Surgery for Port-Wine Stains: A Systematic Review.

BACKGROUND: Port-wine stains (PWS) are congenital low-flow vascular malformations of the skin. PWS tend to become thicker and darker with time. Laser therapy is the gold standard and the first-line therapy for treating PWS. However, some resistant PWS, or PWS that have tissue hypertrophy, do not respond to this therapy. Our aim is to evaluate the role of surgery in the treatment of PWS birthmarks. METHODS: A literature search was performed in PubMed, Scopus, Web of Science (WOS) and Google Scholar for all papers dealing with surgery for port-wine stains, from January 2010 to December 2020 using the search strings: (capillary vascular malformation OR port-wine stains OR Sturge Weber Syndrome OR sws OR pws) AND (surgical OR surgery). RESULTS: Ten articles were identified and used for analysis. They were almost all case series with a short follow up period and lacked an objective-systematic score of evaluation. CONCLUSIONS: Delay in treatment of port wine stains may result in soft tissue and bone hypertrophy or nodules with disfiguring or destructive characteristics. The correction of PWS-related facial asymmetry often requires bone surgery followed by soft tissue corrections to achieve a more harmonious, predictable result.

A Retrospective Evaluation to Assess Reliability of Electrophysiological Methods for Diagnosis of Hearing Loss in Infants.

Background: An electrophysiological investigation with auditory brainstem response (ABR), round window electrocochleography (RW-ECoG), and electrical-ABR (E-ABR) was performed in children with suspected hearing loss with the purpose of early diagnosis and treatment. The effectiveness of the electrophysiological measures as diagnostic tools was assessed in this study. Methods: In this retrospective case series with chart review, 790 children below 3 years of age with suspected profound hearing loss were tested with impedance audiometry and underwent electrophysiological investigation (ABR, RW-ECoG, and E-ABR). All implanted cases underwent pure-tone audiometry (PTA) of the non-implanted ear at least 5 years after surgery for a long-term assessment of the reliability of the protocol. Results: Two hundred and fourteen children showed bilateral severe-to-profound hearing loss. In 56 children with either ABR thresholds between 70 and 90 dB nHL or no response, RW-ECoG showed thresholds below 70 dB nHL. In the 21 infants with bilateral profound sensorineural hearing loss receiving a unilateral cochlear implant, no statistically significant differences were found in auditory thresholds in the non-implanted ear between electrophysiological measures and PTA at the last follow-up (p > 0.05). Eight implanted children showed residual hearing below 2000 Hz worse than 100 dB nHL and 2 children showed pantonal residual hearing worse than 100 dB nHL (p > 0.05). Conclusion: The audiological evaluation of infants with a comprehensive protocol is highly reliable. RW-ECoG provided a better definition of hearing thresholds, while E-ABR added useful information in cases of auditory nerve deficiency.

Effects of rapid maxillary expansion on hearing loss and otitis media in cleft palate children.

PURPOSE: Otitis media with effusion (OME), recurrent acute otitis media and conductive hearing loss (CHL) are significantly prevalent in children with cleft palate (CP) and cleft lip and palate (CLP). Rapid Maxillary Expansion (RME) appears to have a positive effect also on middle ear disorders in these patients. The study aims to offer a prospective evaluation of RME effects in a group of patients with CP/CLP in terms of OME, CHL. METHODS: A prospective observational study was conducted. Thirty-four CP, CLP and submucosa cleft patients who received orthodontic indication to RME treatment for OME or conductive hearing loss in a tertiary institutional Care Unit of San Paolo Hospital, Milan (IT), were included. Twenty-two patients matched for age and with analogous inclusion criteria except for indication to RME treatment were enrolled in the control group. Clinical otolaryngological evaluation, pure tone audiometry and tympanogram were performed at the beginning of treatment (T0), at the end of the expansion (T1) and at 6-month follow-up (T2). MAIN OUTCOME MEASURES: Air-bone gaps and tympanogram results at each time interval. RESULTS: In the main group, RME allowed a statistically significant improvement of air-bone gaps (according to frequency, p < 0.001-0.089 T0 vs. T1 and < 0.001-0.044 T0 vs. T2, Friedman's test) and tympanometry results (p = 0.002 T0 vs. T1 and p < 0.001 T0 vs. T2, Friedman's test). Improvements were stable during follow-up and were significantly better in the main group than in the control group. CONCLUSION: CHL and middle ear effusion improved significantly overtime during RME and after 6 months of follow-up.

