Multicenter Study Investigating Foreign Language Acquisition at School in Children, Adolescents, and Young Adults With Uni- or Bilateral Cochlear Implants in the Swiss German Population.

OBJECTIVES: Evaluation of foreign language acquisition at school in cochlear implant patients. STUDY DESIGN: Multicenter cohort study. SETTING: CI centers. PATIENTS: One hundred twenty-five CI users (10-18 yr) in the German-speaking part of Switzerland were enrolled. Demographic data were obtained by means of written questionnaires. German-speaking children with mainstream foreign language tuition (English and/or French) were enrolled for further testing. The control group of normal-hearing individuals was matched on age, class, and number of foreign language lessons attended. RESULTS: Overall, 100 questionnaires were returned. The 12 CI users without foreign language learning attended special schools. CI users who attended foreign language classes had better German speech comprehension compared with those without foreign language tuition (89 versus 51%; p < 0.05). Thirty-one CI users of different grades were further tested. All (10/10) CI 6th graders attained the school objectives for both English reading and listening skills. French performance at 6th grade for reading was 3/7 and for listening only 1/7. There were 13 matched normal-hearing pairs for English and 10 for French. The total scores were on average 7% higher, with a statistical significance for English reading (p < 0.05). CONCLUSIONS: Almost 90% of CI children in Switzerland learn foreign language(s) at school. All the tested patients reached the current school objectives for English reading. The success rate for French was lower, especially regarding listening tasks. The 13 matched pairs with normal-hearing did not score substantially better.

Introducing the "ChOLE" Classification and Its Comparison to the EAONO/JOS Consensus Classification for Cholesteatoma Staging.

: Many previous attempts have been made to classify or categorize cholesteatomas. Recently, the European Academy of Otology and Neurotology and the Japanese Otological Society proposed a classification system based primarily on extension and complications. The European Academy of Otology and Neurotology/Japanese Otological Society consensus statement makes an effort to standardize reporting of surgical techniques. Internet-based multicenter studies are facilitated by increasing connectivity, but a mutually-agreed framework for reporting is necessary for results to be comparable across sites. New technologies compete with established standardized surgical approaches and need to be validated. It is definitively the right time to find a consensus on how to record and report surgical findings in cholesteatoma surgery. To stimulate this interesting discussion, we propose a ChOLE-classification system, which is based on the differentiation into extension (Ch), status of the ossicular chain at the end of surgery (O), complications (L), and degree of pneumatization and ventilation (E). A numeric rule is used to stage these cholesteatomas from I-III.

[Benefit of Bimodal Stimulation with Cochlear Implant and Hearing Aid in Elderly Patients]. / Cochlea-Implantation ab dem 70. Lebensjahr ­ Nutzen der bimodalen Versorgung mit Hörgerät und CI.

Cochlear implant (CI) surgery is the standard of care for postlingual deafness in adults. A hearing aid (HA) for the non-implanted ear is normally used if residual hearing is available. There is limited data on bimodal stimulation in elderly patients. We compare the outcome of bimodal stimulation and analyzed the correlation of bimodal benefit and residual hearing in elderly patients. 22 patients≥70 years were implanted with a single-sided CI from 2002 till 2014. 17 patients use a HA on the non-implanted ear (bimodal). In a retrospective chart review we analyzed the audiological benefit (pure tone average, speech recognition). Time to follow-up was 6 month to 12 years. The subjective benefit was assessed using a structured questionnaire. The speech recognition (monosyllabic word testing in quiet @ 65 dB SPL) showed a score of 61% in bimodal condition vs. 53% with CI alone (p=0.35). There was no statistically significant correlation between residual hearing in the lower frequencies (250 Hz, 500 Hz and 1 kHz) and bimodal benefit. Most elderly patients benefit from bimodal stimulation. In quiet and in comparison with CI alone, most patients showed some improvement in word recognition scores using bimodal fitting, and all of them were using the contralateral hearing aid all day long. Postlingual deafened elderly patients fitted with a unilateral CI require a short rehabilitation period. In case of contralateral hearing aid fitting, regular control of the hearing aid should not be overlooked.

