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Contains fulltext : 70417.pdf (Publisher’s version ) (Closed access) OBJECTIVE: To study the audiologic outcome of bone-anchored hearing aid (BAHA) application in patients with congenital unilateral conductive hearing impairment. STUDY DESIGN: Prospective audiometric evaluation on 20 patients. SETTING: Tertiary referral center. PATIENTS: The experimental group comprised 20 consecutive patients with congenital unilateral conductive hearing impairment, with a mean air-bone gap of 50 dB. METHODS: Aided and unaided hearing was assessed using sound localization and speech recognition-in-noise tests. RESULTS: Aided hearing thresholds and aided speech perception thresholds were measured to verify the effect of the BAHA system on the hearing acuity. All patients fulfilled the criteria that the aided speech reception thresholds or the mean aided sound field thresholds were 25 dB or better in the aided situation. Most patients were still using the BAHA almost every day. Sound localization scores varied widely in the unaided and aided situations. Many patients showed unexpectedly good unaided performance. However, nonsignificant improvements of 3.0 (500 Hz) and 6.9 degrees (3,000 Hz) were observed in favor of the BAHA. Speech recognition in noise with spatially separated speech and noise sources also improved after BAHA implantation, but not significantly. CONCLUSION: Some patients with congenital unilateral conductive hearing impairment had such good directional hearing and speech-in-noise scores in the unaided situation that no overall significant improvement occurred after BAHA fitting in our setup. Of the 18 patients with a complete data set, 6 did not show any significant improvement at all. However, compliance with BAHA use in this patient group was remarkably high. Observations of consistent use of the device are highly suggestive of patient benefit. Further research is recommended to get more insight into these findings.

Contains fulltext : 70980.pdf (Publisher’s version ) (Closed access) OBJECTIVE: Evaluation of the management and survival of patients treated for temporal bone squamous cell carcinoma. STUDY DESIGN: A retrospective analysis. SETTING: Tertiary care, academic referral center. PATIENTS: Twenty-eight patients underwent primary treatment for squamous cell carcinoma of the temporal bone. INTERVENTIONS: The patients were staged using the modified Pittsburgh staging system. Patients underwent a local resection, lateral temporal bone resection, or a subtotal lateral temporal bone resection usually followed by radiotherapy. MAIN OUTCOME MEASURE: The survival rate of patients grouped by tumor size was calculated. RESULTS: Staging revealed 12 pT1, 2 pT2, 4 pT3, and 10 pT4 tumors. The mean follow-up was 34 months (2-132 mo). The Kaplan-Meier survival curves showed survival rates at 5 years of 83 and 25% for the stages pT1 and pT4, respectively. The pooled survival curves showed survival rates at 5 years of 85 and 46% for the stages pT1p/T2 and pT3/pT4, respectively. CONCLUSION: Long-term prognosis of the carcinoma of the external auditory canal mainly depends on the stage and primary treatment. Surgery may consist of a lateral temporal bone or subtotal temporal bone resection; in T3 and T4 tumors, resection may be combined with a superficial parotidectomy. If disease is diagnosed in the neck or parotid, then a neck dissection and total parotidectomy may also be performed. Additional radiotherapy should be provided in incompletely resected T1 and all T2 and T3 tumors and part of the T4 tumors. T4 tumors may be treated according to their subclassification based on the anatomic extension.

