Sensitivity of the cochlear nerve to acoustic and electrical stimulation months after a vestibular labyrinthectomy in guinea pigs.

Single-sided deafness patients are now being considered candidates to receive a cochlear implant. With this, many people who have undergone a unilateral vestibular labyrinthectomy for the treatment of chronic vertigo are now being considered for cochlear implantation. There is still some concern regarding the potential efficacy of cochlear implants in these patients, where factors such as cochlear fibrosis or nerve degeneration following unilateral vestibular labyrinthectomy may preclude their use. Here, we have performed a unilateral vestibular labyrinthectomy in normally hearing guinea pigs, and allowed them to recover for either 6 weeks, or 10 months, before assessing morphological and functional changes related to cochlear implantation. Light sheet fluorescence microscopy was used to assess gross morphology throughout the entire ear. Whole nerve responses to acoustic, vibrational, or electrical stimuli were used as functional measures. Mild cellular infiltration was observed at 6 weeks, and to a lesser extent at 10 months after labyrinthectomy. Following labyrinthectomy, cochlear sensitivity to high-frequency acoustic tone-bursts was reduced by 16 ± 4 dB, vestibular sensitivity was almost entirely abolished, and electrical sensitivity was only mildly reduced. These results support recent clinical findings that patients who have received a vestibular labyrinthectomy may still benefit from a cochlear implant.

An epidemiological study to investigate the relationship between Meniere's disease and migraine.

OBJECTIVES: A relationship between Meniere's disease and migraine has been postulated previously. This study investigates this relationship further and determines the most influential factors for developing Meniere's disease. DESIGN: Epidemiological study. SETTING: Two tertiary referral Neuro-Otological centres in Sheffield and Sydney. PARTICIPANTS: Adult patients referred to the Neuro-Otology clinic between 2003 and 2010. MAIN OUTCOME MEASURES: Past history and family history of Meniere's disease and migraine. Logistic regression analysis to determine the most influential factors for Meniere's disease. RESULTS: One hundred and eighty-one patients were included in the study, 102 with Meniere's disease and 79 with other balance disorders. Three significant findings were demonstrated. Firstly, a family history of Meniere's disease (33.3% versus 6.3%) or migraine (21.6% versus 9%) is more common in the Meniere's disease group than in the other balance disorders group. Secondly, a history of migrainous headaches is more common in the Meniere's disease group than in the other balance disorders group (45.1% versus 9%). Thirdly, patients with a past history or a family history of Meniere's disease or migraine have a higher likelihood of suffering from Meniere's disease. CONCLUSIONS: There is an overall relationship between Meniere's disease and migraine. A family history of Meniere's disease or migraine is more common in Meniere's disease. A history of migrainous headache is more common in Meniere's disease. Patients with a past history or family history of Meniere's disease or migraine have a higher likelihood of suffering from Meniere's disease.

Cochlear function following implantation with a full electrode array.

OBJECTIVES: Clinicians traditionally advise patients that implantation with a conventional full cochlear implant array will lead to loss of any remaining hearing in the implanted ear. We sought to assess the extent to which 'standard' insertion of a full electrode array affects cochlear function. METHODS: Air conduction pure tone audiometry was performed as part of the routine 6-month postimplantation audiological assessment for 81 adult patients who preoperatively had identifiable pure tone thresholds. All patients were implanted with the nucleus CI24 device, with complete insertion of a straight array in 68 and a contour array in 13 patients. A separate cochleostomy was fashioned anterior to the round window using a 1 mm diamond burr, and the array inserted without lubrication. The operating surgeon was blinded to the inclusion of patients in this study at the time of surgery. RESULTS: Of 81 patients, 58 preserved some residual hearing at 6 months. The rate of preservation of hearing was higher in the low frequencies. At 500 Hz 77 patients had an identifiable preoperative pure tone threshold, still identifiable postoperatively in 48. At 4 kHz only 25 patients had an identifiable preoperative threshold, preserved in 9, and at 8 kHz 6 of 13 patients still had identifiable pure tone thresholds postoperatively. There was no correlation between the depth of insertion and hearing preservation, both groups having a mean of 5 stiffening rings inserted in addition to the 22 active electrodes. The mean deterioration in threshold in those who had residual hearing was 15 dB at 500 Hz, and in 29 patients the deterioration in the threshold was less than 15 dB. CONCLUSION: Insertion of a full conventional electrode array is not as damaging to cochlear function as often assumed. Residual low-frequency hearing may be preserved in approximately two-thirds of patients 6 months postoperatively. This study supports our counselling patients that despite the delicate nature of the inner ear there is a significant chance that their air conduction hearing will not be lost.

