Auditory Skills following Cochlear Implantation in Children with the Charge Syndrome.

OBJECTIVES: To assess the auditory outcomes and skills of pediatric cochlear implant (CI) users with the CHARGE syndrome. To determine the influence of inner ear malformations on the surgical procedure and speech understanding outcomes in this population. STUDY DESIGN: Observational, retrospective study. MATERIALS AND METHODS: Imaging, auditory testing, intraoperative findings, complications, and postoperative auditory skills and outcomes of pediatric CI users with CHARGE syndrome were recorded. RESULTS: 6 children (8 ears) were included, 5 of whom had prelingual deafness. Their mean age at implantation was 37 months. Six of the 8 ears presented cochlear malformation; the most frequent was hypoplasia type III. Intraoperatively, the transmastoid facial recess approach was used in 5 ears, and abnormalities of facial nerve anatomy were found in 5 ears. All electrode insertions were complete. All children were, to a varying degree, able to detect and identify sound. Verbalization skills were developed by 2 children, 1 of whom used oral language as his primary mode of communication. CONCLUSIONS: Cochlear implantation performed by an experienced surgeon in patients with the CHARGE syndrome is a safe procedure with adequate treatment planning. All children had improved auditory skills although the improvement was variable.

Using the HISQUI29 to assess the sound quality levels of Spanish adults with unilateral cochlear implants and no contralateral hearing.

To evaluate cochlear implant (CI) users' self-reported level of sound quality and quality of life (QoL). Sound quality was self-evaluated using the hearing implant sound quality index (HISQUI29). HISQUI29 scores were further examined in three subsets. QoL was self-evaluated using the glasgow benefit inventory (GBI). GBI scores were further examined in three subsets. Possible correlations between the HISQUI29 and GBI were explored. Additional possible correlations between these scores and subjects' pure tone averages, speech perception scores, age at implantation, duration of hearing loss, duration of CI use, gender, and implant type were explored. Subjects derived a "moderate" sound quality level from their CI. Television, radio, and telephone tasks were easier in quiet than in background noise. 89 % of subjects reported their QoL benefited from having a CI. Mean total HISQUI29 score significantly correlated with all subcategories of the GBI. Age at implantation inversely correlated with the total HISQUI29 score and with television and radio understanding. Sentence in noise scores significantly correlated with all sound perception scores. Women had a better mean score in music perception and in telephone use than did men. CI users' self-reported levels of sound quality significantly correlated with their QoL. Cochlear implantation had a beneficial impact on subjects' QoL. Understanding speech is easier in quiet than in noise. Music perception remains a challenge for many CI users. The HISQUI29 and the GBI can provide useful information about the everyday effects of future treatment modalities, rehabilitation strategies, and technical developments.

Validation of the Hearing Implant Sound Quality Index (HISQUI19) to assess Spanish-speaking cochlear implant users' auditory abilities in everyday communication situations.

CONCLUSION: The Spanish-language HISQUI19 is a reliable and easy-to-use tool for quantifying the self-perceived level of auditory benefit that cochlear implant (CI) users experience in everyday listening situations. OBJECTIVES: To validate the Spanish-language version of The Hearing Implant Sound Quality Index (HISQUI19), a questionnaire for quantifying the self-perceived level of auditory benefit that CI users experience in everyday listening situations. METHODS: Adult Spanish-speaking subjects with a CI were asked to complete a Spanish-language version of the HISQUI19. Reliability of the questionnaire was determined using Cronbach's α coefficient; exploratory factor analyses with a rotated (varimax) factor solution was applied to check construct validity; external validity was confirmed using Pearson's correlation. Test-re-test analysis was also performed. The MED-EL Assessment Database was used to assist in the administration, reporting, and data collection. RESULTS: Sixty-six of 77 subjects completed and returned the HISQUI19. The mean score auditory ability score (mean ± standard deviation) of the HISQUI19 was 79.9 ± 24.0, a 'moderate' auditory benefit. An inverse relationship was found between the HISQUI19 score and age at implantation. Gender and side of implantation did not influence self-perceived functioning. The objective measures (disyllables tested without lip-reading and in quiet) were correlated with the Total Score.

Ipsilateral cochlear implantation in patients with sporadic vestibular schwannoma in the only or best hearing ear and in patients with NF2.