Contains fulltext : 50720.pdf (Publisher’s version ) (Closed access) OBJECTIVES: To report hearing impairment and vestibular and ocular features in a Dutch DFNA11 family and to compare these results to reported data on three other DFNA11 families. STUDY DESIGN: Family study. METHODS: Regression analysis was performed in relation to age to outline the development of hearing thresholds and speech recognition scores. Vestibular and ocular functions were examined. RESULTS: First symptoms of hearing impairment started between the ages of 4 and 43 years. Most of the audiograms were symmetric and flat or downsloping. The annual threshold deterioration increased from 0.2 to 2.6 dB per year at 0.25 to 8 kHz in the longitudinal analyses and in the cross-sectional analysis from 0.3 to 0.9 dB per year. The speech recognition score was quite good, deteriorating by 0.9% per year from a 90% score at the age of 36 years onward. Remarkably, extensive ocular examination including corrected visual acuity and refraction measurements, slit-lamp examination, ophthalmoscopy, Goldmann perimetry, electroretinography and electro-oculography revealed signs of subclinical retinal dysfunction. None of the patients showed the classic triad of retinitis pigmentosa. Pure-tone thresholds, phoneme recognition scores, and vestibular responses of the mutation carriers were fairly similar to previously described DFNA11 families. CONCLUSION: Even though the diverse mutations are located in different regions of the myosin VIIa gene, the cochleovestibular phenotype is fairly similar in all DFNA11 families. Surprisingly, only in this family was subclinical retinal dysfunction detected.

Contains fulltext : 47623.pdf (Publisher’s version ) (Closed access) OBJECTIVES: To perform genetic analysis and to analyze cochleovestibular impairment features in a newly identified Dutch family with nonsyndromic autosomal dominant hearing impairment (DFNA9). STUDY DESIGN: Genetic analysis was performed using microsatellite markers and single nucleotide polymorphisms. Audiometric data were collected and analyzed longitudinally. Results were compared with those obtained in previously identified P51S COCH mutation carriers (n = 74). Special attention was also given to a comparison of age-related features such as progressive hearing loss and vestibular impairment. SETTING: Tertiary referral center. PATIENTS: G88E COCH mutation carriers from a Dutch family. MAIN OUTCOME MEASURES: The study of clinical features of a DFNA9 family carrying a G88E COCH mutation and to compare this to the symptoms of those carrying a P51S/COCH mutation. RESULTS: Pure-tone thresholds, phoneme recognition scores, and vestibular responses of the G88E mutation carriers were essentially similar to those previously established in the P51S mutation carriers. Hearing started to deteriorate in G88E mutation carriers from age 46 to 49 years and onward, whereas deterioration of vestibular function started from approximately age 46 years. In the P51S mutation carriers, vestibular impairment started earlier, at approximately age 34 years. However, the difference in age of onset with the G88E mutation carriers was not significant. Remarkably, the proportion of patients who developed complete vestibular areflexia within the age range of 40 to 56 years was significantly lower for the G88E mutation carriers than for the P51S mutation carriers. CONCLUSION: Apart from a significantly lower frequency of vestibular areflexia between the ages of 40 and 56 years, there are no phenotypic differences between carriers of the G88E and P51S mutations in the COCH gene.

Contains fulltext : 47349.pdf (Publisher’s version ) (Closed access) OBJECTIVE: To study the effect of two multipolar electrode configurations on speech perception, pitch perception, and the intracochlear electrical field. STUDY DESIGN: Crossover design; within subject. SETTING: Tertiary referral center. PATIENTS: Eight experienced adult cochlear implant users. INTERVENTION: Each subject used each of three experimental processors for 3 weeks. The following processors were compared that differed only in electrode configuration: 1) monopolar; 2) hybrid quadrupolar, in which half of the current returned to the extracochlear reference electrode and half to two electrodes immediately to the left and right of the active electrode; and 3) flat tripolar +2, which directed all the current to four reference electrodes (two on each side), separated from the active electrode by two inactive electrodes. All the processors used the standard Advanced Bionics HiRes speech-processing strategy, 12 channels, 1,220 pulses per second per channel, and with a pulse width of 33 (micros/phase). RESULTS: The monopolar processors had the largest stimulation efficiency and the smallest dynamic range in linear current units. The reverse was true of flat tripolar +2 processor, whereas the hybrid quadrupolar processor fell in between. Insufficient loudness growth prevented the use of the flat tripolar +2 processor in three subjects. Word recognition did not differ between the clinically used 16-channel monopolar processor and the experimental monopolar processor, regardless of the differences in the number of channels, pulse rate, and duration of experience. Word recognition with the flat tripolar +2 processor was significantly poorer than with the monopolar and hybrid quadrupolar processors; monopolar and quadrupolar processors did not differ. There was no significant interaction between processor type and competing noise type (stationary or fluctuating), but performance at the higher level of fluctuating noise was best with the hybrid quadrupolar processor in almost all the subjects. Pitch scaling showed ceiling performance in five subjects and differed between processors in the two other subjects with imperfect tonotopy. Intracochlear current spread was considerable with the monopolar configuration; it was reduced with the hybrid quadrupolar configuration and virtually absent beyond the active electrodes with the tripolar configuration. CONCLUSION: More confined configurations reduced the longitudinal width of the electrical field, which was expected to enhance channel separation, but no improvement in word recognition was found. More research is needed to test confined configurations that have enhanced efficiency and to evaluate the fundamental effects of configuration on channel discriminability.

