[MRI in patients with auditory implants equipped with implanted magnets-an update : Overview and procedural management]. / MRT bei Patienten mit Hörimplantaten mit Magnetausstattung ­ ein Update : Überblick und prozedurales Management.

BACKGROUND: More than 100,000 patients with cochlear implants live in Germany. In addition, numerous patients have auditory bone conducted, middle-ear conducted or brainstem conducted implants equipped with implanted magnets. At the same time, the number of patients being examined by magnetic resonance imaging (MRI) is increasing. Therefore, MRI compatibility of these implants is an essential quality feature. METHODS: This article provides information about technical innovations and new auditory devices since November 2013 that have medical-technical certification in the European Union and the USA. We communicate the restrictions of the manufacturers and a selective literature search in PubMed using the following keywords: MRI compatibility/MRI safety + cochlear implant/auditory brainstem implant/Bonebridge/Sophono alpha/Vibrand Soundbridge/BAHA attract. We included all publications of this search concerning MRI compatibility of hearing implants complemented by papers cited in the primary articles. RESULTS: In rare cases, high electromagnetic field intensities as used in MRI can cause shearing movements up to dislocation of the implant or the magnet of the device. As a result the implant function could fail. Image artifacts in head MRIs can be reduced by using appropriate MRI sequences. Nevertheless, possible artifacts and the hereby reduced validity of the skull MRI results have to be considered when indicating the examination. Meanwhile, all innovations of these auditory devices are licensed to 1.5 T MRI examination, some implants up to 3.0 T MRI magnetic field intensity. For older devices, the necessary safety measures listed in the article published by Nospes, Mann and Keilmann in November 2013 should be used. CONCLUSION: Respecting the manufacturer's instructions, MRI scans without removal of the magnet in patients with these auditory implants is safe. However, due to possible defects/dislocations of the implant that may occur and the reduced quality of the skull MRI images, the indication for MRI in devices with MRI certification should only be performed under close consultation between the investigating physicians, the implanting team supervising the patient and the radiologist. All other possible diagnostic procedures should be exhausted first.

Alterations of mucosa of the larynx and hypopharynx in patients with mucopolysaccharidoses.

OBJECTIVE: This study aimed to: assess the mucosal alterations of the larynx and hypopharynx typical for mucopolysaccharidoses, in a standardised manner; compare the severity in different subtypes of mucopolysaccharidoses; and monitor the effect of an enzyme replacement therapy. METHODS: A classification for mucosal alterations of the larynx and hypopharynx was developed and utilised in 55 patients with mucopolysaccharidoses. Fifteen patients who started treatment with enzyme replacement therapy were followed longitudinally. RESULTS: The most severe alterations were seen in the posterior region of the larynx and the arytenoids, and in the region of the false vocal folds. The alterations were most severe in patients with mucopolysaccharidosis II. No clear trend was observed in the patients who received enzyme replacement therapy. CONCLUSION: Quantification of mucosal alterations of the hypopharynx and larynx in mucopolysaccharidoses patients can provide information about the disease's natural process and about the efficacy of enzyme replacement therapy.

[Magnetic resonance imaging in patients with magnetic hearing implants: overview and procedural management]. / Magnetresonanztomographie bei Patienten mit magnetversorgten Hörimplantaten: Überblick und prozedurales Management.

BACKGROUND: Every year in Germany approximately 3,500 patients receive a cochlear implant or other hearing implants with an implantable magnet. At the same time more and more patients are examined by magnetic resonance imaging (MRI). For the indications and execution of this imaging modality a number of restrictions and safety measures have to be considered. METHODS: This article is based on the restrictions of the manufacturers and a selective literature search in PubMed using the following keywords: MRI compatibility/MRI safety + cochlea implant/auditory brainstem implant/Bonebridge/Carina/Esteem/Otomag/Sophono alpha/Vibrand Soundbridge. We included all 20 publications of this search concerning the MRI compatibility of the hearing implants complemented by papers cited in the primary articles. RESULTS: High electromagnetic field intensities as used in MRI can cause malfunction and dislocation of the implant or the magnet in the device. Older cochlear implants (CI) and the current CIs produced by Advanced bionics without explantation of the magnet, some CI models produced by the company Cochlear and the middle ear implants Carina®/Esteem® (older models) and Vibrant-Soundbridge® are not approved for MRI examinations. Other hearing prostheses are approved for 0.2 T, 1.0 T or 1.5 T MRI and in exceptional circumstances 3 T MRI. Recommendations of the manufacturers have to be followed, notably wearing a head bandage during the imaging procedure. The longitudinal axis of the patient's head has to be to positioned parallel to the main magnetic field of the scanner. The patient may not move the head laterally during the examination. Possible artefacts and the reduced validity of the results of skull MRI have to be considered when evaluating the indications for the examination. CONCLUSION: For patients wearing hearing implants with an implantable magnet the indications for MRI in devices with MRI certification should be rigorously restricted. Possible defects/dislocation of the implants may occur and the quality of the skull MRI images is reduced. A close contact between the radiologist and the implanting team is required. Other diagnostic procedure options should be exhausted before employing MRI.

