Cochlear implant surgery in patients with otosclerosis can be challenging, with a relatively high number of partial insertions and misplacements of the electrode array demanding revision surgery. A very high proportion of patients experienced facial nerve stimulation mainly caused by the distal electrodes. This must be discussed with patients preoperatively.

The functioning of the nasal valve area is largely determined by the stability and the mobility of the lateral nasal wall. To gain insight into the kinematics of the lateral nasal wall, we studied the functional anatomy of the nasal muscles and the intercartilaginous and osseous-cartilaginous junctions. We performed gross and microscopic nasal dissection and serial sectioning in 15 human cadaveric noses. In addition, two noses were used for three-dimensional reconstruction of the nasal cartilages. We conclude that the lateral nasal wall can be seen as made up of three parts. At the level of the osseous-cartilaginous chain of bone, lateral nasal cartilage, and lateral crus, the lateral nasal wall is relatively stable, limited mobility being allowed by translation and rotation in the intercartilaginous joint and a coupled distortion of the cartilages. At the level of the hinge area the lateral nasal wall is supported by one or more accessory cartilages, embedded in soft tissue, and therefore much more compliant. The alar part of the nasalis muscle, which originates from the maxilla and inserts on these cartilages, may dilate the valve area by drawing this hinge area laterally. The third and most compliant part of the lateral nasal wall is the part that is not supported by cartilage, the ala. The dilatator naris muscle largely occupies the ala and is attached to the lateral crus; it opens the vestibule and nostril. The third nasal muscle that influences the lateral nasal wall is the transverse part of the nasalis muscle. It overlies the nose but is not attached to it. This muscle stabilizes the lateral nasal wall, in particular, the lateral nasal cartilage, the intercartilaginous junction, and the hinge area, by moving the nasal skin.

In all, 20% of the congenitally deaf children in our center study have inner ear abnormalities. Inner ear malformations were limited to incomplete partition of the cochlea; none of the subjects had common cavity malformations. Electrical compound action potentials were successfully recorded in both groups intraoperatively. Speech perception tests on open-set speech yielded an average of 48.8% (SD 21.2%) in the group of children with inner ear malformations vs 54.5% (SD 21.1%) in congenitally deaf children. In four of nine cases with an inner ear malformation we encountered a minor CSF leak.

Electromyographic (EMG) activity of six nasal muscles was monitored in 17 male volunteers without nasal complaints. Surface electrodes were placed on the nasal skin in such a way that they selectively recorded the activity of these muscles. Recordings were made under different breathing conditions and during voluntary nasal movements. Inspiratory EMG activity was observed during nasal and oral breathing in one or more of the following muscles: dilator naris, nasalis muscle (alar and transverse parts) and apicis nasi. EMG activity increased markedly in response to physical exercise and was more often present in subjects with decreased nasal patency. During voluntary nasal movements a combined activity of the six nasal muscles was consistently found. We conclude that the function of the dilator naris, the nasalis muscle and the apicis nasi strongly relates to respiration. These muscles probably contribute to the prevention of collapse of the nasal valve. The role of the procerus and levator labii superioris alaeque nasi seems to be primarily concerned with facial expression.

Objectives: To analyse the speech perception performance of 53 cochlear implant recipients with otosclerosis and to evaluate which factors influenced patient performance in this group. The factors included disease-related data such as demographics, pre-operative audiological characteristics, the results of CT scanning and device-related factors. Methods: Data were reviewed on 53 patients with otosclerosis from 4 cochlear implant centres in the United Kingdom and the Netherlands. Comparison of demographics, pre-operative CT scans and audiological data revealed that the patients from the 4 different centres could be considered as one group. Speech perception scores had been obtained with the English AB monosyllable tests and Dutch NVA monosyllable tests. Based on the speech perception scores, the patients were classified as poor or good performers. The characteristics of these subgroups were compared. Results: There was wide variability in the speech perception results. Similar patterns were seen in the phoneme scores and BKB sentence scores between the poor and good performers. The two groups did not differ in age at onset of hearing loss, duration of hearing loss, progression, age at onset of deafness, or duration of deafness. Conclusions: The clinical presentation of the otosclerosis (rapid or slow progression) did not influence speech perception. Better performance was related to less severe signs of otosclerosis on CT scan, full insertion of the electrode array, little or no facial nerve stimulation and little or no need to switch off electrodes.