The aim of this study was to evaluate the cochlear implant (CI) performances in neurofibromatosis type 2 (NF2) patients with bilateral vestibular schwannoma (VS) and in patients with sporadic VS in the only or better hearing ear. All patients with bilateral VS or sporadic VS in the only or better hearing ear who underwent cochlear implantation, either simultaneous to VS surgery or staged after treatment for VS, in the tumor side were chosen for the study. Postimplantation audiometric scores (sound detection, closed-set and open-set discrimination scores) and device use patterns were the main outcome measures. 15 patients were implanted. Eight patients (53 %) were NF2 and seven patients had VS in the only or better hearing ear. One patient was explanted for cerebrospinal fluid leak. In the CI-only condition, the other 14 patients obtained sound detection, 64 % of them achieving open-set discrimination (mean 70 ± 38 %) and 85 % achieving closed-set discrimination (mean 41 ± 33 %). At the last follow-up 10 patients (67 %) were using the CI. Cochlear implantation provides hearing in particular cases of patients with bilateral VS or VS in the only or better hearing ear. As long as anatomic preservation of the cochlear nerve is achieved, cochlear implantation may offer improvement in communication skills for most patients.

Which ear should we choose for cochlear implantation in the elderly: The poorer or the better? Audiometric outcomes, quality of sound, and quality-of-life results.

CONCLUSIONS: Cochlear implantation in the poorer ear of an elderly patient does not predict poorer post-operative audiological, quality-of-life (QoL), and quality of sound results. OBJECTIVES: To determine whether choosing the 'better' or the 'poorer' ear for cochlear implantation impacts performance outcome in an elderly population. METHODS: Forty-two out of 73 elderly (≥ 60 years) cochlear implant users with some residual hearing in at least one ear were selected. Three criteria were used to group the patients as 'better' or 'poorer' ear implanted; (C1) based on previous use of hearing aid, (C2) pre-operative PTA, and (C3) pre-operative speech discrimination. The Glasgow Benefit Inventory (GBI) and the Hearing Implant Sound Quality Index (HISQUI) were used to measure QoL and quality of sound, respectively. RESULTS: The mean post-operative PTA was 38.7 ± 7.2 dBHL. In quiet, the mean disyllabic SDS at 65dBSPL was 75.4 ± 18.5, whereas the discrimination of sentences was 95.0% ± 6.9. The mean total GBI score was 30.9 ± 21.8, 92.9% of patients reporting a positive score. The mean HISQUI score was 111.3 ± 36.0, which corresponds to 'moderate' sound quality. No significant differences were found between both groups in terms of audiological outcomes, HISQUI scores or GBI scores when considering each of the three criteria.

Fibrous dysplasia of the temporal bone secondary to ear surgery: a case report.

INTRODUCTION: In this report, we describe the clinical course, diagnostic features and management of a patient with fibrous dysplasia of the temporal bone 7 years after middle ear surgery on the same side. CASE PRESENTATION: A 16-year-old Caucasian girl presented to our hospital with a growing bone lesion in the roof of the left temporal bone. She had undergone a previous tympanoplasty at 7 years of age because of a cholesteatoma. At the time of that first surgery, no radiological or histological signs indicated a bone disorder. A computed tomographic scan of the temporal bone showed a lesion with rarefaction areas and lytic images inside that affected the roof of the cavity to the tegmen tympani without alterations in the inner ear. A surgical revision of the ear cavity was performed by resecting the lesion and regularizing the cavity. The histopathologic study confirmed fibrous dysplasia. The patient progressed satisfactorily after surgery with no evidence of recurrence. CONCLUSION: To the best of our knowledge, this is the first report of fibrous dysplasia of the temporal bone secondary to ear surgery.

Pros and Cons of Round Window Vibroplasty in Open Cavities: Audiological, Surgical, and Quality of Life Outcomes.