Resorbable Mesh Cranioplasty Repair of Bilateral Cerebrospinal Fluid Leaks Following Pediatric Simultaneous Bilateral Auditory Brainstem Implant Surgery.

OBJECTIVE: To present a child with cochlear nerve deficiency (CND) who received simultaneous bilateral simultaneous auditory brainstem implants (BS-ABI) and subsequently presented with bilateral cerebrospinal fluid (CSF) leaks unresponsive to standard treatments. To propose a novel rigid retrosigmoid cranioplasty for treating and preventing CSF leaks in children at high risk for this complication. PATIENT: A 3.5-year-old child with CND, vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities, coloboma, heart defect, atresia choanae, retarded growth and development, genital abnormality, and ear abnormality, Arnold Chiari malformation, previous treated tracheo-esophageal fistula underwent BS-ABI. Postoperatively, the child had recurrent bilateral retroauricular fluid collections. A standard revision procedure revealed breaches in the dural closure, migration of the auditory brainstem implantation (ABI) receiver stimulator on both sides and was unsuccessful in stopping the leak. INTERVENTIONS: Bilateral repair with free fat grafting filling the craniectomy space and two absorbable meshes of poly-L-D-lactic (PLDL) acid stabilized with PLDL pins on the surrounding cranium, one to stabilize the fat graft and one to fix the ABI receiver stimulators inside the subperiosteal pockets. MAIN OUTCOME MEASURE: CSF leak recurrence, postoperative computed tomographic (CT) scans, intra- and postoperative simultaneous electrically evoked auditory brainstem responses (EABRs). Subjective and objective assessment of ABI function. RESULTS: No postoperative CSF leaks at 60 days follow-up. EABRs and consistent behavioral responses obtained at initial mapping on both sides. CONCLUSIONS: The use of BS-ABI likely contributed to bilateral CSF leaks requiring revision surgeries in this child. Simultaneous bilateral craniotomies can put patients at risk for CSF leak. A novel cranioplasty technique employed finally proved successful in stopping the CSF leak in this case.

Nervus Intermedius Guides Auditory Brainstem Implant Surgery in Children with Cochlear Nerve Deficiency.

OBJECTIVE: To investigate the anatomic features of the nervus intermedius and cranial nerve VII in children with cochlear nerve deficiency and to verify whether the nervus intermedius can provide an additional landmark to help guide placement of the auditory brainstem implant electrode. STUDY DESIGN: Case series with chart review. SETTING: Tertiary referral center. SUBJECTS AND METHODS: High-definition video was captured during retrosigmoid surgery in 64 children (mean age, 3.91 ± 2.83 years) undergoing auditory brainstem implant placement. These videos were examined with particular reference to the number and variety of nervus intermedius bundles and any associated facial nerve anomalies. RESULTS: Absence of cranial nerves VI, VII, and VIII was observed in 3, 6, and all 64 children, respectively. Fifteen children had several abnormalities of the facial nerve in the cerebellopontine angle. Anatomic identification of the facial nerve and the bundles composing the nervus intermedius was possible in 46 children. In 12 children, identification was possible with the assistance of intraoperative monitoring. The number of bundles composing the nervus intermedius varied from 1 to 6. The nervus intermedius and cranial nerve IX were useful landmarks for identifying the foramen of Luschka of the lateral recess. CONCLUSION: The nervus intermedius provides an additional landmark during auditory brainstem microsurgery since it was identified in all subjects. The nervus intermedius anatomy and its topographic relationship with the neurovascular structures around the foramen of Luschka have been described for the first time in children with cochlear nerve deficiency.

The development of auditory perception in children after auditory brainstem implantation.

