Middle-ear effusion in children with cleft palate: congenital or acquired?

OBJECTIVE: Cleft palates are one of the most common congenital malformations. Because of the loss of Eustachian tube function, middle-ear ventilation is reduced. The aim of this study was to determine if middle-ear effusions were present at birth or at the three-month audiological evaluation. METHOD: A total of 53 children with a cleft palate were included. Data review included the results of newborn hearing screening, microscopic findings, a tympanometry, a free field audiometry and intra-operative findings. RESULTS: A total of 58.4 per cent of patients had a median, 26.4 per cent had a bilateral, 11.3 per cent had a unilateral and 3.8 per cent had a limited soft palate cleft. Newborn hearing screening showed a pass in 83.1 per cent of newborns bilaterally. The first ear microscopy showed a bilateral middle-ear effusion in 90.6 per cent of cases. During cleft surgery, bilateral paracentesis was performed in all cases, and in 90.6 per cent middle-ear effusion was sucked out. CONCLUSION: The majority of children with a cleft palate do not present with middle-ear effusion at birth. It develops within several days or weeks of life.

[Objective audiometry and clinical application]. / Objektive Audiometrie und klinische Anwendung.

Subjective and objective hearing tests are used for evaluation of hearing impairments. Objective methods include impedance measurement with tympanometry and stapedius reflex measurement, otoacoustic emissions (OAE), and auditory evoked potentials (AEP). Combined with statistical analysis, the introduction of auditory steady state responses (ASSR) has enabled objective hearing tests in newborn hearing screening and automated hearing threshold assessment. The type and degree of hearing loss can be determined using the abovementioned methods. Precise interpretation of the test results is helpful to distinguish an auditory synaptopathy/neuropathy from classical sensorineural hearing loss.

[20 Years of Bilateral Cochlear Implantation - an Analysis of the Implanted Patients]. / 20 Jahre bilaterale Cochleaimplantation ­ eine Analyse seither implantierter erwachsener Patienten.

Binaural hearing is essential for localization abilities and improves the speech perception in noise. Since 20 years, bilateral cochlear implantation is routinely performed to restore binaural hearing. In this cross-sectional study, we evaluated speech perception in quiet (Freiburger monosyllables, Hochmair-Schulz-Moser (HSM) sentence test, each at 70 dB) and in noise (HSM test, signal-to-noise ratio 10 dB) in 103 out of 165 adult patients who were bilaterally implanted in Würzburg between 1995 and June 2014. In almost half the patients, the second implanted side showed the better speech perception. Compared to the first implanted side, the average monosyllable scores with bilateral implants were improved from 54 to 63% and the HSM scores from 86 to 96%. In noise the speech perception improved from 47 to 65%. The speech perception of the second implanted side was independent of the time interval between the implantation of both sides in this cohort of postlingually deafened patients. This cross-sectional data underline the importance of bilateral cochlear implantation for speech understanding in quiet and even more in noise and thus, for the everyday life. For this, bilateral cochlear implantation should be the generally accepted standard in the treatment of deaf patients.

[Hearing results after middle fossa removal of small (T1/T2) vestibular schwannomas]. / Hörergebnisse nach transtemporaler Resektion kleiner (T1/T2) Akustikusneurinome.

OBJECTIVE: The aim of this study was to evaluate the hearing results after surgery for T1 and T2 vestibular schwannomas (VS) via the middle cranial fossa (MCF) approach at one institution and to review outcomes in the recent literature. PATIENTS AND METHODS: In our department, 208 consecutive patients have undergone surgery using the MCF approach between December 2005 and February 2015. Audiological testing included pure-tone audiometry, speech-discrimination-tests pre- and postoperatively, as well as a pre- and intraoperative brainstem evoked response audiometry (BERA). Hearing status was categorized according to the American Academy of Otolaryngology - Head and Neck Surgery (AAO-HNS) or Gardner-Robertson classification. Hearing preservation was assessed when postoperative values were still within class A + B. RESULTS: In 167 of the 208 patients, preoperative hearing levels corresponded to class A+B. In 78 tumors, extension of the VS was limited to the internal auditory canal (T1) and in 89 tumors there was an extrameatal growth without touching the brainstem (T2). In 109 patients (65 %), postoperative hearing was still in class A+B. In the group of intrameatal tumors, the hearing preservation rate was even 69 %. The analysis of the literature of the past 16 years revealed comparable results (46-82 %). CONCLUSIONS: Surgery for VS using the MCF approach is an established technique for reliable tumor removal with an adequate rate of hearing preservation. For small tumors without brainstem contact, particularly satisfying results can be achieved. This should be taken into account when discussing the possibility of early hearing preservation surgery.

