Yukihiko Kanda

Sudden hearing loss associated with interferon

With the increasing long-term use of interferon, several new adverse effects have been recognised. We have prospectively assessed auditory function in 49 patients receiving interferon, after we saw a case of sudden sensorineural hearing loss during interferon therapy. Auditory disability (tinnitus, hearing loss, or both) occurred in 22 patients (45%) during treatment with audiometry-documented sensorineural hearing loss in 18 (37%). The auditory disability often developed in the late stage of treatment and resolved in all patients within 7-14 days after discontinuation of interferon.

Music recognition, music listening, and word recognition by deaf children with cochlear implants.

Objectives: To examine the ability of congenitally deaf children to recognize music from incidental exposure and the relations among age at implantation, music listening, and word recognition. Design: Seventeen child implant users who were 4 to 8 yr of age were tested on their recognition and liking of musical excerpts from their favorite television programs. They were also assessed on open-set recognition of three-syllable words. Their parents completed a questionnaire about the children’s musical activities. Results: Children identified the musical excerpts at better than chance levels, but only when they heard the original vocal/instrumental versions. Children’s initiation of music listening at home was associated with younger ages at implantation and higher word recognition scores. Conclusions: Child implant users enjoy music more than adult implant users. Moreover, younger age at implantation increases children’s engagement with music, which may enhance their progress in other auditory domains.

Mutations in the WFS1 gene are a frequent cause of autosomal dominant nonsyndromic low-frequency hearing loss in Japanese

AbstractMutations in WFS1 are reported to be responsible for two conditions with distinct phenotypes; DFNA6/14/38 and autosomal recessive Wolfram syndrome. They differ in their associated symptoms and inheritance mode, and although their most common clinical symptom is hearing loss, it is of different types. While DNFA6/14/38 is characterized by low frequency sensorineural hearing loss (LFSNHL), in contrast, Wolfram syndrome is associated with various hearing severities ranging from normal to profound hearing loss that is dissimilar to LFSNHL (Pennings et al. 2002). To confirm whether within non-syndromic hearing loss patients WFS1 mutations are found restrictively in patients with LFSNHL and to summarize the mutation spectrum of WFS1 found in Japanese, we screened 206 Japanese autosomal dominant and 64 autosomal recessive (sporadic) non-syndromic hearing loss probands with various severities of hearing loss. We found three independent autosomal dominant families associated with two different WFS1 mutations, A716T and E864K, previously detected in families with European ancestry. Identification of the same mutations in independent families with different racial backgrounds suggests that both sites are likely to be mutational hot spots. All three families with WFS1 mutations in this study showed a similar phenotype, LFSNHL, as in previous reports. In this study, one-third (three out of nine) autosomal dominant LFSNHL families had mutations in the WFS1 gene, indicating that in non-syndromic hearing loss WFS1 is restrictively and commonly found within autosomal dominant LFSNHL families.

Clinical characteristics and genotype–phenotype correlation of hearing loss patients with SLC26A4 mutations

Conclusions. The present study confirmed the clinical characteristics of patients with SLC26A4 mutations: congenital, fluctuating, and progressive hearing loss usually associated with vertigo and/or goiter during long-term follow-up. This clarification should help to facilitate appropriate genetic counseling and proper medical management for patients with these mutations, but there was no particular genotype–phenotype correlation among them, suggesting that other factors may contribute to such variability. Objectives. Due to the wide range of phenotypes caused by SLC26A4 mutations, there is controversy with regard to genotype–phenotype correlation. The present study was performed: (1) to determine phenotypic range in patients with biallelic SLC26A4 mutations, and (2) to evaluate whether possible genotype–phenotype correlation exists. Subjects and methods. Phenotypes in 39 hearing loss patients with SLC26A4 mutations were summarized and genotype–phenotype correlation was analyzed. Results. Hearing level varied in the individuals from mild to profound severity. Most of the patients had fluctuating and progressive hearing loss that may have been of prelingual onset. Twenty-four (70.6%) patients had episodes of vertigo, and 10 (27.8%) patients had goiter, which had appeared at age 12 or older. In contrast to such phenotypic variabilities, no apparent correlation was found between these phenotypes and their genotypes.