OBJECTIVE: To evaluate the audiological, surgical, quality of life, and quality of sound outcomes in adults with open cavities implanted with the Vibrant Soundbridge (VSB) implant using round window (RW) vibroplasty approach. STUDY DESIGN: Retrospective study. SETTING: Otolaryngology department, tertiary referral hospital. SUBJECTS AND METHODS: Twelve adult patients with conductive or mixed hearing loss, all with previous middle ear surgery, underwent RW vibroplasty in an open cavity. Compound action potential thresholds were assessed during surgery. Surgical complications were recorded. Subjective benefit was evaluated using the Nijmegen Cochlear Implant Questionnaire (NCIQ), Glasgow Benefit Inventory (GBI), and Hearing Implant Sound Quality Index (HISQUI29) tests. RESULTS: Mean follow-up was 42 months (range 12-76). There was no significant change in bone conduction thresholds after surgery. Mean functional gain was 34.3 dB and speech discrimination score at 65 dB significantly improved from 14 to 83%. Extrusion of the wire link was the main surgical complication in four patients. All NCIQ domains improved after surgery. All patients had a positive overall GBI score (mean 35.0). Mean HISQUI29 score was 152.8, on average the quality of sound being defined as "very good." CONCLUSION: VSB is an effective method of hearing restoration for adults with open cavities suffering from conductive or mixed hearing loss. Intraoperative electrocochleography may be considered of significant help to check the coupling to the inner ear. The high rate of extrusion suggests that middle ear obliteration may be considered in these patients.

Reliability and validity of the Spanish Glasgow Benefit Inventory after cochlear implant surgery in adults.

In recent years, the outcome assessment of subjects undergoing otorhinolaryngological (ORL) intervention has relied increasingly upon patient-reported questionnaires. The English Glasgow Benefit Inventory (GBI) is a reliable, sensitive and validated post-intervention questionnaire that is used to determine health-related quality-of-life (QoL) in patients following ORL intervention. The GBI has been translated into eight languages including Spanish. The present study aimed to determine the internal consistency and validity of the Spanish version of the GBI in patients with a cochlear implant (CI). 121 adult Spanish speaking patients with a CI were questioned retrospectively using the Spanish GBI. Reliability of the questionnaire was determined using Cronbach's α coefficient; factor analyses were performed using principal component extraction with orthogonal rotation; and validity was confirmed using Pearson's correlation coefficient. The mean total score (mean ± standard deviation) of the GBI was 35.1 ± 23.6, 47.6 ± 28.9 for General Health, 17.9 ± 33.2 for Social Benefit and 1.7 ± 27.9 for Physical Health. The reliability was above 0.70 in all test domains. Using factor analyses a 4-factor solution that explained 63.2% of the variance was reached. The objective measures: bisyllables tested without lip-reading and without masking were correlated with the total score and the General Health subscales. The Spanish GBI is a reliable and practicable instrument for the assessment of health-related QoL in Spanish adult CI users.

Reliability and validity of the Nijmegen Cochlear Implant Questionnaire in Spanish.

The Nijmegen Cochlear Implant Questionnaire (NCIQ) is a specific and quantifiable self-assessment health-related quality of life (QoL) questionnaire. It was developed to determine the subjective benefits of a cochlear implant (CI). The present study aimed to determine the validity and reliability of the NCIQ in Spanish. Seventy-six adult, Spanish speaking, CI users and 28 control subjects answered the NCIQ. Reliability of the questionnaire was determined using Cronbach's α coefficient and the validity established using Pearson's correlation coefficient. All questionnaire respondents performed significantly better in all sub-domains with a cochlear implant than before cochlear implantation. The Cronbach's α score exceeded 0.70 in most sub-domains and the total score. The objective measures: bisyllables tested without lip-reading and with masking; and bisyllables, tested without lip-reading and without masking were correlated with the sub-domain 'advanced sound perception'. To determine health-related QoL the NCIQ in Spanish is a reliable self-assessment questionnaire and a valuable instrument in determining the subjective benefit of a CI.

