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Fibrous dysplasia is a rare, benign disease even though the clinical picture may appear severe. It presents a progressive replacement of normal bone structures by fibrous tissue, can involve one or more bone segments and, in some cases, it could be associated with skin lesions and endocrinopathies (McCune-Albright syndrome). It manifests clinically with dysmorphic syndrome, ophthalmologic or otologic signs. We report three cases of temporal polyostotic fibrous dysplasia and we review the literature on this subject. [ABSTRACT FROM AUTHOR]

We conducted a comparison of three DPOAE protocols, testing cubic 2F[1] - F[2] distortion products, in order to define the most feasible protocol for a universal hearing screening programme. The protocols used asymmetrical stimulus intensities (L[1] > L[2] ) with a frequency ratio of 1.22, in the following format: (P1), L[1] = 60, L[2] = 50 dB SPL; (P2), L[1] = 65, L[2] = 55 dB SPL; and (P3), L[1] = 75, L[2] = 65 dB SPL. Linear TEOAE responses evoked by click stimuli of 75 dB p.e. SPL were used as controls of the normal cochlear function. Five 2F[1] - F[2] frequencies, 1.5, 2.0, 3.0, 4.0, 5.0 kHz, were tested with a ILO-92 macro subroutine. The project included randomly selected recordings from 1,200 well-baby nursery (WBN) infants (age 48 hours) and 50 very low birth weight NICU infants. Statistical analyses comparing the signal-to-noise ratios (S/N), at the predefined F[2] frequencies, indicated that the P1 and P2 DPOAE protocols perform similarly. Significant S/N differences were observed in the P3 to P2 and P3 to P1 data-set comparisons. DPOAE scoring criteria were estimated from the P3 data set, using one-sided distribution-free tolerance boundaries. The scoring criteria for a 'pass' were estimated as a minimum S/N of 6.0, 7.0 and 6.0 dB at 2.0, 3.0 and 4.0 kHz respectively. In terms of feasibility, the P3 protocol generated responses in 98% of the WBN and 76% of the NICU infants. All three DPOAE protocols demonstrated smaller time-recording requirements than the TEOAE standard. The false-positive ratio for the NICU infants was estimated as 8%. [ABSTRACT FROM AUTHOR]

Presents recommendations for the description of genetic and audiological data for families with nonsyndromic hereditary hearing impairment. Growth in the localisation and identification of genes for nonsyndromic hearing impairment; Nomenclature and localisation; Mutations that produce the phenotype.

Objective: Evaluation of the long-term safety and performance of an active middle ear implant (AMEI) in the treatment of hearing loss in children and adolescents with a primary focus on improvement in speech discrimination., Study Design: Prospective, multicentric, single-subject repeated-measures design in which each subject serves as his or her own control., Subjects: Thirty-one pediatric subjects aged 5 to 17 years., Intervention: Implantation of an active middle ear implant., Methods: Improvement in word recognition scores, speech reception thresholds (SRT) in quiet and noise, in addition to air conduction, bone conduction, and sound field thresholds were evaluated in two age groups., Results: Residual hearing did not change over time and speech intelligibility significantly improved and remained stable after 36 months. Children aged 5 to 9 improved in WRS from 21.92 to 95.38% and in SRT in quiet and in noise respectively from 62.45 dB SPL (sound pressure level) and +1.14 dB SNR to 42.07 dB SPL and -4.45 dB SNR. Adolescents aged 10 to 17 improved in WRS from 12.78 to 84.71% and in SRT in quiet and in noise respectively from 63.96 dB SPL and +3.32 dB SNR to 35.31 dB SPL and -4.55 dB SNR., Conclusions: The AMEI, under investigation, is a safe treatment for children and adolescents, and significantly improved audiological performance that remains stable on the long-term scale (up to 36 mo postimplantation). In general, all adult-related issues and questions regarding safety and performance can also be applied to the pediatric population, as no apparent specific issues developed.