Auditory brainstem implants (ABIs) can provide useful auditory perception and language development in deaf children who are not able to use a cochlear implant (CI). We prospectively followed up a consecutive group of 64 deaf children up to 12 years following ABI surgery. The etiology of deafness in these children was: cochlear nerve aplasia in 49, auditory neuropathy in 1, cochlear malformations in 8, bilateral cochlear postmeningitic ossification in 3, neurofibromatosis type 2 in 2, and bilateral cochlear fractures due to a head injury in 1. Thirty-five children had other congenital nonauditory disabilities. Twenty-two children had previous CIs with no benefit. Fifty-eight children were fitted with the Cochlear 24 ABI device and 6 with the MedEl ABI device, and all children followed the same rehabilitation program. Auditory perceptual abilities were evaluated on the Categories of Auditory Performance (CAP) scale. No child was lost to follow-up, and there were no exclusions from the study. All children showed significant improvement in auditory perception with implant experience. Seven children (11%) were able to achieve the highest score on the CAP test; they were able to converse on the telephone within 3 years of implantation. Twenty children (31.3%) achieved open set speech recognition (CAP score of 5 or greater) and 30 (46.9%) achieved a CAP level of 4 or greater. Of the 29 children without nonauditory disabilities, 18 (62%) achieved a CAP score of 5 or greater with the ABI. All children showed continued improvements in auditory skills over time. The long-term results of ABI surgery reveal significant auditory benefit in most children, and open set auditory recognition in many.

Improved outcomes in auditory brainstem implantation with the use of near-field electrical compound action potentials.

OBJECTIVE: To compare the outcomes (auditory threshold and open-set speech perception at 48-month follow-up) of a new near-field monitoring procedure, electrical compound action potential, on positioning the auditory brainstem implant electrode array on the surface of the cochlear nuclei versus the traditional far-field electrical auditory brainstem response. STUDY DESIGN: Retrospective study. SETTING: Tertiary referral center. SUBJECTS AND METHODS: Among the 202 patients with auditory brainstem implants fitted and monitored with electrical auditory brainstem response during implant fitting, 9 also underwent electrical compound action potential recording. These subjects were matched retrospectively with a control group of 9 patients in whom only the electrical auditory brainstem response was recorded. Electrical compound action potentials were obtained using a cotton-wick recording electrode located near the surface of the cochlear nuclei and on several cranial nerves. RESULTS: Significantly lower potential thresholds were observed with the recording electrode located on the cochlear nuclei surface compared with the electrical auditory brainstem response (104.4 ± 32.5 vs 158.9 ± 24.2, P = .0030). Electrical brainstem response and compound action potentials identified effects on the neighboring cranial nerves on 3.2 ± 2.4 and 7.8 ± 3.2 electrodes, respectively (P = .0034). Open-set speech perception outcomes at 48-month follow-up had improved significantly in the near- versus far-field recording groups (78.9% versus 56.7%; P = .0051). CONCLUSIONS: Electrical compound action potentials during auditory brainstem implantation significantly improved the definition of the potential threshold and the number of auditory and extra-auditory waves generated. It led to the best coupling between the electrode array and cochlear nuclei, significantly improving the overall open-set speech perception.

The Therapeutic Dilemma of Cochlear Nerve Deficiency: Cochlear or Brainstem Implantation?

OBJECTIVE: To compare the outcomes between 2 age-matched cohorts of children with cochlear nerve deficiency: those receiving auditory brainstem implants (group A) or cochlear implants (group B). STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary referral center. SUBJECTS AND METHODS: Subjects were selected from a pool of 537 children fitted with cochlear implants (n = 443) or auditory brainstem implants (n = 94) over the past 14 years. Performance, examined with the Category of Auditory Performance scale, and complications were compared with a mean follow-up of 5 years. RESULTS: All children had bilateral profound sensorineural hearing loss and cochlear nerve deficiency. Magnetic resonance imaging documented an absent cochlear nerve (n = 12) and a small cochlear nerve (n = 8) in group A and an absent cochlear nerve (n = 11) and a small cochlear nerve (n = 9) in group B (P = 1.000). Children with cochlear implants had Category of Auditory Performance scores spanning from 0 to 3 levels of performance, and all required manual communication mode and visual supplementation. Children with auditory brainstem implants had Category of Auditory Performance scores spanning from 2 to 7, and most patients demonstrated behavioral responses irrespective of inner ear malformations and an absent cochlear nerve or small cochlear nerve (P < .001). CONCLUSIONS: In children with cochlear nerve deficiency, patients fitted with cochlear implants did not develop speech understanding and production. Those fitted with auditory brainstem implants had the opportunity to develop open-set speech perception, acquiring verbal language competence using oral communication exclusively and participating in mainstream education. The overall complication rate of auditory brainstem implants was not greater than that of cochlear implants.