Comparison of outcomes in a case of bilateral cochlear implantation using devices manufactured by two different implant companies (Cochlear Corporation and Med-El).

OBJECTIVE: This paper reports a case of a patient who has had bilateral cochlear implants that have been manufactured by different cochlear implant companies (Cochlear Corporation and Med-El). METHOD: Comparison of speech perception tests following single implant insertion and bilateral insertion (3 and 12 months). The patient was also interviewed to obtain a subjective opinion on their quality of hearing. RESULTS: The patient reported that their Med-El implant had better sound quality than their Cochlear Corporation implant. The speech perception tests however failed to show any difference. CONCLUSION: Despite no difference found with the objective tests hearing is very subjective and therefore the patient's opinion on the quality of sound is important. It is only a matter of time before other patients are fitted with bilateral cochlear implants from different companies and this information should be collated to allow comparison between manufacturers.

Variation in speech perception scores among children with cochlear implants.

OBJECTIVE: The objective of this study was to identify common factors affecting speech perception scores in children with cochlear implants. DESIGN: Speech perception data for 167 implanted children were collected at two cochlear implant centres in Melbourne and Sydney. The data comprised audition-alone scores on open-set word and sentence tests. Children were selected on the basis that they had a Nucleus 22-electrode cochlear implant. The average age of the children was 5 yr. Information was also collected about 12 factors that may have influenced speech perception scores for each child. Analysis of covariance was used to identify factors that significantly affected speech perception scores. Pearson pairwise correlation coefficients were also calculated for all factors analyzed. RESULTS: The analyses in this study identified factors that accounted for 51%, 34%, and 45% of the variance in phoneme, word and sentence perception scores. Scores decreased by 1.4 to 2.4% per year of profound deafness prior to implantation. Children who normally use oral communication scored significantly higher than children normally using sign or simultaneous oral and sign communication. Children implanted in Sydney scored higher on average than children implanted in Melbourne. CONCLUSIONS: The results show that a significant part of the variation in speech perception scores is systematically related to audiological and environmental factors for each child. The reasons for significant differences between children using different communication modes or from different clinics were not identified.

Abnormal positive potentials in round window electrocochleography.

OBJECTIVE: To describe an atypical waveform, termed an abnormal positive potential (APP), on round window electrocochleograms (RW ECochG) of children and to relate its occurrence to clinical history. STUDY DESIGN: APPs were identified prospectively, and a retrospective analysis was made of these patients' clinical histories, audiograms, and auditory outcomes (hearing aid, cochlear implant, or nonauditory communication) SETTING: Tertiary referral teaching hospital, day surgery and clinics. PATIENTS: All 431 children <110 months of age suspected of a severe to profound hearing loss who underwent RW ECochG from January 1993 to August 1997. INTERVENTION: Diagnostic RW ECochG for auditory threshold estimation. MAIN OUTCOME MEASURE: The presence on the RW ECochG of the APP: an early positive potential in the absence of a compound action potential (CAP). RESULTS: An APP was observed in 34 children. The APP was most marked in response to clicks and 8-kHz tones. The APP click threshold averaged 70 dB hearing loss. The brainstem evoked potential of these children showed an absence of waves, or a broad positive wave with no subsequent waves. Twenty-nine of 30 behavioral audiograms obtained were indicative of severe to profound hearing loss. Auditory outcomes were available from 26 children; 45% of them derived no help from a hearing aid, and 8 children received a cochlear implant. Clinical factors frequently associated with APP were prematurity in combination with kemicterus or hypoxia. CONCLUSIONS: APP thresholds were lower than neural thresholds or behavioral thresholds. Children with APP need close follow-up, because half of those studied needed nonauditory strategies to develop effective communication.