Objective: Evaluation of safety and efficacy of the Vibrant Soundbridge in the treatment of hearing loss in children and adolescents with primary focus on improvement in speech discrimination., Study Design: Prospective, single-subject repeated-measures design in which each subject serves as his/her own control., Setting: Tertiary referral center., Patients: Nineteen patients aged 5 to 17 years., Intervention: Implantation of an active middle ear implant., Main Outcome Measure: Improvement in word recognition scores, speech reception thresholds, and signal-to-noise ratios (SNRs) were evaluated, in addition to air and bone conduction. Oldenburger Kids Satztest/Oldenburger Satztest sentences and Göttinger/Freiburger monosyllables at 65-dB hearing level were tested in two age groups., Results: Significant speech discrimination improvement was seen in all patients after 6 months. In children 5 to 9 years old, mean monosyllable recognition improved from 28.9% (unaided) to 95.5% (Soundbridge-aided). Aided 50% sentence discrimination at 44.1 dB and SNR of -4.9 dB were measured. In patients 10 to 17 years old, mean word recognition improved from 18.5% to 89.0%, sentence reception threshold improved to 40.2 dB, and SNR to -3.6 dB. Comparison between age groups indicated a slight trend toward quicker adaptation by older subjects. However, after initial adjustment, a higher level of overall benefit was seen at 6 months in younger children., Conclusions: Currently, the only middle ear implant approved for pediatric patients, the Vibrant Soundbridge, provides an option in cases of congenital aural atresia or disease-induced defects, when surgical intervention and reconstruction is indicated. The 6-month results in this comparatively large study population validated conclusions found in previous trials.

Objective: A "soft surgery" technique was applied, using various types of specifically designed dummy electrodes, to mimic cochlear implantation in a guinea pig model, and the degree of hearing-preservation/cochlear damage was assessed., Methods: Tricolor guinea pigs were divided into 3 groups: group A were implanted with electrodes without any contacts or wires (soft electrode), group B were implanted with electrodes having a metallic wire inside (stiff electrode), and group C underwent a cochleostomy procedure without implantation. Compound action potentials, in the range of 4 to 32 kHz, were used to assess electrophysiologic changes in the hearing function presurgery and postsurgery. Data were collected before surgery, at times t = 0 (immediately after surgery) and at 3, 7, 14, and 30 days., Results: At low frequencies (4-8 kHz), an immediate elevation of hearing threshold was observed in all 3 groups. Higher threshold shifts were more consistent for group B implanted with a stiff electrode, in comparison to the other 2 groups. Animals from group C presented a recovery from hearing loss, starting 3 days after surgery. At high frequencies (16-32 kHz), the elevation of hearing threshold was higher, as compared with the data from the low frequencies. Group C animals presented oscillatory threshold shifts twice, and the recovery to normal threshold values occurred approximately at t = 14 days., Conclusion: The data suggest that cochleostomy is minimally harmful to the inner ear and that a soft electrode might better preserve the inner ear integrity than a rigid electrode.

Objective: Active middle ear implants augment hearing in patients with sensorineural, conductive, and mixed hearing losses with great success. However, the application of active middle ear implants has been restricted to compromised ears in adults only. Recently, active middle ear implants have been successfully implanted in patients younger than 18 years of age with all types of hearing losses. The Vibrant Soundbridge (VSB) active middle ear implant has been implanted in more than 60 children and adolescents worldwide by the end of 2008. In October 2008, experts from the field with experience in this population met to discuss VSB implantation in patients below the age of 18., Methods: A consensus meeting was organized including a presentation session of cases from worldwide centers and a discussion session in which implantation, precautions, and alternative means of hearing augmentation were discussed. At the end of the meeting, a consensus statement was written by the participating experts. The present consensus paper describes the outcomes and medical/surgical complications: the outcomes are favourable in terms of hearing thresholds, speech intelligibility in quiet and in noise, with a low incidence of intra- and postoperative complications., Conclusions: Taken together, the VSB offers another viable treatment for children and adolescents with compromised hearing. However, other treatment options should also be taken into consideration. The advantages and disadvantages of all possible treatment options should be weighed against each other in the light of each individual case to provide the best solution; counseling should include a.o. surgical issues and MRI compatibility., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