Single-Center Study Investigating Foreign Language Acquisition at School in Children, Adolescents, and Young Adults With Uni- or Bilateral Cochlear Implants in the Swiss German Population.

OBJECTIVE: To evaluate foreign language acquisition at school in cochlear implant patients. STUDY DESIGN: Cohort study. SETTING: CI center. PATIENTS: Forty three cochlear implants (CI) patients (10-18 yr) were evaluated. CI nonusers and patients with CI-explantation, incomplete datasets, mental retardation, or concomitant medical disorders were excluded. INTERVENTION(S): Additional data (type of schooling, foreign language learning, and bilingualism) were obtained with questionnaires. German-speaking children with foreign tuition language (English and/or French) at school were enrolled for further testing. MAIN OUTCOME MEASURE(S): General patient data, auditory data, and foreign language data from both questionnaires and tests were collected and analyzed. RESULTS: Thirty seven out of 43 questionnaires (86%) were completed. Sixteen (43%) were in mainstream education. Twenty-seven CI users (73%) have foreign language learning at school. Fifteen of these were in mainstream education (55%), others in special schooling. From 10 CI users without foreign language learning, one CI user was in mainstream education (10%) and nine patients (90%) were in special schooling. Eleven German-speaking CI users were further tested in English and six additionally in French. For reading skills, the school objectives for English were reached in 7 of 11 pupils (64%) and for French in 3 of 6 pupils (50%). For listening skills, 3 of 11 pupils (27%) reached the school norm in English and none in French. CONCLUSIONS: Almost 75% of our CI users learn foreign language(s) at school. A small majority of the tested CI users reached the current school norm for in English and French in reading skills, whereas for hearing skills most of them were not able to reach the norm.

Objective Measurements of Ossicular Chain Mobility Using a Palpating Instrument Intraoperatively.

BACKGROUND: The judgment of a normal or impaired mobility of middle ear ossicles is based on palpation and depends highly on the surgeon's subjective experience. The aim of this study was to develop and test a palpating instrument recording force and vector and allowing to support the surgeon's subjective impression with objective measurement results. STUDY DESIGN: Prospective recordings at surgery. SETTING: Tertiary referral center. PATIENTS AND METHODS: A fiberoptic force-sensing element allowing force measures in three orthogonal directions was integrated into a handheld 45 degree hook and tested in temporal bones. Clinical data series from patients with a functionally normal chain (e.g., cochlear implants (CI)) and impaired ossicles (otosclerosis) were collected. The ossicles were palpated until their first movements out of the resting stage were visualized, the applied force, and vector were recorded by an independent observer. RESULTS: Four CI and 19 otosclerosis patients were further evaluated. The minimum detectable force change of the sensor was 0.2 gF (2 mN). In the otosclerosis patients the average force applied to move the malleus was 9.5 gF, on the incus 8.7 gF. These values were slightly lower after separation of the incudostapedial joint, reaching 8.5 gF and 6.9 gF, respectively. The fixed stapes showed a rigidity of 14.7 gF or higher. The values were lower in the CI group measuring 4.4 gF, 4.1 gF, and 3.3 gF on the three ossicles, respectively. CONCLUSIONS: We were able to produce a disposable, easy-to-handle palpating probe that enables the otologist to record continuously tip contact forces in three dimensions during his standard palpation of each ossicle. Normative values were reproduced for each ossicle, as well as increased rates for stapes fixation in otosclerosis.

Incus necrosis after irradiation.

