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.

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.

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.

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.

Intraoperative observation of changes in cochlear nerve action potentials during exposure to electromagnetic fields generated by mobile phones.

BACKGROUND: The rapid spread of devices generating electromagnetic fields (EMF) has raised concerns as to the possible effects of this technology on humans. The auditory system is the neural organ most frequently and directly exposed to electromagnetic activity owing to the daily use of mobile phones. In recent publications, a possible correlation between mobile phone usage and central nervous system tumours has been detected. Very recently a deterioration in otoacoustic emissions and in the auditory middle latency responses after intensive and long-term magnetic field exposure in humans has been demonstrated. METHODS: To determine with objective observations if exposure to mobile phone EMF affects acoustically evoked cochlear nerve compound action potentials, seven patients suffering from Ménière's disease and undergoing retrosigmoid vestibular neurectomy were exposed to the effects of mobile phone placed over the craniotomy for 5 min. RESULTS: All patients showed a substantial decrease in amplitude and a significant increase in latency of cochlear nerve compound action potentials during the 5 min of exposure to EMF. These changes lasted for a period of around 5 min after exposure. DISCUSSION: The possibility that EMF can produce relatively long-lasting effects on cochlear nerve conduction is discussed and analysed in light of contrasting previous literature obtained under non-surgical conditions. Limitations of this novel approach, including the effects of the anaesthetics, craniotomy and surgical procedure, are presented in detail.

Evidence of gadolinium distribution from the endolymphatic sac to the endolymphatic compartments of the human inner ear.

To verify whether injection of substances into the endolymphatic sac (ES) diffuses into the endolymphatic compartments of the human inner ear and in particular to the endolymphatic space of the scala media (ESp-SM), as demonstrated in animals, an exploratory investigation with magnetic resonance imaging (MRI) and intraoperative electrocochleographic recordings (ECoG) was conducted in patients with Ménière's disease (MD) treated with ES decompression. A mixture of dexamethasone and gadolinium (GD) in solution was injected into the ES of 4 patients. The results of the ES injection procedure were compared with administration of the same solution intratympanically (IT, 1 patient) and via a platinotomy in 2 patients. The study was conducted retrospectively at a tertiary referral center. Main outcomes measures were pre- and postintervention complete audiological and neuro-otological evaluation; intraoperative ECoG investigation with evaluation of the morphology of acoustically elicited compound action potentials (CAPs) and 1.5 T MRI evaluations at different follow-up times. Distribution of GD from the ES injection procedure was observed first in the ES, after 24 h in the vestibule and semicircular canals, and after 24-48 h in the ESp-SM in all patients. High signal was detected within the inner ear for 1 week or more (mean: 10 days; range: 7-16 days). Changes in morphology and latency of CAPs were observed within 30 min of the dilatory injection into the ES in all patients. Administration of GD into the vestibule and the IT approach did not distribute the contrast in the ES and GD was observed in the perilymphatic space of the vestibule, cochlea and semicircular canals. No side effects relating to administration of GD into the ES, IT or into the vestibule were observed. To the best of our knowledge this is the first demonstration in humans that drugs injected into the endolymphatic structure of the ES diffuse to the cochlea, presumably into the ESp-SM. The possibility of injecting substances into the endolymphatic space might open up new prospects in the treatment of inner ear disorders. Further studies will be needed to define the limitations of this approach.

Auditory results after vestibular nerve section and intratympanic gentamicin for Ménière's disease.