Contains fulltext : 48927.pdf (Publisher’s version ) (Closed access) OBJECTIVE: To present the course of tympanic membrane pathology in childhood and young adulthood after otitis media (OM) in early life. STUDY DESIGN: Prospective follow-up study. SETTING: Community study of a birth cohort. PATIENTS: Three hundred fifty-eight subjects with a positive and negative history of OM (OM+ or OM-) or ventilation tube insertion (VT+ or VT-) derived from a birth cohort that had been followed-up from preschool to adult age. METHODS: Standardized otomicroscopic examination performed at ages 8 and 18 years. MAIN OUTCOME MEASURES: Tympanic membrane abnormalities (i.e., tympanosclerosis, atrophy, atelectasis and retraction pockets of the pars tensa, and retraction of the pars flaccida). RESULTS: At the age of 8 years, tympanic membrane pathology was highly prevalent in the both OM+ subcohorts (OM+VT+, 92% and OM+VT-, 46%), whereas in the OM- ears (11%), tympanic membrane abnormalities were rare. In the subsequent 10-year period, many tympanic membrane abnormalities disappeared spontaneously, although the prevalence of tympanosclerosis remained substantial in the OM+VT+ cohort. CONCLUSION: The natural course of most tympanic membrane pathology associated with OM in early life is favorable over time, suggesting an intrinsic repair capacity of the tympanic membrane. Tympanosclerosis, the most prevalent sequelae of OM and treatment with VT, however, shows little tendency of resolution.

Objective Disturbance of any of the ET functions may contribute to the development of otitis media. Sonotubometry measures the ventilatory function using sound. The qualities of sonotubometry as a test for eustachian tube ventilatory function have been studied by various investigators. The development of the method is described in the review, and a summary of the study results is provided to make an estimate of the diagnostic potential of this eustachian tube function test. Data sources The English-language literature on the topic was searched systematically by Medline and Pubmed using the following key words: ventilatory function, eustachian tube, sonotubometry, and function test. There were no limits for the year of publication. Study selection Articles that described the method itself (validity, reproducibility, diagnostic value) were studied in detail. Data extraction All the articles described in study selection were used for this review. Conclusions The technique of sonotubometry has been improved gradually over the years. The results of sonotubometry are at least as good as those of other function tests. However, because the results still tend to be ambiguous in children and otitis media is most common in this population, the reproducibility and application of sonotubometry must be evaluated further. Sonotubometry has great advantages over other function tests, but it is not used routinely to assess eustachian tube ventilatory function because its value for clinical practice has not yet been adequately demonstrated. The review showed that sonotubometry can be improved further and that efforts to do so seem justified because it forms a particularly promising method to assess eustachian tube function in children with suspected eustachian tube disease.

