Early cochlear implantation in prelingual profound hearing loss in Italy, analyzed by means of a social media survey.

Objective: To assess newborn hearing screening (NHS) impact on timing of cochlear implant (CI) surgery of patients with prelingual bilateral profound hearing impairment (BPHI), in order to evaluate whether the NHS ultimately serves the needs of the target population in Italy. Methods: An online questionnaire was created to survey subjects affected by prelingual BPHL born between 1990 and 2018. Questions focused on age at BPHI diagnosis, first and second CI surgery (if performed), and the region in which the surgery was performed. The survey was distributed to potential participants via social media communities used by hearing impaired people or their family members for sharing advice and offering support. Responses were analyzed using descriptive statistics. Results: Among the 318 respondents who completed the questionnaire, 276 (87%) reported having chosen CI surgery, 2/3 of them bilaterally. In the vast majority (97%) of cases the CI is used on a daily basis. Most of the people residing in the center (65%) and southern Italy (71%) had to move from their region of residence to perform the surgery. Late CI surgery was associated with failure to perform NHS (p = 0.007), birth before 2011 (p = 0.009), definitive diagnosis of BPHI after 6 months of life (p = 0.002), and progressive hearing impairment (p < 0.001). Conclusion: The worldwide scientific approval of the NHS as the current best opportunity for early diagnosis and CI treatment for prelingual BPHI is confirmed by what patients and families reported via the online questionnaire used for this study. In recent years, early bilateral cochlear implantation has become increasingly available in Italy, but late diagnosis, progressive hearing loss, failure to perform the NHS and lack of follow-up are still open questions. A large proportion of families had to move from the region of residence to have their child undergo CI surgery, revealing inequalities in terms of geographical disparities. Social media has proved to be a valuable, fast and inexpensive tool for gathering information on the effectiveness of health prevention programs, involving a large sample of individuals in a short amount of time.

The Role of Autosensitivity Control (ASC) in Cochlear Implant Recipients.

The purpose of the study was to examine the subjective and objective potential advantage for speech understanding in noise achieved by cochlear implant (CI) recipients when using the autosensitivity control (ASC) input signal processing in combination with the adaptive dynamic range optimization (ADRO). Eighteen subjects (8 females, 10 males, mean age 17.7 ± 6.7) were enrolled in a prospective open blinded comparative study between the ASC + ADRO condition vs. the ADRO alone; 16 were sequential binaural and 2 were monoaural CI recipients. All patients had been wearing their CI for at least 3 years, had no additional disabilities, had an age-appropriate receptive and expressive language. Word recognition performances in noise (at signal-to-noise ratio +5 dB HL) were significantly better in the ADRO-alone condition than in the ADRO + ASC condition. (p = 0.03) These objective outcomes were in agreement with the subjective reports. No significant difference was found in quiet. Our results, apparently in contrast with other reports in the literature, suggest that the decision of adding the slow-acting automatic reduction in microphone sensitivity provided by ASC should be limited to selected CI recipients.

Linguistic and pragmatic skills in toddlers with cochlear implant.

BACKGROUND: An increasing number of deaf children received cochlear implants (CI) in the first years of life, but no study has focused on linguistic and pragmatic skills in children with CI younger than 3 years of age. AIMS: To estimate the percentage of children who had received a CI before 2 years of age whose linguistic skills were within the normal range; to compare linguistic skills of children implanted by 12 months of age with children implanted between 13 and 26 months of age; and to describe the relationship among lexical, grammar and pragmatic skills. METHODS & PROCEDURES: The participants consisted of children who were included on the patient lists of the Service of Audio-Vestibology of the Circolo Hospital in Varese, Italy, and met the following criteria: chronological age between 18 and 36 months; CI activated between 8 and 30 months of age; absence of other reported deficits; hearing parents; and not less than 6 months of CI experience. Language development was evaluated through MacArthur-Bates CDI; pragmatic skills (assertiveness and responsiveness) were evaluated through the Social Conversational Skills Rating Scale. The scores obtained were transformed into z-scores and compared with normative data. The relationship among lexical, grammar and pragmatic skills were tested using Spearman Rho correlations. Children with CI were divided into groups based on the age at CI activation and the differences between the two groups were tested using the Student's t-test. OUTCOMES & RESULTS: Data from 23 deaf children were collected. Fewer than half of the children were within the normal range for lexical production and use of sentences; more than one-third of them fell below the normal range for both lexical and grammar skills. No significant difference was found in vocabulary size or early grammar skills when comparing children who received the CI by 12 months of age with those implanted during the second year of life. Despite the strong relationship among lexical, grammar and pragmatic skills, the delays found for grammar and pragmatic skills were greater than expected based on the vocabulary size. Age at diagnosis of hearing loss was the only predictor of vocabulary size. CONCLUSIONS & IMPLICATIONS: CI may provide deaf children with a good opportunity to develop language skills, but severe difficulties in early social experiences and interaction mediated by language still remain. Delays in these aspects suggest that interventions improving pragmatic skills are recommended even on very young children with CI.