Background: The aim of the study was to compare the estimation of hearing threshold values by behavioral and electrophysiological (ASSR) methods in subjects with normal hearing and those with sensorineural hearing impairment., Material/methods: Thirty-two subjects (17 male and 15 female) were tested, with a total of 61 ears. Of these, 11 (22 ears) presented normal hearing threshold values (0-19 dBHL) and 21 (39 ears) sensorineural deficits., Results: The data showed that for the normal-hearing subjects the mean +/-SD ASSR threshold was approximately 20+/-11 dB for frequencies of 0.25-1.0 kHz. For higher frequencies the ASSR threshold increased to 40+/-12.5 dB at 8.0 kHz. Regression analysis confirmed that the difference between the ASSR-estimated and behavioral threshold values decreased significantly with the amount of hearing loss. The data showed that for a 10-dB increment of the behavioral threshold, the ASSR threshold increased by 7 dB. The difference of about 27 dB between the two methods observed in normal subjects tends to cancel in hearing loss greater than 95-100 dBHL., Conclusions: The results of these analyses indicate that the threshold estimates are rather discordant with the behavioral thresholds. in particular it seems that the correction factor applied here does not rely on factors adequately modeled (in terms of instrumentation) to compensate for the effects of hearing loss on ASSR thresholds. The threshold estimation is adequately modeled for high levels of hearing loss, particularly for patients requiring a cochlear implant.

Introduction: Middle ear surgery is primarily concerned with resolving the discharging pathology, in the case of chronic otitis media (COM), or with complete eradication, in case of cholesteatoma. Either of these procedures may require repeated surgeries, often resulting in severe mixed hearing impairment. A middle ear implant may be indicated in these cases instead of a hearing aid because the anatomical conditions in such cases often impede an adequate acoustic coupling. The objective of this study was to evaluate MED-EL Vibrant Soundbridge (VSB) implantation in patients with severe conductive and mixed hearing loss occurring after middle ear surgery for cholesteatoma or chronic otitis media (COM)., Materials and Methods: Over a 2-years period, the VSB system was implanted in 40 patients between 35 and 81 year old (mean: 59.5). Surgery was performed with comparable technique in 3 regional hospitals in Italy: Rovereto (n=16), Meran (n=12) and Tortona (n=12). The 40 candidates for implantation had a history of 1-5 previous surgeries. Of those, 20 patients suffered from COM and 20 from, cholesteatomas. The floating mass transducer (FMT) of the VSB was placed and stabilized on the round window niche in 32 cases; alternative positioning was necessary in 8 cases. Bone conduction (BC) was tested 1 day post-operatively. At 1 month post-surgery and between 6-9 months, open-field warble tones threshold in VSB-off and VSB-on conditions and open-field speech audiometry for words in quiet were conducted., Results: Results of BC audiometry one day post-operatively showed no significant changes in hearing. Unaided mean pure tone average (PTA4) was 82.38 dB SPL with a mean speech recognition threshold (SRT) of 94.28 dB SPL. Results obtained after a minimum of three months post-operatively were evaluated in terms of aided thresholds and functional gain. At VSB activation, the mean PTA4 was 50.63 dB SPL with a mean SRT of 61.68 dB. After 6-9 months, the group had a mean PTA4 of 47.89 dB SPL and a mean SRT of 53.33 dB SPL., Conclusions: Implantation of the VSB with its direct driver of the inner ear fluids appears promising for auditory rehabilitation of severe mixed hearing loss associated with sequelae of cholesteatoma surgery. Patients' results improved over time, allowing us to assume a positive effect of consolidation of the coupling related to fibrosis. Results reported here refer to 6-9 months of observation and do not provide evidence of long term stability.