Effect of cochlear implants on children’s perception and production of speech prosody

Japanese 5- to 13-yr-olds who used cochlear implants (CIs) and a comparison group of normally hearing (NH) Japanese children were tested on their perception and production of speech prosody. For the perception task, they were required to judge whether semantically neutral utterances that were normalized for amplitude were spoken in a happy, sad, or angry manner. The performance of NH children was error-free. By contrast, child CI users performed well below ceiling but above chance levels on happy- and sad-sounding utterances but not on angry-sounding utterances. For the production task, children were required to imitate stereotyped Japanese utterances expressing disappointment and surprise as well as culturally typically representations of crow and cat sounds. NH 5- and 6-year-olds produced significantly poorer imitations than older hearing children, but age was unrelated to the imitation quality of child CI users. Overall, child CI users imitations were significantly poorer than those of NH children, but they did not differ significantly from the imitations of the youngest NH group. Moreover, there was a robust correlation between the performance of child CI users on the perception and production tasks; this implies that difficulties with prosodic perception underlie their difficulties with prosodic imitation.

Cochlear Implantation in Children With Congenital Cytomegalovirus Infection

Objective: To assess the impact of cochlear implantation (CI) on children with cytomegalovirus (CMV)-induced deafness. Study Design: Retrospective chart review. Patients: Four children with congenital CMV-related deafness (CMV group) and 17 children with congenital deafness without CMV infection as the cause of deafness (non-CMV group). The age at CI ranged from 2.0 to 3.3 years (mean, 2.6 yr) in the CMV group and from 1.8 to 3.6 years (mean, 2.6 yr) in the non-CMV group. Their follow-up period ranged from 3.0 to 4.3 years (mean, 3.3 yr) in the CMV group and from 1.6 to 4.3 years (mean, 3.3 yr) in the non-CMV group. Methods: Evaluation and comparison of preoperative and postoperative hearing levels, motor, social, and language development, Infant-Toddler Meaningful Auditory Integration Scale, and Enjoji Scale of Infant Analytical Development between the 2 groups. Results: Within 12 months after CI, the mean score of both language perception and production was poorer in the CMV group than in the non-CMV group, and the difference in the language production was statistically significant. However, 12 months after CI, the language perception and production showed good progress at levels similar to that of the non-CMV group. Conclusion: Long-term results of the language perception and production after CI was satisfactory in Japanese children with congenital CMV-related deafness compared with in subjects deafened by other causes. CMV alone, as a cause of deafness, is not likely a contraindication for CI.

Interferon-lnduced Sudden Hearing Loss: Original Paper

With the increasing long-term use of interferon (IFN), several new adverse effects have been recognized. Very little attention, however, has been paid to auditory acuity. We encountered 3 cases of sudden hearing loss associated with IFN. We then co nducted a prospective study to assess the auditory function of 73 patients receiving IFN. Auditory disability (tinnitus and/or hearing loss) occurred in 32 patients (43.8%) during IFN therapy, among which audiometry documented sensorineural hearing loss in 27 cases (36.9%); 17 (48.6%) of the 35 patients receiving IFN-β had auditory disability, including hearing loss in 13 cases (37.1%), and 15 (39.5%) of 38 patients receiving IFN-α suffered from auditory disability. There was not much difference between the influences of IFN-α and -β. Auditory disability frequently developed in the later stages of treatment, and most patients recovered 7-14 days after the discontinuation of IFN. The results demonstrate that sudden hearing loss can occur as a side effect of trea...With the increasing long-term use of interferon (IFN), several new adverse effects have been recognized. Very little attention, however, has been paid to auditory acuity. We encountered 3 cases of sudden hearing loss associated with IFN. We then conducted a prospective study to assess the auditory function of 73 patients receiving IFN. Auditory disability (tinnitus and/or hearing loss) occurred in 32 patients (43.8%) during IFN therapy, among which audiometry documented sensorineural hearing loss in 27 cases (36.9%); 17 (48.6%) of the 35 patients receiving IFN-beta had auditory disability, including hearing loss in 13 cases (37.1%), and 15 (39.5%) of 38 patients receiving IFN-alpha suffered from auditory disability. There was not much difference between the influences of IFN-alpha and -beta. Auditory disability frequently developed in the later stages of treatment, and most patients recovered 7-14 days after the discontinuation of IFN. The results demonstrate that sudden hearing loss can occur as a side effect of treatment with IFN. This may reveal the association between autoimmunity and sudden hearing loss.