European Bilateral Pediatric Cochlear Implant Forum consensus statement.

This is a consensus statement on pediatric cochlear implantation by the European Bilateral Pediatric Cochlear Implant Forum. The consensus statement was determined by review of current scientific literature to identify areas of scientific and clinical agreement of current understanding of bilateral cochlear implantation. The statement is "Currently we feel that the infant or child with unambiguous cochlear implant candidacy should receive bilateral cochlear implants simultaneously as soon as possible after definitive diagnosis of deafness to permit optimal auditory development; an atraumatic surgical technique designed to preserve cochlear function, minimize cochlear damage, and allow easy, possibly repeated re-implantation is recommended."

Cochlear implant in the second year of life: lexical and grammatical outcomes.

PURPOSE: The authors studied the effect of the cochlear implant (CI) on language comprehension and production in deaf children who had received a CI in the 2nd year of life. METHOD: The authors evaluated lexical and morphosyntactic skills in comprehension and production in 17 Italian children who are deaf (M = 54 months of age) with a CI and in 2 control groups of children with normal hearing (NH; 1 matched for chronological age and the other whose chronological age corresponded to the duration of CI activation). The authors also compared children with unilateral CI to children with bilateral CI. RESULTS: Children with CI appeared to keep pace with NH children matched for time since CI activation in terms of language acquisition, and they were similar to same-age NH children in lexical production. However, children with CI showed difficulties in lexical comprehension when a task required phonological discrimination as well as in grammar comprehension and production. Children with bilateral CI showed better comprehension than did children with unilateral CI; the 2 groups were similar for production. CONCLUSIONS: Activation of CI in the 2nd year of life may provide children who are deaf with a good opportunity to develop language skills, although some limitations in phonological and morphological skills are still present 3 years after auditory reafferentation.

An underestimated issue: unsuspected decrease of sound processor microphone sensitivity, technical, and clinical evaluation.

OBJECTIVE: To describe the rate of occurrence of unsuspected decrease in sensitivity of the sound processor microphone and to evaluate its effect on the patient's audiological performance in terms of reduction in speech recognition scores. DESIGN: Speech processor microphones were tested by connecting the speech processor acoustic monitor circuit to a hearing aid analyzer. The response curves were compared with those obtained from fully working microphones. During a 6-month investigation period, microphone response curves were measured from a group of cochlear implant recipients who had not reported any problems. Despite the absence of any subjective problem, some microphones were found to show a loss of sensitivity. Their users, aged between 4 and 67 years, were tested both with the defective and a working microphone in order to calculate the correlation between the degree of microphone failure and the decline in audiological performance. To quantify the effect of microphone failure, patients' speech recognition skills were measured by live voice connected discourse tracking series administered in different conditions and by recorded sentences lists. RESULTS: A total of 120 apparently fully functioning sound processors were tested in the investigation: 33 (27.5%) were affected by a subjectively unreported sensitivity decrease. Speech-tracking scores correlated significantly with the loss of microphone sensitivity in all test conditions (r = 0.69-0.77, P < 0.05). A high degree of correlation was also found for speech audiometry tests (r = 0.70-0.73, P < 0.05). Microphone sensitivity loss affected speech recognition skills, especially without lip reading and in the presence of background noise. CONCLUSION: The results indicate that any reduction in sound processor microphone sensitivity causes a degree of hearing decline that negatively affects the cochlear recipient's clinical performance. Microphone faults are often unreported events, and their occurrence rate is underestimated. To establish that the microphone is providing correct input to the speech processor a standard control procedure, including technical and clinical checks, is needed in clinical practice.