Nueva entidad en el diagnóstico diferencial de los tumores del ganglio geniculado: lesión del tejido conectivo fibroso del nervio facial / A new entity in the differential diagnosis of geniculate ganglion tumours: fibrous connective tissue lesion of the facial nerve

Dentro del diagnóstico diferencial de las lesiones del ganglio geniculado nos encontramos principalmente con los schwannomas, hemangiomas y meningiomas. Se presenta el caso de un paciente cuya clínica y hallazgos radiológicos imitaban la presentación de un schwannoma del nervio facial. Los estudios anatomopatológicos revelaron una lesión con fascículos nerviosos desestructurados por intensa colagenización, por lo que se denominó lesión fibrosa del tejido conectivo fibroso del nervio facial (AU)

Differential diagnosis of geniculate ganglion tumours includes chiefly schwannomas, haemangiomas and meningiomas. We report the case of a patient whose clinical and imaging findings mimicked the presentation of a facial nerve schwannoma. Pathological studies revealed a lesion with nerve bundles unstructured by intense collagenisation. Consequently, it was called fibrous connective tissue lesion of the facial nerve (AU)

Is there an age limit for cochlear implantation?

OBJECTIVES: We evaluated the quality of life following cochlear implantation in elderly postlingually deaf adults. METHODS: Data were studied concerning demographics and audiometric evaluation in postlingually deaf adults at least 60 years of age who underwent cochlear implantation in 3 institutions. The Glasgow Benefit Inventory was used to quantify the quality of life. The patients were divided into 2 groups (those less than 70 years of age and those at least 70 years of age), and the results were also compared to those of younger adult cochlear implant recipients (less than 60 years of age). RESULTS: Eighty-one patients were included in this study. The mean age at implantation was 68 years (range, 60 to 82 years). Cochlear implantation significantly improved the patients' audiometric outcomes (pure tone average and speech perception; p < 0.05). The Glasgow Benefit Inventory showed a benefit overall (+36) and on the individual subscales (+49, +20, and +1). The difference in quality of life was not significant between those less than 70 and those at least 70 years of age (p = 0.90). The results were similar to those of younger postlingually deaf implant recipients. CONCLUSIONS: Elderly cochlear implant users experience an improvement in their quality of life, with outcomes similar to those achieved in younger adults. Particular attention must be paid to the possibility of age-related conditions in the elderly that may increase the risks of surgery.

Calidad de vida de pacientes implantados con el dispositivo BAHA según su indicación / Quality of life in patients implanted with the BAHA device depending on the aetiology

Introducción y objetivos: Cuantificar la mejoría en la calidad de vida en los pacientes osteoimplantados, teniendo en consideración tanto la indicación del implante como su uso y la presencia de acúfenos. Métodos: Este estudio incluye a 69 pacientes implantados entre junio de 2004 y noviembre de 2010. La edad media de los pacientes fue de 40 años. El instrumento empleado para la medición de la calidad de vida fue el Glasgow Benefit Inventory (GBI) y un cuestionario con preguntas abiertas, uso del BAHA, modificación del acúfeno y presencia o no de dolor. Resultados: La puntuación media del GBI total fue de 38, y en las subescalas general, social y física: 51, 15 y 7 respectivamente. La media de horas al día de utilización fue de 11. No se encontró relación significativa entre el sexo, edad y bilateralidad o unilateralidad del proceso con la calidad de vida. En cambio se observaron diferencias significativas entre los pacientes con hipoacusia transmisiva y con cofosis unilateral, si bien en ambos grupos los resultados fueron positivos. La presencia de acúfeno disminuyó de un 37,5 a un 20,8%, siendo esta diferencia estadísticamente significativa. Conclusiones: Nuestros resultados muestran que el uso del BAHA se asocia a una clara mejoría en la calidad de vida de los pacientes, menor en cofosis unilaterales, siendo necesario individualizar su indicación en este grupo. Asimismo muestran que el uso del BAHA tiene un efecto beneficioso sobre el acúfeno (AU)

Introduction and objectives: Assess the improvement of quality of life in osseointegrated implanted patients, taking into account the indication as well as the use of the implant, and the presence of pre- and postoperative tinnitus. Methods: Sixty-nine patients implanted between June 2004 and November 2010 were included. The average age of the patients was 40 years. The instruments used to quantify the change in quality of life were the Glasgow Benefit Inventory and a questionnaire including open questions, bone anchored hearing aid (BAHA) use, change in tinnitus and postoperative pain. Results: The average total benefit score with the Glasgow Benefit Inventory was 38, and the general, social, and physical scores were 51, 15 and 7, respectively. There was no significant association between sex, age and bilaterality or unilaterality of the process with quality of life. Nevertheless, there were significantly better results in patients with conductive hearing loss than in those with unilateral deafness, the results were positive although in both groups. The tinnitus rate went from 37.5 to 20.8% following BAHA, with this difference being significant. Conclusions: Our results show that the use of BAHA is associated with a great improvement in quality of life for patients with conductive hearing loss, whereas indications in unilateral deafness have to be individually studied. Moreover, the study shows that BAHA has a positive effect upon tinnitus (AU)

A new entity in the differential diagnosis of geniculate ganglion tumours: fibrous connective tissue lesion of the facial nerve.