The influence of residual high-frequency hearing on the outcome in congenitally deaf cochlear implant recipients.

OBJECTIVE: To show that congenitally deaf children who receive a cochlear implant between 10 and 15 years of age find it significantly more difficult to learn the new signal, and that a history of sound detection at high frequencies with hearing aids is predictive of better outcomes in these children. STUDY DESIGN: A retrospective study using a within-subjects design. SETTING: Children's Cochlear Implant Centre, Sydney (CCIC), Royal Prince Alfred Hospital, and the New Children's Hospital in Westmead are tertiary referral centers. PATIENTS: Forty-five congenitally deaf patients were grouped according to their age (in years) at implantation into group 1 (aged 10-15), group 2 (aged <10), group 2a (aged 6-9), and group 2b (aged 3-5). Within each group, individuals with previous hearing between 2 and 4 kHz before receiving a cochlear implant were identified, and their mean results were compared with those in their respective age-matched groups. INTERVENTIONS: Surgical implantation, intensive weekly habilitation at the CCIC. MAIN OUTCOME MEASURES: Speech perception, speech production, and language measures were compared. Questionnaires and telephone interviews were conducted. RESULTS: Group 2 (age <10 years) consistently outperformed group 1 (10-15 years) on all outcome measures, and most of them learned to converse without lipreading. In group 1, children with previous aided hearing at high frequencies displayed exceptional gains in speech perception and speech production, with reduced dependence on lipreading. Previous high-frequency hearing does not benefit group 2. CONCLUSIONS: Implant recipients aged 10 to 15 years experience more difficulty than younger children during the initial periods of device use. A history of high-frequency hearing before implantation in these individuals correlates with more rapid improvement.

Electrocochleography in the diagnosis of Meniere's disease.

This study prospectively analyses electrocochleography (ECoG) recordings obtained from ears demonstrating symptoms highly suggestive of Meniere's disease. Comparison is made with ECoG recording from ears in which the diagnosis of Meniere's is considered unlikely (control ears). Electrocochleograph recordings were made through a transtympanic recording needle, situated in the round window niche. Analysis was made of the 1 kHz tone burst summation potential (SP) and the summation potential:action potential (SP/AP) ratio response to a 90 dB click. All patients were prospectively awarded a Meniere's score based on a 10-point scale, and comparison was made between ears awarded a score of 7 or greater (Meniere's ears; n > 500), and ears awarded a score 3 or less (control ears; n > 900). Results demonstrate a significant difference in the 1 kHz SP response, and the SP/AP ratio, between "control" and "Meniere's" ears. Furthermore, we show that SP negativity is proportional to the amplitude of the AP click response, and in patients producing an AP click amplitude exceeding 10 V, sensitivity of the tone burst test approaches 85%. This study provides further evidence suggesting the useful role of electrocochleography in determining a diagnosis of endolymphatic hydrops. and demonstrates improved sensitivity of the 1 kHz SP response, compared with SP/AP click ratio, in the diagnosis of Meniere's disease.

Performance after cochlear implantation: a comparison of children deafened by meningitis and congenitally deaf children.