Clinical evaluation of cochlear implant sound coding taking into account conjectural masking functions, MP3000™.

Efficacy of the SPEAK and ACE coding strategies was compared with that of a new strategy, MP3000™, by 37 European implant centers including 221 subjects. The SPEAK and ACE strategies are based on selection of 8-10 spectral components with the highest levels, while MP3000 is based on the selection of only 4-6 components, with the highest levels relative to an estimate of the spread of masking. The pulse rate per component was fixed. No significant difference was found for the speech scores and for coding preference between the SPEAK/ACE and MP3000 strategies. Battery life was 24% longer for the MP3000 strategy. With MP3000 the best results were found for a selection of six components. In addition, the best results were found for a masking function with a low-frequency slope of 50 dB/Bark and a high-frequency slope of 37 dB/Bark (50/37) as compared to the other combinations examined of 40/30 and 20/15 dB/Bark. The best results found for the steepest slopes do not seem to agree with current estimates of the spread of masking in electrical stimulation. Future research might reveal if performance with respect to SPEAK/ACE can be enhanced by increasing the number of channels in MP3000 beyond 4-6 and it should shed more light on the optimum steepness of the slopes of the masking functions applied in MP3000.

Residual hearing conservation and electroacoustic stimulation with the nucleus 24 contour advance cochlear implant.

OBJECTIVE: To assess the conservation of residual hearing in recipients of the Nucleus 24 Contour Advance cochlear implant (CI) and the benefits of combined electrical and acoustic stimulation. STUDY DESIGN: Prospective multicenter study. SETTING: CI clinics in Western Europe. PATIENTS: Adult candidates for conventional cochlear implantation with a minimum preoperative word recognition score of 10% in the ear to be implanted. INTERVENTION: "Soft-surgery" protocol, including a 1- to 1.2-mm anterior and inferior cochleostomy hole with the electrode array, inserted 17 mm using the "advance-off-stylet" technique. Patients with postoperative pure-tone hearing threshold levels (HTLs) of 80 dB hearing loss or less at 125 and 250 Hz and 90 dB hearing loss or less at 500 Hz were refitted with an in-the-ear hearing aid for combined ipsilateral electrical and acoustic (El-Ac) stimulation. MAIN OUTCOME MEASURES: A questionnaire to collect information regarding surgery. Pure-tone HTLs measured at intervals. Word recognition tested in quiet and sentence recognition tested in noise at 10 and 5 dB signal-to-noise ratio (SNR). HEARING CONSERVATION RESULTS: HTL data were available for 27 patients. HTLs were conserved within 20 dB of preoperative levels for 33, 26, and 19% of patients for 125, 250, and 500 Hz, respectively. However, the recommended soft-surgery protocol was strictly followed in only 12 of 27 patients. For these 12 patients, hearing thresholds were conserved within 20 dB for 50, 50, and 33% of patients. Median threshold increases were 40 dB (range, 250-500 Hz) for the whole group and 23 dB for the strict surgery group. Ten patients retained sufficient HTLs to enter the El-Ac user group. SPEECH RECOGNITION RESULTS: Group mean recognition scores for nine El-Ac users for words presented at 65 dB sound pressure level were 45% for CI alone and 55% for CI + ipsilateral hearing aid (p < 0.05, paired t). For sentences presented in noise at 5 dB SNR, mean word scores were 46% CI alone and 56% CI + ipsilateral hearing aid (p < 0.01, paired t). CONCLUSION: Hearing was conserved for conventional candidates for cochlear implantation where the recommended soft-surgery protocol was strictly adhered. Combined ipsilateral electrical and acoustic stimulation provided considerable benefits for speech recognition in noise, equivalent to between 3 and 5 dB SNR, compared with CI alone.

Preservation of residual hearing with cochlear implantation: how and why.