[Preoperative auditory evaluation and postoperative follow-up in cochlear implantees : The role of objective measures]. / Präoperative audiologische Evaluation und postoperative Verlaufsdiagnostik bei Cochleaimplantatversorgung : Bedeutung objektiver Testverfahren.

The primary diagnostic aim prior to cochlear implantation is establishment of a comprehensive and multidisciplinary diagnosis, in order to subsequently begin therapy as early as possible. Audiological evaluation prior to implantation employs a test battery-approach, including subjective and objective procedures. Objective measures show high reliability and therefore play a major role in the diagnosis of difficult-to-test subjects such as infants and young children. During postoperative follow-up, objective measures offer a valid method for analyzing the effects of different stimuli on the auditory system. Particularly in infants, children, and uncooperative patients, the results of these tests enable the speech processor settings to be optimized, the hearing benefit to be assessed, and treatment to be adapted accordingly. Auditory brainstem responses (ABR) offer an excellent test/retest and inter-/intrarater reliability and validity, and are the most commonly used method for objective hearing threshold estimation and evaluation of the functional integrity of the lower auditory pathway. The use of narrow-band stimuli allows frequency-specific threshold estimation; analysis of stationary auditory steady state potentials (ASSR) adds the advantages of automated objective detection. Electrocochleography and electrically evoked ABR give valuable information in special cases. The use of cortical potentials (CAEP) in response to speech stimuli is quite promising, although the high response variability currently limits this method's clinical application. An audiological test-battery approach combining the results of subjective and objective measures leads to significantly increased reliability of preoperative diagnosis and postoperative follow-up in cochlear implantees.

[Patient Satisfaction after Pitch Elevation and Development of a Therapy Algorithm]. / Zufriedenheit nach Stimmerhöhung bei Transsexualität ­ Entwicklung eines Therapiealgorithmus.

Objective: Voice feminization is an important step in the therapy of male-to-female transsexualism. Approaches are conservative voice therapy and surgical interventions. The most powerful parameter of gender perception is the fundamental frequency. Besides the vocal pitch, there are other parameters influencing gender perception of a voice, e. g. intonation, prosody or formant frequencies. Material and methods: In 21 male to female transgender persons after surgical elevation of the vocal pitch the Voice Handicap Index (VHI), the Life Satisfaction Questionnaire (FLZ) and a new addendum were used. A new algorithm for voice feminization in male-to-female transsexualism was deduced. Results: After elevation of the vocal pitch, the self-confidence of the male-to-female transgender persons has increased. Despite of an elevated pitch some persons were not satisfied with their voice. Conclusion: Surgical intervention changes only the pitch of a voice. To change other parameters, conservative voice therapy is necessary. If the transgender persons are able to reach a satisfying female voice with conservative voice therapy alone, surgical intervention is not indicated.

[Electrocochleography using Transtympanic, Ear Drum and Ear Canal Electrode in Diagnosis of Morbus Menière]. / Elektrokochleografie mit Trommelfell-, Gehörgangs- und transtympanaler Elektrode bei V.a. Morbus Menière.

