Bilateral cochlear implant users can at least qualitatively benefit from the effects that are known from normal-hearing subjects, that is, head shadow, summation, and squelch effect. Bilateral cochlear implantation also reduces the performance gap between cochlear implant users and normal-hearing subjects.

These results support a growing consensus that bilateral implantation provides functional benefits beyond those of unilateral implantation. Longitudinal data suggest that some aspects of binaural processing continue to develop up to 1 yr after implantation. The squelch effect, often reported as absent or rare in previous studies of bilateral cochlear implantation, was present for most subjects at the 1 yr measurement interval.

Bilateral cochlear implants offer a substantial benefit in sound localization to late-deafened, late-implanted subjects. The very limited data from early-deafened subjects implanted at a later age could suggest that these subjects may not benefit in sound localization from bilateral cochlear implants. It is possible that early implantation for early deafened subjects might allow better acquisition of spatial hearing, thus leading to improved localization performance.

We report the genetic analysis of one large Belgian and two small Dutch families with autosomal dominant non-syndromic progressive sensorineural hearing loss associated with vestibular dysfunction. Linkage studies in the Belgian family mapped the disease to the DFNA9 locus on chromosome 14. Mutation analysis of the COCH gene, which is responsible for DFNA9, revealed a missense mutation changing a highly conserved residue. One of the patients, who had an earlier age of onset in comparison with most of the affected family members, was shown to be homozygous for the mutation. After the mutation was found in the Belgian family, we discovered that the same missense mutation was also present in two Dutch families with similar cochleo-vestibular symptoms. In all three families with hearing loss and imbalance problems, >25% of the patients showed additional symptoms, including episodes of vertigo, tinnitus, aural fullness and hearing loss. Clinically, these symptoms are consistent with the criteria for Menière's disease. The importance of genetic factors in Menière's disease has been suggested on many occasions, but this study is the first report of a mutation in a gene leading to the symptoms of Menière's disease in a significant portion of the carriers. The COCH gene may be one of the genetic factors contributing to Menière's disease and the possibility of a COCH mutation should be considered in patients with Menière's disease symptoms.

Objective: To investigate whether the residual hearing of severely hearing-impaired children and adults could be preserved using the soft surgery approach. Patients and Methods: This project employed a prospective study design. All testing and surgery took place in the Institute of Physiology and Pathology of Hearing, Warsaw, Poland. Twenty-six patients (7 children and 19 post-lingually deafened adults) with residual hearing were assessed. Subjects were assessed using conventional pure-tone audiometry at least 1 month prior to surgery. Cochlear implant surgery with a Med-El Combi 40/40+ standard electrode array was conducted, using the soft surgery approach. Pure-tone audiometry thresholds were re-assessed at least 1 month after surgery. The researchers assessed change in auditory thresholds using pure-tone audiometry to determine preservation of residual hearing. Results: Sixteen of 26 patients (62%) retained their residual hearing within 5 dB HL of pre-operative scores. Only 5 of 26 patients (19%) lost all measurable residual hearing after cochlear implantation. This suggests that surgeons are often able to preserve residual hearing during cochlear implant surgery using the soft surgery technique. Conclusions: Preservation of residual hearing is an important consideration in cochlear implantation in the light of changing selection criteria for cochlear implant candidates, and as younger children are receiving implants. This is important, as we do not know yet the long-term effects of inner ear damage due to traumatic insertions of electrodes. This finding suggests a good prognosis for future possibilities of re-implantation.