Binaural hearing has been shown to support better speech perception in normal-hearing listeners than can be achieved with monaural stimulus presentation, particularly under noisy listening conditions. The purpose of this study was to evaluate whether bilateral electrical stimulation could confer similar benefits for cochlear implant listeners. A total of 26 postlingually deafened adult patients with short duration of deafness were implanted at five centers and followed up for 1 yr. Subjects received MED-EL COMBI 40+ devices bilaterally; in all but one case, implantation was performed in a single-stage surgery. Speech perception testing included CNC words in quiet and CUNY sentences in noise. Target speech was presented at the midline (0 degrees), and masking noise, when present, was presented at one of three simulated source locations along the azimuth (-90, 0, and +90 degrees). Benefits of bilateral electrical stimulation were observed under conditions in which the speech and masker were spatially coincident and conditions in which they were spatially separated. Both the "head shadow" and "summation" effects were evident from the outset. Benefits consistent with "binaural squelch" were not reliably observed until 1 yr after implantation. 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.

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 COCHgene, 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.

This study evaluated the use of landline and mobile phones in an international sample of cochlear implant users. A custom-designed survey was mailed to cochlear implant users from four different countries. A link to the survey was posted on the MED-EL website, with responses from a further six countries. Results from 196 surveys show that there is a significant shift from pre-operative non-use of a telephone to use of a telephone post-operatively. Seventy-one percent of MED-EL cochlear implant users are able to use a landline telephone to some extent and 54% are able to use a mobile phone to some extent. Talking to familiar speakers about familiar topics is the easiest listening condition on the telephone, and it is easier to recognize a voice using the landline. Many respondents found it difficult to make a call without some assistance. Most respondents could manage to call someone in an emergency, even on a mobile phone. Data obtained should provide useful information in the counselling and rehabilitation of cochlear implant recipients and candidates.

To report the clinical, auditory, and vestibular characteristics of a nonsyndromic otovestibular dysfunction in a large Belgian family caused by a missense mutation of the DFNA9 gene: COCH. Retrospective study of the clinical, audiologic, and vestibular data of 60 genetically affected cases. Tertiary referral center. All members of a Belgian kindred who carry the genetic (P51S) defect linked to the inherited hearing and vestibular impairment. Diagnostic otologic, audiometric, and vestibular analysis and imaging. Pure tone audiometry, supraliminary audiometry. and vestibular investigation. The autosomal dominant inherited impairment was characterized by peripheral degeneration of the inner ear, leading to total deafness and bilateral vestibular areflexia. The genetically affected persons of a Belgian family shared a progressive sensorineural hearing loss starting between the third and sixth decade. Vestibular symptoms started at about the same age as the hearing loss. The vestibular symptoms consisted of instability in darkness, a tendency to fall sideways, light-headiness, a drunken feeling, and attacks of vertigo. Most of the patients reported tinnitus, and half of them reported pressure in the ears. Clinically, 9 of the 60 patients met the criteria for definite Ménière's disease, and another 13 and 17 patients met the criteria for probable or possible Ménière's disease, respectively. All 9 were older than the age of 35, but only 1 was older than 55 years, so more than 30% of the patients were between 35 and 55 years old. A specific pattern could be recognized in the evolution of the otovestibular impairment. Under the age of 35 years, almost all the affected family members had normal hearing, whereas above the age of 55 years, the hearing loss was at least moderate, and vestibular hypofunction occurred. In between, there was a transition period of two to three decades, when deterioration of the cochleovestibular function occurred, with a temporary audiometric and vestibular asymmetry.

The objectives of this study were to assess the factors which contribute to individuals’ health motivation to address hearing loss and to gather baseline data that could then be used to measure the impact of an awareness campaign. An online questionnaire with 13 closed set questions was completed by 100 subjects in each country including Austria, Germany, France, Sweden, and the United Kingdom. The questionnaire was based around the Health Belief Model, which describes how, in order to take action to address a medical problem, the individual must perceive that the condition presents a threat to their well-being that exceeds any barriers to treatment. Good hearing was regarded as being important in all countries. There was agreement that the main sign of hearing loss was turning up the TV or radio. In most countries, hearing aids were thought to be not particularly visible, not require much maintenance, a hindrance while doing sport, and must be removed before bed. Perceptions of hearing implants were that they were permanently fitted, not externally visible, and do not need to be removed before bed. Medical issues were mostly researched through a doctor and then via the Internet. In this sample, there was a good understanding of the consequences and signs of hearing loss, but although hearing implants were viewed as being different to hearing aids, there was little understanding that the external speech processor was similar to a hearing aid in its physical characteristics. When actions were taken, the key professionals consulted about hearing problems were the general practitioner and ear, nose, and throat specialist.