OBJECTIVE: Hearing loss is a possible complication of vestibular neurectomy and intratympanic gentamicin administration in Ménière's disease. The aim of this study was to compare the incidence of this complication with the two treatments. STUDY DESIGN: Retrospective review. SETTING: Tertiary referral center. PATIENTS: The initial study population reviewed consisted of 24 patients receiving gentamicin injections and 209 patients undergoing vestibular nerve section. Comparison of data was performed among all 24 intratympanic gentamicin patients and the last 24 vestibular neurectomy patients operated on via the retrosigmoid approach. INTERVENTIONS: Intratympanic gentamicin administration (26-156 mg) and retrosigmoid vestibular neurectomy. MAIN OUTCOME MEASURE: Auditory and vertigo results were evaluated according to the American Academy of Otolaryngology 1995 criteria. RESULTS: The mean preoperative pure-tone average for patients undergoing vestibular nerve section was 48.5 dB, with a speech discrimination score of 85%. In these patients, the postoperative pure-tone average was 50.3 dB, and the speech discrimination score was 82%. Patients undergoing gentamicin injection had a mean pretreatment pure-tone average of 50.1 dB and a speech discrimination score of 87%. The posttreatment pure-tone average and discrimination score for the gentamicin group were 74.7 dB and 65%, respectively. The amount of postprocedure hearing loss was significantly greater in the gentamicin group (p = 0.03). Excellent control of vertigo (classes A and B) was obtained in 95.8% of the patients treated with vestibular nerve section and in 75% of the patients in the gentamicin group. CONCLUSION: Gentamicin administration and vestibular neurectomy are both effective for relieving vertigo in Ménière's disease. The incidence of hearing loss is significantly higher after gentamicin injection.

Beneficial auditory and cognitive effects of auditory brainstem implantation in children.

CONCLUSION: This preliminary study demonstrates the development of hearing ability and shows that there is a significant improvement in some cognitive parameters related to selective visual/spatial attention and to fluid or multisensory reasoning, in children fitted with auditory brainstem implantation (ABI). The improvement in cognitive paramenters is due to several factors, among which there is certainly, as demonstrated in the literature on a cochlear implants (CIs), the activation of the auditory sensory canal, which was previously absent. The findings of the present study indicate that children with cochlear or cochlear nerve abnormalities with associated cognitive deficits should not be excluded from ABI implantation. OBJECTIVES: The indications for ABI have been extended over the last 10 years to adults with non-tumoral (NT) cochlear or cochlear nerve abnormalities that cannot benefit from CI. We demonstrated that the ABI with surface electrodes may provide sufficient stimulation of the central auditory system in adults for open set speech recognition. These favourable results motivated us to extend ABI indications to children with profound hearing loss who were not candidates for a CI. This study investigated the performances of young deaf children undergoing ABI, in terms of their auditory perceptual development and their non-verbal cognitive abilities. PATIENTS AND METHODS: In our department from 2000 to 2006, 24 children aged 14 months to 16 years received an ABI for different tumour and non-tumour diseases. Two children had NF2 tumours. Eighteen children had bilateral cochlear nerve aplasia. In this group, nine children had associated cochlear malformations, two had unilateral facial nerve agenesia and two had combined microtia, aural atresia and middle ear malformations. Four of these children had previously been fitted elsewhere with a CI with no auditory results. One child had bilateral incomplete cochlear partition (type II); one child, who had previously been fitted unsuccessfully elsewhere with a CI, had auditory neuropathy; one child showed total cochlear ossification bilaterally due to meningitis; and one child had profound hearing loss with cochlear fractures after a head injury. Twelve of these children had multiple associated psychomotor handicaps. The retrosigmoid approach was used in all children. Intraoperative electrical auditory brainstem responses (EABRs) and postoperative EABRs and electrical middle latency responses (EMLRs) were performed. Perceptual auditory abilities were evaluated with the Evaluation of Auditory Responses to Speech (EARS) battery - the Listening Progress Profile (LIP), the Meaningful Auditory Integration Scale (MAIS), the Meaningful Use of Speech Scale (MUSS) - and the Category of Auditory Performance (CAP). Cognitive evaluation was performed on seven children using the Leiter International Performance Scale - Revised (LIPS-R) test with the following subtests: Figure ground, Form completion, Sequential order and Repeated pattern. RESULTS: No postoperative complications were observed. All children consistently used their devices for >75% of waking hours and had environmental sound awareness and utterance of words and simple sentences. Their CAP scores ranged from 1 to 7 (average =4); with MAIS they scored 2-97.5% (average =38%); MUSS scores ranged from 5 to 100% (average =49%) and LIP scores from 5 to 100% (average =45%). Owing to associated disabilities, 12 children were given other therapies (e.g. physical therapy and counselling) in addition to speech and aural rehabilitation therapy. Scores for two of the four subtests of LIPS-R in this study increased significantly during the first year of auditory brainstem implant use in all seven children selected for cognitive evaluation.