Vestibulotomy with ossiculoplasty versus round window vibroplasty procedure in children with oval window aplasia.

OBJECTIVE: To review the surgical procedures and outcomes in children with bilateral oval window aplasia (OWA). STUDY DESIGN: Retrospective cohort review. SETTING: Tertiary referral center. PATIENTS: Children suffering from OWA between 1990 and 2010. INTERVENTION: Vestibulotomy with ossiculoplasty (V-OPL) or round window vibroplasty (RWV). MAIN OUTCOME MEASURES: Findings at radiology and surgery, preoperative and postoperative bone conduction (BC), air conduction (AC), and RWV-air conduction (RWV-AC) thresholds and speech discrimination scores (SDSs). RESULTS: Among 23 children, 11 underwent V-OPL and 8 RWV. Four children in the V-OPL group had aborted surgery and were excluded from the study. In all the remaining 19 children, the 6-month follow-up time showed postoperative AC and SDS values significantly better than the preoperative thresholds in both groups. At the 36-month long-term follow-up, AC and SDS were stable in the RWV group but showed a significant worsening in the V-OPL children compared with the 6-month follow-up results. Preoperative versus postoperative BC values showed a significant difference between the 2 groups at 36 months; 5 of the V-OPL group underwent revision following the same surgical principles, which did not result in improved outcome. CONCLUSION: In children with OWA, V-OPL provides modest long-term results and carries higher risks of BC degradation compared to RWV. Both procedures are technically challenging but considering the respective hearing results and morbidity of primary and revision surgery, we have abandoned the V-OPL procedure in favor of RWV. In infants and children younger than 5 years with OWA previously not considered candidates for hearing restoration, we consider RWV as the first-choice surgery. It has shown to provide significantly better hearing outcomes than traditional atresia surgery with minimal complication rate.

Effect of Bluetooth headset and mobile phone electromagnetic fields on the human auditory nerve.

OBJECTIVES/HYPOTHESIS: The possibility that long-term mobile phone use increases the incidence of astrocytoma, glioma and acoustic neuroma has been investigated in several studies. Recently, our group showed that direct exposure (in a surgical setting) to cell phone electromagnetic fields (EMFs) induces deterioration of auditory evoked cochlear nerve compound action potential (CNAP) in humans. To verify whether the use of Bluetooth devices reduces these effects, we conducted the present study with the same experimental protocol. STUDY DESIGN: Randomized trial. METHODS: Twelve patients underwent retrosigmoid vestibular neurectomy to treat definite unilateral Ménière's disease while being monitored with acoustically evoked CNAPs to assess direct mobile phone exposure or alternatively the EMF effects of Bluetooth headsets. RESULTS: We found no short-term effects of Bluetooth EMFs on the auditory nervous structures, whereas direct mobile phone EMF exposure confirmed a significant decrease in CNAPs amplitude and an increase in latency in all subjects. CONCLUSIONS: The outcomes of the present study show that, contrary to the finding that the latency and amplitude of CNAPs are very sensitive to EMFs produced by the tested mobile phone, the EMFs produced by a common Bluetooth device do not induce any significant change in cochlear nerve activity. The conditions of exposure, therefore, differ from those of everyday life, in which various biological tissues may reduce the EMF affecting the cochlear nerve. Nevertheless, these novel findings may have important safety implications.

Auditory brainstem implantation after unsuccessful cochlear implantation of children with clinical diagnosis of cochlear nerve deficiency.