OBJECTIVE: Aim of this study was to explore the clinical value of non-invasive recordings of electrocochleography in comparison to the use of invasive electrodes for the diagnosis of endolymphatic hydrops in patients with suspicious Morbus Menière. MATERIAL AND METHODS: 15 patients with clinical suspicion of Morbus Menière underwent electrocochleography with transtympanic needle-electrode as well as tympanic membrane electrode. 10 patients received electrocochleography with transtympanic needle-electrode and ear canal electrode. A control group of 12 healthy adults was evaluated using tympanic membrane electrode. The negative electrode was placed on the ipsilateral mastoid, the ground electrode in the middle of the forehead. For stimulation broadband click stimulus as well as long tone bursts of 1, 2 and 4 kHz were applied. In addition the impedance of the electrode was registered. RESULTS: While transtympanic electrocochleography gave reliable results in 100% of these cases, extratympanic recordings often manifested poor wave morphology. The electrocochleographic measures following stimulation with 1 and 2 kHz long tone bursts were the best measures to identify endolymphatic hydrops. This was the case with transtympanic as well as with tympanic membrane electrodes. There was no correlation between impedance and wave morphology. CONCLUSIONS: For the evaluation of Morbus Menière non-invasive electrocochleography showed good results using a tympanic canal electrode with 1 and 2 kHz tone bursts. Yet in unclear cases transtympanic electrocochleography should be preferred.

DFNB16 is a frequent cause of congenital hearing impairment: implementation of STRC mutation analysis in routine diagnostics.

Increasing attention has been directed toward assessing mutational fallout of stereocilin (STRC), the gene underlying DFNB16. A major challenge is due to a closely linked pseudogene with 99.6% coding sequence identity. In 94 GJB2/GJB6-mutation negative individuals with non-syndromic sensorineural hearing loss (NSHL), we identified two homozygous and six heterozygous deletions, encompassing the STRC region by microarray and/or quantitative polymerase chain reaction (qPCR) analysis. To detect smaller mutations, we developed a Sanger sequencing method for pseudogene exclusion. Three heterozygous deletion carriers exhibited hemizygous mutations predicted as negatively impacting the protein. In 30 NSHL individuals without deletion, we detected one with compound heterozygous and two with heterozygous pathogenic mutations. Of 36 total patients undergoing STRC sequencing, two showed the c.3893A>G variant in conjunction with a heterozygous deletion or mutation and three exhibited the variant in a heterozygous state. Although this variant affects a highly conserved amino acid and is predicted as deleterious, comparable minor allele frequencies (MAFs) (around 10%) in NSHL individuals and controls and homozygous variant carriers without NSHL argue against its pathogenicity. Collectively, six (6%) of 94 NSHL individuals were diagnosed with homozygous or compound heterozygous mutations causing DFNB16 and five (5%) as heterozygous mutation carriers. Besides GJB2/GJB6 (DFNB1), STRC is a major contributor to congenital hearing impairment.

New outcomes with auditory brainstem implants in NF2 patients.

OBJECTIVE: To determine factors related to high levels of speech recognition in patients with the auditory brainstem implant (ABI). STUDY DESIGN: Retrospective case review. SETTING: International multicenter data from hospitals and tertiary referral facilities. PATIENTS: Patients with neurofibromatosis type 2 (NF2) and bilateral vestibular schwannomas. INTERVENTION: ABIs were placed after the removal of vestibular schwannomas. MAIN OUTCOME MEASURES: Demographic and surgical data were collected from 26 patients with ABIs who achieved scores of better than 30% correct identification of sentences presented in quiet listening conditions and without lipreading cues. RESULTS: Scores better than 30% speech recognition of standard sentence test materials (HINT or equivalent) in quiet listening conditions were obtained in 26 of the 84 NF2 patients (31%). ABI speech recognition was correlated with surgical position, length of deafness, the number of distinct pitch electrodes, perceptual levels, and ABI stimulation rate, but not correlated with tumor size, tumor stage, the number of electrodes used, or electrophysiological recordings. This paper presents the consensus opinion from a meeting of surgeons to compare outcomes across ABI surgical centers. CONCLUSIONS: The consensus opinion was that brainstem trauma is a primary factor in the variability of outcomes in NF2 patients. The significant co-factors in outcomes implied that ABI surgery should be accomplished with great care to minimize physical and venous trauma to the brainstem. It is clear that high levels of speech recognition, including high levels of open-set speech recognition, are possible with the ABI even in patients with NF2 and large tumors.

[Auditory evoked potentials: basics and clinical applications]. / Akustisch evozierte Potenziale: Grundlagen und klinische Anwendung.

