Audiology and Neurotology

Critical Factors for Binaural Hearing in Children with Bilateral Sequential Cochlear Implantation: First Implant Performance and Inter-Implant Interval

08-08-2019 –

Objectives: This study evaluated the influence of the performance of the first cochlear implant (CI-1) on the second implant (CI-2) and the significance of inter-implant intervals in children with sequential cochlear implantation. Method: Thirty-four patients were included for speech perception outcome and 38 patients were included for subjective hearing satisfaction in daily life in this study. The patients were classified into 3 groups according to inter-implant interval: group I, #x3c;4 years; group II, 4–7 years; group III, #x3e;7 years. Open-set speech perception scores before and at 3, 6, 12, and 24 months after the second implantation was compared among the CI-1, CI-2, and bilateral CI conditions. Subjective hearing benefits in daily life were measured using a questionnaire of Speech, Spatial, and Qualities of Hearing Scale (SSQ) for parents of children with impaired hearing, and device use and preference were evaluated (by phone interview.) Results: The speech perception of children using the CI-2 only improved quickly within 3 months postoperatively and were similar to those using the CI-1 only. The results of monosyllabic and disyllabic word tests using CI-2 at 3 months after second implantation were not significantly different from those in patients using the CI-1 only. In the sentence test, the scores using the CI-2 only were not significantly different from those using the first implant only at 6 months after second implantation. SSQ scores were similar among groups and the worst score in each section was shown from the questions about performance under noisy conditions in each section. Device usage habits showed that 17 (44.7%) and 16 (42.1%) patients preferred bilateral and the CI-1, respectively. Only 5 (13.2%) patients wanted the CI-2 rather than the CI-1. While group I and II showed a prominent positive attitude to bilateral use, group III, for which the inter-implant interval was 7 years or more, showed a definite preference for the CI-1 (p #x3c; 0.01). Conclusions: Functional binaural benefits were achieved in patients who were good performers with the CI-1 after the second implantation irrespective of the inter-implant interval. Sequential CI should be strongly recommended for patients with unilateral CI showing a good performance.
Audiol Neurotol

Audiovestibular Phenotypes and Advanced Magnetic Resonance Imaging Features of Cochlin Gene Mutation Carriers

07-08-2019 – G Conte,F Lo Russo,L Caschera,D Zanetti,P Castorina,C Sina,F Triulzi,F Di Berardino

Journal Article

Objective: To describe clinical and imaging findings in a group of patients affected by nonsyndromic deafness A9 (DFNA9), using advanced magnetic resonance imaging (MRI) with 3-dimensional (3D) fluid-attenuated inversion recovery (FLAIR) sequence. Method: A retrospective case review was conducted in a tertiary referral center in Italy. Four sequential adult DFNA9-affected patients, who had undergone MRI at our Department between January 2017 and June 2018, were enrolled (male = 2, female = 2; median age: 65.6 years; 8 diseased ears analyzed). Three patients were relatives; the fourth was unrelated. The main outcome measures – age, sex, records of audiological and vestibular testing, genetic assessment, MRI findings – were analyzed. Results: All subjects suffered from bilateral progressive sensorineural hearing loss, more severely at the high frequencies and with a typical clinical pattern of bilateral chronic degenerative cochleovestibular deficit. Aural fullness was reported at the onset of the disease. All patients revealed a pathogenic heterozygous mutation in the Limulus factor C, Coch-5b2 and Lgl1 domain of cochlin. None of the patients showed a significant vestibular and cochlear endolymphatic hydrops at MRI, while high bilateral contrast enhancement on 4-h delayed postcontrast 3D FLAIR sequence was observed in all ears. Conclusions: Increased perilymph enhancement on 4-h delayed postcontrast 3D FLAIR sequence is the common imaging feature of DFNA9 ears, suggesting that blood-labyrinthine barrier breakdown may play the main role in the pathophysiology of this disease. Significant hydrops has been excluded by MRI. This finding might be clinically useful in differentiating DFNA9 disease from other pathologies with similar clinical findings like Ménière’s disease.
Audiol Neurotol

Impairment of Vestibular Function and Balance Control in Patients with Type 2 Diabetes