OBJECTIVES: Osteoradionecrosis (ORN) of the temporal bone is a rare, late complication of radiotherapy to the temporal bone region for head and neck or skull base tumours. ORN can occur as a localized or a diffuse type, according to the extension of the affected temporal bone. It can lead to otitis externa, otitis media, aseptic labyrinthitis and may lead to serious intracranial complications. Hearing loss may be conductive, sensorineural, or mixed. A few case studies report of previously irradiated patients presenting with conductive hearing loss presumably caused by ORN of the ossicular chain. In only one case report of diffuse ORN, ORN of the ossicles was histologically proven, leading to the conclusion that ossicular chain involvement as the sole entity of ORN would not exist. However, the presented case report disputes this. PATIENTS: A 13-year-old boy presenting with a unilateral mixed hearing loss as the sole otological complaint, 10 years after radiotherapy for an anaplastic ependymoma. RESULTS: Middle ear inspection revealed ORN of the incus which was confirmed by histological investigation. CONCLUSION: ORN of the ossicular chain as a late complication can occur as an isolated entity and may present as conductive hearing loss predominantly in the low frequencies. Middle ear inspection and ossicular chain reconstruction should be attempted, bearing in mind the risk of iatrogenic lacerations of a thin tympanic membrane and possibly a delayed wound healing.

Surgical management of benign idiopathic osteonecrosis of the external auditory canal.

BACKGROUND/AIMS: Benign idiopathic osteonecrosis of the external ear canal remains a challenging management problem, with many patients experiencing a prolonged chronic course requiring frequent debridement. The technique of bony sequestrectomy, followed by rotation of a vascularized fascial flap and then a free full-thickness skin graft is presented. METHODS: Success with this technique is described in a retrospective case series format in addition to a review of the existing literature on surgical techniques for the treatment of this condition. RESULTS: The described technique was performed on 5 patients following prolonged periods of failed medical therapy (minimum 8 months). All 5 patients achieved a healed external auditory canal, with no ongoing skin ulceration or bony sequestration. Follow-up data (median 22 months) suggests that the technique provides a robust and long-lasting therapy. CONCLUSION: Symptomatic benign idiopathic osteonecrosis of the tympanic bone is well treated by provision of vascularized tissue to maximize the chance of bone healing and subsequent cure. It should be considered early in the management protocol to avoid lengthy symptomatic periods, the symptoms of which ultimately may not heal with medical therapy alone.

Mucoepidermoid carcinoma of the parotid infiltrating the chorda tympani nerve.

We present here the first case report of a mucoepidermoid carcinoma of the parotid infiltrating the chorda tympani nerve and also discuss why an initial diagnosis of Bell palsy may be misleading.

Tympanoplasty in chronic otitis media patients with an intact, but severely retracted malleus: a treatment challenge.

OBJECTIVE: To analyze the outcome of patients with chronic otitis media (COM) with an intact, but markedly medialized ossicular chain, treated by removing the malleus head and interposing an autologous incus and then an underlay myringoplasty. STUDY DESIGN: Retrospective clinical study. SETTING: Tertiary referral center. PATIENTS: The search criteria within the prospective surgical database was COM with a central perforation (without cholesteatoma) with a markedly medialized malleus handle (the umbo adherent to the promontory) with an intact ossicular chain (study, n = 15) or an incus necrosis at the lenticular process (incus, n = 23). Only primary surgeries performed at our otorhinolaryngology department were included. INTERVENTION: All patients underwent the same surgical procedure consisting of an autologous incus interposition and underlay myringoplasty with temporalis fascia. MAIN OUTCOME MEASURE: The patients' audiological and follow-up data were retrieved from the database. The postoperative audiogram (0.5-3 kHz) with the longest follow-up was used. RESULTS: The preoperative air-conduction thresholds were less impaired in the study group than in the incus group. After their surgery, all, except 3 patients, improved their hearing, and 97% had an intact tympanic membrane at a mean follow-up of 2 years. The air-bone gap was closed within 20 dB in 80% (study) and in 87% (incus), in one third of all patients even within 10 dB. Although the largest improvement was seen in the lower frequencies, closure of the air-bone gap at 4 kHz was difficult to achieve. CONCLUSION: Patients presenting with COM, a (central) perforation, a medially rotated malleus and intact ossicular chain are a treatment challenge. Lateralizing the malleus handle may require disconnection of the ossicular chain and an autologous incus interposition to bring back the reconstructed tympanic membrane in its original position and improve the hearing.