Auditory evoked potentials (AEPs) are elicited at various levels of the auditory system following acoustic stimulation. Electrocochleography is a technique for recording AEPs of the inner ear. The recording is performed by means of a needle electrode placed on the promontory or non-invasive with tympanic membrane or ear canal electrodes. Clinically, electrocochleography is used for the diagnosis of auditory neuropathy spectrum disorder (ANSD) and endolymphatic hydrops. According to their latencies, AEPs of the central auditory pathway are subdivided into early, middle and late (cortical) AEPs. These AEPs are recorded via surface scalp electrodes. Normally, the larger EEG masks AEPs. For unmasking the AEP, several techniques are applied. Early AEPs or auditory brainstem responses (ABR) are the most widely used AEPs for functional evaluation of the auditory pathway. In contrast to otoacoustic emissions, early AEPs can detect ANSD. Thus, they are more suitable for hearing screening in newborns. For this purpose automated procedures are implemented.

[Speech perception in noise with the "wuerzburg speech test for children"]. / Sprachverstehen im Störlärm mit dem Würzburger Kindersprachtest.

BACKGROUND: Patients with hearing loss complain of a reduced speech perception in noise. For school children and adults there are several standardized audiometric tests available to measure the outcome in background noise like the HSM-Test, OlSa or OlKiSa. However, for kindergarden and preschool children a comparable speech perception test in noise does not exist. The aim of this study was to evaluate and standardize the recently developed "wuerzburg speech test for children" in noise. MATERIALS AND METHODS: 24 children aged 5-9 years with bilateral normal hearing were included. They have been tested with the test IV of the "wuerzburg speech test for children" in noise. This test consists of 3 lists, each with 20 two-syllable words. For the background noise, a 32 time delayed interference of the speech items were used. RESULTS: The speech perception threshold (SRT) at 60 dB (SPL) depending on the signal to noise ratio (SNR) was measured for each child. The overall averaged SNR was -8.3 dB. Furthermore a small, non-significant influence by the age of the children to the test results was seen. CONCLUSION: The "wuerzburg speech test for children" is suitable to measure the individual speech perception in noise. It is appropriate for kindergarden, preschool and school children. The test material consists of the uptodate vocabulary of children. The average SRT was -8.3 dB SNR, which is comparable with the published literature.

[Evaluation of the Freiburg monosyllabic speech test in background noise]. / Evaluation des Freiburger Einsilbertests im Störschall.

BACKGROUND: The Freiburg speech test has been the gold standard in speech audiometry in Germany for many years. Previously, however, this test had not been evaluated in assessing the effectiveness of a hearing aid in background noise. Furthermore, the validity of particular word lists used in the test has been questioned repeatedly in the past, due to a suspected higher variation within these lists as compared to the other word list used. PATIENTS AND METHODS: In this prospective study, two groups of subjects [normal hearing control subjects and patients with SNHL (sensorineural hearing loss) that had been fitted with hearing aid] were examined. In a first group, 113 control subjects with normal age- and gender-related pure tone thresholds were assessed by means of the Freiburg monosyllabic test under free-field conditions at 65 dB. The second group comprised 104 patients that had been fitted with hearing aids at least 3 months previously to treat their SNHL. Members of the SNHL group were assessed by means of the Freiburg monosyllabic test both with and without hearing aids, and in the presence or absence of background noise (CCITT-noise; 65/60 dB signal-noise ratio, in accordance with the Comité Consultatif International Téléphonique et Télégraphique), under free-field conditions at 65 dB. RESULTS: The first (control) group exhibited no gender-related differences in the Freiburg test results. In a few instances, inter-individual variability of responses was observed, although the reasons for this remain to be clarified. Within the second (patient) group, the Freiburg test results under the four different measurement conditions differed significantly from each other (p>0.05). This group exhibited a high degree of inter-individual variability between responses. In light of this, no significant differences in outcome could be assigned to the different word lists employed in the Freiburg speech test. CONCLUSION: The Freiburg monosyllabic test is able to assess the extent of hearing loss, as well as the effectiveness of a fitted hearing aid, in the presence or absence of background-noise (CCITT-noise). The present study could not evidence statistically significant differences in outcome when using the different word lists in this test battery.

[Direct recording from cochlear nerve via a ball-electrode in transtemporal acoustic neuroma surgery]. / Direkte Ableitung vom Nervus cochlearis bei transtemporaler Resektion von Vestibularisschwannomen.