OBJECTIVES: We compared the perceptual auditory abilities of 21 children with suspected cochlear nerve deficiency (CND) and a surgically verified absent cochlear nerve (CN) who first underwent cochlear implantation (CI) and subsequently underwent auditory brainstem implantation (ABI). METHODS: In this retrospective cohort study, from 2000 to 2011, 21 children initially underwent CI at an outside institution and failed to progress in their perceptual auditory abilities. Before CI, all of the children had severe to profound sensorineural hearing loss and a diagnosis of CND. Magnetic resonance imaging (MRI) documented an absent CN in 13 children and a small CN in 8 children. We performed explantation of the cochlear implant and simultaneous ABI on the same side. We performed MRI if no previous MRI results were available. All surgical videos were reviewed to determine the presence or absence of the CN. Measures of the patients' perceptual auditory abilities obtained after CI and after ABI were converted to the Category of Auditory Performance (CAP) scale. RESULTS: At surgery, all patients demonstrated an absent CN. After CI, all patients had a CAP score of 2 or less (mean, 0.52 +/- 0.68). After ABI, all patients had a CAP score of 2 or more (mean, 4.33 +/- 1.68); the improvement was statistically significant (p < 0.001). The complication rates were similar for CI and ABI. CONCLUSIONS: In this cohort of patients who had poor performance after CI, ABI achieved significantly improved performance as measured by the CAP and was shown to successfully rehabilitate hearing. Cases of a small CN may in reality represent an absent CN. Although this cohort was selected from patients with failed CI, the results have implications for the selection of device for patients with CND, in that ABI is a potential alternative to CI in select cases. In patients who fail to progress with intensive rehabilitation with CI or who have no progression in evoked auditory brainstem response, ABI must be considered early.

Long-term outcome of round window Vibrant SoundBridge implantation in extensive ossicular chain defects.

OBJECTIVE: To evaluate retrospectively the long-term safety and efficacy of the first 50 patients, all suffering from severe ossicular chain defects and with moderate to severe mixed hearing loss, who received the Vibrant SoundBridge with the floating mass transducer located on the round window membrane. To evaluate differences in outcome versus etiology and age of the patient population. STUDY DESIGN: Case series with planned data collection. SETTING: Tertiary referral medical center. SUBJECTS AND METHODS: Patients eligible for implantation of the floating mass transducer on the round window membrane ranged in age from 2 months to 74 years with a moderate to severe conductive or mixed hearing loss from different etiologies. For each adult patient, preoperative versus postoperative bone and air conduction thresholds, air-bone gaps, and speech understanding scores were evaluated at 24-month follow-up. At 60-month follow-up, data were available from 33 patients. Preoperative and postoperative free-field auditory brainstem responses were studied in infants and children. Intraoperative and short- and long-term postoperative complications are presented. RESULTS: There were significant improvements in speech perception and pure-tone audiometry in adults and auditory brainstem response thresholds in infants immediately after surgery and at follow-up examinations (12 to 71 months). No significant complications or device extrusions were observed in the present series. CONCLUSIONS: Infants, children, and adults with moderate to severe conductive or mixed hearing loss obtained substantial benefit from implantation of the floating mass transducer on the round window membrane regardless of the etiology of hearing loss and previous surgery.

Auditory brainstem implants for neurofibromatosis type 2.

PURPOSE OF REVIEW: Neurofibromatosis type 2 (NF2) produces benign Schwann cell tumors on many cranial nerves, in particular on the vestibular portions of the VIIIn bilaterally. Removal of these vestibular schwannomas usually severs the auditory portion of the VIIIn, thus deafening the patients. The auditory brainstem implant (ABI) was designed to provide prosthetic electric stimulation of the cochlear nucleus in the brainstem to restore some hearing sensations to patients deafened by bilateral removal of vestibular schwannomas. This study will review the new developments and improving outcomes of the ABI. RECENT FINDINGS: From its initial application in 1979 until about 2005, the ABI provided modest but useful auditory sensations to NF2 patients. However, application of the ABI in non-NF2 populations and in children with congenital malformations demonstrated better results, showing that the ABI could provide high levels of speech recognition. Recent results show excellent speech recognition in NF2 patients as well. This study will discuss the potential causes of the variability in ABI outcomes. SUMMARY: ABIs activate neurons in the cochlear nucleus to recreate hearing sensations in people who have become deaf as a result of the loss of the auditory nerve. Most NF2 patients show functional hearing benefit from the ABI, with awareness and recognition of environmental sounds and enhancement of lipreading. It is now clear that ABIs can produce excellent speech recognition in some patients with NF2, allowing even conversational telephone use. Although the factors leading to this improved performance are not completely clear, these new results show that excellent hearing is possible for NF2 patients with the ABI.

Cochlear implants in children younger than 6 months.