19-07-2019 – J Li,J Jiang,Y Zhang,B Liu,L Zhang

Journal Article

Background: Recent evidence suggests that falls are a major complication of diabetes in elderly patients, leading to disability and preventable death. However, the potential risk factors leading to falls in patients with type 2 diabetes are not fully understood. This study was designed to explore the characteristics of vestibular dysfunction and balance control in patients with type 2 diabetes and to analyse the risk factors associated with falls. Methods: The study recruited 51 patients with type 2 diabetes and 43 controls who underwent vestibular function tests and balance control capability tests between January 2013 and December 2015. Vestibular function and balance control capability assessment was based on slow-phase velocity, canal paresis, Sensory Organisation Test (SOT) score, Limits of Stability Test (LOS) score, and Motor Control Test (MCT) score. Results: In all, 56.7% of the diabetic patients had vestibular dysfunction, compared with 27.9% of the controls (p = 0.005). Vestibular dysfunction was dependent on the duration of the disease and serum Hb
A1c levels. There were no significant differences between the two groups with respect to the balance test results for SOT score, somatosensory subtest score, vestibular subtest score, or LOS score. However, the visual system and MCT scores were significantly lower in the diabetic patients than in the controls (p = 0.032 and p = 0.018, respectively). Conclusions: Patients with type 2 diabetes have a higher incidence of vestibular dysfunction. Vestibular dysfunction, visual system impairment, and a decline in motion control may be the risk factors that can lead to falls, and thus need to be managed accordingly in diabetic patients.
Audiol Neurotol 2019;24:154–160

Fluctuating Sensorineural Hearing Loss

17-07-2019 – H Liu,K Zhou,X Zhang,KA Peng

Journal Article, Review

Background: Several otologic conditions can present with fluctuating sensorineural hearing loss, including Ménière’s disease, autoimmune inner ear disease, and enlarged vestibular aqueduct. Although these 3 etiologies vary greatly, distinguishing between these conditions at initial presentation can be challenging. Furthermore, initial treatment of these conditions is often similar. In this review, we discuss historical and current perspectives on diagnosis and treatment of these conditions. Summary: A literature search was performed regarding fluctuating hearing loss, and current treatment of these etiologies of fluctuating hearing loss was summarized. Immediate measures at the onset of acute hearing loss include corticosteroid therapy, while preventative and chronic therapies, which can limit disease severity and frequency, vary based on the specific condition treated. Key Messages: Fluctuating hearing loss can represent a range of pathologies, but the precise etiology may not be clear at initial presentation. Timely treatment and long-term follow-up, along with appropriate diagnostics, are necessary to optimize long-term hearing.
Audiol Neurotol 2019;24:109–116

Cochlear Nerve Hypoplasia: Audiological Characteristics in Children and Adults

15-07-2019 – BC Cinar,E Tahir,MO Batuk,M Yarali,G Sennaroglu,L Sennaroglu

Journal Article

Background: Cochlear nerve deficiency is a general term used to describe both cochlear nerve hypoplasia (CNH) and cochlear nerve aplasia. Although these two conditions can have similar results on audiological evaluation, CNH yields more variation in audiological tests. Objectives: To describe the audiological characteristics of the CNH cases in our series in relation to radiological findings. Methods: We reviewed the medical charts, audiological findings, and radiological findings on cases with CNH. We included cases with CNH in one ear or both ears. Out of 90 subjects with CNH, we included a total of 40 individuals (21 women and 19 men; 49 ears) in the current study. We reviewed and analyzed the participants’ audiological test results according to the radiological findings. Results: Cases with CNH showed variations according to the cochlear structure. There were 13 normal cochleae, 4 with incomplete partition type I, and 32 with cochlear hypoplasia. The accompanying cochlear apertures also showed variation: 17 were normal, 28 stenotic, and 4 aplastic cochlear apertures. The subjects displayed hearing loss ranging from moderate to profound; furthermore, 4 subjects had no response to sound whatsoever. The degree of hearing loss was not statistically significantly different with regard to the presence or absence of cochlear malformation with CNH (p #x3e; 0.005). We observed both sensorineural hearing loss and mixed-type hearing loss among the CNH cases. Conclusions: CNH is the presence of a cochlear nerve that is smaller in diameter than the facial nerve. It can be accompanied with other associated inner ear malformations of different degrees of severity. We observed degrees of hearing loss ranging from moderate to profound.
Audiol Neurotol 2019;24:147–153