Differential diagnosis of geniculate ganglion tumours includes chiefly schwannomas, haemangiomas and meningiomas. We report the case of a patient whose clinical and imaging findings mimicked the presentation of a facial nerve schwannoma.Pathological studies revealed a lesion with nerve bundles unstructured by intense collagenisation. Consequently, it was called fibrous connective tissue lesion of the facial nerve.

[Quality of life in patients implanted with the BAHA device depending on the aetiology]. / Calidad de vida de pacientes implantados con el dispositivo BAHA según su indicación.

INTRODUCTION AND OBJECTIVES: Assess the improvement of quality of life in osseointegrated implanted patients, taking into account the indication as well as the use of the implant, and the presence of pre- and postoperative tinnitus. METHODS: Sixty-nine patients implanted between June 2004 and November 2010 were included. The average age of the patients was 40 years. The instruments used to quantify the change in quality of life were the Glasgow Benefit Inventory and a questionnaire including open questions, bone anchored hearing aid (BAHA) use, change in tinnitus and postoperative pain. RESULTS: The average total benefit score with the Glasgow Benefit Inventory was 38, and the general, social, and physical scores were 51, 15 and 7, respectively. There was no significant association between sex, age and bilaterality or unilaterality of the process with quality of life. Nevertheless, there were significantly better results in patients with conductive hearing loss than in those with unilateral deafness, the results were positive although in both groups. The tinnitus rate went from 37.5 to 20.8% following BAHA, with this difference being significant. CONCLUSIONS: Our results show that the use of BAHA is associated with a great improvement in quality of life for patients with conductive hearing loss, whereas indications in unilateral deafness have to be individually studied. Moreover, the study shows that BAHA has a positive effect upon tinnitus.

Osteoblastoma of the temporal bone.

Benign osteoblastoma is an uncommon primary tumor of the bone. Any area of the skeleton may be affected by this tumor, but its occurrence in the temporal bone and middle ear is extremely rare. Clinical symptoms are nonspecific, even in the middle ear, and the diagnosis is often difficult in spite a complete physical and radiological examination. A biopsy is usually necessary for definitive diagnosis. Because of its potential for recurrence, local invasion, and, rarely, malignant transformation, a complete surgical excision remains the treatment of choice for osteoblastoma. We report a case of benign osteoblastoma involving the temporal bone and the middle ear and a review of the literature.

Benefits from a mobile telephone adapter in Combi40+ cochlear implant users.

CONCLUSIONS: Telephone adapters can help cochlear implanted patients to enhance their telephonic conversations. However, these devices should be further developed to be useful for daily life. OBJECTIVES: To test a mobile telephone adapter to improve speech discrimination by mobile telephone in Combi40+ users. To evaluate the influence of the mobile telephone in communication. SUBJECTS AND METHODS: Seventeen Combi40+ implantees were tested with bisyllabic words presented through mobile telephones with and without a telephone adapter in quiet and noisy environments. Speech audiometries in open field were obtained for every patient. RESULTS: Mean speech discrimination without the adapter in a quiet environment was 51.2%. In a noisy environment, mean discrimination increased significantly with the adapter from 30.3% to 42.9%. No statistically significant difference was found between speech discrimination in the quiet environment without the adapter and speech discrimination in the noisy environment with the adapter. When comparing speech discrimination in open field without lip-reading with equivalent telephonic conditions a statistically significant difference was found in favour of the open field.

[Musical perception and enjoyment in post-lingual patients with cochlear implants]. / Percepción y disfrute de la música en pacientes poslocutivos con implante coclear.