CONCLUSIONS: Hearing may be conserved in adults after implantation with the Nucleus Contour Advance perimodiolar electrode array. The degree of hearing preservation and the maximum insertion depth of the electrode array can vary considerably despite a defined surgical protocol. Residual hearing combined with electrical stimulation in the same ear can provide additional benefits even for conventional candidates for cochlear implantation. OBJECTIVES: We present preliminary results from a prospective multicentre study investigating the conservation of residual hearing after implantation with a standard-length Nucleus Contour Advance perimodiolar electrode array and the benefits of combined electrical and acoustic stimulation. MATERIAL AND METHODS: The subjects were 12 adult candidates for cochlear implantation recruited according to national selection criteria. A "soft" surgery protocol was defined, as follows: 1-1.2-mm cochleostomy hole anterior and inferior to the round window; Nucleus Contour Advance electrode array inserted using the "Advance-off-stylet" technique; and insertion depth controlled by means of three square marker ribs left outside the cochleostomy hole. These procedures had been shown to reduce insertion forces in temporal bone preparations. Variations in surgical techniques were monitored using a questionnaire. Pure-tone thresholds were measured pre- and postoperatively. Patients who still retained thresholds <90 dB HL for frequencies up to 500 Hz were re-fitted with an in-the-ear (ITE) hearing aid. Word recognition was tested in quiet and sentence perception in noise for the cochlear implant alone and in combination with an ipsilateral hearing aid. RESULTS: Hearing threshold level data were available for 12 patients recruited from 6 of the centres. Median increases in hearing threshold levels were 23, 27 and 33 dB for the frequencies 125, 250 and 500 Hz, respectively. These median increases include the data for two patients who had total loss of residual hearing due to difficulties encountered during surgery. "Cochlear view" X-ray images indicated that the depth of insertion varied between 300 and 430 degrees, despite modest variations in the length of the electrode inserted (17-19 mm). The insertion angle had some influence on the preservation of residual hearing at frequencies of 250-500 Hz. Six of the 12 patients retained sufficient hearing for effective use of an ipsilateral ITE hearing aid (< or = 80 dB HL at 125 and 250 Hz; < or = 90 dB HL at 500 Hz). Word recognition scores in quiet were improved from 10% to 30% with the cochlear implant plus ipsilateral hearing aid in 3 patients who had at least 3 months postoperative experience. Signal:noise ratio thresholds for sentence recognition were improved by up to 3 dB. Patients reported that they experienced greatly improved sound quality and preferred to use the two devices together.

Far advanced otosclerosis: stapes surgery or cochlear implantation?

OBJECTIVE: Nowadays, two surgical alternatives are to be considered in patients with far advanced otosclerosis: stapedectomy and cochlear implantation. The aim of this study was to analyze and compare the hearing and communicative results obtained in two groups of patients affected with far advanced otosclerosis, who were treated with the two different surgical procedures, to assess the proper surgical approach for these patients. DESIGN: Retrospective study. SETTINGS: University hospital and referral audiologic centre. METHODS: We submitted 11 adult patients, affected by far advanced otosclerosis and exhibiting unsatisfactory communicative abilities with hearing aids, to two different procedures: 5 to cochlear implantation, and 6 to stapedotomy followed by hearing aid fitting. The benefits afforded by the two procedures were assessed by means of pure-tone audiometry and speech audiometry in an open field and by means of a speech perception test. MAIN OUTCOME MEASURES: Hearing and communicative outcomes, measured 1 year after intervention. RESULTS: The results achieved in the patients subjected to stapedotomy were unpredictable and variable: very good in some cases but unsatisfactory in others, whereas those who had cochlear implantation all achieved satisfactory results with regard to speech recognition. CONCLUSIONS: Patients who underwent cochlear implantation achieved better hearing and communicative results than those who underwent stapedotomy. Even if the number of patients reported in this study is too small to provide definitive results, and despite the reportedly better results afforded by cochlear implantation, in our opinion, correction of far advanced otosclerosis should first be attempted via stapedotomy, and cochlear implantation should instead be reserved for cases in which stapedotomy is unsuccessful or for which imaging techniques unequivocally reveal a fenestral or cochlear obstruction.