The management of peripheral facial nerve palsy: "paresis" versus "paralysis" and sources of ambiguity in study designs.

OBJECTIVE: Conservative management of idiopathic or herpetic acute peripheral facial palsy (herpes zoster oticus, HZO) often leads to a favorable outcome. However, recent multicenter studies have challenged the necessity of antivirals. Whereas large numbers of patients are required to reveal statistical differences in a disease with an overall positive outcome, surprisingly few studies differentiate between patients with paresis and paralysis. Analyzing our own prospective cohort of patients and reviewing the current literature on conservative treatment of Bell's palsy and HZO, we reveal the importance of initial baseline assessment of the disease course to predict the outcome and to validate the impact of medical treatment options. STUDY DESIGN AND DATA SOURCE: Prospective analysis of consecutive patients referred to 2 tertiary referral centers and research on the Cochrane Library for current updates of their previous reviews and search of MEDLINE (1976-2009) for randomized trials on conservative treatment of acute facial palsy were conducted. METHODS: One hundred ninety-six patients with Bell's palsy or HZO were followed up prospectively until complete recovery or at least for 12 months. The numeric Fisch score (FS) was used to classify facial function, and patients were separated between incomplete palsy (=paresis) and complete paralysis. Electroneuronography (ENoG) was used to further subdivide patients with paralysis. The treatment protocol was independent of the ongoing investigation including prednisone and valacyclovir in most patients. A total of 250 previous studies on facial palsy outcome were evaluated regarding their distinction between different severity scores at baseline and its impact on treatment outcome. Trials not making the distinction between paresis and paralysis at baseline and with an insufficient follow-up of less than 12 months were excluded. RESULTS: In the Bell's and HZO paresis group, all except 1 patient recovered completely, most of them within 3 months, independent of the treatment regimen. In the Bell's paralysis group, 38 patients (70%) recovered completely after 1 year, including 94% of patients with a denervation by ENoG of less than 90%. Thirty percent of Bell's paralysis patients recovered incompletely, revealing the worst outcome in patients with a 100% denervation on ENoG. None of the 4 patients with HZO and ENoG denervation of more than 90% recovered to normal facial function. We found a highly significant difference regarding the time course and final outcome in patients with incomplete palsies versus total paralysis; however, only 3 of 250 studies make this distinction. CONCLUSION: The time course for improvement and the extent of recovery is significantly different in patients presenting with an incomplete facial nerve paresis compared with patients with a total paralysis. Whereas the term "palsy" includes both entities, the term "paralysis" should only be used to describe total loss of nerve function. Patients with incomplete acute Bell's palsy (paresis) should start to improve their facial function early (1-2 wk after onset) and are expected to recover completely within 3 months. These patients do not benefit from antiviral medications and most likely do not profit from systemic steroids. Mixing patients with different severity of palsies will always lead to controversial results.

Impact of the SMAS on Frey's syndrome after parotid surgery: a prospective, long-term study.

BACKGROUND: Clinical observations indicate that creation of the superficial musculoaponeurotic system (SMAS) flap during parotid surgery decreases postoperative gustatory sweating (Frey's syndrome) and improves cosmesis after surgery. METHODS: On the basis of their previous study with 23 patients where no SMAS flap was used, the authors performed a prospective, long-term study of 6(1/2) years in 25 patients, using the SMAS flap, and compared these results with the postoperative results of their initial study. Twenty-two patients of the SMAS flap group were available for reassessment of gustatory sweating and symptoms. The Minor starch test was used to document the extent and intensity of postoperative sweating. Satisfaction with postoperative cosmetic results was assessed through a semiquantitative questionnaire as used in the previous study. RESULTS: The incidence of symptomatic Frey's syndrome was significantly higher in the no-SMAS flap group after 23 months (43 percent versus 0 percent; p = 0.003). The surface extent of Frey's syndrome after 23 months was significantly reduced in the SMAS flap group (p = 0.006). At final follow-up, the incidence and extent of symptomatic Frey's syndrome showed no significant differences between the two groups (41 percent versus 56 percent; p = 0.42). The rate of satisfactory cosmetic results was significantly higher in the SMAS flap group (96 percent versus 35 percent; p < 0.05). CONCLUSIONS: Creation of the SMAS flap in parotid surgery for benign lesions delays the onset and reduces the intensity and extent, but does not prevent the occurrence, of Frey's syndrome. It reliably improves the cosmetic results by reducing the retromandibular depression after parotid surgery.