The speech perception and speech production performance following cochlear implantation of congenitally deaf children and children deafened by meningitis were analysed. Three groups consisting of 70 congenitally deaf children, 22 children deafened by meningitis before two years of age and 14 children deafened by meningitis after two years of age were compared. The group deafened by meningitis after two years of age demonstrated significantly better speech perception than the other two groups. Their speech production appeared better but did not achieve statistical significance compared with the other two groups. There was no significant difference in either speech perception or speech production between the congenitally deaf group and the group deafened by meningitis before two years of age.

Meniere's disease: the incidence of hydrops in the contralateral asymptomatic ear.

OBJECTIVE: This study analyzes the incidence of endolymphatic hydrops in the asymptomatic contralateral ear of patients with classic Meniere's disease. STUDY DESIGN: A retrospective study of 3000 subjects who underwent electrocochleography (ECOG) from 1988 to 1998. METHODS: The presence of endolymphatic hydrops was determined by use of ECOG recordings, which were made through a transtympanic recording needle situated in the round window niche. Analysis was made of the 1-kHz toneburst summation potential (SP), and comparison was made between asymptomatic contralateral "Meniere's ears" (n = 144) and asymptomatic normal "control ears" (n = 114). RESULTS: Results demonstrated that more than 10% of the contralateral asymptomatic Meniere's ears have an ECOG recording that is highly suggestive of the presence of endolymphatic hydrops. In contrast, less than 2% of the control population demonstrate abnormal ECOG recordings. Furthermore, 15% of the population of contralateral Meniere's ears lie above the 95th percentile of the control population for 1-kHz tone-burst (100 dB) SP negativity. CONCLUSIONS: This study suggests that a high percentage of patients who have what appears to be unilateral Meniere's disease have evidence of endolymphatic hydrops in the contralateral asymptomatic ear. This finding has important clinical relevance for the management of patients in whom destructive surgery is planned and further highlights the importance of electrocochleography in the diagnosis and management of this disease process.

Positioning of the receiver-stimulator for the CI-24M cochlear implant in infants.

A new cochlear implant (CI-24M) has recently been released by Cochlear Ltd. The shape and size of the receiver-stimulator differs from that of the CI-22M. Infants as young as one year of age are now receiving cochlear implants. We have examined the likely effect of skull growth following the implantation of a CI-24M cochlear implant in an infant of this age.

Surviving cochlear function in the presence of auditory nerve agenesis.

This case reports electrophysiological evidence for cochlear function in a child with radiological evidence of bilateral auditory nerve agenesis or severe hypoplasia. The diagnosis of auditory nerve agenesis was supported by a bilateral atresia of internal auditory canals on computed tomography (CT) scan and magnetic resonance imaging (MRI) absent auditory brainstem responses and absent behavioural responses to sound. Despite the apparent absence of an auditory nerve or spiral ganglion, electrocochleography revealed surviving cochlear function at 70-80 db HL and an abnormal electrocochleographic waveform. This case demonstrates that cochlear function may develop without afferent, or efferent innervation. It also emphasizes that cochlear function may occur in the presence of profound deafness.

Hearing loss associated with large internal auditory meatus: a report of five paediatric cases.

This paper describes the abnormality of a large internal auditory meatus (LIAM). Computed tomography (CT) scans show the otic capsule to be affected by a widened, bulbar internal auditory meatus with loss of or reduction of the bony wall dividing the lateral fundus of the meatus from the cochlea. The vestibule is abnormally dilated. We report five cases of children with LIAM and profound hearing loss. Three of these children are girls and two children were boys. Three had congenital progressive hearing loss, one of these had an accompanying large vestibular aqueduct and dysplasia of the cochlea. Two patients had had meningitis resulting in profound loss.

Cochlear origin of hearing loss in MELAS syndrome.