Contains fulltext : 48452.pdf (Publisher’s version ) (Closed access) OBJECTIVE: To evaluate the benefit of a bone-anchored hearing aid contralateral routing of sound hearing aid (BAHA CROS hearing aid) in 29 patients with unilateral inner ear deafness. STUDY DESIGN: Prospective clinical follow-up study. SETTING: Tertiary referral center. PATIENTS: Thirty patients were recruited. There were 19 patients with a history of acoustic neuroma surgery and 11 patients with unilateral inner ear deafness due to other causes; 1 patient was excluded. The first 21 patients had also participated in a previous evaluation. INTERVENTION: Audiometric measurements were taken before intervention, when fitted with a conventional CROS, and after BAHA implementation. Patients' subjective benefit was quantified with four different hearing aid-specific instruments: the Abbreviated Profile of Hearing Aid Benefit, the Glasgow Hearing Aid Benefit Profile, the International Outcome Inventory for Hearing Aids, and the Single-Sided Deafness questionnaire. MAIN OUTCOME MEASURES: The same instruments were used at a mean long-term follow-up of 1 year after BAHA implantation. RESULTS: Sound localization in an audiologic test setting was no different from chance level. The main effect of the BAHA CROS that was found was the "lift the head shadow" effect in the speech-in-noise measurements. All instruments also showed positive results in favor of the BAHA CROS at long-term follow-up. CONCLUSIONS: The poor sound-localization results in an audiologic test setting illustrated the inability of patients with unilateral inner ear deafness to localize sounds. The speech-in-noise measurements demonstrated the efficacy of the BAHA CROS to lift the head shadow. Patients were still satisfied at 1-year follow-up, according to the four instruments.

Hypothesis and Background: The Eustachian tube has three important functions with respect to the middle ear: ventilation, clearance, and protection. Surfactants are assumed to be important to maintain these functions. The administration of exogenous surfactant may therefore be effective to improve the function of the Eustachian tube. This randomized, doubleblind, placebo-controlled study was designed to investigate the effect of exogenous surfactant on the function of the Eustachian tube in rats. Materials and Methods: Exogenous surfactant was administered into the middle ear of 10 otologically healthy rats, and 10 other rats received placebo. The effect on the opening and closing pressure (passive ventilatory function) and the dye clearance time (clearance function) of the rat’s Eustachian tube was measured. Results: A significant decrease in the opening pressure was seen after the administration of surfactant. Both surfactant and placebo caused an increase in the closing pressure. A serious disturbance of the dye clearance time was induced in 13 rats, and the test failed in 1 rat. In the remaining 6 rats, no significant differences in the dye clearance time were observed between the two groups. Conclusions: Exogenous surfactant decreased the closing forces of the Eustachian tube even in otologically healthy rats. No significant effect on the mucociliary clearance was observed, but this may have resulted from the small number of rats. Additional randomized, double-blind, placebo-controlled trials should be conducted to determine the clinical relevance of these changes and to further assess the effect of surfactant on the function of the Eustachian tube. Key Words: Surfactant— Eustachian tube—Ventilatory function—Clearance function. Otol Neurotol 24:6–10, 2003.

Objective: To evaluate the results of stapes surgery in osteogenesis imperfecta. Study Design: Audiological and clinical analysis of the results of stapes surgery in a consecutive series of 15 ears in 13 patients with osteogenesis imperfecta. Setting: Tertiary referral center. Results: Stapedotomy was successful in 11 of 12 ears in 11 patients with osteogenesis imperfecta type I. Air-bone gaps were within 10 dB in four ears and within 20 dB in the seven remaining ears. In one ear, revision surgery with malleovestibulopexy was needed to achieve this result. In another ear, the initial good result within 20-dB air-bone gap decreased to 28 dB; therefore, it is not considered as a success. In one other patient, revision surgery resulted in only marginal improvement in the hearing level. In one case of, probably, osteogenesis imperfecta type III, surgery resulted in a dead ear. Conclusion: Stapedotomy successfully improved the hearing of patients with osteogenesis type I. These results are in accordance with those reported in a few previous series. It is questionable whether stapes surgery will be as successful in osteogenesis imperfecta types other than type I. Stapedotomy-Osteogenesis imperfecta-Genetic hearing impairment-COLIA1 gene.