Objective: To describe our experience with the retrosigmoid-transmeatal (RS-TM) approach in auditory brainstem implantation (ABI) as well as the anatomosurgical guidelines for this route., Study Design: Retrospective case review., Setting: Ear, Nose, and Throat Department of the University of Verona., Patients: Five patients with neurofibromatosis type 2 (NF2) were operated on for vestibular schwannoma removal with ABI implantation from April 1997 to June 1999. The patients were four men and one woman, whose ages ranged from 22 to 37 years. The tumor sizes ranged from 12 to 30 mm. The records of a total of 179 patients operated on for vestibular schwannoma (VS) removal via the RS-TM approach from January 1990 to June 1999 were also evaluated. Their ages ranged from 18 to 88 years (average 54 years). The tumor sizes ranged from 4 to 50 mm. Five patients had a solitary VS in the only hearing ear., Intervention: The classic RS-TM approach was used in all patients. After tumor excision, for ABI implantation, the landmarks (seventh, eighth, and ninth cranial nerves, choroid plexus) for the foramen of Luschka were carefully identified. The choroid plexus was then partially removed, and the tela choroidea was divided and bent back. The floor of the lateral recess of the fourth ventricle and the convolution of the dorsal cochlear nucleus became visible. The electrode array was then inserted into the lateral recess and correctly positioned with the aid of electrically evoked auditory brainstem responses (EABRs)., Main Outcome Measures: Intraoperative EABR and postoperative speech perception evaluation., Results: Auditory sensations were induced in all patients with various numbers of electrodes. Different pitch sensations could be identified with different electrode stimulation., Conclusions: In the authors' experience, the RS-TM approach is the route of choice for patients who are candidates for ABI when there is a chance of hearing preservation during surgery. If auditory function is lost during surgery, anatomical preservation of the cochlear nerve may allow hearing restoration with a cochlear implant. Direct intraoperative recording of cochlear nerve action potentials (CNAPs) and round window electrical stimulation are mandatory for these purposes. In addition, decompression of the intrameatal portion of the vestibular schwannoma and planned partial tumor resection with hearing preservation are also possible with the RS-TM approach.

The middle fossa approach was used in 11 patients with profound bilateral hearing loss for insertion of a cochlear implant. Fibroadhesive otitis media (n = 1), bilateral cavity radical mastoidectomy (n = 1), autoimmune inner ear disease (n = 2), previous cranial trauma (n = 1), genetic prelingual deafness (n = 5), and otosclerosis (n = 1) were the causes of deafness. A cochleostomy was performed on the most superficial part of the basal turn, and the electrode array was inserted up to the cochlear apex. Speech perception tests (1-9 months after cochlear implant activation) yielded better results in these patients compared with a homogeneous group of postlingually deaf patients operated on through the traditional transmastoid route. Insertion of the implant through the middle fossa cochleostomy furnishes the possibility of stimulating areas of the cochlea (ie, the middle and apical turns) where a greater survival rate of spiral ganglion cells is known to occur, with improvement of information regarding the formants relevant for speech perception.

From April 1997 to December 1999, six patients (five men and one woman), ranging in age from 22 to 37 years with neurofibromatosis type 2 (NF2) were operated on via the classic retrosigmoid-transmental (RS-TM) approach for removal of a vestibular schwannoma (VS) (tumor size from 12 to 40 mm) and for auditory brain stem implantation (ABI). After tumor removal, the floor of the lateral recess of the fourth ventricle and the convolution of the dorsal cochlear nucleos were reached, and the ABI was inserted. More recently, an ABI was implanted via the retrosigmoid approach in a 4-year-old boy with a cochlear malformation (common cavity) associated with cochlear nerve aplasia. Electrically evoked auditory brain stem responses (EABRs) and neural response telemetry (NRT) were performed to verify the correct positioning of the inserted electrodes. No major complications related to ABI were observed. ABI has been activated to date in five of the NF2 patients. Auditory sensations with various numbers of electrodes were evoked in all patients. We consider the RS-TM approach the route of choice for ABI insertion in patients with NF2 and good hearing, offering a chance of hearing preservation, and in patients with complete cochlear ossification, severe head trauma and cochlear fracture, or nerve disruption, or a combination of these. A new indication for ABI implantation via the RS approach is presented by patients with bilateral cochlear nerve aplasia.