There have been few studies investigating the mechanism and nature of the hearing loss that occurs in the mitochondrial disorders. We studied 18 patients with the MELAS A3243G point mutation from four different kindreds. Pure tone audiometry, speech discrimination testing, acoustic reflexes, tympanometry, and brain stem auditory evoked responses were performed to localize the site of pathology in the auditory pathways. In 12 patients, we performed electrocochleography and otoacoustic emissions to assess cochlear involvement. Neuroimaging and promontory nerve stimulation were performed to exclude retrocochlear pathology. Audiological testing confirmed sensorineural hearing loss in 14 of the 18 patients studied; hearing loss was usually gradual in onset, was symmetrical, and initially affected the higher frequencies. In some patients, there were features that distinguished the hearing loss from presbyacusis, including a young age at onset, asymmetrical involvement, stepwise progression, and partial recovery. We treated one patient who had profound bilateral hearing loss with cochlear implantation; this restored good functional hearing. Hearing loss in MELAS syndrome appears to be due to dysfunction of the cochlea, probably resulting from metabolic failure of the stria vascularis and outer hair cells. Cochlear implantation is a therapeutic option worth considering in those patients who become deaf.

Speech perception results for children with implants with different levels of preoperative residual hearing.

OBJECTIVE: Many reports have established that hearing-impaired children using the Nucleus 22-channel cochlear implant may show both significant benefits to lipreading and significant scores on open-set words and sentences using electrical stimulation only. These findings have raised questions about whether severely or severely-to-profoundly deaf children should be candidates for cochlear implants. To study this question, postoperative results for implanted children with different levels of preoperative residual hearing were evaluated in terms of speech perception benefits. STUDY DESIGN/SETTING: A retrospective study of the first 117 children, sequentially, to undergo implantation in the Melbourne and Sydney Cochlear Implant Clinics was undertaken. All children had been assessed by and received their implants in a tertiary referral centre. MAIN OUTCOME MEASURES: To assess aided residual hearing, the children were grouped into four categories of hearing on the basis of their aided residual hearing thresholds measured preoperatively. To assess benefits, the scores of children on standard speech perception tests were reviewed. As different tests were used for children with different ages and language skills, children were grouped into categories according to the level of postoperative speech perception benefit. RESULTS: The results showed that children in the higher categories of aided preoperative residual hearing showed significant scores on open-set word and sentence perception tests using the implant alone. For children in lower categories of aided residual hearing, results were variable within the groups. More than 90% of children with implants with aided residual hearing thresholds in the speech range above 1 kHz achieved open-set understanding of words and sentences. CONCLUSION: While the results of this preliminary study confirm previous findings of differential outcomes for children with different levels of preoperative residual hearing, they suggest that children with severe to profound hearing impairments should be considered for cochlear implantation.

Use of transtympanic round window electrocochleography for threshold estimations in children.

OBJECTIVE: This study aimed to evaluate the efficacy of round window electrocochleography for the estimation of hearing thresholds in difficult-to-test children. STUDY DESIGN: The study was a retrospective analysis. SETTING: A standard day-stay operating room was used. PATIENTS: Round window electrocochleography was performed on 198 children between January 1993 and January 1996. INTERVENTION: The intervention was diagnostic. MAIN OUTCOME MEASURE: Clinically reliable pure-tone audiograms were obtained in 101 patients (50.9%) for comparisons of electrocochleography and behavioral thresholds. RESULTS: The mean differences between electrocochleography and behavioral thresholds at 0.5, 1, 2, and 4 kHz were less than 6 dB. Ninety-seven percent of the results were within +30 to -30 dB, and approximately 86% of the results were within +20 to -20 dB. Good correlation coefficients of 0.83, 0.84, 0.91 and 0.88 were found between electrocochleography and behavioral thresholds at 0.5, 1, 2, and 4 kHz, respectively. Only 2 of 395 ears were complicated postoperatively by suppurative otitis media. There were no anesthetic complications. CONCLUSION: Round window electrocochleography can be used in conjunction with other audiometric methods for threshold estimations in the difficult-to-test children and as part of the preoperative assessment for cochlear implantation.