[Symptoms and diagnosis of auditory processing disorder]. / Symptome und Diagnosestellung auditiver Verarbeitungs- und Wahrnehmungsstörungen.

The definition of an auditory processing disorder (APD) is based on impairments of auditory functions. APDs are disturbances in processes central to hearing that cannot be explained by comorbidities such as attention deficit or language comprehension disorders. Symptoms include difficulties in differentiation and identification of changes in time, structure, frequency and intensity of sounds; problems with sound localization and lateralization, as well as poor speech comprehension in adverse listening environments and dichotic situations. According to the German definition of APD (as opposed to central auditory processing disorder, CAPD), peripheral hearing loss or cognitive impairment also exclude APD. The diagnostic methodology comprises auditory function tests and the required diagnosis of exclusion. APD is diagnosed if a patient's performance is two standard deviations below the normal mean in at least two areas of auditory processing. The treatment approach for an APD depends on the patient's particular deficits. Training, compensatory strategies and improvement of the listening conditions can all be effective.

[Hearing disorders in aphasia]. / Hörstörungen bei Aphasie.

Aphasia is an acquired communication disorder that often involves receptive language abilities. After clinical assessment it is often not clear if this is partially due to a hearing loss, which can be compensated by hearing aids facilitating the rehabilitative process.In the present study the hearing ability of 88 male and female patients with aphasia after stroke, all of whom suffered from a left-hemispheric ischemia was assessed in the rehabilitative setting.We found that a majority of patients (72, 82%) was able to perform pure tone audiometry. 15 aphasic patients (21%) showed a hearing loss and were not fitted with hearing aids.Patients with aphasia are due to their central speech disorders in their communication skills limited, so that the therapeutic success is further reduced by an existing hearing loss. Due to the demographic development of our people and with the age increasing prevalence of hearing impairment hearing screening in the post-acute phase in aphasic patients is justified by pure tone audiometry.

[Stroboscopy findings: a comparison of flexible CCD-videostroboscopy and rigid stroboscopy]. / Stroboskopie-Befunde: Flexible CCD-Videostroboskopie und klassische Lupenstroboskopie im Vergleich.

OBJECTIVE: After exclusion of morphologic laryngeal alterations by laryngoscopy the prospective study compared stroboscopy findings using a flexible distal charge-coupled device chip-optic (CCD-optic) and a rigid 70° - or 90° -laryngoscope. MATERIAL AND METHODS: 52 patients with functional dysphonia and 47 candidates for speech therapy education were checked with both examination methods. The stroboscopy results were rated randomized and pseudonymized by 3 experts assessed by a study protocol according to the European laryngological society basic protocol 2001. RESULTS: The interrater-reliability was moderate to good. Using the flexible videolaryngoscopy less gaging, less supraglottic contraction during phonation, more often a complete glottal closure and more often a normal mucosal wave movement were found. CONCLUSION: To get an optimal endoscopy result the combination of rigid laryngoscopy and flexible videolaryngoscopy and -stroboscopy will be recommended. Because of the variety of stroboscopic findings for the diagnosis of functional dysphonia additional the case history and functional voice examinations are necessary.

GJB2 mutations and genotype-phenotype correlation in 335 patients from germany with nonsyndromic sensorineural hearing loss: evidence for additional recessive mutations not detected by current methods.

We report on 335 patients (319 families) with mild-to-profound nonsyndromic sensorineural hearing loss. We identified 178 mutated GJB2 alleles representing 29 different sequence changes (including 3 novel mutations: Q7P, N14D, H100Q), and 2 alleles with the deletion del(GJB6-D13S1830) of the GJB6 gene. Eleven GJB2 mutations (119 mutated alleles) were truncating (T), and 18 mutations (59 alleles) were nontruncating (NT). Biallelic GJB2 mutations were found in 71 patients (21.2%; 67 families; 25 different genotypes). Audiograms of 62 patients (56 families) with biallelic GJB2 mutations typically indicated a profound hearing loss with T/T mutations, moderate hearing loss with T/NT mutations, and mild hearing impairment with NT/NT mutations (p < 0.01, Student's t test). From 37 patients (34 families) with biallelic GJB2 mutations, audiograms at different ages were available and indicated progressive hearing loss (>15 dB) in 10 patients (27.0%, 10 families). Interestingly, we identified an unexpectedly large subset of patients (n = 29; 8.7%) presenting with only one GJB2 mutation (n = 14 T/wild-type; n = 15 NT/wild-type). This strongly suggests the presence of additional recessive mutations that are not detected by current GJB2 mutation and GJB6 deletion analyses.