S Passetti

Preservation of residual hearing following cochlear implantation: comparison between three surgical techniques.

The preservation of residual hearing is becoming a high priority in cochlear implant surgery. It allows better speech understanding and ensures long-lasting and stable performance; it also allows the possibility, in selected cases, of combining electro-acoustic stimulation in the same ear. We present the results of a retrospective study of the conservation of residual hearing in three different groups of patients who had undergone cochlear implantation using three different cochlear implant electrode arrays, combined with three different surgical techniques for the cochleostomy. The study aimed to evaluate which approach allowed greater preservation of residual hearing. The best residual hearing preservation results (i.e. preservation in 81.8 per cent of patients) were achieved with the Contour Advance electrode array, using the Advance Off-Stylet technique and performing a modified anterior inferior cochleostomy; this combination enabled reduced trauma to the lateral wall of the cochlea during electrode insertion.

Mitochondrial non-syndromic sensorineural hearing loss: a clinical, audiological and pathological study from Italy, and revision of the literature.

Over the last decade, a number of distinct mutations in the mtDNA (mitochondrial DNA) have been found to be associated with both syndromic and non-syndromic forms of hearing impairment. Their real incidence as a cause of deafness is poorly understood and generally underestimated. Among the known mtDNA mutations, the A1555G mutation in the 12S gene has been identified to be one of the most common genetic cause of deafness, and it has been described to be both associated to non-syndromic progressive SNHL (sensorineural hearing loss) and to aminoglycoside-induced SNHL. In the present study, we have investigated the presence of mtDNA alterations in patients affected by idiopathic non-syndromic SNHL, both familiar and sporadic, in order to evaluate the frequency of mtDNA alterations as a cause of deafness and to describe the audiological manifestations of mitochondrial non-syndromic SNHL. In agreement with previous studies, we found the A1555G mutation to be responsible for a relevant percentage (5.4%) of cases affected with isolated idiopathic sensorineural hearing impairment.

Fully implantable Otologics MET Carina™ device for the treatment of sensorineural and mixed hearing loss: Audio-otological results

Abstract Conclusion: Our results attest that the Otologics MET Carina™ is a viable treatment for moderate to severe sensorineural hearing loss (SNHL) and for cases of mixed hearing loss, and that in selected cases it could represent an alternative to conventional hearing aids. Objectives: To describe our experience with the fully implantable Carina™ in eight adult patients, seven with moderate to severe SNHL and one with mixed hearing loss. Methods: Eight implanted adult patients were submitted to a comprehensive audiological evaluation. Results: We did not record any surgical relevant complication in any of the patients, nor any significant postoperative variation in hearing thresholds, for air conduction or bone conduction, indicating the absence of surgical damage to the cochlea. All the patients demonstrated improvements in speech perception abilities with the device functioning and reported subjective benefits. With regard to the postoperative adverse effects, we had problems with feedback noise, which resolved with minor fitting adjustments in seven cases, while it required a second surgery to change the microphone position in the other patient. In one case a minimal extrusion of the microphone cable occurred requiring a revision surgery, a device failure occurred in one case, requiring substitution, and one patient decided on explantation of the device owing to psychological problems.

A rare association between neurofibromatosis type I and Cogan's syndrome: Case report

Abstract Sensorineural hearing loss (SNHL) has been rarely reported in neurofibromatosis type 1 (NF type 1) and literature data concerning its pathogenesis are lacking. We report a patient affected by NF type 1, who several years later developed Cogans syndrome, with a quickly progressive bilateral SNHL, leading to total deafness. Cochlear implantation was carried out in a partially ossified cochlea, with a good outcome. On the basis of our findings, we underscore the importance of comprehensive evaluation of patients with NF type 1 who develop SNHL, to accurately evaluate the pathogenesis of the hearing loss and explore other possible causes of hearing impairment especially with the emergence of new symptoms. If a retrocochlear lesion is excluded, a cochlear implantation is a viable option for those NF type 1 patients who develop a severe to profound SNHL. We also highlight the importance of a prompt and accurate radiological study of the petrous bone in patients with Cogans syndrome considered for cochlear implantation, in order to plan a proper surgical approach and the importance of a thorough post-implant medical follow-up.