone conduction thresholds measured in the first and second months after surgery showed a gradual recovery with overclosure as the end result. CONCLUSIONS: Our results confirm the transient depression of inner ear function in the immediate postoperative period, with recovery within the first weeks after surgery. In the studied population, no statistically significant difference was found between the two techniques that were used to make the calibrated hole (laser versus microdrill). These results demonstrate that both techniques possess the same early effect regarding inner ear function. The authors hypothesize that an early inflammatory reaction could be the cause of the transient bone conduction shift.

The use of malleus allografts in ossiculoplasty.

OBJECTIVE: To assess the functional performance of remodeled malleus allografts in a malleus-footplate assembly in terms of hearing results and mid long-term stability. STUDY DESIGN: A retrospective study of 60 consecutive patients who underwent a malleus allograft ossiculoplasty from 1993 until 2000. In all cases the incus and the stapedial arch were missing as the result of cholesteatoma (49), chronic otitis (5), incus necrosis resulting from stapes prosthesis (5), and congenital ossicular malformations (1). In all cases malleus allografts were remodeled to form a malleus-stapes assembly. RESULTS: The audiometric results, using such an ossiculoplasty, revealed an overall median gain of 18.3 dB at 2 months, 22.3 dB at 6 months, and 25 dB 1 year postoperatively on Fletcher frequencies. An air-bone gap closure within 20 dB was achieved in 81% of all cases 1 year postoperatively. No cases of extrusion have been seen in our series. CONCLUSION: Our findings suggest that malleus allografts are capable of generating good and stable functional results as malleus-stapes assembly.