BACKGROUND: Intraoperative monitoring (IOM) of the cochlear nerve function during acoustic neuroma surgery is employed to assist in preserving hearing. So far, Auditory Brain-stem Response (ABR) is considered to be an optimal method for intraoperative monitoring in transtemporal approach. The aim of this study was to perform direct recording of the cochlear nerve action potential after resection of the tumor by using a ball-electrode and to evaluate the use of this method in predicting the postoperative hearing. The obtained data were compared to the simultaneous ABR results and to the postoperative hearing. MATERIAL AND METHODS: In 2009, 38 patients have undergone acoustic Neuroma Surgery at the ENT University hospital, Wuerzburg. In 33 patients an intraoperative ABR as well as a direct measurement from the cochlear nerve using a ball electrode were performed. In 5 patients the postoperative hearing was predicted using the direct measurement at the cochlear nerve only. RESULTS: The direct recording from the cochlear nerve gave very robust responses. Even in cases where ABR recording was not possible, the identification of clear cochlear nerve action potential could still be reached. Using the direct recordings from the cochlear nerve to predict the post operative hearing turned out to have a sensitivity of 100% and a specificity of 70%. CONCLUSIONS: These results show that intraoperative monitoring with direct recording from the cochlear nerve via a ball-electrode in transtemporal approaches offers a valuable method to predict the postoperative hearing. Further investigation will be made to provide additional information.

[Short and long term results of endolymphatic sac surgery: a patient-questionnaire based study]. / Die Saccotomie in der Behandlung des M. Ménière: Beurteilung der kurz-, mittel- und langfristigen Ergebnisse anhand eines Patientenfragebogens.

INTRODUCTION: The endolymphatic sac surgery for the treatment of Meniere's disease has been described since the 1920s. The success rate of this technique in terms of vertigo control has been reported to be 50-80%. However, the value of this treatment method remained controversial. Furthermore, the reliable identification of the endolymphatic sac intraoperatively can be challenging in some cases. This study examines the short-, middle- and long-term results in a larger cohort of patients. MATERIALS AND METHODS: In 74 patients, vertigo control, tinnitus and degree of satisfaction was evaluated by means of a questionnaire retrospectively. Additionally, the diagnostic value of the electrocochleography (EcochG) was determined. RESULTS: The overall vertigo control rate was more than 70% in patients followed up for two years and has reached 81% in patients followed up for more than two years. Hearing preservation rate was 61%. Tinnitus has disappeared in 11% and improved in 23% of the patients. In 47% of the patients it was unchanged and in 19% worsened. The difference in EcochG results pre- versus postoperative was highly significant. CONCLUSIONS: ELSS is a useful tool in the management of Ménière's disease, in particular in patients that do not benefit sufficiently from conservative therapy.

[Clinical and audiological findings in children with auditory neuropathy]. / Klinische und audiologische Befunde von Kindern mit auditorischer Neuropathie und ihre Versorgung mit einem Cochlea-Implantat.

BACKGROUND: Auditory neuropathy is a disorder characterised by preservation of outer hair cells function with normal otoacoustic emissions (OAEs), but with absent auditory brainstem responses (ABR). Perisynaptic synchronisation disorder is one of the possible pathogenesis underlying auditory neuropathy. In this paper we describe the clinical presentation and audiological findings in pediatric auditory neuropathy and its management. PATIENTS AND METHODS: 9 children with auditory neuropathy could be included in the study. An audiological evaluation was performed in all children including behavioural audiometry, measurement of the OAEs as well as electrocochleography (ECoG) and ABR recordings. Children who failed to get any benefit from conventional amplification received a cochlear implant. Prior to implantation the responses to electrical stimuli were examined with the promontory test and with the electrically evoked ABR. RESULTS: One child showed auditory neuropathy only on one side with normal hearing thresholds on the contralateral ear. Another child had normal hearing thresholds after the follow up period. Four children received a hearing aid. But variable hearing reactions were observed. Thus in three cases a CI is planned. In three children cochlea implantation was done. Following implantation a remarkable improvement in hearing/speech capabilities with the CI compared to conventional hearing aids were observed in all three cases. Beside, these three children developed open set speech discrimination and are using now oral language for communication. CONCLUSIONS: Auditory neuropathy is a disorder which presents with different clinical and audiological findings. Thus the management of this disorder must be an individual one. In light of our findings we support the use of cochlear implants as an option for children with auditory neuropathy in cases where conventional amplification does not work sufficiently.