Auditory Skills following Cochlear Implantation in Children with the Charge Syndrome

10-07-2019 – P Aragón-Ramos,MF Pedrero-Escalas,J Gavilán,R Pérez-Mora,B Herrán-Martin,L Lassaletta

Journal Article

Objectives: To assess the auditory outcomes and skills of pediatric cochlear implant (CI) users with the CHARGE syndrome. To determine the influence of inner ear malformations on the surgical procedure and speech understanding outcomes in this population. Study Design: Observational, retrospective study. Materials and Methods: Imaging, auditory testing, intraoperative findings, complications, and postoperative auditory skills and outcomes of pediatric CI users with CHARGE syndrome were recorded. Results: 6 children (8 ears) were included, 5 of whom had prelingual deafness. Their mean age at implantation was 37 months. Six of the 8 ears presented cochlear malformation; the most frequent was hypoplasia type III. Intraoperatively, the transmastoid facial recess approach was used in 5 ears, and abnormalities of facial nerve anatomy were found in 5 ears. All electrode insertions were complete. All children were, to a varying degree, able to detect and identify sound. Verbalization skills were developed by 2 children, 1 of whom used oral language as his primary mode of communication. Conclusions: Cochlear implantation performed by an experienced surgeon in patients with the CHARGE syndrome is a safe procedure with adequate treatment planning. All children had improved auditory skills although the improvement was variable.
Audiol Neurotol 2019;24:139–146

Audiological Evaluation of Vestibular Schwannoma Patients with Normal Hearing

02-07-2019 – N Salem,A Galal,V Mastronardi,M Talaat,O Sobhy,M Sanna

Journal Article

Objective: To evaluate the audiological aspects of vestibular schwannoma (VS) patients with normal hearing. Study Design: Retrospective study. Setting: Quaternary referral center for skull base pathologies. Patients: The records on 4,000 patients who had been diagnosed with VS between 1986 and December 2017 were retrospectively reviewed. The patients included in the study were the ones who complied with the strict audiological normality criteria, as follows: a pure tone hearing threshold (at the 6-octave-spaced frequencies from 250 to 8,000 Hz) ≤25 d
BHL; a word recognition score #x3e;90%; and interaural differences ≤10 d
B at each frequency. Interventions: Auditory brainstem response (ABR) testing and radiological imaging. Main Outcome Measures: The incidence of normal objective hearing among VS patients, and the diagnostic utility of the ABR and the effect of tumor size and site on the response. Results: The incidence of normal hearing among VS patients was 4.2%. Tinnitus and vertigo were the most common symptoms across tumor grades; 5.6% of the tumors were large and giant tumors. The ABR yielded a sensitivity of 73.6%, with a false negative rate of 26.3% using a cutoff point of 0.2 ms for interaural latency differences. Conclusions: The diagnosis of VS should not be based on audiometric thresholds alone. Alarming signs of VS should be clear to the physician in order not to miss or delay the diagnosis of the disease. The ABR is useful in the diagnosis of VS, but normal results do not exclude the occurrence of the disease in patients with normal hearing.
Audiol Neurotol 2019;24:117–126

How Does Nonverbal Reasoning Affect Sentence Recognition in Adults with Cochlear Implants and Normal-Hearing Peers?