OBJECTIVES: (1) To determine the long-term outcomes of cochlear implantation in children implanted younger than 6 months and (2) to evaluate auditory-based performance in very young children compared with older children, all with profound sensorineural bilateral hearing loss. STUDY DESIGN: Prospective cohort study. SETTING: Tertiary referral center. SUBJECTS AND METHODS: Twelve subjects aged 2 to 6 months, 9 aged 7 to 12 months, 11 aged 13 to 18 months, and 13 aged 19 to 24 months, all with profound bilateral hearing loss, were fitted with cochlear implants and followed longitudinally for 4 years. Subjects were developmentally normal with no additional disabilities (visual, motor, or cognitive). Auditory-based communication outcomes included tests for speech perception, receptive language development, receptive vocabulary, and speech production. RESULTS: Age at cochlear implantation was a significant factor in most outcome measures, contributing significantly to speech perception, speech production, and language outcomes. There were no major complications and no significantly higher rates of minor complications in the younger children. CONCLUSION: This article reports an uncontrolled observational study on a small group of infants fitted with cochlear implants following personal audiological criteria and, up to now, with limited literature support due to the innovative nature of the study. This study shows, for the first time, significantly improved auditory-based outcomes in children implanted younger than 6 months and without an increased rate of complications. The data from the present study must be considered as explorative, and a more extensive study is required.

Electrocochleography during cochlear implantation for hearing preservation.

OBJECTIVE: To determine whether intraoperative electrocochleography during cochlear implant surgery provides online feedback to modify surgical procedure, reduce trauma, and increase preservation of residual hearing. STUDY DESIGN: Prospective cohort study. SETTING: Tertiary referral center, Otolaryngology Department, University of Verona. SUBJECTS AND METHODS: Twenty-seven adult patients undergoing cochlear implant surgery who had low- to mid-frequency (0.25-2 kHz) auditory thresholds measured preoperatively were enrolled. Fifteen subjects had compound action potentials measured to assess cochlear function during surgery. In those patients, surgery was modified according to electrocochleographic feedback. Twelve control subjects underwent cochlear implant surgery with blinded electrocochleographic monitoring. RESULTS: The average preoperative pure-tone audiometry thresholds (0.25-2 kHz) were 74.3 ± 10.2 and 81.5 ± 12.7 dB hearing level (HL) in the electrocochleographic feedback and control cohorts, respectively (P > .05). Compound action potential recordings showed a mean maximum latency shift of 0.63 ± 0.36 ms and normalized amplitude deterioration of 59% ± 19% during surgery. All of these changes reverted to normal after electrode insertion in all but 1 subject in the electrocochleographic feedback group. The average shifts in postoperative pure-tone average threshold (0.25-2 kHz), evaluated before activation, were 8.7 ± 4.3 and 19.2 ± 11.4 dB HL in the electrocochleographic feedback and control cohorts, respectively (P = .0051). Complete hearing preservation (loss of ≤10 dB) at 1 month before activation was achieved in 85% (11/13) of electrocochleographic feedback subjects and in 33% (4/12) of control patients (P = .0154). CONCLUSION: Monitoring cochlear function with electrocochleography gives real-time feedback during surgery, providing objective data that might help in modifying the surgical technique in ways that can improve the rate of hearing preservation.

Electrocochleography in round window Vibrant Soundbridge implantation.

OBJECTIVE: To determine the role of intraoperative electrocochleography to optimize the fitting of the floating mass transducer of the Vibrant Soundbridge on the round window membrane in patients with conductive and mixed hearing loss. STUDY DESIGN: Prospective cohort study. SETTING: Tertiary referral center, Otolaryngology Department, University of Verona, Verona, Italy. SUBJECTS AND METHODS: Twenty-six adult patients suffering from chronic otitis media with moderate to severe conductive and mixed hearing loss, all with previous unsuccessful functional surgery, underwent round window vibroplasty. Thirteen subjects had intraoperative compound cochlear action potentials measured to assess vibroplasty coupling during and after surgery. In these patients, surgery was modified according to electrocochleographic feedback. The other 13 had vibroplasty without electrocochleography monitoring. RESULTS: The average preoperative air conduction and bone conduction thresholds (0.5-4 kHz) were not statistically significantly different between the 2 cohorts (P > .05). Compound action potential recordings indicated specific surgical modalities to optimize coupling of the floating mass transducer with the round window membrane. The average postoperative Vibrant Soundbridge-aided air conduction threshold improvements (0.5-4 kHz) were 54.6 ± 8.9 and 41.7 ± 11.1 dB HL, respectively, in the monitored and unmonitored cohorts (P = .0032). CONCLUSION: Improved round window vibroplasty outcomes are observed when the surgeon is promptly informed of the compound action potential changes induced by the floating mass transducer round window membrane vibroplasty and alters surgery accordingly. The key point for optimal coupling is a floating mass transducer in full contact with the round window membrane, free to vibrate without any contact with the surrounding bony structures and mobile footplate.