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.

Cochlear implantation at under 12 months: report on 10 patients.

OBJECTIVES: There is growing evidence that early application of a cochlear implant in children affected by profound congenital hearing loss is of paramount importance for the development of an adequate auditory performance and language skills. For these reasons and as a result of advances in audiologic diagnosis and an enhanced awareness of the safety of cochlear implants, the age of implantation has substantially decreased over recent years. Children aged as little as 12 months are now being implanted in some centers. On the basis of our experience with very young children, we believe that the date of implantation may be further reduced to only 4 to 6 months of age. STUDY DESIGN: Over the period from November 1998 to April 2004, 103 children have been fitted with cochlear implants and 11 with auditory brainstem implants in our department, including 65 children aged below 3 years. The present study focuses on 10 children aged less than 12 months fitted with cochlear implants from November 1998 to December 2003. METHODS: The children's ages ranged from 4 to 11 (mean 9.5) months. Five were males and five females. All received a Nucleus CI 24 M. Postoperative auditory performance, as evaluated at the latest follow-up, was based on the category of auditory performance (CAP). The results obtained in these 10 children were compared with those obtained with cochlear implants in children belonging to older age brackets. The criteria used to assess speech performance were onset of babbling onset and babbling spurt, and the results observed were compared with those of a control group of 10 normally hearing children. RESULTS: Surgery was uneventful, and no immediate or delayed complications were encountered. Auditory performance was seen to increase as function of early age of implantation and length of implant use. All 10 children had a CAP score of 3 within 6 months of cochlear implant activation. The onset of babbling occurred very early (i.e., within 1 to 3 months of activation of the implant in all 10 patients), regardless of age at implantation, whereas the babbling spurt was recorded at times ranging from 3 to 5 months after implant activation. The positive impact of early implantation on babbling was clearly shown by the fact that the earlier the activation of the cochlear implant, the closer the results were to the outcomes of normally hearing children. CONCLUSIONS: We encourage very early implantation to facilitate a series of developmental processes occurring in the critical period of initial language acquisition. The indices we used in the present study (i.e., CAP and babbling) suggest that early cochlear implantation tends to yield normalization of audio-phonologic parameters, which enables us to consider the performance of children implanted very early as being similar to that of their normally hearing peers.

Auditory brainstem implant (ABI): new frontiers in adults and children.

UNLABELLED: Previous studies have considered only patients with neurofibromatosis type 2 (NF2) older than 12 years as candidates for an auditory brainstem implant (ABI). Our study expands the potential criteria to include both children and adult subjects with other cochlear or cochlear nerve malfunctions who either would not benefit at all from a cochlear implant (eg, cochlear nerve aplasia or avulsion) or whose benefit was or would be severely compromised (eg, cochlear ossification, cochlear fracture). STUDY DESIGN: In our department, over the period from April 1997 to September 2002, 29 patients, 20 adults and 9 children, were fitted with ABIs. Their ages ranged from 14 months to 70 years. Thirteen subjects had tumors, 10 NF2 and 3 solitary vestibular schwannoma, and 16 patients had a variety of nontumor (NT) cochlear or cochlear nerve diseases. A retrosigmoid-transmeatal approach was used in T and a retrosigmoid approach in NT patients. The electrode array was inserted into the lateral recess of the fourth ventricle and correct electrode positioning was monitored with the aid of electrically evoked auditory brainstem responses (EABRs). RESULTS: Correct implantation was achieved in all patients. No complications were observed due to implantation surgery or related to ABI activation or long-term use. Auditory sensations were induced in all patients with various numbers of electrodes (from 5 to 15). Different pitch sensations were identifiable with different electrode stimulation. Closed-set word recognition, open-set sentence recognition, and speech tracking scores achieved by the patients are reported in detail. The auditory performance of the patients showed significantly better outcomes than controls (Multicentric European clinical investigations on ABI with NF2). CONCLUSION: We have shown that the indications for the ABI can be extended to include NT patients with severe cochlear and/or cochlear nerve abnormalities. The degree of auditory benefit varies as a function of the underlying pathological conditions, with NT subjects exhibiting significantly better outcomes than the T patients.