A checklist for surgical exposure in stapes surgery: how to avoid misapprehension.

The goal of middle ear exploration in stapes surgery is to identify impairments of function along the entire ossicular chain. The endaural approach with an extended tympanomeatal flap and an almost routinely performed anterosuperior canalplasty allow adequate exposure to identify the anterior malleal ligament and process (1), the inferior incudomalleal joint (2), the entire stapes including the pyramidal process (3), and the round window niche (4). With this checklist at hand the otologic surgeon can define the exact location of the hearing impairment and choose the proper technique for hearing reconstruction in primary and revision surgeries.

Phenotypic characterization of DFNA24: prelingual progressive sensorineural hearing impairment.

This article describes the hearing impairment (HI) phenotype which segregates in a large multi-generation Swiss-German family with autosomal dominant nonsyndromic HI. The locus segregating within this pedigree is located on chromosome 4q35-qter and is designated as DFNA24. For this pedigree, audiometric data on 25 hearing-impaired family members are available. It was demonstrated that within this kindred the HI is sensorineural, bilateral, prelingual in onset, and progressive throughout life. Age-related typical audiograms depict steeply down-sloping curves, with moderate high-frequency HI at birth, then steady progression to moderate HI in the low frequencies, severe HI at mid-frequencies and profound HI at high frequencies by age 70. Annual threshold deterioration was approximately 0.5 dB/year at 1-2 kHz after correction for presbycusis.

Round window atresia and its effect on sound transmission.

OBJECTIVE: To document isolated round window atresia and to discuss its impact on current theories of inner ear function. PATIENTS AND STUDY DESIGN: Retrospective analysis of isolated round window atresia suggesting an autosomal dominant inheritance pattern and review of current concepts of cochlear macromechanics. SETTING: Tertiary referral center. RESULTS: The unexpected finding of isolated round window atresia in two female patients of the same family was confirmed intraoperatively as well as postoperatively using high-resolution computed tomography. The current audiograms and the review of the literature highlight a mixed, but predominantly conductive, hearing impairment with thresholds at 30 to 40 dB. Implying that there is no pressure release mechanism for inner ear fluid displaced by the stapes footplate, a total conductive hearing loss would be expected. CONCLUSIONS: The rare finding of round window atresia can be overlooked at surgery because of insufficient exposure of the round window niche. High-resolution computed tomography confirms the round window obliteration. It seems that an alternative way of cochlear stimulation takes place besides the concept of fluid bulk shifting. Surgery seems not to guarantee favorable results.

Arachnoid cyst of the fallopian canal: a surgical challenge.

OBJECTIVE: Spontaneous cerebrospinal fluid (CSF) leaks from the fallopian canal are exceedingly rare, with only 6 reports appearing in the world literature. We report a seventh case that is unique in that it involves an arachnoid cyst of the fallopian canal and an associated facial nerve palsy. STUDY DESIGN: Case report. SETTING: International tertiary care referral center. CONCLUSION: CSF otorhinorrhea associated with a facial nerve palsy may be indicative of an arachnoid cyst of the fallopian canal. These fistula are extremely rare. Surgical management involves sealing the fistula while preserving facial nerve function and is extremely challenging.