OBJECTIVES: To evaluate music perception and enjoyment following cochlear implantation and its impact on quality of life (QOL). MATERIAL AND METHOD: Eighty-eight adult post-lingually deaf cochlear implant users entered the study. The music questionnaire analyzed musical background, listening habits, and quality of musical sound through the cochlear implant. Music perception was evaluated with the Primary Measures of Music Audiation (PMMA). The Glasgow Benefit Inventory evaluated the quality of life (QOL). The music questionnaires, the PMMA and the GBI were completed by 84 %, 74 %, and 82 % of patients, respectively. RESULTS: Subjective enjoyment of music and listening habits decreased post-implantation when compared with prior to deafness. Mean scores for music perception were: tone 71 %, rhythm 78 %, timbre 61 %, songs 59 %, and melodies 23 %. The quality of music sound through the cochlear implant was associated with music enjoyment and with QOL. CONCLUSIONS: Music enjoyment and perception are possible with a cochlear implant. The quality of music sound through the cochlear implant influences post-operative QOL.

Percepción y disfrute de la música en pacientes poslocutivos con implante coclear / Musical perception and enjoyment in post-lingual patients with cochlear implants

Objetivos: Evaluar la percepción y el disfrute de la música tras la implantación coclear y su impacto en la calidad de vida. Material y método: Participaron en el estudio 88 adultos con sordera poslocutiva y portadores de un implante coclear. El cuestionario de la música analizó la experiencia musical, los hábitos musicales y la calidad del sonido musical a través del implante coclear. Para estudiar la percepción de la música se empleó el PMMA (Primary Measures of Music Audiation). La calidad de vida fue evaluada mediante el GBI (Glasgow Benefit Inventory). El cuestionario de la música, el PMMA y el GBI fueron contestados por el 84, el 74, y el 82 % de los pacientes, respectivamente. Resultados: El disfrute de la música y los hábitos musicales disminuyeron tras el implante coclear cuando se compararon con la situación previa a la sordera. Los valores medios de percepción de la música fueron: tono, el 71 %; ritmo, el 78 %; timbre, el 61 %; canciones, el 59 %, y melodías, el 23 %. La calidad del sonido de la música a través del implante coclear se relacionó con el disfrute de la música y con la calidad de vida. Conclusiones: Es posible disfrutar y percibir la música con un implante coclear. La calidad del sonido de la música a través del implante influye en la calidad de vida

Objectives: To evaluate music perception and enjoyment following cochlear implantation and its impact on quality of life (QOL). Material and method: Eighty-eight adult post-lingually deaf cochlear implant users entered the study. The music questionnaire analyzed musical background, listening habits, and quality of musical sound through the cochlear implant. Music perception was evaluated with the Primary Measures of Music Audiation (PMMA). The Glasgow Benefit Inventory evaluated the quality of life (QOL). The music questionnaires, the PMMA and the GBI were completed by 84 %, 74 %, and 82 % of patients, respectively. Results: Subjective enjoyment of music and listening habits decreased post-implantation when compared with prior to deafness. Mean scores for music perception were: tone 71 %, rhythm 78 %, timbre 61 %, songs 59 %, and melodies 23 %. The quality of music sound through the cochlear implant was associated with music enjoyment and with QOL. Conclusions: Music enjoyment and perception are possible with a cochlear implant. The quality of music sound through the cochlear implant influences post-operative QOL

Changes in listening habits and quality of musical sound after cochlear implantation.

OBJECTIVE: To evaluate listening habits and quality of musical sound after cochlear implantation. STUDY DESIGN: A total of 89 consecutive, adult, postlingually deafened, cochlear implant recipients entered the study. Music questionnaire included questions about musical background, listening habits, and quality of musical sound through cochlear implants. Music perception was evaluated with the Primary Measures of Music Audiation (PMMA). The questionnaire and the PMMA were administered to 67 and 65 patients, respectively. RESULTS: Listening habits significantly decreased postimplantation. The mean score (0-100) for the adjective pairs "like-dislike," "sounds like music-doesn't sound like music," "natural-mechanical," and "easy to follow-difficult to follow" was 60, 59, 50, and 40, respectively. The adjective pairs were associated with music enjoyment. Mean PMMA scores were 71 and 78 percent for tone and rhythm, respectively, with no association with music enjoyment. CONCLUSION: Music perception through a cochlear implant is a complex phenomenon that depends on audiological and non-audiological factors.