02-07-2019 – AC Moberly,JK Mattingly,I Castellanos

Journal Article

Background: Previous research has demonstrated an association of scores on a visual test of nonverbal reasoning, Raven’s Progressive Matrices (RPM), with scores on open-set sentence recognition in quiet for adult cochlear implant (CI) users as well as for adults with normal hearing (NH) listening to noise-vocoded sentence materials. Moreover, in that study, CI users demonstrated poorer nonverbal reasoning when compared with NH peers. However, it remains unclear what underlying neurocognitive processes contributed to the association of nonverbal reasoning scores with sentence recognition, and to the poorer scores demonstrated by CI users. Objectives: Three hypotheses were tested: (1) nonverbal reasoning abilities of adult CI users and normal-hearing (NH) age-matched peers would be predicted by performance on more basic neurocognitive measures of working memory capacity, information-processing speed, inhibitory control, and concentration; (2) nonverbal reasoning would mediate the effects of more basic neurocognitive functions on sentence recognition in both groups; and (3) group differences in more basic neurocognitive functions would explain the group differences previously demonstrated in nonverbal reasoning. Method: Eighty-three participants (40 CI and 43 NH) underwent testing of sentence recognition using two sets of sentence materials: sentences produced by a single male talker (Harvard sentences) and high-variability sentences produced by multiple talkers (Perceptually Robust English Sentence Test Open-set, PRESTO). Participants also completed testing of nonverbal reasoning using a visual computerized RPM test, and additional neurocognitive assessments were collected using a visual Digit Span test and a Stroop Color-Word task. Multivariate regression analyses were performed to test our hypotheses while treating age as a covariate. Results: In the CI group, information processing speed on the Stroop task predicted RPM performance, and RPM scores mediated the effects of information processing speed on sentence recognition abilities for both Harvard and PRESTO sentences. In contrast, for the NH group, Stroop inhibitory control predicted RPM performance, and a trend was seen towards RPM scores mediating the effects of inhibitory control on sentence recognition, but only for PRESTO sentences. Poorer RPM performance in CI users than NH controls could be partially attributed to slower information processing speed. Conclusions: Neurocognitive functions contributed differentially to nonverbal reasoning performance in CI users as compared with NH peers, and nonverbal reasoning appeared to partially mediate the effects of these different neurocognitive functions on sentence recognition in both groups, at least for PRESTO sentences. Slower information processing speed accounted for poorer nonverbal reasoning scores in CI users. Thus, it may be that prolonged auditory deprivation contributes to cognitive decline through slower information processing.
Audiol Neurotol 2019;24:127–138

Clinical Implications of Poloxamer 407 as Packing Material in an Animal Model

17-06-2019 – JE Choi,JH Lee,SY Chang,MY Lee,JY Jung

Journal Article

Background: Endoscopic ear surgery has recently increased, but it is still inconvenient and time-consuming to place packing material in the middle ear with one hand. Poloxamer 407 (P407) is a thermo-reversible gel that can be easily administered with one hand into the middle ear cavity in liquid form. Upon warming to body temperature, the gel form of P407 can support the graft in the target position and is known to prevent postsurgical tissue adhesion. Objectives: We aim to investigate the feasibility of P407 as packing material in an animal model. Male Hartley guinea pigs (350 and 400 g) were utilized in this study. Method: The animals were randomly divided into 3 groups according to the packing material: the control group, the P407 group, and the gelatin group. To assess the role of packing material on bacterial colonization, left ears were inoculated with Streptococcus pneumoniae through the tympanic membrane using a 0° endoscope. Five days after inoculation, the middle ear cavity was packed through a transbullar approach using 18% P407 or gelatin in both ears. In the control group, no ear pack was inserted. The tympanic membrane was examined every week using a 0° 1.9-mm endoscope until 6 weeks. Half of the animals in each group were sacrificed 6 weeks after placement of the packing materials. Results: Compared with the absorbable gelatin sponge, the P407 group showed little inflammation or fibrosis in the tympanic membrane and middle ear mucosa regardless of bacterial inoculation. The gelatin group showed severe otorrhea or perforation until 2 weeks in the right ear (2 of 4) and the left ear (1 of 4). Even though the endoscopic findings were similar between both packing groups at 6 weeks, histological analysis showed persistent packing material, inflammatory cells, and fibrosis in the gelatin group compared to the P407 group. Conclusions: This study suggested that P407 is feasible as a packing material to handle with one hand and to prevent adhesion, especially in infected middle ear mucosa. Although there is a lack of data on how well P407 supports grafts, we suggest that P407 could be a candidate for packing material in endoscopic ear surgery.
Audiol Neurotol 2019;24:100–107

Tophaceous Gout of the Middle Ear: Case Reports and Review of the Literature

05-06-2019 – J Saliba,H Sakano,RA Friedman,JP Harris

Journal Article, Review

Tophaceous gout of the middle ear is a rare occurrence that presents as a granular white-colored mass. It is frequently misdiagnosed as cholesteatoma or tympanosclerosis in patients who otherwise may not manifest any clinical or biochemical signs of gout. While uncommon, it can lead to clinically significant disease such as conductive hearing loss. The present report describes 2 cases of middle ear gouty tophi initially mistaken for another entity. Both patients underwent surgery, and the diagnosis of gout was revealed after final histopathological analysis. A review of the literature is also presented.
Audiol Neurotol 2019;24:51–55