Cochlear implantation on prelingually deafened adults.

OBJECTIVE To evaluate the validity of cochlear implantation (CI) on prelingually deafened adults who have been trained by auditory-verbal/oral communication since childhood. METHODS Preoperative and postoperative data was investigated regarding the rehabilitation, hearing level, and educational experience of eight prelingually deafened adults. All eight patients were diagnosed with severe to profound sensorineural hearing loss (preoperative hearing levels were over 100 dB). All used hearing aids (HA) before the age of two and were trained by auditory-verbal/oral communication since childhood. The average age of the patients at the time of their CI operations was 23.3 ranging from 18 to 29 years of age. The average postoperative observation time was 55.4 months ranging from 11 to 90 months. RESULTS Improvement was achieved not only on the pure-tone hearing threshold, but also in speech perception on tests using the Japanese video speech discrimination score (SDS) system. All of them now use CI very well in their daily lives and play important roles in society. CONCLUSION It was demonstrated that even prelingually deafened adult patients could achieve considerable improvement through CI when they were trained well by auditory-verbal/oral communications since childhood. The indications of CI for prelingually deafened adults must be determined carefully, but all of them do not have to be rejected only because they are prelingually deafened. In other words, CI could be recommended for prelingually deafened adult patients if they received habilitation well with consistent auditory-verbal/oral training using well-fitted HAs.

Hearing preservation and clinical outcome of 32 consecutive electric acoustic stimulation (EAS) surgeries

Abstract Conclusions: Our results indicated that electric acoustic stimulation (EAS) is beneficial for Japanese-speaking patients, including those with less residual hearing at lower frequencies. Comparable outcomes for the patients with less residual hearing indicated that current audiological criteria for EAS could be expanded. Successful hearing preservation results, together with the progressive nature of loss of residual hearing in these patients, mean that minimally invasive full insertion of medium/long electrodes in cochlear implantation (CI) surgery is a desirable solution. The minimally invasive concepts that have been obtained through EAS surgery are, in fact, crucial for all CI patients. Objectives: This study was conducted to evaluate hearing preservation results and speech discrimination outcomes of hearing preservation surgeries using medium/long electrodes. Methods: A total of 32 consecutive minimally invasive hearing preservation CIs (using a round window approach with deep insertion of a flexible electrode) were performed in 30 Japanese patients (two were bilateral cases), including patients with less residual hearing. Hearing preservation rates as well as speech discrimination/perception scores were investigated on a multicenter basis. Results: Postoperative evaluation after full insertion of the flexible electrodes (24 mm, 31.5 mm) showed that residual hearing was well preserved in all 32 ears. In all patients, speech discrimination and perception scores were improved postoperatively.

Superficial Siderosis of the Central Nervous System

This is a case of superficial siderosis of the central nervous system (SSCN). The diagnosis of SSCN was based on the result of T2-weighted magnetic resonance imaging and on suggestive clinical manifestations. The pure-tone audiogram showed bilateral progressive sensorineural hearing loss with a poor speech discrimination score and Jerger type IV. The remarkable elevation of the detective threshold of cochlear microphonics on electrocochleography was found and distortion product otoacoustic emission (DPOAE) showed no response: These electrophysiologic examinations, including electrocochleography and DPOAE, revealed that the progressive sensorineural hearing loss in this case was caused by both retrocochlear and cochlear damages.