[Development of a new test for speech audiometry in children and results in CI children]. / Entwicklung eines neuen Kindersprachtests und Erprobung an CI-Kindern.

BACKGROUND: Speech audiometry plays an important role for hearing evaluation in children. Hearing impaired children, such as those with cochlear implant (CI) who frequently undergo testing, learn the test words by heart. Furthermore, the standard tests use words of former time. PATIENTS AND METHODS: Using the most frequently words of modern children's literature a speech assessment test was developed for children between 1 to 8 years. The material was first evaluated on normal hearing children. In a second step the phoneme and word perception scores of 35 CI children were measured using 60 - 100 dB SPL according to the most comfortable level (MCL) in free sound field. RESULTS: The performance-intensity function for the single words were highly comparable with those of the Freiburger or Göttinger speech perception test. The average speech reception threshold in CI children was 44 % (test I), 36 % (test II), 63 % (test III) and 69 % (test IV), respectively. Compared to the Mainzer and Göttinger speech perception test the new words were more difficult to understand than those of the known tests. However, a statistically significant difference could be noted only for test II. CONCLUSIONS: The new developed "Würzburger speech perception test" appears to be a useful additional tool for the evaluation of hearing impaired children in addition to the standard test procedures.

Bilateral cochlear implants: a way to optimize auditory perception abilities in deaf children?

OBJECTIVES: The Würzburg bilateral cochlear implant (CI) program was started with the aim to improve the patients' communicative abilities in complex listening situations. In this study, the auditory skills of children using bilateral cochlear implants were evaluated. STUDY DESIGN AND SETTINGS: Qualitative data based on free observations in the rehabilitation setup were collected in 39 bilaterally implanted children. A speech discrimination in noise test was performed in 18 of these children; lists of bisyllabic words were presented in noise at a signal to noise ratio (SNR) of +15 dB. RESULTS: Qualitative and quantitative data show clearly that bilateral CI improves the children's communicative behaviour, especially in complex listening situations. Children examined with the speech in noise test scored significantly better under the bilateral condition compared to the unilateral condition. Integration of the second implanted side and use of binaural information was observed to be easier and faster in children with a short time lag between both implants. CONCLUSIONS: To be able to obtain optimal benefit from bilateral cochlear implants, an intensive rehabilitation program is necessary. The important aspects of such a program are creating realistic expectations in older children before implantation; performing the first processor fitting of the second side with the first side switched on; and separate intensive training with the new system in order to balance out the hearing competence of the second CI with that of the first.

[The effects of quinine on active motile responses and fine structure of isolated outer hair cells from the Guinea pig cochlea]. / Der Einfluss von Chinin auf aktive Motilität und Feinstruktur isolierter äusserer Haarzellen der Meerschweinchenkochlea.

BACKGROUND: Large doses of quinine (as well as of salicylate) are known to produce reversible hearing loss and tinnitus. Cochlear outer hair cells seem to be the common site for the ototoxic effect of both drugs. METHODS: Isolated outer hair cells from the guinea pig cochlea were exposed to ototoxic doses of quinine hydrochloride (0.05-1.5 mmol/l). The cells were examined using tight-seal whole-cell recording techniques and transmission electron microscopy. RESULTS: Quinine exposure led to a hyperpolarization followed by a depolarization of the hair cells' membrane potential. It also caused a diminution of evoked rapid motile responses that was more apparent in response to hyperpolarizing than to depolarizing pulses. These effects were largely dose dependent and reversible. Ototoxic doses of quinine were not found to induce changes in turgor, shape or fine structure of outer hair cells such as those reported with ototoxic doses of salicylates in vitro. CONCLUSIONS: The present in vitro findings show that quinine (as well as salicylate) directly and reversibly affects cochlear outer hair cells. They also indicate that the underlying mechanisms of quinine ototoxicity are considerably different to that of salicylate although both substances clinically lead to identical symptoms.