Auditory brainstem implant as a salvage treatment after unsuccessful cochlear implantation.

OBJECTIVE: The present article investigates on an individual basis the performance achieved with the auditory brainstem implant in patients who had been treated unsuccessfully with a cochlear implant. STUDY DESIGN: An intrasubject comparison between results achieved with the cochlear implant and the auditory brainstem implant is reported. SETTING: Tertiary referral care. PATIENTS: Five subjects were fitted with an auditory brainstem implant in our department because of the poor results achieved with cochlear implants. Two were children, one with bilateral cochlear nerve aplasia and one suffering from auditory neuropathy. Three were adults with complete cochlear ossification. INTERVENTION: A retrosigmoid approach was used in all subjects. Electrically evoked auditory brainstem responses and neural response telemetry were used to monitor electrode positioning. RESULTS: No complications were observed due to implantation surgery or related to activation or long-term use of the auditory brainstem implant. Auditory sensations were induced in all patients with varying numbers of electrodes (from 9-16). In all three adults, the cochlear implant did not allow either word/sentence discrimination or speech tracking, whereas the auditory brainstem implant permitted discrimination of two- or three-syllable words with scores from 85 to 100%. In the two adults with a follow-up of 5 and 6 months after auditory brainstem implant activation, the open-set sentence recognition scores (auditory-only mode) were 70% and 100%, respectively, and the speech-tracking scores were 27 and 40 words/min, respectively. One patient with a follow-up of only 3 months scored 0% in both sentence recognition and speech tracking. The two children who had achieved no hearing ability with the cochlear implant were already able to detect sounds and words as early as 2 months after activation of the auditory brainstem implant and are showing progressive improvement in their performance. CONCLUSION: Auditory brainstem implantation may be a very powerful rehabilitative treatment after cochlear implant failure. The possibility of using the auditory brainstem implant as first-choice therapy in some categories of deaf patients (e.g., subjects with auditory neuropathy or cochlear ossification) who are currently treated with cochlear implantation is discussed.

Cochlear implant failure: is an auditory brainstem implant the answer?

OBJECTIVE: To investigate the auditory rehabilitative results achieved in five patients with cochlear implants (CIs) who subsequently received, due to poor results, auditory brainstem implants (ABIs). MATERIAL AND METHODS: Between April 1997 and March 2003, 37 patients (age range 14 months to 70 years) were fitted with ABIs in our ENT Department. Fourteen subjects had neurofibromatosis type 2 and 23 were non-tumor patients who had cochlea or cochlear nerve disease. Five subjects had previously been treated with a CI and received an ABI owing to the poor results achieved. One child had bilateral undiagnosed cochlear nerve aplasia and one was suffering from auditory neuropathy; three adults had total cochlear ossification. RESULTS: The open-set sentence recognition score (auditory-only mode) 6-8 months after ABI activation ranged from 0% to 100% in adults. In 1 subject the speech-tracking score was 56 words/min with the ABI. The two children who had achieved no hearing ability with their CI were able to detect sounds and words as early as 3 months after activation of the ABI. CONCLUSION: CI failure as a result of anatomical abnormalities can be remedied by an ABI.

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.