The tectorial membrane is an extracellular matrix of the inner ear that contacts the stereocilia bundles of specialized sensory hair cells. Sound induces movement of these hair cells relative to the tectorial membrane, deflects the stereocilia, and leads to fluctuations in hair-cell membrane potential, transducing sound into electrical signals. Alpha-tectorin is one of the major non-collagenous components of the tectorial membrane. Recently, the gene encoding mouse alpha-tectorin (Tecta) was mapped to a region of mouse chromosome 9, which shows evolutionary conservation with human chromosome 11q (ref. 3), where linkage was found in two families, one Belgian (DFNA12; ref. 4) and the other, Austrian (DFNA8; unpublished data), with autosomal dominant non-syndromic hearing impairment. We determined the complete sequence and the intron-exon structure of the human TECTA gene. In both families, mutation analysis revealed missense mutations which replace conserved amino-acid residues within the zona pellucida domain of TECTA. These findings indicate that mutations in TECTA are responsible for hearing impairment in these families, and implicate a new type of protein in the pathogenesis of hearing impairment.

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.

After more than 25 years of clinical experience, the BAHA (bone-anchored hearing aid) system is a well-established treatment for hearing-impaired patients with conductive or mixed hearing loss. Owing to its success, the use of the BAHA system has spread and the indications for application have gradually become broader. New indications, as well as clinical applications, were discussed during scientific roundtable meetings in 2004 by experts in the field, and the outcomes of these discussions are presented in the form of statements. The issues that were discussed concerned BAHA surgery, the fitting range of the BAHA system, the BAHA system compared to conventional devices, bilateral application, the BAHA system in children, the BAHA system in patients with single-sided deafness, and, finally, the BAHA system in patients with unilateral conductive hearing loss.

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.

Submillimetric gradient-echo images (eg, 3DFT-CISS) should always be used to exclude aplasia or hypoplasia of the cochlear branch of the VCN in all cochlear implant candidates and patients with congenital deafness. This entity, which can occur with or without associated labyrinthine malformation, should be confirmed in two planes.

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.

All children with congenital deafness who underwent implantation before the age of 6 years appeared to benefit from the implant. However, these data add evidence to the importance of early implantation (before the age of 2 years). Intervention before the age of 4 years seemed to be critical to avoid irreversible auditory performance losses, and intervention before the age of 2 years seemed to be critical to achieve optimal results.

Purpose:To retrospectively compare non-echo-planar (non-EP) diffusion-weighted (DW) imaging, delayed gadoliniumenhanced 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, specifi city, 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 fi tted. Results were compared with surgical results, which were regarded as the standard of reference. Results:Sensitivity, specifi city, NPV, and PPV were signifi cantly different between the three methods ( P , .005). Sensitivity and specifi city, 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 specifi city 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 signifi cant increases in sensitivity, specifi city, NPV, or PPV over the use of the non-EP DW imaging sequence alone.q RSNA, 2010 Supplemental material: http://radiology.rsna.org/lookup /suppl