The present paper reports our experience with the surgical retrosigmoid-transmastoid (RS-TM) technique for implanting auditory brainstem implants (ABIs). From April 1997 to August 1998, four patients with neurofibromatosis type 2 (NF2) were operated on for vestibular schwannoma removal with ABI implantation. The subjects (three men and one women) ranged in age from 22 to 31 years. Tumour size ranged from 12 to 30 mm. A classical RS-TM approach was performed. After tumour excision, identification of landmarks (VIIth, VIIIth and IXth cranial nerves, choroid plexus) to the foramen of Luschka was carefully carried out. The choroid plexus was partially removed and the tela choroidea divided and deflected. The floor of the lateral recess of the fourth ventricle and the convolution of the dorsal cochlear nucleus became visible. The electrode array was then inserted into the lateral recess and placed in the correct position with the help of electrically-evoked auditory brain stem responses. Auditory sensations were induced in all patients with various numbers of electrodes. Different pitch sensations could be identified with different electrode stimulation. Details of the results are presented. In our series, the RS-TM approach represents the elective route for ABI insertion.

The present article reports on our experience with hearing preservation during 158 acoustic neuroma (AN) operations via the retrosigmoid-transmeatal (RS-TM) approach with the aid of intraoperative auditory monitoring. Several auditory monitoring methods are described. Of these, the bipolar cochlear nerve action potential (CNAP) was found to be the most helpful in preserving hearing. Of 106 patients with useful hearing preoperatively, more than 50% had useful hearing after surgery. Electrical auditory brainstem responses were useful in the placement of an auditory brain stem implant (ABI) in 4 patients with neurofibromatosis type 2 (NF2). All 4 reported speech perception benefit and use their ABIs regularly in their lives. It is our firm belief that intraoperative auditory monitoring has a pivotal role in the preservation and restoration of hearing in AN surgery.

The preliminary results of insertion of a cochlear implant via the middle fossa in nine patients with profound bilateral hearing loss are described. Aetiologies included a bilateral radical mastoidectomy cavity, adhesive otitis media, autoimmune inner ear disease, previous cranial trauma, genetic pre-lingual deafness, and otosclerosis. A classic middle fossa approach was adopted. A small cochleostomy measuring 1.5 mm in diameter was performed on the most superficial part of the basal turn. A Nucleus 24M cochlear implant system (Cochlear Corporation) was inserted in four patients, a Lauraflex implant (Philips Hearing Implants) was used in three patients and a Combi 40+ (Med-el) with a double electrode array in two. Single electrode arrays were inserted from the cochleostomy to the cochlear apex and occupied a portion of the basal turn, as well as the middle and apical turns. Double electrode arrays were inserted, one towards the apex and one into the basal turn of the cochlea towards the round window. The receiver stimulator was positioned in a bone well previously drilled in the temporal squama and the electrode carrier was inserted in the fenestrated cochlea. The activity of the inserted electrodes was tested by means of telemetry and intraoperative recording of electrically evoked auditory brainstem responses (EABR). Speech recognition tests, performed over a period of time ranging from one to six months after cochlear implant activation, yielded better results in these patients compared with those obtained in postlingually deaf patients operated on via the traditional transmastoid route. Cochlear implant insertion via the middle fossa approach is a technique which is suitable for the implantation of patients with bilateral radical mastoidectomy cavities, chronic middle ear disease, middle ear malformations, or with partial obliteration of the cochlea in the basal turn. However, the main advantage of inserting the implant through the middle fossa cochleostomy consists in the possibility of stimulating, with the single array, areas of the cochlea, i.e. part of the basal, middle and apical turns, where a greater survival rate of spiral ganglion cells is known to occur. In addition, with the double array total occupation of the cochlea is possible, providing the possibility of replicating the tonotopic organization of the cochlea. This new approach has led to major improvements in speech recognition in all patients compared with patients operated on via the transmastoid approach and, given the present state of the art, may be the elective approach for optimal implantation outcomes.