Estimated net saving to society from cochlear implantation in infants: a preliminary analysis.

OBJECTIVES/HYPOTHESIS: Although it is clear that cochlear implants (CIs) are highly cost-effective in adults and children, the possible additional economic benefit of implantation at younger ages has to be fully established to verify whether the costs and outcomes of CIs differ between infants and older children. STUDY DESIGN: Retrospective cohort study. METHODS: Comprehensive data of CI costs were obtained in four groups of children (age 2-11, 12-23, 24-35, and 72-83 months) from parent questionnaires, national healthcare and educational systems, and retail prices for materials used. Outcomes are compared in terms of receptive language level (Peabody Picture Vocabulary Test-Revised [PPVT-R]), with follow-up to the chronological age of 10 years. RESULTS: Implantation in infants was associated with a lower total cost for the first 10 years of life. The net savings to society ranged from around 21,000€ in the two younger classes to more than 35,000€ when comparing infants against children in the oldest group. When implantation was delayed, family costs played an important role in the increase in expenses. Children in the 2- to 11-month group scored significantly better at the PPVT-R than those in the other age groups (P < .05, P < .01, and P < .001, respectively; Dunn's test) at 10 years of age. The cost per 1-year gain in vocabulary age at the PPVT-R showed a substantial difference between the youngest and oldest age groups (13,266€/year, 17,719€/year, 20,029€/year, and 28,042€/year, respectively). CONCLUSIONS: CIs for patients under 1 year of age afford significantly improved performance and a net savings to society.

Treatment of the atretic ear with round window vibrant soundbridge implantation in infants and children: electrocochleography and audiologic outcomes.

OBJECTIVE: To evaluate the long-term outcomes of the first 5 infants and 9 children with congenital aural atresia (CAA) who had undergone hearing rehabilitation using the MED-EL Vibrant Soundbridge with intraoperative assistance of electrocochleography (ECoG) for optimal fitting of the floating mass transducer (FMT) on the round window (RW) membrane. STUDY DESIGN: Tertiary referral medical center; retrospective case series. PATIENTS: Infants and children ranging in age from 2 months to 16 years with a moderate-to-severe conductive or mixed hearing loss with CAA. For comparison, the study population was divided into 2 groups: older children (≥5 yr of age; 5 patients) and younger children/infants (<5 yr of age; 9 subjects) who were submitted to different audiologic tests appropriate for their age and general condition. INTERVENTION: RW implantation. MAIN OUTCOME MEASURES: Compound action potential threshold and amplitude were assessed as a function of different methods for stabilizing the FMT on the RW. Pure tone audiogram at 0.5, 1, 2, and 4 kHz, free-field speech testing (older children), bone conduction and free-field auditory brainstem response (ABR; younger children and infants), intraoperative and postoperative complications, and FMT displacement or extrusion rate. RESULTS: Statistically significant differences were observed with ECoG recordings between pre- and post-FMT-RW membrane optimization with fascia and cartilage (p < 0.001). Significant improvements were observed in speech perception and pure-tone and ABR threshold, immediately after surgery and at follow-up intervals (12-65 mo) in children and infants (p < 0.01). No complications or instances of device extrusion were observed. CONCLUSION: Infants and children with moderate-to-severe conductive or mixed hearing loss, not suitable or unwilling to accept Bone-Anchored Hearing Aids and who would not tolerate traditional bone and air conduction hearing aids, obtain substantial benefit with the FMT-RW implantation procedure. Intraoperative ECoG is of significant help in achieving the best FMT-RW fitting.